Introduction

This electronic handbook contains a excerpts from the Pediatric Surgery Not a Textbook (NaT) that are most applicable to general surgery residents and medical students rotating on the pediatric surgery service. Marjorie Arca, Ken Gow and Joseph Iocono edited this content.

see also STEPS presentations

Abdominal Mass

Problem: abdominal mass

Introduction

Children of any age can present with an abdominal mass. Understanding the shift in differential diagnosis based on the location of the mass and the patient’s age and sex is critical to the work up to reach the correct diagnosis. Presentation may range from prenatal diagnosis by screening ultrasound to patients with increasing abdominal distention due to an enlarging mass found by caretakers or clinicians.

Pathophysiology

Obtaining a timely pathologic diagnosis is the first step to understanding the treatment. Masses in neonates are often due to congenital abnormalities whereas older children are more likely to have neoplasms. While patients may be seen for the chief complaint of an "abdominal mass" it is important to remember that the mass may be due to organomegaly, congenital masses, tumors and even infections. The most common solid tumor in infants is neuroblastoma, followed by Wilms tumor, rhabdomyosarcoma, germ cell tumors and hepatoblastoma. After two years of age, Wilms tumor and germ cell tumors become more common.

Key history points

The abdominal mass can be identified by the caretaker (e.g. parents), general practitioner or seen on imaging obtained for other reasons. Abdominal masses can be seen in any age ranging from neonates to adolescents. Knowing the most common diagnoses based on age at presentation is important. The history depends on the age, sex and location of mass. A comprehensive history including the possibility of pregnancy and other identifying risk factors such as trauma, infectious disease, and malignancies can help guide the work up. Familial syndromes or genetic predispositions may also help to the diagnosis.

Key physical exam findings

A general examination with focused assessment of the abdominal exam is critical. The origin of the mass may be made apparent by physical exam. Liver and renal tumors are generally identified as a palpable mass. Look carefully for other associated syndromes which may place patients at higher risk of certain malignancies such as Wilms tumor in Beckwith-Wiedemann syndrome.

Differential diagnosis

The patient’s presentation will help guide your work up of the wide differential diagnosis including congenital abnormalities, malignancy and infection.

All masses should be worked up with a complete blood count, electrolytes, liver function tests, urinalysis, lactate dehydrogenase and uric acid. Depending on the concern for specific tumors, additional laboratory tests may need to be ordered. Ultrasound (US), computed tomography (CT) of abdomen and pelvis, and magnetic resonance imaging (MRI) may need to be ordered depending on the location of the mass. Usually an ultrasound is the first imaging study obtained.

Neuroblastomais the most common extracranial solid tumor in infants and children. Patient may have asymptomatic localized disease or present with advanced, metastatic disease. Serum laboratory markers to order include ferritin, neuron specific enolase, lactate dehydrogenase and urine catecholamines. CT will help evaluate tumor extent and resectability. MRI and metaiodobenzylguanidine (MIBG) scan is useful when evaluating any bone or spinal involvement.

Wilms tumoris most common renal malignancy in infants or children usually presenting by 5 years of age. Almost all present with palpable mass, although may also have hypertension or hematuria. Associated with Wilms tumor, aniridia, genitourinary anomalies, & retardation (WAGR) syndrome, Denys-Drash syndrome, Beckwith-Wiedemann syndrome, Perlman syndrome, sporadic aniridia, and hemihypertrophy. Need further imaging with contrast enhanced CT to evaluate contralateral kidney and chest.

Hepatoblastoma is most common pediatric liver malignancy usually presenting between 1 and 4 years of age. Increased risk with very low birth weight, Beckwith-Wiedemann syndrome, and familial adenomatous polyposis. Patients often present with palpable mass, and can have an elevated alpha fetoprotein (AFP). Many are chemo-sensitive and will benefit from neoadjuvant chemotherapy to downstage disease.

Genitourinary (GU) anomalies are the most common palpable masses in infants and include primary renal cystic disease and hydronephrosis leading to cystic changes of the kidney. Patients may present with renal dysfunction and imaging with ultrasound should be obtained.

Periappendiceal abscesspresents in a patient with prolonged perforated appendicitis with a mass which can be palpated in the right lower quadrant. These can be further evaluated with US or CT for possible percutaneous drainage.

Ovarian cysts are the most commonly diagnosed abdominal cyst. Most are asymptomatic and undergo spontaneous resolution. Surgical excision indicated if complex, symptomatic, increasing in size or persist for more than six months.

Gastrointestinal cysts include duplication (most common found in ileum followed by jejunum and duodenum), mesenteric and omental cysts. Depending on their presentation and symptoms cysts likely warrant excision.

Choledochal cysts are more common in females. The Todani classification is based on location and saccular changes. Type I are most common and require cyst excision with Roux-en-Y hepaticojejunostomy. The classic triad at presentation includes a right upper quadrant palpable mass, pain and jaundice.

Indications for surgery

The indication for surgical excision of the mass depends on the pathologic diagnosis of the abdominal mass. Surgery may involve removal or biopsy of primary tumor and may also include placement of durable intravenous access for chemotherapy.

Preoperative preparation

Prophylactic intravenous antibiotics should be given within one hour prior to laparotomy. All available imaging should be reviewed just prior to incision. The skin should be prepared with chlorhexidine based prep in the operating room.

Operative consent points

The risks of the procedure when addressing an abdominal mass depend on the etiology of the mass. Procedures for resection of malignancy should be discussed with consulting teams in detail for perioperative planning. Potential unexpected findings at the time of exploration should be discussed with the family. The risk of aborting an operation due to these findings should also be made apparent.

If the child has diagnosis of cancer additional procedures may be performed at the time of operation.

Complications

Surgical residents should have a high suspicion for postoperative bleeding and infection as with other procedures. Depending on the etiology of the mass other organ systems will need to be monitored. Patients undergoing bowel resection with anastomosis should be monitored for tolerance of diet and risk of potential anastomotic leak. Major resection of the hepatobiliary system should be monitored for bleeding, bile leaks and postoperative liver function. .

Postoperative care

Care in the patient after a resection of an abdominal mass can be challenging. The etiology, location of the mass and resection will guide the postoperative care of these patients. Patients with major resection of intra-abdominal organs will need to be monitored closely in an intensive care unit. A multidisciplinary approach to the care of these patients should be employed.

When to call the next level

A consult for any child with an intra-abdominal mass should be reviewed with the senior resident or attending promptly. It is important to initially determine if the patient is "sick". If the patient is in need of active resuscitation or with respiratory compromise call right away. Otherwise, the site of the mass, all imaging and the status of basic labs should be known when talking to your senior. Seeing the patient first can be difficult as any family is worried about the presence of cancer. Deferring most questions to your senior or attending may be best and assure the family that you are gathering as much information as possible to help the surgery team to provide the best care of the child and family.

Author: Rodrigo Interiano

Acute Abdominal Pain

Problem : Acute Abdominal Pain

Likely diagnoses: appendicitis, intussusception, Meckel diverticulitis, incarcerated hernia, ovarian torsion, intestinal obstruction (e.g. Hirschsprung disease), volvulusand nonsurgical causes

There are many causes of abdominal pain. The goal is to determine the specific cause and whether it is life threatening. The differential diagnosis includes

Abdominal pain - newborn to one month of age

Pain is difficult to assess in the newborn. Most of the time, surgery will be consulted for irritability and abdominal distention. There are many causes of abdominal distention and irritability. The goal is to determine the specific cause and whether it is life threatening. Differential Diagnosis:

Key history points

Most of the history will be obtained from nurse or parent. Obtain a careful feeding history such as when the feedings started at what quantity, frequency, route and type of formula or breast milk.

Are there bowel movements, when was first meconium seen, when was last bowel movements and what was the quality, amount and consistency? Any blood?

Is there bilious or nonbilious emesis?

Is there abdominal distention? When did distention start?

What is the birth history (estimated gestational age, complications, medications, viral infections (i.e. TORCH), prenatal care)?

Are there signs of sepsis (apnea, bradycardia, hypothermia)?

NEC is more common with premature neonates. Volvulus commonly presents with bilious emesis. HD is associated with a failure to pass meconium in first 48 hours. Duodenal atresia is often diagnosed prenatally and frequently seen in patients with trisomy 21.

Key physical exam findings

Abdominal distension (location and severity)

Abdominal wall erythema or edema

Tenderness

Capillary refill (to assess volume status)

Differential diagnosis

Necrotizing enterocolitis (NEC), malrotation with volvulus, Hirschsprung disease (HD), duodenal atresia, intestinal atresia (jejunal, ileal, or colonic),meconium ileus/plug, nonsurgical causes (e.g. sepsis with ileus, feeding intolerance)

Imaging

A two view abdominal radiograph is useful to differentiate between the different causes of abdominal pain and distention. NEC will have pneumatosis intestinalis and loops of bowel that remain fixed and unchanged in position on subsequent radiographs. Look for signs of perforation (i.e. pneumoperitoneum). HD and intestinal atresias will show dilated loops of bowel. Duodenal atresia will show a double bubble sign.

An upper gastrointestinal contrast series can show signs of malrotation such as ligament of Treitz on the right side, corkscrew appearance of the duodenum or bird’s beak appearance of the duodenum if there is volvulus and obstruction.

A contrast enema is useful in differentiating HD, intestinal atresia and meconium ileus/plugs. In HD a transition zone may be present where a narrow aganglionic rectum gives way to a dilated colon more proximally. Intestinal atresias and meconium ileus will show a microcolon due to disuse of the colon. Contrast enema will identify meconium plugs and will often be therapeutic.

Indications for surgery

Immediate surgery is indicated if there is concern for perforation. For extremely sick neonates with NEC who may not tolerate laparotomy under general anesthesia an alternative is bedside primary peritoneal drainage which consists of placement of a penrose drain into the abdomen under local anesthetic. Immediate surgery is also indicated for patients with malrotation and midgut volvulus.

In patients without signs of perforation, the timing of surgery will be determined based on the diagnosis and need for additional studies.

Patients with possible HD need to have the diagnosis confirmed with suction rectal biopsy. Patients with duodenal atresia may require an echocardiogram prior to surgery (particularly if they have a murmur or desaturation). Patients with intestinal atresia typically go to the operating room within 24 hours. Patients with meconium ileus should have an attempt at decompression with contrast enema. If not successful, then surgery is indicated urgently. Patients with a meconium plug rarely requires surgery but consideration should be given for a suction rectal biopsy to rule out HD.

Preoperative preparation

The initial treatment should include intravenous (IV) fluids, oro- or nasogastric tube decompression. The required laboratory studies include a complete blood count with differential, basic metabolic panel with consideration for arterial or venous blood gases.

Patients with significant ileus should begin parenteral nutrition.

Broad spectrum antibiotics should be started for those with NEC or perforation.

Operative consent points

The specific surgery will depend on the diagnosis. In addition to the standard risks of bleeding, infection, injury to surrounding structures and need for reoperation discuss

NEC - a laparotomy is performed with resection of any necrotic segments of bowel. Usually an ileostomy and mucous fistula are created.

Malrotation and midgut volvulus - a Ladd procedure is performed which consists of untwisting of the volvulus in a counter clockwise fashion (or resection if grossly necrotic), division of the Ladd bands, appendectomy, widening the base of the mesentery and placing the small bowel on the right and colon on the left. Also consent for possible bowel resection in case there is ischemic bowel from the volvulus.

HD - A one or two stage operation can be performed. A two stage operation consists of first performing a leveling colostomy. A one stage pull through operation can be performed open, laparoscopically, or trans-anally. The decision depends on surgeon preference.

Duodenal atresia - A duodenoduodenostomy is performed. Also consent for possible Ladd procedure if malrotation is found. The same operation is performed if an annular pancreas is found.

Jejunoileal atresia - Usually a limited bowel resection and primary anastomosis is performed. The consent should also include a possible ostomy and discussion of the risk of leak or stricture.

Other procedures - It is common to place durable intravenous access at the time of surgery - either a tunneled (i.e.Broviac) or nontunneled catheter. Check with team and if planned, make sure it is on the consent with its associated complications including bleeding, collapsed lung and infection.

When to call the next level

In general, all newborn consults should be discussed immediately with a senior resident, fellow or attending surgeon. If the neonate is stable and there is low concern for perforation the call can be made after imaging studies are obtained. If there is high concern for perforation on exam or if the baby is unstable then call immediately.

Abdominal pain - infant (one month to one year), toddler (one to five years), preschooler

Infants and toddlers may have difficulty communicating that they are in pain. Often the parent will say he/she is irritable, walking funny or inconsolable.

Key physical exam findings

The most common physical findings are abdominal distension and tenderness to palpation. A rigid abdomen may be present if there is perforation and peritonitis. Intussusception may have a sausage shaped mass on the right side of the abdomen. Incarcerated hernia will have a tender bulge with possible overlying erythema in the groin. Ovarian torsion will have tenderness, minimal peritoneal signs and possibly a palpable mass. Volvulus will have tenderness and possible rebound and guarding if ischemic bowel is present.

Differential diagnosis

Intussusceptionis more common in children less than three years of age. Appendicitis becomes more common in children over three years of age. Incarcerated inguinal hernia is more common in infants.

Imaging

Ultrasound (US) is often used as a screening tool. It will show a noncompressible tubular structure in acute appendicitis. A wall thickness greater than two mm and total diameter greater than 6 mm is consistent with apendicitis. An appendicolith may also be seen.

In intussusception US will show a target sign. An air or contrast enema is both diagnostic and therapeutic for ileocolic intussusception once the diagnosis is made. Radiographic reduction of the intussusception is the treatment of choice if there is no concern for perforation. US will also be able to diagnose ovarian torsion. If ovarian torsion is present an ovarian mass or cyst may also be identified.

If the ultrasound is nondiagnostic for acute appendicitis, a computerized tomography (CT) scan with intravenous contrast may be performed. In addition to an enlarged appendix, the CT may show an appendicolith, fat stranding and possible phlegmon or abscess. CT scans are rarely utilized in children of this age.

A Meckel scan is a nuclear medicine study that identifies ectopic gastric mucosa if it is present in the Meckel diverticulum.

A contrast enema is useful in diagnosing HD as it may show a transition zone where a narrow aganglionic rectum gives way to a dilated colon more proximally.

Indications for surgery

Immediate surgery is indicated if there is a concern for intestinal perforation. Incarcerated hernias that cannot be reduced also require urgent intervention. Sedation may be administered in the emergency department with reattempt at reduction. An asymptomatic Meckel diverticulum found incidentally on imaging does not need surgery. Meckel diverticulitis will often be diagnosed at the time of surgery for presumed appendicitis. It is rare that a bleeding Meckel diverticulum will require an urgent operation. Intussusception requires surgery if it cannot be otherwise radiographically reduced by contrast enema. Acute appendicitis typically requires appendectomy. Exceptions to this include early appendicitis that may be treated with antibiotics if preferred by the family and patients with an abscess who are well appearing. Ovarian torsion requires urgent operative reduction.

Observation with serial abdominal exams is a reasonable choice if the patient has equivocal findings of appendicitis.

Preoperative preparation

Empiric therapy includes IV fluids, antibiotics for perforation or acute appendicitis. Required labs include a CBC with differential, basic metabolic panel, urinalysis and possibly C-reactive protein. Most patients will benefit from imaging prior to operative intervention.

Operative consent points

In addition to the standard risks of bleeding, infection and injury to surrounding structures the specific risks of surgery will depend on the diagnosis. Most of the operations can be performed laparoscopically, but all patients should be also consented for possible open procedures. For Meckel diverticulum, a diverticulectomy can be performed or a segmental bowel resection with a primary anastomosis. Acute appendicitis is treated surgically with appendectomy. If an intussusception can not be reduced radiographically, operative reduction is performed. If this is unsuccessful, resection and anastomosis is necessary. If an incarcerated hernia cannot be reduced at the bedside, it must be surgically reduced and herniorraphy performed. A bowel resection may be required if there is strangulated bowel. For ovarian torsion, the ovary is reduced and left in place. The ovary should not be removed unless there is an obvious solid mass that is the cause of the torsion. For suspected HD, an open rectal biopsy will typically be required to make the diagnosis. Once the diagnosis is made, a leveling colostomy is typically performed followed by a pull through procedure at a later date.

Complications

There is a small risk for wound infection or abscess formation following surgery. This risk is higher for perforated appendicitis or perforated viscous. The risk of recurrent intussusception is roughly five percent. The recurrence rate is similar following radiographic and surgical reduction of intussusception.

Postoperative care

After operative reduction of the intussusception, the infant should be monitored for 12 to 24 hours in the hospital so that diet can be advanced and to look for recurrence. All postoperative patients should be monitored for pain control and advancement of diet. Antibiotics are typically discontinued unless there was a perforation or abscess.

When to call the next level

If the infant, toddler or child is stable and there is low concern for perforation, the call can be made after initial imaging studies are obtained. If there is a high concern for perforation on exam or hemodynamic instability then call immediately.

Abdominal pain – child five to 13 years

There are many causes of abdominal pain and the goal is to determine the specific cause and whether it is life threatening. At beginning consultation, keep in mind diagnoses that require quick intervention and escalate the consult accordingly for worrisome signs.

Key history points

Most surgical problems are likely to have associated gastrointestinal symptoms (e.g. nausea, vomiting, anorexia) and abdominal distension. In severe cases, patients may present with signs and symptoms of sepsis.

Appendicitis- pain that migrates to the right lower quadrant over 12 to 18 hours associated with fever and vomiting

Cholecystitis- colicky postprandial right upper quadrant pain becoming constant with vomiting, fever, jaundice, clay colored stools, dark urine, history of parenteral nutrition as an infant or newborn and bleeding disorders

Obstruction – bilious emesis, history of abdominal surgery, when was last bowel movement or flatus?

Ovarian torsion– sudden onset of pain with few gastrointestinal symptoms

Renal colic – also sudden onset usually in flank with few gastrointestinal symptoms except nausea with pain episodes

Pancreatitis– epigastric pain radiating to the back, history of gallstones, jaundice

Key physical exam findings

Look for tachycardia and fever. Malaise and poor perfusion may be present. The abdominal exam should focus on distension, tenderness, rebound tenderness, and guarding. A positive Murphy sign indicates cholecystitis. Small bowel obstruction will have abdominal distention with minimal tenderness. If tenderness is present in patients with intestinal obstruction consider possible ischemic bowel. Ovarian torsion will have tenderness, minimal peritoneal signs and possibly a palpable mass. Pancreatitis will have tenderness in the epigastric region.

Differential diagnosis

Appendicitis, cholecystitis, small bowel obstruction (SBO), ovarian torsion, nonsurgical causes (e.g.pancreatitis, inflammatory bowel disease). This is partial list and the key is determining quickly if early surgical intervention is needed (e.g. perforated appendicitis) or if the work up can progress while diagnostic (e.g. imaging, labs, repeat exams) and therapeutic (e.g.fluid resuscitation) interventions are initiated.

Nonsurgical causes of abdominal pain

The common causes of abdominal pain in this age group that elicit pediatric surgery consults include dehydration from infectious enteritis, right lower lobe pneumonia causing abdominal pain, kidney stones, pyelonephritis and pancreatitis. These are very common so taking care of the patient by hydrating them and taking a careful hiistory will help to cure the disease while making the diagnosis in many instances.

Imaging

Abdominal radiographs may show distended bowel and air fluid levels in a small bowel obstruction. Otherwise, plain films are not helpful. Residents should know which films are needed for each age group that constitute an acute abdominal series.

Ultrasound is a good initial study and will show a noncompressible tubular structure in acute appendicitis with a wall thickness greater than two mm and total diameter greater than six mm. An appendicolith may also be seen. In patients with cholecystitis, US will show gallstones, a gallbladder wall greater than four mm and edema. US may identify dilated common bile or intrahepatic ducts. Ultrasound will also be able to diagnose ovarian torsion. If ovarian torsion is present, an ovarian mass or cyst may be the etiology. Ultrasound can also visualize an edematous pancreas suggestive of pancreatitis.

If an US is nondiagnostic for acute appendicitis, a CT scan with IV and oral contrast may be performed. Also consider serial abdominal exams if the patient has equivocal findings of appendicitis. CT may show an enlarged appendix, appendicolith, fat stranding or a possible phlegmon/abscess. In intestinal obstruction, a CT scan may show proximally dilated bowel with a transition point. Noncontrast CT scans are much less sensitive in diagnosing the cause of abdominal pathology.

Indications for surgery

Immediate surgery is indicated if there is a concern for intestinal perforation. Acute appendicitis requires surgery. Exceptions to this include early appendicitis that may be treated with antibiotics if preferred by the family, and patients with an abscess who are well appearing. If bowel rest and decompression are insufficient in resolving a small bowel obstruction then surgery will be necessary. Patients with cholecystitis will need a cholecystectomy performed but if they are too sick to tolerate surgery they may first undergo percutaneous drainage. Ovarian torsion requires urgent operative reduction.

Preoperative preparation

Empiric therapy includes IV fluids (20 mL/kg bolus which may be repeated), nasogastric tube for decompression of obstruction if bilious vomiting, antibiotics for perforation, acute appendicitis or cholecystitis. Labs include a CBC with differential, basic metabolic panel, urinalysis, liver function tests, lipase/amylase and consider a C-reactive protein and lactate if severely ill. Most patients will benefit from some additional imaging prior to operative intervention. Imaging should correspond to history and physical exam.

Operative consent points

In addition to the standard risks of bleeding, infection and injury to surrounding structures, the specific risks will depend on the diagnosis and procedure. Most of the operations are done laparoscopically, but all patients should be also consented for possible open procedures. Acute appendicitis is treated surgically with appendectomy. Obstruction is treated with lysis of adhesions and possible bowel resection and anastomosis. Cholecystitis is treated with cholecystectomy with a possible intraoperative cholangiogram or possible common duct exploration if deemed necessary by the surgeon. For ovarian torsion, the ovary is reduced and left in place. The ovary should not be removed unless there is an obvious solid mass that is the cause of the torsion. If there is evidence of a systemic inflammatory response or diffuse peritonitis, ask your chief or attending if central venous access should be part of the consent.

Complications

There is a small risk for wound infection or abscess formation following surgery. This risk is higher for perforated appendicitis or perforated viscous. Complications following cholecystectomy include, bile leak, common bile or common hepatic duct injury and retained common bile duct stone. If a bowel resection is required for treatment of intestinal obstruction the complications include anastomotic leak or stricture.

Postoperative care

All postoperative patients should be monitored for pain control and advancement of diet. Antibiotics are usually discontinued unless there was a perforation or abscess.

When to call the next level

If the child is stable and there is low concern for perforation, the call can be made after the initial labs and imaging studies are obtained. If there is high concern for perforation on exam or if the child is unstable then call immediately.

Abdominal pain – adolescent male

There are many causes of abdominal pain, the goal is to determine the specific cause and most importantly whether it is life-threatening.

Key history points

Most surgical problems are likely to have associated gastrointestinal symptoms (e.g. nausea, vomiting, anorexia) and abdominal distension. In severe cases, patients may present with signs and symptoms of sepsis.

Appendicitis- pain that migrates to the right lower quadrant over 12 to 18 hours associated with fever and vomiting

Cholecystitis- colicky postprandial right upper quadrant pain becoming constant with vomiting, fever, jaundice, clay colored stools, dark urine, history of parenteral nutrition as an infant or newborn and bleeding disorders

Obstruction – bilious emesis, history of abdominal surgery, when was last bowel movement or flatus?

Testicular torsion– sudden onset of scrotal pain with few gastrointestinal symptoms

Renal colic – also sudden onset usually in flank with few gastrointestinal symptoms except nausea with pain episodes

Pancreatitis– epigastric pain radiating to the back, history of gallstones, jaundice

Key physical exam findings

Look for tachycardia and fever. Malaise and poor perfusion may be present. The abdominal exam should focus on distension, tenderness, rebound tenderness, and guarding. A positive Murphy sign indicates cholecystitis. Small bowel obstruction will have abdominal distention with minimal tenderness. If tenderness is present in patients with intestinal obstruction consider possible ischemic bowel. Testicular torsion will have tenderness, swelling and color change of scrotal skin, absence of cremasteric reflex. Pancreatitis will have tenderness in the epigastric region.

Differential diagnosis

Appendicitis, cholecystitis, small bowel obstruction (SBO), testicular torsion, nonsurgical causes (e.g.pancreatitis, inflammatory bowel disease). Remember that teenagers may present with abdominal pain from a trauma but be unwilling to admit the trauma that caused the injury. This is partial list and the key is determining quickly if early surgical intervention is needed (e.g. perforated appendicitis) or if the work up can progress while diagnostic (e.g. imaging, labs, repeat exams) and therapeutic (e.g.fluid resuscitation) interventions are initiated.

Nonsurgical causes of abdominal pain

The common causes of abdominal pain in this age group that elicit pediatric surgery consults include dehydration from infectious enteritis, right lower lobe pneumonia causing abdominal pain, kidney stones, urinary tract infection such as pyelonephritis and pancreatitis. These are very common so taking care of the patient by hydrating them and taking a careful history will help to cure the disease while making the diagnosis in many instances.

Imaging

Abdominal radiographs may show distended bowel and air fluid levels in a small bowel obstruction. Otherwise, plain films are not helpful. Residents should know which films are needed for each age group that constitute an acute abdominal series.

Ultrasound is a good initial study and will show a noncompressible tubular structure in acute appendicitis with a wall thickness greater than two mm and total diameter greater than six mm. An appendicolith may also be seen. In patients with cholecystitis, US will show gallstones, a gallbladder wall greater than four mm and edema. US may identify dilated common bile or intrahepatic ducts. Ultrasound will also be able to diagnose testicular torsion. Ultrasound can also visualize an edematous pancreas suggestive of pancreatitis.

If an US is nondiagnostic for acute appendicitis, a CT scan with IV and oral contrast may be performed. Also consider serial abdominal exams if the patient has equivocal findings of appendicitis. CT may show an enlarged appendix, appendicolith, fat stranding or a possible phlegmon/abscess. In intestinal obstruction, a CT scan may show proximally dilated bowel with a transition point. Noncontrast CT scans are much less sensitive in diagnosing the cause of abdominal pathology.

Indications for surgery

Immediate surgery is indicated if there is a concern for intestinal perforation. Acute appendicitis requires surgery. Exceptions to this include early appendicitis that may be treated with antibiotics if preferred by the family, and patients with an abscess who are well appearing. If bowel rest and decompression are insufficient in resolving a small bowel obstruction then surgery will be necessary. Patients with cholecystitis will need a cholecystectomy performed but if they are too sick to tolerate surgery they may first undergo percutaneous drainage. Testicular torsion requires urgent operative reduction.

Preoperative preparation

Empiric therapy includes IV fluids (20 mL/kg bolus which may be repeated), nasogastric tube for decompression of obstruction if bilious vomiting, antibiotics for perforation, acute appendicitis or cholecystitis. Labs include a CBC with differential, basic metabolic panel, urinalysis, liver function tests including bilirubin, lipase/amylase and consider a C-reactive protein and lactate if severely ill. Most patients will benefit from some additional imaging prior to operative intervention. Imaging should correspond to history and physical exam.

Operative consent points

In addition to the standard risks of bleeding, infection and injury to surrounding structures, the specific risks will depend on the diagnosis and procedure. Most of the operations are done laparoscopically, but all patients should be also consented for possible open procedures. Acute appendicitis is treated surgically with appendectomy. Obstruction is treated with lysis of adhesions and possible bowel resection and anastomosis. Cholecystitis is treated with cholecystectomy with a possible intraoperative cholangiogram or possible common duct exploration if deemed necessary by the surgeon. For testicular torsion, the testis is reduced may be left in place. If there is evidence of a systemic inflammatory response or diffuse peritonitis, ask your chief or attending if central venous access should be part of the consent.

Complications

There is a small risk for wound infection or abscess formation following surgery. This risk is higher for perforated appendicitis or perforated viscous. Complications following cholecystectomy include, bile leak, common bile or common hepatic duct injury and retained common bile duct stone. If a bowel resection is required for treatment of intestinal obstruction the complications include anastomotic leak or stricture.

Postoperative care

All postoperative patients should be monitored for pain control and advancement of diet. Antibiotics are usually discontinued unless there was a perforation or abscess.

When to call the next level

If the teenager is stable and there is low concern for perforation, the call can be made after the initial labs and imaging studies are obtained. If there is high concern for perforation on exam or if the child is unstable then call immediately. Suspicion of a testicular torsion justifies an immediate call for help to minimize delay to surgery.

Abdominal pain – adolescent female

There are many causes of abdominal pain and the goal is to determine the specific cause and whether it is life threatening.

Key history points

Most surgical problems are likely to have associated gastrointestinal symptoms (e.g. nausea, vomiting, anorexia) and abdominal distension. In severe cases, patients may present with signs and symptoms of sepsis.

Appendicitis- pain that migrates to the right lower quadrant over 12 to 18 hours associated with fever and vomiting

Cholecystitis- colicky postprandial right upper quadrant pain becoming constant with vomiting, fever, jaundice, clay colored stools, dark urine, history of parenteral nutrition as an infant or newborn and bleeding disorders

Obstruction – bilious emesis, history of abdominal surgery, when was last bowel movement or flatus?

Ovarian torsion– sudden onset of pain with few gastrointestinal symptoms

Renal colic – also sudden onset usually in flank with few gastrointestinal symptoms except nausea with pain episodes

Pancreatitis– epigastric pain radiating to the back, history of gallstones, jaundice

Key physical exam findings

Look for tachycardia and fever. Malaise and poor perfusion may be present. The abdominal exam should focus on distension, tenderness, rebound tenderness, and guarding. A positive Murphy sign indicates cholecystitis. Small bowel obstruction will have abdominal distention with minimal tenderness. If tenderness is present in patients with intestinal obstruction consider possible ischemic bowel. Ovarian torsion will have tenderness, minimal peritoneal signs and possibly a palpable mass. Pancreatitis will have tenderness in the epigastric region. Pelvic inflammatory disease and ectopic pregnancy can have pelvic pain and cervical motion tenderness. Pancreatitis will have tenderness in the epigastric region.

Differential diagnosis

Appendicitis, cholecystitis, small bowel obstruction, ovarian torsion and nonsurgical. Many key differential diagnoses in the teenage female relate to the reproductive system.

Nonsurgical causes of abdominal pain

The common causes of abdominal pain in this age group that elicit pediatric surgery consults include dehydration from infectious enteritis, right lower lobe pneumonia causing abdominal pain, pregnancy, pelvic inflammatory disease, inflammatory bowel disease, renal stones, urinary tract infection such as pyelonephritis and pancreatitis. These are very common so taking care of the patient by hydrating them and taking a careful history will help to cure the disease while making the diagnosis in many instances.

Preoperative preparation

Empiric therapy includes IV fluids (20 mL/kg bolus which may be repeated), nasogastric tube for decompression of obstruction if bilious vomiting, antibiotics for perforation, acute appendicitis or cholecystitis. Labs include a CBC with differential, basic metabolic panel, urinalysis, liver function tests including bilirubin, lipase/amylase and consider a C-reactive protein and lactate if severely ill. Most patients will benefit from some additional imaging prior to operative intervention. Imaging should correspond to history and physical exam.

Imaging

Abdominal radiographs may show distended bowel and air fluid levels in a small bowel obstruction. Otherwise, plain films are not helpful. Residents should know which films are needed for each age group that constitute an acute abdominal series.

Ultrasound is a good initial study and will show a noncompressible tubular structure in acute appendicitis with a wall thickness greater than two mm and total diameter greater than six mm. An appendicolith may also be seen. In patients with cholecystitis, US will show gallstones, a gallbladder wall greater than four mm and edema. US may identify dilated common bile or intrahepatic ducts. Ultrasound may also be able to diagnose ovarian torsion but the presence of ovarian blood flow is unreliable. Ultrasound can also visualize an edematous pancreas suggestive of pancreatitis.

If an US is nondiagnostic for acute appendicitis, a CT scan with IV and oral contrast may be performed. Also consider serial abdominal exams if the patient has equivocal findings of appendicitis. CT may show an enlarged appendix, appendicolith, fat stranding or a possible phlegmon/abscess. In intestinal obstruction, a CT scan may show proximally dilated bowel with a transition point. Noncontrast CT scans are much less sensitive in diagnosing the cause of abdominal pathology.

Indications for surgery

Immediate surgery is indicated if there is a concern for intestinal perforation. Acute appendicitis requires surgery. Exceptions to this include early appendicitis that may be treated with antibiotics if preferred by the family, and patients with an abscess who are well appearing. If bowel rest and decompression are insufficient in resolving a small bowel obstruction then surgery will be necessary. Patients with cholecystitis will need a cholecystectomy performed but if they are too sick to tolerate surgery they may first undergo percutaneous drainage. Ovarian torsion requires urgent operative reduction. Ectopic pregnancy justifies a referral to a gynecology experienced service.

Operative consent points

In addition to the standard risks of bleeding, infection and injury to surrounding structures, the specific risks will depend on the diagnosis and procedure. Most of the operations are done laparoscopically, but all patients should be also consented for possible open procedures. Acute appendicitis is treated surgically with appendectomy. Obstruction is treated with lysis of adhesions and possible bowel resection and anastomosis. Cholecystitis is treated with cholecystectomy with a possible intraoperative cholangiogram or possible common duct exploration if deemed necessary by the surgeon. For ovarian torsion, the ovary is reduced and left in place. The ovary may be biopsied or a cyst drained but not removed unless there is an obvious solid mass that is the cause of the torsion. If there is evidence of a systemic inflammatory response or diffuse peritonitis, ask your chief or attending if central venous access should be part of the consent.

Complications

There is a small risk for wound infection or abscess formation following surgery. This risk is higher for perforated appendicitis or perforated viscous. Complications following cholecystectomy include, bile leak, common bile or common hepatic duct injury and retained common bile duct stone. If a bowel resection is required for treatment of intestinal obstruction the complications include anastomotic leak or stricture.

Postoperative care

All postoperative patients should be monitored for pain control and advancement of diet. Antibiotics are usually discontinued unless there was a perforation or abscess.

When to call the next level

If the teenager is stable and there is low concern for perforation, the call can be made after the initial labs and imaging studies are obtained. If there is high concern for perforation on exam or if the child is unstable then call immediately. Suspicion of an ovarian torsion justifies an immediate call for help to minimize delay to surgery.

Authors: Genia Dubrovsky, Wolfgang Stehr and Steven Lee

Anorectal Malformations

Problem: newborn infant without an anus

Likely diagnosis: Imperforate anus

An anorectal malformation (ARM) is a congenital defect in the formation of the normal anal canal that can affect males or females and is the consequence of abnormal events that take place during formation of the caudal end of the embryo between the fourth and eighth week of gestation. The overall incidence approaches one in 5000 live births and the distance from the normal perineal anal orifice to the end of the hindgut correlates with the degree of associated anomalies and outcome for ultimate fecal continence.

Key history points

Typically, the surgical service is asked to evaluate a newborn in whom a normal anus is not present during the newborn evaluation. Patients with an anorectal malformation may also present with abdominal distention and failure to pass meconium. The presence of meconium in the perineum does not necessarily mean the newborn has a normal anal orifice and one must carefully examine the perineum to determine that there is a normal caliber anal orifice centered within the anal sphincter complex.

Patients with an ARM have a higher incidence of associated anomalies - usually within the VACTERL complex of malformations (vertebral 30%, esophageal or duodenal atresia or tracheoesophageal fistula 10%, genitourinary 30 to 50%, cardiovascular 30%, gynecologic 10%) and the surgical consultant should also address the possibility of these potential malformations during the initial evaluation.

The prenatal history may be significant for polyhydramnios (which suggests an associated esophageal atresia as isolated anorectal malformations do not generally produce amniotic fluid problems), cardiac abnormality, distended bladder, bowel or pelvic mass. In most cases, the prenatal history is normal. The family history may reveal ARM as there is a two percent risk in patients with first degree relatives of patient with ARM. The neonatal history prior to consultation may show immediate choking and gagging with feeding (suggesting an esophageal malformation), cyanosis or murmur (suggesting a cardiac malformation).

Key physical exam findings

Although a complete physical examination is warranted, one should focus on anomalies within the VACTERL spectrum as up to 60% of patients will have an associated anomaly.

General - Look for signs of Down Syndrome (i.e. trisomy 21, the most common in association with an ARM) include low muscle tone, flat facial features, epicanthal folds, flat nasal bridge, small and dysplastic ears. Also note any other signs of a possible genetic syndrome.

Esophagus - If the infant has not fed and there is no visible anal opening, passage of an orograstric tube is warranted to confirm esophageal continuity and decompress the gastrointestinal tract until a decision has been made about fecal diversion or primary repair. If the infant has fed successfully, placement of an orogastric tube is still reasonable if there is no obvious anal opening.

Cardiac - Note murmur, capillary refill time, respiratory effort and skin color (cyanosis may indicate severe cardiac anomaly).

Abdomen - Note muscle integrity (laxity compatible with prune belly syndrome), distention, masses, umbilical anomalies or drainage or urine from the umbilicus. A lower midline mass in a female may represent hydrocolpos or a distended bladder in the setting of a cloacal malformation.

Extremities - Note obvious anomalies of the fingers, hands, distal forearms (radial aplasia).

Perineum, buttocks and lower back - Note the general appearance of the buttocks and the gluteal crease. Is the bottom “flat" or normal in contour? Is there a sacral pit or an abnormal gluteal crease? Is this cloacal exstrophy (i.e. omphalocele, extrophied hemibladders with midline cecal plate, prolapsed ileal “trunk” and an imperforate anus)? Is there an anus located in the normal location? Is the anus patent? Is there an orifice which is located outside of the sphincter complex whereby meconium is expressed (a small perineal opening or an opening in the vestibule in a female)? A “anteriorly displaced” anus may represent a normal variant so long as the orifice is of adequate size, lined by mucosa and centrally located within the sphincter complex. If the anus is small in caliber is there a visible dentate line or is it lined by skin (i.e. anal stenosis and/or funnel anus)? If the anus is normal in appearance, a passage of a small caliber lubricated dilator may elicit an evacuation of meconium (suggesting Hirschsprung disease) or, if there is obstruction to passage, rectal atresia. A normal size neonatal anus in a term infant should admit a lubricated 11/12 Hegar dilator. In patients who are premature you should call your next level before inserting a dilator.

Male - Are both testis descended? Is there hypospadias (i.e. dorsally hooded foreskin, urethral orifice not located at tip of glans)? Note any beading of meconium at urethral orifice. Is there a “bucket handle deformity” (i.e. a midline skin bridge)?

Female - Examine the posterior fourchette in attempt to identify a fistula posterior to the vagina. This is best performed by retracting the labia upward and laterally. Also note if there are separate urethral and vaginal orifices in a female – a single orifice in the setting of no anal opening represents a cloacal anomaly.

Preoperative preparation

Studies to order upon suspicion of an anorectal malformation (at time of initial consultation) - Plain film radiographs (e.g. chest film or “babygram” to look for vertebral or rib anomalies), echocardiogram, renal ultrasound. Routine laboratory studies are generally not necessary.

Studies to consider after discussion with next level or attending staff (depends on nature of anomaly an institutional guidelines) - Invertogram - (i.e. lateral prone plain radiograph, reserved for the male patient) - place radio-opaque marker on perineal skin in location where anus should be and take film 24 hours after birth. If gas is present below the coccyx then primary repair may be an option.

Spinal ultrasound for screening for tethered spinal cord, is accurate before three months of age) and evaluation of presacral mass.

Voiding cystourethrogram (VCUG), pelvic or perineal ultrasound is especially helpful in females with a cloaca to evaluate for hydrocolpos

Anteroposterior/lateral sacral radiogrpahs to calculate the sacral ratio are ideally performed after three months once the bony pelvis has ossified but often obtained in neonatal period.

Magnetic resoance imaging of lumbosacral spine is usually performed at three to six months of age to evaluate for spinal anomalies.

The principal neonatal concern is the evaluation of other potentially life threatening anomalies, or other anomalies which may take operative precedence over the anorectal malformation. Thereafter, surgical decision making largely depends on these factors as well as the potential malformation in question and whether the anal orifice or fistula can be dilated with adequate decompression of stool. In general, one need not make an operative decision within the first 24 hours of life which allows for a measured assessment and plan.

Perineal fistula - Many term these lesions as a “low” ARM whereby definitive neonatal repair may be possible and long term outcome is generally favorable. In a male or female with a perineal fistula and no contraindications to neonatal repair, an anorectoplasty can be performed safely in the neonatal period without a protective colostomy. In a male or female with a perineal fistula but a contraindication to immediate repair (e.g. complex congenital heart disease) or another more pressing medical or surgical issue (e.g. esophageal atresia) and the fistula can be dilated, repair can be deferred to later date with ensuing dilations. The size of dilator should be determined by the next level surgeon, but generally a size five Hegar is a reasonable size to start with and the ultimate size to allow for adequate decompression dictated by the patient. Alternatively, some surgeons prefer to dilate and bring the patient back on an elective basis for definitive repair so you should inquire about what your attending surgeon prefers.

In male patients with no identifiable fistula after 24 hours, an invertogram that demonstrates gas above the coccyx, obvious meconium in the urine or at the urethral meatus or there are other life threatening situations and dilation is not an option, a diverting colostomy is indicated. This is generally completed during daylight hours after 24 hours of life.

In female patients with a rectovestibular fistula, the surgeon will decide whether primary repair without a colostomy or an initial diverting colostomy is preferred. Alternatively, some will delay repair if the fistula can be dilated in a similar fashion to the patient with a perineal fistula. In females with a cloaca (i.e. a single perineal opening) an evaluation of the genitourinary tract is tantamount to determine if hydrocolopos is present. If so, drainage of the vagina along with a colostomy will be required. If there is no hydrocolpos a colostomy alone is sufficient.

Operative Consent points

Consent for different types of anoplasty or anorectoplasty operations should be addressed by attending staff.

A colostomy may be done laparoscopic or open although a traditional colostomy is performed as a completely diverted proximal sigmoid colostomy performed through a left lower quadrant oblique incision whereby the proximal functional colostomy is matured through the lateral aspect of the skin incision and the mucous fistula matured as a diminutive opening at the medial side of the incision. The purpose of the procedure is to divert the fecal stream and irrigate all of the meconium from the distal bowel. The diminutive opening in the distal colon as a mucous fistula allows for ease of ostomy appliance placement, reduces the risk of prolapse and facilitates the performance of a high pressure distal colostogram at a later date. Risks include bleeding, infection, injury to surrounding structures, fascial stenosis at colostomy exit, stoma prolapse or retraction (long term) and need for stoma revision.

When to call next level

In most cases, you should call your next level once you have seen and evaluated the patient to confirm which studies should be ordered and the timing of these evaluations. The attending surgeon should be notified promptly so that a definitive management plan can be instituted and the family can be approached about the nuances of the work up and potential operative interventions. The patient should remain NPO until a definitive operative plan has been made.

Author: Casey Calkins

Biliary Atresia

Problem: White, acholic stools

Likely diagnosis: Biliary atresia

Epidemiology

Biliary atresia (BA) is the most common cause of end stage liver disease in infants. The estimated incidence in the United States is 0.73 per 10,000 live births with a much higher incidence in Asian and Pacific nations. There are three types of BA, with the majority (85%) being sporadic and isolated BA. The other 15% are syndromic, either with laterality defects (biliary atresia splenic malformation (BASM) syndrome) such as situs inversus or intestinal malrotation. In addition to this classification, BA can also be classified anatomically. Type I is located at the common bile duct (distal type, ~12%). Type II is located at the common hepatic duct (proximal type, ~2%). Type III, the most common type, involves the porta hepatis (~85-90%).

anatomic classification

used with permission Ann Surg 254:4, 577-585, 2011. Copyright Wolters Kluwer Health, Inc.

Key history points

Usually a term infant with normal birth weight who develops progressive jaundice and acholic stools. This jaundice persists beyond the physiologic two week period. Stools are classically acholic, though meconium may initially be passed and stools may be yellow in some patients. Dark urine from hyperbilirubinemia can sometimes make acholic stools difficult to identify in a mixed diaper.

Key physical exam findings

The liver may be large, palpable and firm. There may be splenomegaly if the process is advanced.

Differential diagnosis

The differential diagnosis of conjugated hyperbilirubinemia in infants less than two months of age is extensive. Common conditions that must be considered are neonatal hepatitis, drug- induced/toxic cholestasis (especially PNALD) and perinatal infections (such as the TORCH pathogens). Hereditary syndromes such as Alagille and progressive familial intrahepatic cholestasis (PFIC) must also be considered. The workup proposed below can be useful to eliminate some of these diagnoses although ultimately a cholangiogram is necessary to arrive at a definitive diagnosis. Even liver biopsy is not always able to differentiate biliary atresia from neonatal hepatitis and PNALD.

Imaging

An abdominal ultrasound should be performed to assess the liver, evaluate for the presence of a normal gallbladder (absence is quite specific for BA) and look for other sources of extrahepatic biliary obstruction. In addition, intrahepatic biliary dilation is not a feature of BA and should prompt workup for other conditions. Hepatobiliary scintigraphy (i.e. HIDA scan) can demonstrate patency of the bile ducts if excretion all the way to the intestine is seen. This would rule out BA. However, in the setting of profound hyperbilirubinemia, several days of pretreatment with phenobarbital is needed and there can still be a high false positive rate due to poor bile flow. Thus scintigraphy can be useful to rule out BA but not necessarily to definitively diagnose BA. Other imaging studies such as endoscopic retrograde cholangiopancreatography (ERCP) or magnetic resonance cholangiopancreatography (MRCP) have been utilized but are poorly studied, have limited availability, significant cost and risk (in the case of ERCP) and are not routinely utilized. The gold standard for diagnosis is cholangiography. The demonstration of patent bile ducts with the flow of contrast into the intestine and refluxing into the intrahepatic bile ducts rules out BA. This final confirmatory test can be done percutaneously or, more frequently, intraoperatively at the time of planned surgical intervention.

Preoperative preparation

The work up of biliary atresia should be performed in any newborn with conjugated hyperbilirubinemia and acholic stools. This needs to be done efficiently. There is no uniformly endorsed strategy for evaluation. While each individual test has limited sensitivity and specificity, the test results can be pieced together to come to a likely diagnosis. Laboratory evaluation will demonstrate elevated conjugated (direct) bilirubin as early as 24 hours of life in most patients with BA but this normally peaks between 4 and 6 mg/dL rather than continuing to climb higher as in some other conditions. Patients will also have elevated hepatic transaminases and gamma glutamyl transferase (GGT) levels. Coagulation parameters should be obtained due to the risk of poor vitamin K absorption and coagulation protein synthesis. In addition, blood cultures should be obtained to assess for infection. Additional workup includes laboratory tests to assess for other possible diagnoses, such as genetic tests, TORCHS (toxoplasmosis, Other infections, Rubella, Cytomegalovirus, Herpes, Syphilis) titers and alpha-1 antitrypsin assessment. Standard screening studies should also be obtained including ultrasound and tests for hypothyroidism, cystic fibrosis and galactosemia. Liver biopsy may also be obtained during the workup for BA but is limited because the histopathological findings of BA (e.g. expanded portal tracts, bile duct proliferation, portal fibrosis, portal tract edema with inflammation) can overlap with neonatal hepatitis and parenteral nutrition associated liver disease (PNALD).

Indications for surgery

Biliary atresia should be addressed surgically as soon as the diagnosis is made. In many institutions, surgical repair is planned at the same time as confirmatory cholangiogram. Thus, some surgeons will approach the initial assessment laparoscopically with a laparoscopic cholangiogram to confirm the diagnosis. Surgical treatment with portoenterostomy (i.e. Kasai procedure) has been shown in randomized assessment to have better outcomes when performed with a standard laparotomy and most surgeons will start “open” or convert to “open” once the definitive plan for Kasai has been made. In addition, given the rarity of the disease and the importance of appropriate surgical and multidisciplinary care, centralization of care to high volume centers has been shown to be helpful in improving outcomes.

The Kasai procedure involves the placement of a Roux-en-Y connection from the portal plate (identified by following the scarred and obliterated hepatic ducts up to the liver at the bifurcation of the portal vein) to the jejunum (portoenterostomy). The jejunum is typically transected at the first easily mobile loop of bowel distal to the ligament of Treitz. The distal end is brought up to the portal plate and an anastomosis fashioned in an end to side configuration. The proximal end is used to create a jejunojejunostomy - typically 40 cm distally. A drain at the level of the portoenterostomy may be left to control lymphatic or biliary leak.

Operative consent points

The goal of the operation is to remove the atretic biliary tree and reconstruct with an Roux-en-Y connection to the portal plate. The risks include bleeding, infection, anesthesia, leakage from the new anastomoses and bowel obstruction.

Complications

Short term complications include vascular injury to the hepatic artery or portal vein and lymphatic or biliary leakage. Cholangitis is the most common complication and can occur at any time following creation of the portoenterostomy. It is characterized by fever, abdominal pain, leukocytosis and elevated liver function tests. Aggressive treatment with antibiotics is necessary. Long term complications include poor nutrition due to poor fat absorption with resultant decreased central nervous system development and consequences of poor fat soluble vitamin absorption such as pathologic fractures due to low vitamin D function. Ultimately, portal hypertension and/or liver failure may develop if biliary drainage is not accomplished with the portoenterostomy. This will result in clinical findings such as ascites, thrombocytopenia, splenomegaly and variceal bleeding.

Prognosis

Success after Kasai portoenterostomy can be defined as adequate biliary drainage. This is traditionally thought of in thirds: one third of patients will never drain adequately, one third will drain well initially but go on to progressive biliary fibrosis, recurrent jaundice and ultimate liver failure and the final third will drain well and have long term successful biliary drainage with the portoenterostomy. Factors predicting improved likelihood of success include younger age at diagnosis and surgery (less than 45 day of age), absence of the biliary atresia-splenic malformation syndromic variant and less fibrosis on liver biopsy at the time of surgery. Ultimately, for those failing drainage and developing progressive liver failure, transplant is necessary for survival.

When to call next level

When faced with this consult, the baby is usually not acutely ill so the work up can be initiated following a good history and physical exam. If the baby appears ill or has unstable vital signs, pointing to a diagnosis other than biliary atresia, an earlier phone call to a senior resident or attending is warranted.

Author: Biren Modi

Branchial Anomalies

Problem: draining neck sinus or mass

Likely diagnosis: branchial anomaly

Branchial arches, clefts and pouches form during the fourth to seventh week of gestation. Some of these arches, clefts and pouches regress; others give rise to structures in the head, neck and upper chest. Incomplete regression results in cysts, sinuses, fistulae or cartilaginous remnants termed branchial anomalies. These anomalies account for 25% of cervical masses in children with second branchial anomalies being the most common (approximately 95%). In order to understand branchial anomalies it is critical to understand the embryology and anatomy. Each of the arches normally develops into specific structure identified below. If there is an incomplete regress a cyst, sinus or fistula develops and one may predict where it will present and what surrounding structures will be at risk during a dissection.

Arches develop into:

First - ear, eustachian tube, mastoid air cells, tympanic membrane, external auditory canal, maxillary artery, muscles of mastication, trigeminal nerve, Meckel cartilage (stapes, styloid process, portion of hyoid bone)

Second - facial nerve, muscle of facial expression, stapedial artery, tonsillar fossa, palatine tonsil, Reichert cartilage (stapes, styloid process, hyoid bone)

Third - inferior parathyroid gland, thymus, carotid artery, stylopharyngeus muscle, glossopharyngeal nerve, hyoid

Fourth - superior parathyroid, thyroid c-cells, cricothyroid muscle, most of the pharynx and palate, vagus, superior laryngeal nerve, thyroid and epiglottic cartilages

Fifth - regresses

Sixth- pulmonary artery, muscle of larynx except cricopharygeus, vagus and recurrent laryngeal nerve, cricoid cartilage, arytenoid complex

Key history points

The chief complaint depends on the location.

First - Type 1 - duplications of the external auditory canal; Type 2 - ectodermal and mesodermal elements and pass medial to the facial nerve. Cyst, sinuses and fistula form between the external auditory canal and angle of the mandible. The may be symptomatic or painful and cause parotid inflammation or chronic clear or purulent drainage from the ear.

Second - Type 1 - anterior to the sternocleidomastoid (SCM); Type 2 - anterior and deep to the SCM, anterior or posterior to the carotid sheath; Type 3 - between the internal and external carotid, adjacent to pharynx; Type 4 - medial and deep carotid sheath, next to the pharynx and tonsillar fossa opening into the pharynx. They present as a cyst, sinus or fistula anterior to the SCM in the lower neck.

Third and fourth - These may present with recurrent neck infections or abscess, suppurative thyroiditis and enlarging cyst or abscess may cause airway compromise, torticollis or dysphagia. It is unclear if fourth branchial anomalies truly exist.

While these are developmental anomalies and often diagnosed in infancy or childhood, especially if a sinus or fistulae, some do not present until adulthood.

It is important to determine the location of mass, cyst, sinus, or fistula, duration, past infections, changes over time, difficulty swallowing, ear infections or drainage. Approximately 20% will have previously been infected.

Key physical exam findings

The location, size and shape of cyst, mass, sinus or fistula should be noted. It is important to examine the inside of the ear, oropharynx including the pyriform sinus and tonsillar fossa. Does the mass move with swallowing or when patient extends his/her tongue? Is there drainage? if so what is the amount, character and color. Are there signs of infection such as erythema, induration, pus or tenderness? Sudden enlargement of a branchial lesion may be associated with stridor, respiratory distress, torticollis or dysphagia.

Differential diagnosis

Thyroglossal duct cyst, dermoid, lymphatic malformation and parotid cyst (first branchial lesions).

Indications for surgery

Branchial anomalies should be excised due to the possibility of infection, drainage, skin changes, neck swelling, respiratory difficulty due to airway compromise and possible malignant transformation.

Preoperative preparation

Laboratory or imaging studies are typically not needed. If the diagnosis is unclear, one may consider obtaining an ultrasound. Ultrasound, computerized tomography or magnetic resonance imaging may be helpful if the lesion is complex or recurrent. An upper level resident or the attending should be asked before ordering studies.

An infection should be treated prior to surgery. Incision and drainage many be needed for an abscess but resection should be delayed until after infection has resolved in order to limit damage to nearby structures, recurrence or incomplete resection.

Operative consent points

First - The cyst or track must be excised while protecting the facial nerve and parotid gland. Paralysis is avoided in order to use a nerve monitor. The tracks may extend to deep to the facial trunk and may require parotid resection or may extend to the middle ear.

Second - An elliptical skin incision is made around sinus orifice and carefully traced using a lacrimal duct probe or suture. The tract is closely followed proximally to avoid injury to carotid artery, hypoglossal and glossopharyngeal nerves and pharynx.

Third - An endoscopic identification with contrast may assist in identification of the tract. The skin incision is made around the sinus. The tract may be difficult to discriminate from a second branchial anomaly. A third anomaly will be deep to internal carotid and glossopharyngeal nerve, pierce thyroid membrane and enter the pyriform sinus. Thyroid lobectomy may be needed.

Complications

There is a risk of bleeding, infection (five to 10%), damage to nearby structures, recurrence (two to 12%, up to 20% if infected during resection), incomplete resection, and/or need for further procedures. The definition of nearby structures varies based on the type of branchial anomaly.

First - hearing loss, damage to ear, damage to facial nerve, facial droop; first branchial anomalies require an average of 2.4 surgeries with facial nerve injury during from 10 to 40%

Second and third - damage to carotid artery, pharynx, tonsillar fossa, tongue deviation due to damage to glossopharyngeal nerve, damage to hypoglossal, damage to vagus, hoarse voice due to injury of the superior laryngeal nerve

Outcomes

Overall patients tolerate the procedure well and without long term complications. Parents should be educated on signs of infection and recurrence.

When to call the next level

Call early if the diagnosis is unclear, imaging is contemplated, airway compromise, stridor or an expanding neck hematoma is present. Otherwise, call when evaluation and physical exam is completed.

Author: Diana Diesen

Burns

Problem: Burn

Burns are significant cause of injury related death in the pediatric population. Scald burns account for the majority of burn wounds - especially in the age group two years and under. The typical mechanism of sustaining these burns involve a young child reaching up and pulling a pot with hot liquid from the stove, spilling a container of hot liquid or bathing with hot water. Cooking grease burns are example of a burn that carries a higher morbidity than other scald burns. In studying the 2000 Kids’ Inpatient Database of 10,000 children hospitalized for a burn, the highest mortality occurred with flame or fire burns. Compared to the scald patient, the flame patient sustained a significantly larger burn surface area and was more likely to be a third degree burn.

Types of burn - scald (most common), contact with thermal surface/object, direct fire/flame, chemical, electrical, smoke inhalation

Knowing the anatomy of the skin allows determination of the burn severity in terms of burn surface area, burn degree and burn depth. The resulting calculated total body surface area, degree and depth of the burn are then used to determine management. Burn injury to the skin continues to evolve during the first 36 hours.

Layers of the skin - epidermis, dermis, subcutaneous

Burn degree

First degree – injury to epidermis; painful, blanching erythema of the skin; typically spontaneously heal in several days without any scarring

Second degree – injury to epidermis and dermis; painful, intact or nonintact blistering of the skin; takes weeks to heal and in more severe cases significant scarring occurs

Third degree – injury to epidermis, dermis and subcutaneous layer; white, leathery appearance to the area burned, insensate; takes months to heal with significant scarring and contracture if no surgical intervention as intact wound edge epithelial cells migrate into the wound

Fourth degree – injury through all skin layers with exposure to the depth of muscle, tendons or bone; complex surgical intervention to cover wound (e.g. full thickness or rotational tissue flap grafting)

Burn depth

Burn injury evolves in the first 36 hours. On initial physical examination, what appears to be a superficial burn may turn out to be a deep or full thickness injury on repeat examination

Superficial partial thickness – involves injury to the upper dermis; typically heal spontaneously in two to three weeks

Deep partial thickness – involves injury to the lower dermis but not yet the subcutaneous layer; typically take much longer to heal and are associated with more significant scarring

Full thickness - injury through epidermis and dermis which results in lack of the needed skin appendages to produce new epithelium within the wound (i.e. third degree)

Key history points

Time the burn occurred

Source of the burn (water, grease, explosion, fire, hot object, chemical, electrical wire/line)

Duration of exposure to the source (house fire with smoke exposure)

Associated trauma (involved in motor vehicle crash; fell off a roof and hit electrical power line), the work up for the trauma will need to be in parallel to the work up for the burn

Volume of fluids given by the first responding team

Key physical exam findings

Blistering or pale, leathery appearance

Circumferential injury

Intact sensation over burn wound

Signs of mental status changes

Signs of respiratory distress or stridor

Soot in nasal passages or oropharynx

Anterior sparing of the body from any burn injury (suspicious for nonaccidental trauma)

Determine the percent total body surface area (TBSA)

Because of key anatomical differences in children compared to adults, the “Rule of Nines” is not used for pediatric patients. The calculated percentage TBSA is used to determine if the child requires specific fluid resuscitation. The Lund Browder chart takes into account the larger surface area of the head for children.

total body surface area

Lund Browder chart

Imaging

Typically, imaging is not needed primarily for the burn. It is more likely part of any other sustained traumatic mechanism. Imaging may be needed for evaluating for possible concomitant traumatic brain injury or for prognosis.

Management

If there is no concomitant trauma and the patient is hemodynamically stable with a stable airway, timely characterization of the burn wound with regards to degree, depth and % TBSA are key in providing optimal management and therapy.

Perform the initial early assessment of extent of burn and early gentle debridement.

Re-evaluate burn in the first 24 to 36 hours to ensure accuracy of initial characterization and to note any evolution of the injury that would require a change in therapy.

The child should be up to date on tetanus. The use of intravenous antibiotics empirically or with known burn wound infection is controversial.

Encourage enteral feeding in children showing poor oral intake or experiencing multiple periods of NPO status for optimal wound healing.

The following criteria from the American Burn Association should prompt early transfer to a center with burn experience for optimal outcomes - burns greater than 10% TBSA, any third degree burn, high voltage electrical burns, smoke inhalation, burns involving face, hands, feet, genitalia, perineum or joints and burns in patients with a complex medical history or medical conditions.

Less than 15% TBSA - Typically, fluid resuscitation is not needed as the extent of this injury typically does not stimulate a systemic inflammatory response syndrome with resultant fluid shifts.

15% TBSA - Fluid resuscitation using formula (Parkland or Galveston) in addition to maintenance intravenous fluid rate. The goal is to maintain urine output of 0.5 to 1 mL/kg/hour

Parkland formula 4 mL x kg x %TBSA = total volume of lactated Ringers. Give half of total volume over first eight hours from the time the burn was sustained Example: if initiating IV fluids 5 hours since the burn, give half of the volume over 3 hours then: Give remaining half over next 16 hours

Galveston formula 5000 mL/meter² x %TBSA = total volume of lactated Ringers. Give half of total volume over first eight hours from the time the burn was sustained and give the remaining half over next 16 hours.

Intensive care unit admission to monitor fluid resuscitation. Avoid over or under resuscitation and prolonged and multiple NPO periods – restore and optimize nutrition as early as possible.

Burn wound care

The goals are to prevent wound infection, control pain, preserve function, optimize wound healing time, scar management and maintain excellent nutrition

The initial debridement is performed immediately after patient is stabilized. The child may need sedation. True full thickness/third degree burns are insensate and sedation is not necessary. Use saline moistened sponge to gently remove necrotic or nonintact skin overlying the burn wound area to expose the true depth and surface area extent of the injury. Avoid hypothermia with prolonged skin exposure or excessive saline use. Determine burn degree, depth and %TBSA expeditiously. Apply topical ointment/cream directly to burn wound and cover with a nonadherent dressing. Re-evaluate the wound in 24 to 36 hours.

Topical dressings

Ointments/creams typically need to be applied once or twice daily to the burn wound.

Bacitracin - typically used for face; has great Gram positive bacterial coverage

Silver sulfadiazine - Gram positive and gram negative bacterial coverage

Mafenide for ears

Silver impregnated dressings are typically left in place over the burn wound for three to five days

Skin substitutes can be used as a topical dressing if topical creams/ointments are not available. They are more expensive and often used as a temporary graft to cover the burn wound bed after excision when autologous skin is not readily available.

Biological dressings (e.g. xenograft, cadaveric skin) is used for temporary coverage for several days to two weeks. Due to being nonautologous, these dressing eventually slough from the wound.

Bio-engineered dressings are most expensive and can not be applied to an infected burn.

Indications for surgery

Early (less than 48 hours from injury) excision and grafting requires experienced burn surgeons to determine which major burns would benefit from this. It decreases morbidity associated with SIRS and infection.

Later excision and grafting is an option for deep partial thickness or full thickness burns that with significantly prolonged healing.

Sharp debridement is required for infected burn wounds.

Excision and Standard Split Thickness Grafting

General anesthesia is required. Sharply excise the burn wound tissues leaving behind viable bleeding wound bed. Harvest a sheet of autologous skin from patient (typically the upper thigh or buttocks) using dermatome. Mesh or leave whole the harvested autologous sheet graft. Lay graft sheet in wound bed and secure with desired dressing. Leave graft in place undisturbed for five to seven days before doing the first dressing change.

Complications

Acute

Burn wound infection/sepsis

Bleeding from burn wound sharp debridement down to healthy bleeding tissues

Topical dressing with gram negative coverage - Pseudomonas is the most common culprit in infections, frequent re-evaluation of the burn wound. Skin grafting may be needed but only once the burn wound infection is controlled

Long term

Hypergranulation of the healing burn wound should be treated with silver nitrate application or surgical sharp debridement.

Scar contracture may require scar contracture release with grafting and splinting.

Psychosocial, behavioral, emotional and social difficulties can occur due to the acute trauma, pain of the injury and any permanent disfigurement.

When to call next level

Characterizing a burn on initial physical exam has been shown to be challenging in children. This is likely due to their wide range in size, development, etiologies of the burn and severe anxiety. Often, depth and %TBSA determinations are inconsistent and even significantly inaccurate between providers. This is true even in the more experienced providers who care for pediatric burn patients. Only a handful of centers throughout the country have met the verifications specifically for pediatric burn care. Thus, most pediatric burn patients should have early higher level physician involvement.

Author: Kathryn Bernabe

Congenital Diaphragmatic Hernia

Problem: Respiratory distress in newborn

Likely Diagnosis: Congenital diaphragmatic hernia

Congenital diaphragmatic hernia (CDH) is one of the hallmark pediatric surgical diseases. It is reported to occur in as high as 1:2000 births. One third of patients have an associated congenital anomaly. There is a wide spectrum of associated anomalies including chromosomal anomalies or as part of a defined syndrome (Simpson-Golabi-Behemel and Beckwith-Weidemann). For the most part, genetic predisposition is incompletely understood.

The majority of defects are left sided – right-sided (10 to 20%) and bilateral defects (less than one percent) are rare and have worse outcomes. It is more common in males. “Bochdalek” – type defects are located posterior and lateral and account for 90% CDH cases.

If a prenatal diagnosis is made, referral should be made for care at a facility with advanced neonatal intensive care capabilities. Chromosomal/genetic screening should be offered (e.g. CVS, amniocentesis), and the family should be counseled regarding treatment options and outcomes.

Fetal diaphragm repair is currently not performed routinely in the US. Other fetal treatments such as temporary tracheal occlusion are still experimental.

CDH is a consequence of abnormal pleuro-peritoneal separation during the fourth to eighth weeks of development. The pleural and peritoneal cavities are known to communicate during that time. Closure of this communication is complex and failure to do so results in CDH.

Pulmonary hypoplasia amongst other complex factors leads to pulmonary hypertension and persistent fetal pulmonary circulation that can be medically refractory and lethal. Medical treatment strategies are all based on the reduction of pulmonary hypertension

types of CDH

Some anomalies are associated with CDH while others are a consequence of the disease.

Associated anomalies (40 to 60% of cases) - cardiovascular, urogenital, musculoskeletal and central nervous system anomalies may be observed

Consequential anomalies (occur along with CDH diagnosis) - lung hypoplasia, intestinal malrotation and patent ductus arteriosus.

Key history points

Prenatal diagnosis Approximately 50 to 70% of CDHs are diagnosed during pregnancy. The majority of these are seen on ultrasound with the characteristic findings of polyhydramnios, bowel loops in the chest, echogenic chest mass and an intrathoracic stomach. Fetal MRI can aid in characterizing the defect and prognosis. Poor prognostic indicators include liver herniation into the chest (“liver up” presentation), low lung-to-head ratio (LHR) with LHR less than 1.35 associated with decreased survival.

Postnatal presentation Infants display the symptoms of respiratory distress immediately or within 48 hours - tachypnea, chest wall retractions, grunting, cyanosis and pallor

Key physical exam findings

Physical exam may show a scaphoid abdomen, increased chest diameter and auscultation of bowel sounds in the chest.

Differential diagnosis

The differential includes bronchopulmonary malformations (e.g. congenital pulmonary airway malformation (CPAM), bronchogenic cysts, bronchial atresia, bronchopulmonary sequestrations), mediastinal lesions (e.g. enteric, neuroenteric or thymic cysts) and diaphragmatic pathology (e.g. diaphragmatic eventration).

Imaging

Chest radiograph usually confirms the diagnosis. An echocardiogram is obtained as a baseline to assess the degree of pulmonary hypertension and repeated as necessary after any medical interventions.

left CDH radiograph

Preoperative preparation

The initial management focuses on stabilization – intubation and ventilator support for respiratory distress, placement of an enteric decompression tube, venous and arterial access for monitoring arterial blood pressure and blood gasses as well as central venous pressure (e.g. umbilical vein catheter). Acid-base balance and oxygenation-ventilation status should be trended.

Conventional modes of ventilation are usually attempted first in pressure-controlled mode. It is important to minimize barotrauma (peak inspiratory pressure (PIP) should be limited to less than 25 cm H₂O. Permissive hypercapnia may be tolerated and the PaCO₂ should range between 45 and 60 mm Hg and a pH greater than 7.2 should be maintained. The FiO₂ should be titrated to a preductal PaO₂ greater than 60 mm Hg with a preductal SaO₂ greater than 85%. Additional ventilation strategies include high frequency oscillation with or without the addition of pulmonary vasodilating agents (e.g. inhaled nitric oxide, sildenafil, remodulin).

Extracorporeal membrane oxygenation (ECMO) can be used in patients failing to respond to the above or as an adjunct – the use is institution specific. Blood is passed through a circuit where it is oxygenated. Bleeding can be a consequence of the anticoagulation required. Relative contraindications to ECMO include significant congenital anomalies, lethal chromosomal anomalies, intracranial hemorrhage, birth weight less than 2 kg and gestational age less than 34 weeks .

ECMO survival

Indications for surgery

Historically CDH was considered a surgical emergency. Currently, nonemergent surgery is performed in most centers after the establishment of cardiopulmonary stability – loosely defined as stable ventilator support with evidence of resolving pulmonary hypertension by echocardiogram. The most important factors are improved pulmonary compliance and decreased pulmonary hypertension. These criterion can also be applied to patients on ECMO.

The surgical goals are the reduction of the abdominal viscera from the pleural space with relief of mass effect on intrathoracic structures and restoration of normal pleuroperitoneal separation.

The repair can be accomplished from an abdominal or a thoracic approach. Both approaches can be performed open or minimally invasively. Classically, left sided CDH repair is performed through a left subcostal incision. The abdominal contents are placed back in the peritoneal cavity and the diaphragmatic defect is closed. If the defect cannot be closed primarily with sutures, a number of biologic and synthetic mesh options are available. Flap/tissue repairs have also been described. Chest tubes are not routinely left in place postoperatively.

Operative consent points

The procedure is closure of diaphragm. Depending on the scenario, this may be done open or with a minimally invasive procedure. The risks are infection, failure of the repair and need for other procedures.

Complications

A lack of intra-abdominal domain prior to repair can lead to abdominal compartment syndrome. In some cases the abdomen may be left open if intraoperative PIP is greater than 25 mmHg. Patients on ECMO and who are anticoagulated are at risk for bleeding. Pulmonary status may worsen temporarily after or during repair.

Postoperative care

Survival rates range from 25 to 83%. This reflects a range amongst institutions and a range of disease presentation. Birth weight, APGAR scores, associated anomalies and defect size have all been linked to prognosis.

CDH survivors may have a higher rate of chronic lung disease, neurodevelopmental problems, sensorineural hearing loss, gastroesophageal reflux requiring fundoplication, scoliosis and other chest wall abnormalities.

Recurrence of the hernia is common (15 to 40%) with both patch and primary repairs and requires surgical intervention.

When to call next level

A consult for a baby with CDH should prompt an immediate call to your senior resident or attending as you gather information and examine the patient. The baby will be acutely ill.

Authors: A. Francois Trappey, Rebecca Stark and Shinjiro Hirose

Congenital Pulmonary Airway Malformations

Problem: Respiratory distress or prenatal diagnosis

Likely diagnosis: Congenital Lung Malformations

Anatomy

There are four common congenital lung malformations, all of which are developmental anomalies of bronchopulmonary tree maturation. These include congenital pulmonary airway malformations (CPAM), bronchopulmonary sequestrations (BPS) which are broken down further by intrapulmonary sequestration and extrapulmonary sequestration, congenital lobar emphysema (CLE) and bronchogenic cysts. The estimated incidence of all congenital lung malformations combined is between 1 in 2,000 to 12,000 live births. CPAM is the most common lesion, representing approximately 60% of cases. CPAMs, BPS and bronchogenic cysts are more commonly diagnosed prenatally on ultrasound while CLE is infrequently diagnosed prenatally because they do not produce mass effect until respirations begin after birth.

CPAMs are benign cystic pulmonary lesions composed of macrocysts (greater than five mm) and/or microcysts (less than five mm). Neither microcystic nor macrocystic CPAMs contain normal alveoli and therefore function poorly with respect to gas exchange. CPAMs are located in one of the lower lobes in approximately 75% of cases.

BPS is similar to CPAM but are distinguished based on its lack of communication to the native airways and systemic arterial blood supply. BPS lesions are classified as either intralobar (75%) or extralobar (25%). Intralobar BPS lesions have pulmonary venous drainage while extralobar BPS lesions have systemic arterial and venous drainage.

CLE most frequently affects the left upper lobe which typically appears overinflated.

Bronchogenic cysts are typically located in the mediastinum and are cysts containing cartilaginous elements which are lined with ciliated mucus secreting columnar epithelium that can fill the cyst with mucinous secretions.

Pathophysiology

Congenital lung malformations likely arise during aberrant pulmonary development which begins as early as 4 to 8 weeks gestation.

Large fetal lung masses can cause mediastinal compression which can manifest as hydrops and fetal demise in utero. Impairment in fetal swallowing because of esophageal obstruction can lead to polyhydramnios which increases the risk of preterm labor. Postnatally, large lung malformations can cause severe respiratory distress at delivery. In most cases of CLE there is an anatomic or functional bronchial obstruction proximal to abnormal lung parenchyma. This can lead to progressive air trapping as gas enters freely into the CLE but has difficulty evacuating upon exhalation.

Key history points

Most congenital lung malformations are diagnosed prenatally at the time of the anatomic survey and are asymptomatic after birth (85 to 90%). Most cases initially diagnosed in the postnatal period present with either respiratory distress as infants (10 to 15%) or pneumonia in older children.

Key physical exam findings

Large or symptomatic congenital lung malformations can cause significant respiratory distress, tracheal deviation, mediastinal shift and a lack of breath sounds on the affected side. Asymptomatic lesions typically have normal physical exam findings.

Differential diagnosis

The differential diagnosis of prenatal congenital lung lesions include the four main lesions discussed here in addition to less common lesions such as bronchial atresia, fetal lung interstitial tumor, pulmonary vascular malformations and hybrid lesions. If diagnosed later in life the most common differential diagnosis is lobar pneumonia.

Imaging

A symptomatic infant should undergo plain chest radiography and then a CT angiogram of the chest if stable enough preoperatively. Echocardiography may be of value to document pulmonary hypertension and allow for proper preoperative and intraoperative anesthetic management.

An asymptomatic infant who can tolerate feeds and has no respiratory distress only needs a plain chest radiograph after birth. A CT angiogram can be performed at two to four months of age to confirm the persistence of the lesion as well as assist in preoperative planning.

Indications for surgery

Patients who experience respiratory distress as a result of a congenital lung lesion typically do so as a result of the space occupying nature of the mass. Removal of the mass is undertaken to improve respiratory function or alleviate mediastinal compression and improve cardiac performance. In asymptomatic patients, resection is undertaken to prevent future complications of infection, pneumothorax, heart failure or malignancy. Performing the operation early during the alveolar phase of lung growth theoretically allows for compensatory lung growth. Resection may be deferred in patients with multifocal CPAMs in an asymptomatic patient where multiple lobectomies or segementectomies may compromise pulmonary function, an older patient with a small lesion who already has compromised pulmonary function or the patient with high operative risk with marginal benefit.

Preoperative preparation

In the symptomatic infant, basic laboratory studies and a type and cross in the event of needing an urgent thoracotomy are recommended.

In patients with asymptomatic lesions at birth, no specific laboratory testing is required. In an older patient or in the setting of recurrent respiratory infections, tuberculin skin testing, sputum culture and additional pertinent infectious workup studies are performed.

Antibiotics to clear any infectious process should be administered if applicable. The surgery should be performed in a hospital that is capable of providing high level care including the presence of a pediatric intensive care unit, minimally invasive operative equipment and pediatric anesthesiology in order to provide single lung ventilation if necessary. Preoperative antibiotics (e.g.cefazolin) are recommended as lobectomy is a clean contaminated case.

Operative consent points

Generalized risks to discuss include bleeding, infection and injury to surrounding structures. Major bleeding during a neonatal lobectomy can be life threatening and if being performed thoracoscopically may mandate converting the operation to a thoracotomy which should always be discussed with parents. Most patients will have a chest tube postoperatively so complications from chest tubes including clogging, dislodgement and/or prolonged air leaks should be discussed as well. Wound infections are rare (less than five percent) but it is important to stress the role of adequate pulmonary toilet postoperatively to avoid pneumonia.

Complications

Complications after lung resection include bleeding, prolonged mechanical ventilation, surgical site infections, prolonged air leak, bronchial plug and temporary or permanent nerve injury. A pneumothorax can occur after removal of the chest tube, which may either require further observation or a repeat chest tube placement. Recurrent or persistent cystic lesions may be seen in one to 15% of cases - more commonly with segmental resections. These typically require repeat imaging and possibly further surgical resection.

Outcomes

The long term outcome depends on the extent of the pulmonary resection. Since there is a potential for compensatory lung growth, earlier resection may lead to improved pulmonary function. Lung function after lobectomy is normal using current pulmonary function testing

When to call the next level

Call your chief or attending early for any infant with respiratory distress, cyst larger than 30% of the affected side, any mediastinal shift, or any infant where the need for stat intubation is occurring or has happened. Otherwise, call after careful history and physical is completed including any prenatal imaging reports that can be obtained.

Author: Jill Whitehouse

Esophageal Atresia and Tracheoesophageal Fistula

Problem: choking in a newborn

Likely diagnosis: Esophageal atresia and tracheoesophageal fistula (EA/TEF)

Most neonates with EA/TEF present with excessive oral secretions and feeding intolerance. Many will also have coughing or choking with feeds. Symptoms depend on the anatomic classification but most patients will have some form of proximal esophageal atresia.

Anatomy

EA/TEF occurs in 1/3500 births. 40% are born premature. 50 to 70% have at least one associated congenital anomaly. VACTERL (vertebral, anorectal, cardiac, tracheoesophageal, renal, limb deformities) is a pneumonic of commonly associated congenital anomalies.

There are five anatomic variations in patients with EA/TEF.

• type A (8%) - isolated or “pure” esophageal atresia with no tracheoesophageal fistula and associated with a long gap
• type B (1%) - proximal tracheoesophageal fistula with distal esophageal atresia
• type C (86%) - proximal esophageal atresia with distal tracheoesophageal fistula
• type D (1%) - esophageal atresia with tracheoesophageal fistula to both pouches
• type E (4%) - tracheoesophageal fistula without esophageal atresia (H or N type)

spectrum of EA/TEF

Key history points

Most patients with EA/TEF will have profound feeding difficulties. Some patients are discovered when an orogastric tube can not be passed. Neonates with obvious VACTERL anomalies (e.g. limb deformities, imperforate anus) may also have EA/TEF. Patients with type A through D present in the neonatal period. Type E may present months to years after birth with recurrent aspiration.

Key physical exam findings

Neonates may exhibit signs of respiratory distress due to aspiration and hypoventilation such as cyanosis and chest wall retractions. Abdominal distension may be seen in neonates with a distal tracheoesophageal fistula (type C and D). Neonates with type A or B often have a scaphoid abdomen. Associated anomalies which should be detected on exam include limb deformities and imperforate anus.

Differential diagnosis

The diagnosis of EA/TEF is often based on the inability to successfully pass an orogastric tube. Perforation of the cervical esophagus by orogastric tubes is well described in small, premature neonates and creation of such a false tract could be interpreted as inability to pass the orogastric tube and confused with EA/TEF. Other causes of difficulty feeding in the early neonatal period include gastroesophageal reflux, duodenal atresia, laryngeal clefts. Congenital esophageal stenosis is a rare condition that could be considered but usually presents at about six months of age.

Imaging

Imaging is used to identify the subtype of EA/TEF and diagnose associated anomalies (VACTERL). All neonates with EA/TEF should undergo plain chest and abdomen radiograph (i.e. babygram) in order to determine the position of the proximal esophageal pouch and identify the presence or absence of bowel gas. Patients with bowel gas have a distal tracheoesophageal fistula and those without bowel gas have distal esophageal atresia without fistula. A plain radiograph can also evaluate for bony spine anomalies. All neonates with EA/TEF should undergo prompt echocardiogram, renal ultrasound, and spine ultrasound. The echocardiogram is important to evaluate for congenital heart disease and determine the laterality of the aortic arch.

Indications for surgery

All patients with any variant of EA/TEF will require surgical repair. Most patients with type C EA/TEF undergo elective repair through the right chest within the first few days of life. Surgical management of neonates with type A EA/TEF varies widely between institutions but often involves placement of a gastrostomy tube and delayed definitive repair. Patients with severe respiratory compromise, profound congenital heart disease or other significant medical comorbidities may undergo division of a distal tracheoesopageal fistula and delayed reconstruction of the esophagus. Type E EA/TEF is usually repaired through a cervical incision.

Preoperative preparation

The initial evaluation will determine the degree of physiologic compromise to the patient and the anatomic subtype of the EA/TEF anomaly. All neonates with EA/TEF should be made NPO and have the tip of an 8 to 10 F oral or nasal sump suction tube left in the proximal esophageal pouch. This tube should be placed to low intermittent suction in order to prevent aspiration of oral secretions. The head of bed should be elevated to 45 degrees in order to minimize gastric reflux through a distal tracheoesophageal fistula. Patients with profound respiratory embarrassment may require emergent control of the distal tracheoesophageal fistula. If possible, intubation and positive pressure ventilation should be avoided as this may increase flow through a distal tracheoesophageal fistula with subsequent distension and possible perforation of the gastrointestinal tract. Stable patients should undergo a work up for associated anomalies prior to surgical repair (VACTERL). Most patients will need central venous access for parenteral nutrition. Stable patients usually undergo surgical repair within 24 to 72 hours after birth.

Operative consent points

The procedure will be either a thoractotomy or thoracoscopy to repair the TEF and esophagus. Regardless of the approcah, the main risks are bleeding, infection, leak from the repair of the fistula or the esophageal anastomosis as well as recurrent fistula. Long term complications include stricture, reflux and tracheomalacia.

Complications

The most common surgical complications following the repair of EA/TEF include anastomotic stricture (40%), leak (20%) and recurrent tracheoesophageal fistula (5%). Other rare complications include injury to the airway, inadvertent ligation of the right main stem bronchus and vocal cord dysfunction. During the immediate postoperative period, manipulation of the endotracheal tube or reintubation should be done with great care to avoid disruption of the tracheal closure (typically near the carina).

Outcomes

Most modern series report excellent survival in patients with EA/TEF (greater than 95%). The most important predictor of survival following repair of EA/TEF is the presence of congenital heart disease. The presence of renal anomalies also plays a significant role in long term morbidity and mortality. Tracheomalacia and gastroesophageal reflux are common in patients with EA/TEF. Chronic respiratory symptoms such as cough, asthma, wheezing and bronchitis may persist into adulthood.

When to call the next level

Prior to surgery, alert the fellow or attending surgeon if the patient requires intubation as this may necessitate emergency decompression of the GI tract through gastrostomy tube placement. After surgery, it is important to report if a previously extubated patient requires reintubation. In this circumstance, it is also important to take note of a new air leak from the chest tube or pneumothorax on chest radiograph as this could indicate disruption of the tracheal closure.

Author: John Horton

Gastroesophageal Reflux

Problem: Nonbilious vomiting with failure to thrive or "this kid needs a wrap and a feeding tube"

Likely Diagnosis: gastroesophageal reflux disease

Gastroesophageal reflux is an important and common cause of nonbilious vomiting in the infant. Over two thirds of healthy infants regurgitate more than once a day. Reflux becomes pathologic (i.e. gastroesophageal reflux disease, GERD) when the patient exhibits poor weight gain, respiratory complications, aspiration pneumonia or brief resolved unexplained event (BRUE, formerly referred to as an apparent life threatening events, ALTE).

The lower esophageal sphincter (LES) is a physiologic region made up of the inner circular muscle layer of the esophagus that extends onto the stomach. An appropriate length of intra-abdominal esophagus and the diaphragmatic crura contribute to create a region of higher basal pressure. Reflux occurs when the LES transiently relaxes or as a result of inadequate tone. The reflux of gastric contents exposes the esophagus to acid which can lead to esophagitis, strictures, laryngeal/pharyngeal inflammation, recurrent pneumonia and apnea.

Key History points

Chief Complaint: Vomiting, non bilious, non-infectious

GERD presents differently at different ages. In infants, regurgitation or vomiting is the most common presentation. Pulmonary symptoms, dysphagia, irritability due to pain and failure to thrive are also common. Older children may complain of regurgitation, cough, vomiting, heartburn, abdominal pain, or recurrent respiratory infections.

Obtaining a prenatal, birth and growth history is important. What are the type and route of feedings? When and how much does the patient vomit - every feed? Are their neurological development challenges?

Key Physical Exam Findings

Patients may display signs of malnutrition, delayed development or abnormalities on lung exam.

Differential Diagnosis

Hiatal hernia, rotational anomalies, hypertrophic pyloric stenosis, diffuse esophageal spasm, achalasia, overfeeding

Imaging

Several imaging and diagnostic studies are available - which of theses are completed and in which order varies among institution. Check with your senior or attending before ordering any of these tests.

Upper gastrointestinal series – reflux can be seen on this study but the absence of reflux does not rule GERD out as a cause of the patient’s symptoms. Useful for assessing gastric anatomy and to rule out rotational anomalies. A very poor determinant of the severity of reflux.

24 hour pH monitoring – good study to quantitatively measure esophageal acid exposure. The electrode is placed above the gastroesophageal junction and measures the pH in the distal esophagus. It is able to document number of reflux events, number of events lasting longer than five minutes and the longest episode. The reflux index is the percentage of time that the pH was less than four.

Impedance – used in combination with 24 hour pH monitoring. It measures exposure to weakly acidic and nonacidic reflux.

Esophagogastroduodenoscopy (EGD) – useful in assessing esophageal abnormalities, inflammation and assessing gastric anatomy. Biopsies can be done to evaluate for eosinophilic esophagitis, Barrett esophagitis and H. pylori.

Manometry – used to rule out other causes such as achalasia, diffuse esophageal spasm or other motility disorders.

Nuclear scintigraphy – most commonly ordered as a gastric emptying studies, these can be used to document presence of tracer in the respiratory tract which indicates GERD as a cause of pulmonary problems

Indications for surgery

Nonmedical and medical treatments precede surgical intervention.

Nonmedical treatment

The first steps for treating GERD in infants are nonmedical. A large number of patients will improve with postural and behavioral changes such as positioning the child semiupright during and after feeds, frequent burping, reduction of feeding volumes and increasing caloric density and thickening of feeds

Medical treatment

Histamine-2 receptor antagonists (e.g. cimetidine, ranitidine) - block the effect of histamine on gastrin release

Proton pump inhibitors (e.g. omeprazole, pantoprazole) - block the final pathway of acid secretion from parietal cells and are the most effective agents against acid secretion

Promotility agents (e.g. metoclopramide, erythromycin, domperidone, cisapride) – each agent has different mechanism of action. Erythromycin acts on the motilin receptor and has minimal effectiveness in GERD. Domperidone and cisapride are not approved for use in the United States due to side effects.

Surgical treatment

Surgery is indicated in patients who have failed to improve on medical therapy. Patients with esophageal damage despite maximal medical therapy are candidates for antireflux surgery.

Nissen fundoplication – This is a 360 degree wrap of the fundus of the stomach around the GE junction. This is the most common type of fundoplication performed and is performed open and laparoscopically depending on surgeon preference and patient selection.

Partial Fundoplication – Thal Fundoplication refers to anterior partial fundoplication. Toupet refers to a posterior partial fundoplication. These procedures are sometimes used in patients with poor esophageal motility but are associated with a higher risk of recurrent reflux.

Operative consent points

The operative procedure is perform fundoplication. Often a feeding gastrostomy is also placed. Ask your senior which type of fundoplication and which approach (laparoscopic or open) is planned. Regardless of type or approach, complications to discuss include bleeding, damage to intra-abdominal organs (e.g. esophagus and spleen), infection, failure of the wrap and need for revision at later date. The risks of a gastrostomy tube placement include leak and tube dislodgement. All of these are rare but need to be mentioned.

Complications

Fundoplication is well tolerated. Reoperation rates range from six to 12% and are related to wrap migration, wrap dehiscence and hiatal hernia. The number of patients on GERD medication decreases after fundoplication but up to 75% of patients will restart medication one year after surgery. The use of antireflux medication is unchanged in neurologically delayed patients. Other rare complications include wound infection, gastric perforation, dysphagia, gas bloat, recurrent GERD and wrap migration.

Postoperative Care

Infants are usually started on feeds either several hours after the surgery or by the next morning. Older children are recommended to be on a soft diet for the first few weeks as the swelling in the fundoplication resolves.

When to call next level

A consult on a patient with reflux can be discussed with your senior or attending after you obtain a good history and physical exam. They are rarely urgent or emergent operations and patients are usually referred to the clinic when medical management is failing. You should understand why the patient is failing nonoperative management when you present the patient. Also, if the patient appears ill or if you think the vomiting may be caused by a more urgent diagnosis, call your senior or attending earlier. Finally, bilious vomiting or gastrostomy output in a patient with a previous fundoplication is an emergency.

Author: Pavan Brahmamdam

Gastrointestinal Foreign Bodies

Problem: Foreign body in the gastrointestinal tract

Young children, especially toddlers, are busy exploring the world around them. They are drawn to small, shiny objects and frequently put things in their mouths which can lead to foreign body ingestion or aspiration. Poor swallowing and talking, playing or crying while eating can contribute to aspiration. In countries where fish is not the primary staple of the diet, coins are the most commonly ingested foreign body. The objects most commonly aspirated by children include food items such as nuts and seeds.

Ingestion

Foreign bodies can become impacted in the esophagus. There are three areas of physiologic narrowing.

• level of the cricopharyngeus
• mid esophagus where there is compression from the aortic arch and left mainstem bronchus
• gastroesophageal junction

In patients with a history of prior esophageal surgery other areas of stenosis may be present. Most foreign bodies are inert and require urgent rather than emergent removal. Button batteries, however, cause an electrical discharge in the esophagus which can lead to burn and perforation. Even a “dead” battery can have sufficient remaining charge to cause serious injury. Therefore button batteries must be emergently removed from the esophagus.

Foreign bodies distal to the esophagus will usually transit the remainder of the gastrointestinal (GI) tract uneventfully. However, if multiple magnets have been swallowed and are still present in the stomach, they should be removed. Multiple magnets in the distal GI tract can cause bowel perforation secondary to pressure necrosis of the bowel wall trapped between two magnets.

Aspiration

Foreign bodies are most commonly found in the main bronchi although they can be found anywhere from the subglottis to the periphery of the lung. Multiple foreign bodies may be present.

Key history points

Chief Complaint

Ingestion - The foreign body ingestion may not have been witnessed. Patients may be asymptomatic or may present with drooling or feeding intolerance.

Aspiration - The choking event may not have been witnessed. Patients may be asymptomatic or can present with coughing, gagging, wheezing, or respiratory distress. In patients with a prolonged aspiration history, there may be complaints related to an underlying pneumonia or abscess.

Was the ingestion or choking event witnessed?

When did the ingestion/ aspiration take place?

What was ingested/ aspirated?

How many were ingested/ aspirated?

Is there a history of prior esophageal or tracheal surgery?

Key physical exam findings

Ingestion - Exam findings are often limited; excessive drooling may be seen.

Aspiration - Exam findings may be limited; stridor, retractions, dyspnea, unilateral hyperresonance and decreased breath sounds.

Differential diagnosis

If radiopaque there is usually enough evidence to know if the foreign body is smooth, has a sharp edge and its location by imaging studies and history.

Imaging

Ingestion - A posteroanterior (PA) and lateral chest radiograph will identify radiopaque foreign bodies in the esophagus which will most commonly be coins. However, it is critically important to differentiate between a coin and a button battery. On the PA projection, a coin appears as a solid disc whereas a button battery has a halo or ring around the periphery. On the lateral projection, a coin has a uniform thickness whereas a battery has a step off because the negative side of the battery is narrower than the positive. An impacted button battery is a surgical emergency.

An upper gastrointestinal series may delineate the presence of a radiolucent foreign body.

Aspiration - PA and lateral chest radiograph will not demonstrate the majority of airway foreign bodies as they are not typically radiopaque. Hyperinflation, hyperlucency and mediastinal shift may be present. Evidence of pneumonia, atelectasis and lung opacification may also be present. Inspiratory and expiratory films may increase the diagnostic yield.

Indications for surgery

Any foreign body in the esophagus is eligible for removal. These are an urgent and not an emergent case unless the object is a battery. If the foreign body is smooth such as a coin and is in the distal esophagus at the gastroesophageal junction, passing an nasogastric tube to try and move the foreign body into the stomach (and avoiding the need for endoscopic removal) has been described.

Operative consent points

Ingestion - Procedure: rigid or flexible (provider/ institution preference) esophagoscopy with foreign body removal. Risks: sore throat, hoarseness, rare bleeding or perforation of the esophagus. Patients who have ingested button batteries are at risk for tracheoesophageal fistula, aortoenteric fistula and discitis. There can be late presentation of these complications.

Aspiration - Procedure: rigid bronchoscopy with foreign body removal. Risks: sore throat, hoarseness, rare tracheal injury. Patients with delayed presentation can develop pneumonia, bronchiectasis and lung abscess.

Complications

Other than described in the risks above, anesthesia and delayed stricture if foreign body present for long time before removal.

Postoperative care

Ingestion - Most patients can be allowed to drink and eat once they have been recovered from anesthesia. If they are able to drink well they can be discharged to home. A longer hospital course may be warranted for button battery ingestions as postoperative imaging of the esophagus may be indicated.

Aspiration - In most patients, there should be a brief period of observation to ensure airway patency prior to discharge. Steroids may be indicated if there is evidence of airway edema. In patients where a foreign body has been present for a longer period of time, prolonged hospitalization may be warranted to address secondary lung pathology.

When to call the next level

Ingestion - a senior should be notified immediately for button battery ingestions. Otherwise, patients should be admitted to the ward overnight in anticipation of endoscopy the following morning. Patients should be made NPO at the appropriate time prior to surgery and started on maintenance intravenous fluids. Some advocate thick foods such a bread or peanut butter in an effort to push the foreign body into the stomach. Either way, a repeat chest radiograph should be obtained prior to the operating room to ensure the impaction persists.

Aspiration - a senior should be notified for all airway foreign bodies.

Author: Colleen M. Fitzpatrick

Gastroschisis

Problem: Abdominal wall defect

Likely diagnosis: Gastroschisis

Neonates with gastroschisis are born with an obvious abdominal wall defect that is recognized immediately after delivery. The intra-abdominal contents such as bowel, stomach or gonads are readily visible as they herniate through the gastroschisis defect. The eviscerated organs are not covered with a membrane or sac. Most patients in developed countries will be identified prenatally on routine 20 week ultrasound.

Epidemiology

The incidence of gastroschisis in the United States is increasing and currently stands at approximately 4.4 per 10,000 live births. 28% are born premature and most exhibit intrauterine growth retardation. Intestinal atresia is the most common associated anomaly and can be found in 6.9 to 28% of patients. Malrotation occurs in nearly every patient with gastroschisis but midgut volvulus is not commonly seen. Less common anomalies include cardiac, renal, musculoskeletal and central nervous system abnormalities.

Key history points

Patients with gastroschisis have a dramatic abdominal wall abnormality easily appreciated by most health care providers. It is important to query the obstetric team regarding any trauma to the bowel during delivery. In addition, reports of meconium stained fluid should raise suspicion for the presence of intestinal atresia or hollow viscous perforation.

Key physical exam findings

After the patient has demonstrated adequate perfusion and respiratory effort the exam should focus on the exposed viscera. Neonates with gastroschisis do not have a membrane or sac covering the exposed viscera. The abdominal wall defect is usually located to the right of the umbilical cord. Identification of exposed viscera should be attempted. The exposed viscera may be covered with a think inflammatory peel. Bowel loops may be densely adherent making it impossible to distinguish one loop of bowel from another. The bowel should be closely inspected for evidence of perforation, vascular compromise and the presence of intestinal atresia. The size of the abdominal wall defect should be noted. Gentle probing of the distal rectum with the tip of a cotton swab can reveal the presence or absence of meconium. Meconium in the rectum favors intestinal continuity and the absence of intestinal atresia.

Differential Diagnosis

Omphalocele is also an abdominal wall defect and can sometimes be confused with gastroschisis. The viscera of an omphalocele is usually covered with a membrane or sac. Occasionally this membrane can rupture causing some difficulty differentiating omphalocele from gastroschisis. Patients with omphalocele have a higher incidence of congenital anomalies than gastroschisis patients.

Imaging

Imaging is not usually necessary unless additional anomalies are suspected. The presence of intestinal atresia can often be established upon close examination of the bowel and imaging immediately after birth it does not help to elucidate the continuity of the gastrointestinal (GI) tract. A small bowel follow through or contrast enema may be helpful later in order to clarify the anatomy, identify occult atresias or diagnose a mechanical bowel obstruction. These studies are not usually performed until several weeks after all the bowel is returned to the peritoneal cavity.

Indications for Surgery

All patients with gastroschisis will require surgical intervention. The basic tenets of surgical evaluation and treatment are

• insure the mesentery is not kinked, twisted or compromised.
• resect nonviable bowel
• return the viscera to abdominal cavity and close the facial defect
• re-establish GI continuity

For patients with simple gastroschisis (i.e. viable bowel, no atresias) this approach distills down to returning the bowel to the abdomen and closing the facial defect. Surgeons can accomplish these two therapeutic goals in patients with simple gastroschisis through the following approaches.

• placement of Silastic® silo with staged reduction of the viscera over several days followed by delayed facial closure with suture
• complete reduction of the viscera shortly after birth and primary facial closure with suture
• complete reduction of the viscera shortly after birth and covering of the defect with an umbilical cord flap

Preoperative preparation

All patients with gastroschisis should undergo prompt evaluation by the surgical team shortly after birth. An orogastric tube should be placed to decompress the GI tract and intravenous access obtained. After thorough inspection of the exposed viscera, the neonate should be placed on their right side to prevent kinking of the mesentery. The bowel should be covered with moist gauze. Evaporative fluid losses can be reduced by wrapping the bowel in plastic wrap or placing the torso of the baby in a plastic bag. The timing and technique for repair varies widely between institutions but perforated or compromised bowel must be dealt with emergently. Patients should receive a 10 mL/kg fluid bolus of lactated Ringers or normal saline followed by maintenance intravenous fluids at a standard rate. Aggressive fluid resuscitation is not usually necessary and can result in detrimental outcomes. Eventually, neonates with gastroschisis will need durable central venous access (e.g. Broviac® or PICC line) for administration of parenteral nutrition.

Operative consent points

Silo placement - The abdominal contents are partially reduced into the abdomen and a temporary prosthetic "silo" is placed to protect the bowel while it is slowly reduced into the abdomen. The risks are bleeding, infection, anesthesia, need for further procedures, damage to the abdominal contents and need for revision.

Closure (primary or staged) - All of the abdominal contents are reduced into the abdomen and the fascia and skin defect is closed. A "sutureless" closure involves reduction and placement of the umbilical cord over the unclosed defect. Risks are bleeding, infection, anesthesia, need for further procedures, damage to the abdominal contents and need for revision.

Broviac® insertion - The exact vein used may vary due to the venous anatomy. Therefore, do not commit on laterality or vein to be used. Risks involve bleeding and injury to the surrounding structures. If planning a subclavian or jugular vein approach, a pneumothorax is a rare but possible complication. Common issues afterwards include bleeding, line dysfunction or dislodgement and line infection.

Complications

Abdominal compartment syndrome (ACS) can occur within the first few days following reduction and closure of a gastroschisis defect. ACS may manifest as increasing pulmonary pressures in ventilated patients, low urine output, differential cyanosis (bluish appearing legs caused by impaired venous return) and hypotension. Ten to 15% of patients with gastroschisis develop necrotizing enterocolitis (NEC). NEC can manifest as feeding intolerance, abdominal distension, hematochezia and/or overall clinical deterioration. Intestinal atresia can initially go unrecognized in 12 to 37% of patients and should be suspected in patients with persistent bilious emesis, feeding intolerance or abdominal distension several weeks after closure. Most patients with gastroschisis have malrotation but volvulus is less common. However, this devastating complication is certainly possible. Adhesive small bowel obstruction is another source of morbidity and need for reoperation in patients with gastroschisis.

Outcomes

Patients with simple gastroschisis typically do relatively well and have a low mortality rate. Long term problems include gastroesophageal reflux, intestinal dysmotility, cryptorchidism, hearing loss, low IQ scores and the need for feeding tubes. Up to 60% of children with gastroschisis report some psychosocial stress related to an abnormal or absent umbilicus. Complex gastroschisis is associated with a mortality rate as high as 28% and these patients often require multiple procedures. Patients with complex gastroschisis may suffer from intestinal failure and parenteral nutrition induced cholestatic liver disease necessitating liver and/or small bowel transplantation.

When to call the next level

A neonate with dwindling urine output and differential cyanosis following reduction and closure of a gastroschisis may have abdominal compartment syndrome and an exam by an experienced clinician is essential to determine if abdominal decompression is needed. A patient with gastroschisis who was previously at full feeds and begins to have abdominal distension, emesis and frequent apnea/bradycardic episodes may represent NEC and requires prompt recognition and treatment as it can be easily confused with the routine feeding intolerance seen in many neonates with gastroschisis.

Author: John Horton

Hirschsprung Disease

Problem: Newborn with inability to pass meconium or any child with severe refractory constipation

Likely Diagnosis: Hirschsprung Disease

Hirschsprung disease (HD) is a congenital absence of the ganglion nerves in myenteric and submucosal plexus of distal bowel. It affects bowel in continuous fashion starting distally in the rectum then progressing more proximally. The most common area of involvement is the rectum or rectosigmoid colon. HD can extend proximally varying lengths with five to 10% of patients having total colonic HD. The aganglionic segment lacks peristalsis thus causing a functional intestinal obstruction.

The overall incidence is one in 5000 live births with children of parents with HD generally having more extensive disease.

Key history points

The chief complaint in infants is the failure to spontaneously pass meconium in first 24 to 48 hours of life (90%). Fifty to 90% of patients present as neonates with abdominal distension, bilious emesis and feeding intolerance consistent with distal intestinal obstruction.

Older children present with chronic constipation - particularly those who are exclusively breast fed. Older children more often have short segment involvement associated with chronic abdominal distension, failure to thrive and dependence on enemas without significant encopresis.

Ten percent of patients will present with Hirschsprung disease associated enterocolitis (HAEC) with fever, abdominal distension and diarrhea. HAEC can be chronic or severe and life threatening. Enterocolitis is the most common cause of death in children with HD. Patients with longer segment disease and trisomy 21 are at higher risk.

Key physical exam findings

The abdomen will be distended consistent with a distal intestinal obstruction. An explosive amount of stool and gas can be produced with a rectal exam.

Differential diagnosis

Small left colon syndrome(in diabetic mothers or those administered tocolytics such as magnesium), meconium plug syndrome, meconium ileus, intestinal atresia and idiopathic constipation in older children.

Imaging

An abdominal radiograph with a left lateral decubitus film is used to evaluate for an obstructive bowel gas pattern. HD displays dilated colon and some small bowel. Significant small bowel dilation can often indicate extensive disease. A water soluble contrast enema should identify the transition zone between normal colon and the aganglionic segment with a reversed rectosigmoid ratio. The dilated proximal bowel is the normal ganglionated bowel. The presence of contrast within the colon on the 24 hour postevacuation film is also suggestive of HD. The definitive diagnosis is made with rectal biopsy.

Preoperative preparation

A suction rectal biopsy can be performed in infants at the bedside. In older infants and children a full thickness strip rectal biopsy is performed in the operating room. The biopsy is taken at least 1 to 1.5 cm above the dentate line depending on size of child. If the biopsy is taken too low it can be nondiagnostic as this can contain transitional epithelium from anal mucosa which normally has paucity of ganglion cells. In patients with HD, the biopsy will show an absence of ganglion cells in myenteric and submucosal plexuses, hypertrophied nerves, staining for acetylcholinesterase and calcitonin. An experienced pediatric pathologist is required.

Prior to surgery, the patient requires adequate resuscitation, rectal decompression (i.e. rectal irrigations) and intravenous antibiotics covering enteric bacteria if there is evidence of colitis.

Some older children with large dilated colon may require decompressive colostomy prior to definitive corrective surgery.

In the infant who is otherwise well and without evidence of colitis, the timing of the surgery may be delayed for a short period as long as the child may be maintained with irrigations to keep the bowel decompressed. Many infants will be eligible for single stage repair but those with enterocolotis or a delayed diagnosis with dilated proximal colon may benefit from a two stage approach with an initial diverting colostomy in order to decrease the dilation of the proximal colon and improve nutrition before surgery.

Operative consent points

The goals of surgery are to resect the aganglionic segment and restore intestinal continuity using normal bowel anastomosed to the anus leaving the sphincter mechanism intact.

Multiple types of operative techniques are described.

Swensen - resection of entire aganglionic segment of bowel with end to end anastomosis above the anal sphincter. It can be done via a combined abdominal and perineal approach. Risks include damage to pelvic nerve plexus and surrounding pelvic structures.

Soave - endorectal submucosal resection of aganglionic bowel leaving distal muscular cuff. Normal bowel is then brought through the dividied aganglionic muscular cuff and an anastomosis performed.

Duhamel - normal bowel is delivered posterior to the aganglionic bowel and a side to side anastomosis created thus forming a large reservoir with aganglionic bowel wall anteriorly and normal ganglionic bowel wall posteriorly.

Multiple variations of these are currently being practiced based on surgeon preference and include transanal endorectal pull through, laparoscopic or open colonic mobilization and biopsies followed by perineal pull through, complete laparoscopic assisted or open removal and reconstruction.

The procedure can start transanal if disease is rectosigmoid – start dissection 0.5-1cm proximal to dentate line (depending on age of child). Otherwise, laparoscopy or laparotomy is performed to identify transition zone.

Longer aganglionic segments require mobilization and/or a diverting stoma if the segment is unable to reach to perineum.

Once the transition zone is identified the distal bowel is resected. Frozen section pathology is used to insure that normal ganglionated bowel is used in the reconstruction. If long segment disease is present a leveling colostomy is performed to allow the baby to grow prior to pull through. A long segment of aganglionic bowel be left in situ as this can result in enterocolitis.

Complications

Bowel obstruction from intra-abdominal adhesions, anastomotic stricture, retained or acquired intestinal aganglionosis, twisting of the pulled through segment of bowel, disordered intestinal motility and internal sphincter achalasia,. The parents should be educated that patients are still at risk for enterocolitis after a good operation. Vomiting and diarrhea are sometimes the only signs. Enterocolitis is treated with NPO, rectal irrigations and broad spectrum antibiotics to include anaerobic coverage.

Postoperative care

Patients generally do well with acceptable bowel function though they should always be monitored for enterocolitis, bowel obstruction and soiling (which usually resolve by 5 years of age). Fecal soiling is more common in those with longer segment disease and trisomy 21. There is a poorer prognosis in those with other comorbidities including congenital heart disease and congenital central hypoventilation syndrome.

Patients should follow-up with surgeon yearly to ensure appropriate bowel management until toilet training is complete. Toilet training may be delayed and parents should be reassured that this may occur.

When to call the next level

Suspicion of enterocolitis (both pre- and postoperatively), bowel obstruction or a sick patient with bilious emesis.

Author: Grace Mak

Hypertrophic Pyloric Stenosis

Problem: Nonbilious vomiting in an infant

Likely diagnosis: Hypertrophic pyloric stenosis

Key history points

Nonbilious vomiting in the newborn or infant that begins like gastroesophageal reflux but worsens to be all or nearly all feeds and results in dehydration. Usually not associated with signs of infection (i.e. afebrile). The baby is liley hungry, feeds well and then vomits. Over time the vomiting becomes forceful (projectile). Wet diapers become less frrequent. Patients often present with having multiple formula changes by the primary care practitioner prior to consulting pediatric surgery.

The age at presentation is usually two to 10 weeks of age. Hypertrophic pyloric stenosis (HPS) is caused by hypertrophy of the muscle fibers in the pylorus - principally the circular layer. It is very common and occurs in 1:300 live births. The incidence is more common in males (4:1). Hypertrophic pyloric stenosis has a propensity for first born males and an may run in families.

Patients presenting later in the disease process will have evidence of more severe dehydration and electrolyte disturbances that lead to symptoms of lethargy and poor urine output.

Key physical exam findings
Patients usually display signs of dehydration such as a sunken fontanel, poor perfusion and dry mucous membranes. The abdominal exam may reveal upper abdominal distension. Experienced examiners may feel a palpable olive in the upper abdomen in a quiet baby.

Differential diagnosis
Severe gastroesophageal reflux, sepsis and gastroenteritis are all capable of causing severe vomiting. Although less likely, malrotation may have nonbilious vomiting.

Imaging
Imaging is used to confirm the diagnosis. Ultrasound is the gold standard and measures the length and width of the pyloric channel. Widths greater than four mm wide and lengths greater than 14 mm are worrisome for pyloric stenosis but each hospital will have slightly criteria. When ultrasound is unavailable, an upper gastrointestinal series may be used for diagnosis which will show complete obstruction at pylorus.

Indications for surgery
Surgery is indicated once the diagnosis is confirmed by ultrasound. The timing of surgery is contingent upon correction of dehydration and metabolic alkalosis. A bicarbonate level less than 30 mEq/L and a chloride greater than 95 mEq/L are needed before general anesthesia.

Preoperative preparation
Empiric therapy is directed at treating dehydration with fluid boluses (20 mL/kg) of normal saline. Rates higher than maintenance are often required and the boluses are repeated up to three times or until the urine output has returned to normal. Laboratory examination includes a basic metabolic panel which will usually show a hypochloremic, hypokalemic metabolic alkalosis. Placement of a nasogastric tube is discouraged. Laboratory studies are repeated after resuscitation (12 hr later is reasonable) and until they are normal.

Operative consent points
The pyloromyotomy may be done via a laparoscopic or open approach- ask which is planned before obtaining consent. The purpose of the procedure is to cut the serosal and muscular layer of the pyloric channel without cutting into the mucosa in order to relieve the stenosis yet prevent leakage of the feedings into the peritoneal cavity. The risks of the procedure are bleeding, infection, anesthesia, damage to pyloric channel (perforation rate less than 1:1000), postoperative apnea and a need for other procedures.

Complications
After pyloromyotomy complications are rare. Intraoperative complications include incomplete myotomy (fixed with increasing myotomy) or perforation. If recognized, perforation can be fixed intraoperatively and a new myotomy created. Aspiration and pneumonia occurs less than one percent of cases. Wound infection and hernia are also rare.

Postoperative care
All infants should be on a monitor for at least 12 to 18 hours to assess for possible postoperative apnea . Feedings should start after recovery from anesthesia. Feedings should be ad lib formula or breast milk unless directed by your institution’s feeding regimen. Intravenous fluids should continue until the baby is tolerating feeds. The prognosis is excellent and babies usually have no long term issues after pyloromyotomy. Acetaminophen is usually adequate for pain and babies rarely need narcotics after this operation and they should not be ordered unless specifically requested by the chief resident or attending.

When to call the next level
If the evaluation is straightforward a call can be made after fluid boluses have been started and after the ultrasound and lab results are known so that planning of timing of surgery can be done. Senior residents should be called earlier if the diagnosis is unsure (e.g. fever, any question of bilious vomiting, baby looks toxic), inability to obtain intravenous access or if the infant doesn’t respond to fluid resuscitation.

Author: Joseph Iocono

Inguinal Hernia

Problem: Inguinal bulge

Likely Diagnosis: Inguinal hernia/hydrocele

The failure of obliteration of the processus vaginalis is the underlying pathogenesis of indirect pediatric inguinal hernias. Narrowing of the processus allowing only fluid passage results in a communicating hydrocele; complete obliteration of the proximal portion of the processus can result in a noncommunicating hydrocele. An indirect hernia passes lateral to the inferior epigastric vessels and exits into the inguinal canal via the internal ring.

Risk factors for pediatric hernias include male sex, prematurity, history of an undescended testicle, history of a hydrocele, positive family history, disease causing increased intra-abdominal pressure and connective tissue disorders. The incidence of indirect inguinal hernias in infants and children is estimated to range from one to five percent. Premature infants are at an increased risk for inguinal hernia with rates of two percent in females and seven to 30% in males.

Direct hernias bulge through the inguinal floor medial to the inferior epigastric vessels. This type of inguinal hernia is rarely seen in pediatric patients.

Key history points

The chief complaint is usually a groin bulge.

Inguinal hernias present as an asymptomatic or symptomatic swelling in the inguinal canal that may extend to the scrotum or labia. Most hernias in children are minimally symptomatic and may bulge with increasing intra-abdominal pressure such as crying or straining. The bulge often resolves during the night while the baby is sleeping. Hernias present on the right side in 60%, left in 30% and bilateral in 10% of patients.

A hydrocele is a fluid filled sac which is sometimes found adjacent to the testicle or along the cord structure. It most commonly presents as a swelling in the scrotum but can also present with swelling along the inguinal canal. Hydroceles can communicate with the peritoneal cavity, resulting in variability in size over time, or may be noncommunicating.

Symptomatic hernias may present with incarceration (unable to be reduced) or strangulation (loss of blood supply) with a bowel obstruction. In this event the child will present with pain, abdominal distension and emesis.

Key physical exam findings

Palpating along the inguinal canal at the external ring is an optimal method to examine the canal for hernias. The examination often reveals a palpable smooth mass (e.g. loop of intestine or ovary) originating from the external ring lateral to the pubic tubercle. The mass may only be noticeable after maneuvers that increase intra-abdominal pressure and it should reduce easily. Occasionally, the hernia is difficult to reproduce. The presence of the silk glove sign (thickening of the cord compared to the contralateral side) supports the possible presence of an ipsilateral hernia.

If an incarcerated hernia is identified on examination, a reduction should be attempted if there is no concern for strangulation. Pain medication, sedation and the Trendelenburg position may assist in reduction. Straightening the canal by pushing the lower abdominal wall toward the scrotum with one hand while gently squeezing the hernia sac with the other may help appropriate incremental reduction of the incarcerated bowel loop.

Differential diagnosis

Other diagnoses in the differential include orchitis, torsion of the appendix testis and testicular torsion.

Imaging

Imaging is rarely needed to make this diagnosis. In the setting of an incarcerated hernia, an abdominal radiograph may clarify the diagnosis by revealing dilated intestinal loops or air in the hernia sac lateral and inferior to the inguinal ligament. An ultrasound may help differentiate between a hydrocele and an inguinal hernia or between an incarcerated hernia and an enlarged inguinal lymph node. Surgical exploration may be necessary to confirm the diagnosis between inguinal lymphadenopathy and an incarcerated hernia.

Indications for surgery

Inguinal hernias in children do not spontaneously heal and they must be surgically repaired because of the risk of incarceration. Most noncommunicating hydroceles spontaneously resolve and do not require repair unless they persist (or first appear) after one year of age.

Preoperative preparation

Once the diagnosis of an inguinal hernia is established in an infant or child, surgical treatment is recommended. The procedure can be performed electively in asymptomatic patients with a reducible hernia. Urgent operative exploration should be performed in any patient with a suspected strangulated inguinal hernia.

A communicating hydrocele is treated in the same fashion as an inguinal hernia with a scheduled elective operative repair.

Operative consent points

Procedure: inguinal hernia repair - may be done laparoscopic or open, ask which is planned before obtaining consent. The purpose of the procedure is to isolate the patent processus vaginalis/inguinal hernia sac and ligate it at its base (i.e. high ligation).

Complications

The risks of the procedure are bleeding, infection, anesthetic complications, damage to testicular vessels or vas deferens, testicular atrophy and recurrence. Hernia recurrence rates range between one and five percent.

Postoperative care

For infants and young children it is not necessary to limit activities after inguinal hernia repair. Most patients are treated with acetaminophen and/or nonsteroidal anti-inflammatory medications for a few days after surgery. A small dose of oral narcotic pain medicine may be needed for older children.

When to call the next level

Call your senior resident or attending if you

• cannot identify a hernia but the history from the parent is consistent with a hernia
• have difficulty differentiating between a hydrocele versus an inguinal hernia
• have difficulty reducing an incarcerated inguinal hernia
• have difficulty assessing whether a reduction of an incarcerated hernia should be attempted because of concern for strangulation
• see a patient with an incarcerated hernia who also appears obstructed, with peritonitis or is septic.

Author: Eunice Huang

Intestinal Rotational Abnormalities

Problem: Bilious vomiting in the newborn

Likely Diagnosis: Malrotation with midgut volvulus

Anomalies of intestinal rotation encompass a spectrum of abnormal rotation and fixation of the intestine which may predispose the individual to midgut volvulus - a potentially catastrophic event with associated morbidity and potential mortality. Therefore, it is critical to promptly recognize and treat malrotation - particularly if complicated by acute volvulus.

Anatomy

Beginning the fourth to sixth weeks of gestation, rapid elongation of the primitive gut leads to physiologic herniation of the intestine into the umbilical cord with duodenojejunal and cecocolic limbs oriented along the axis of the superior mesenteric artery (SMA). The intestine subsequently proceeds through a counterclockwise rotation of 270 degrees around the SMA axis, simultaneous with reduction into the abdominal cavity during the tenth gestational week. This process positions the duodenum under the SMA, the ligament of Treitz in the left upper quadrant and the cecum assumes a final resting position in the right lower quadrant. The duodenum, ascending colon, and descending colon are fixed within the retroperitoneum by the twelfth week of gestation. The small bowel mesentery from the ligament of Treitz to the cecum is thereby broadly fixed obliquely across the abdomen from the left upper to the right lower quadrant. An error at any stage of this complex intestinal development can lead to a rotational anomaly whereby the fixation of the small bowel mesentery may be narrow. and thus predisposed to volvulus.

Key history points

A newborn with bilious vomiting is a surgical emergency.

Bilious vomiting in any age is an urgent consult to pediatric surgery. Bilious vomiting is the cardinal sign of neonatal bowel obstruction. Other symptoms and signs may include feeding intolerance, abdominal pain and abdominal distension. Late signs of volvulus with ischemia include hematemesis, melena or bloody stools, abdominal wall erythema and peritonitis.

Key physical exam findings

The newborn with intestinal malrotation may initially appear well with nonspecific mild abdominal distension alone. The abdomen may be flat because the obstruction is proximal. The abdomen distends during late ischemia and perforation. Beware, the infant may deteriorate rapidly, with progression to peritonitis, sepsis and hypovolemic shock.

Differential diagnosis

The differential diagnosis for bilious vomiting in the newborn includes other forms of intestinal obstruction including intestinal atresia, meconium disease, Hirschsprung disease, incarcerated inguinal hernia and anorectal malformations. Also beware of the infant who is a neonatal intensive care unit graduate as they may have had septic episodes or nonoperative necrotizing enterocolitis that may predispose them to ischemic or adhesive bowel obstructions.

Imaging

Abdominal radiographs are often the initial imaging evaluation but are not sufficiently sensitive to achieve the diagnosis. Most plain are normal or show a gasless abdomen in early intestinal malrotation, even with volvulus, or may demonstrate an obstructive pattern or pneumatosis intestinalis.

Use of ultrasound with Doppler to diagnose malrotation has been described, delineating the relationship of the superior mesenteric artery and vein to determine the diagnosis.

An upper gastrointestinal series (UGI) demonstrating an abnormally positioned ligament of Treitz is confirmatory; a corkscrew appearance or bird’s beak tapering of the proximal small bowel is concerning for associated volvulus.

Indications for surgery

Emergent abdominal exploration is indicated as soon as the diagnosis is confirmed by UGI. It may be appropriate to proceed with surgical exploration without confirmatory imaging when the diagnosis is likely on clinical grounds alone - particularly if a newborn presents in extremis with suspected advanced intestinal ischemia.

Preoperative preparation

Prompt resuscitation with intravenous fluids, placement of a decompressive nasogastric tube and expeditious confirmation of the diagnosis is crucial. An emergent upper gastrointestinal contrast study remains the gold standard to diagnose intestinal malrotation. Once the diagnosis is established, emergent corrective surgery should follow. Fluid resuscitation, correction of electrolyte abnormalities and metabolic acidosis and initiation of antibiotics should occur simultaneously but should not delay urgent operative intervention.

Operative consent points

The classic surgical repair of intestinal malrotation with volvulus is known as the Ladd procedure. The bowel is reduced in a counterclockwise direction, Ladd bands originating from the cecum and crossing over the duodenum are divided to relieve duodenal obstruction, the base of the mesentery is broadened, an incidental appendectomy is performed (to avoid future confusion and missed diagnosis of appendicitis) and the bowel is returned to the abdomen in a nonrotated position (with the small bowel in the right abdomen and the colon in the left abdomen). The procedure can be approached open or laparoscopically but at present there is minimal evidence to support the use of laparoscopy in cases of known volvulus or neonates.

The primary objective of the procedure is to reduce the bowel and restore perfusion. The remaining components of the procedure relieve additional obstruction and discourage recurrent volvulus. Risks include bleeding, infection and anesthesia. In cases where advanced ischemia is suspected, it is prudent to outline the possibility of bowel resection and potential stoma formation. At the time of surgery, if the entire midgut is ischemic, despite reduction and restoration of perfusion, consideration should be made to silo placement and second look laparotomy.

Complications

Complications are rare after straightforward Ladd’ procedure for malrotation without bowel ischemia. However, malrotation with volvulus and complete midgut infarction can lead to extensive bowel resection resulting in short bowel syndrome or death. Long term complications following Ladd procedure include adhesive bowel obstruction (five percent) and recurrent volvulus (one percent).

Postoperative care

If able to save the intestine, the baby will have increased fluid requirements for 24 to 48 hours postoperatively. The ileus after a Ladd procedure may be prolonged if the preoperative ischemic time is long.

When extensive bowel resection is performed supportive care for short bowel syndrome is required including total parental nutrition, prevention of dehydration, treatment of dumping syndrome and support of the adaptive potential of the remaining gut. Ultimately, multivisceral organ transplant may be required.

When to call next level

Since prompt recognition and operative intervention are critical in intestinal malrotation with volvulus, early notification of senior members of the pediatric surgery team is appropriate such that the newborn can be assessed, the UGI obtained emergently and mobilization of operating room resources can be initiated. This is not a consult that can wait. If you are unable to see the consult promptly due to other duties, this is one of the situations where the upper level can be called even before you see the patient. If advanced intestinal ischemia is suspected, discussion and counseling of the parents should be left to the pediatric surgery attending as this must be approached with utmost sensitivity.

Author: Andrea Lo

Jejunoileal and Colonic Atresia

Problem: Bilious vomiting in a newborn

Potential Diagnosis: Intestinal atresia

Intestinal atresia is a congenital defect in the lumen of the small or large bowel causing a complete or partial obstruction in a newborn. There are five different types of atresia described - intestinal length is usually preserved in types I and II whereas the small bowel tends to be foreshortened in types III and IV. Intestinal atresia occurs in about 1:5000 live births and can sometimes be detected prenatally. Small bowel and colonic atresias are generally believed to be due to vascular compromise to the bowel in utero. Duodenal atresia if thought to be a result of failure of the intestinal lumen to recannulate.

types of intestinal atresia

Key history points

Bilious emesis or bilious gastric aspirate is present if the atresia is distal to the second part of the duodenum. Duodenal atresia can present as either bilious or nonbilious emesis

In general, intestinal atresia presents in the newborn period although some type I atresias consist of a mucosal web with small perforations acting as a partial obstruction which leads to a later presentation - usually in the first few weeks to months of life.

Prenatally there may be a history of polyhydramnios if proximal atresia or sonographic evidence of dilated or echogenic bowel.

There can still be passage of meconium in the presence of an atresia depending on when the atresia occurred during fetal development.

Sometimes a type I atresia consists of a mucosal web with perforation and can present with intermittent emesis/feed intolerance.

Key physical exam findings

The baby may be dehydrated with decreased urine output. If the abdomen is significantly distended there may be respiratory compromise.

If there has been perforation of the intestine secondary to atresia there may be hemodynamic instability and abdominal wall erythema. if the atresia is quite proximal the abdomen may be nondistended or even scaphoid.

It is important to check the patency of the anus and also make sure there are no inguinal hernias as both of these may cause obstructive symptoms.

Differential diagnosis

Other causes of neonatal bowel obstruction include Hirschsprung disease, small left colon syndrome, meconium ileus,meconium plug, midgut volvulus or incarcerated inguinal hernia.

Associated causes of duodenal obstruction include annular pancreas or preduodenal portal vein.

Imaging

Abdominal radiographs are usually the first imaging study. A babygram and left lateral decubitus film provide the most information. Duodenal atresia often demonstrates a double bubble on plain radiograph (gastric air and air within the proximal duodenum between the pylorus and the atresia). In the case of more distal atresia there will be several dilated loops of air filled bowel.

If the baby is having bilious emesis or gastric aspirates, an upper gastrointestinal series is indicated to look for malrotation and volvulus. It can sometimes be difficult to differentiate volvulus from duodenal atresia. A contrast enema can be useful prior to surgery to rule out colonic atresia which can be hard to assess in the operating room through a small incision.

Indications for surgery

All children with intestinal atresia (duodenal, small bowel and/or colonic) will need surgical correction. If there is any question of malrotation with volvulus proceed to the operating room emergently.

Preoperative preparation

Prior to surgery it is important to ensure the baby is well hydrated and has a functional oro- or nasogastric tube placed to suction. Check electrolytes and a complete blood count. Often the diagnosis can be made based on the abdominal radiograph findings and prenatal history.

Operative consent points

The objective of the operation is to identify the area of obstruction and restore intestinal continuity.

Duodenal atresiacan be repaired laparoscopically or through an open incision. Most commonly a duodenoduodenostomy is performed to bypass the obstruction. Most small bowel atresias are repaired through an open incision with a resection and primary anastomosis. If there is significant proximal bowel dilation, intestinal resection or tapering may be warranted to achieve a less significant size mismatch and improve intestinal motility.

Stomas are rarely required but should be mentioned.

Central venous access may be required. Ask if durable intravenous access is planned (Broviac) before obtaining consent so that it can be added to it as needed.

Complications

Risks include anastomotic leak or stricture, prolonged ileus/intestinal dysmotility, wound infection and incisional hernia. In some cases the intestine can be quite foreshortened resulting in short bowel syndrome and extended parenteral nutrition dependence.

Postoperative care

For most small bowel atresias it will take two to four weeks before full enteral nutrition is achieved depending on the degree of intestinal dilation and dysmotility. Mortality is highest for babies with additional congenital anomalies who weigh less than two kg.

When to call the next level

Any baby that has bilious emesis should prompt an early call to the senior resident or attending as soon as you get the consult. Also call if the baby is not sufficiently decompressed with oro- or nasogastric tube, poor urine output or requiring hemodynamic support.

If non of the above are present, you can call after placing the gastric tube, obtaining an abdominal radiograph, standard labs and starting intravenous fluids.

Author: Hannah Piper

Necrotizing Enterocolitis

Problem: Abdominal distention, intolerance of feeds, bilious vomiting, bloody stools in a newborn

Likely diagnosis: necrotizing enterocolitis

Necrotizing enterocolitis (NEC) is one of the most devastating diseases to affect infants in the neonatal intensive care unit and a leading cause of morbidity and mortality. It is an acquired inflammatory disease of the intestine of neonates. It is the most common newborn surgical emergency.

NEC has a multifactorial etiology of intestinal mucosal compromise, pathogenic bacteria and enteral feedings in a susceptible host leading to bowel injury and the initiation of the inflammatory cascade. The most consistent risk factors include prematurity and enteral formula feeds. The highest risk is in infants less than 1000 g and less than 28 wk gestation.

NEC primarily affects the vascular watershed regions and is in a single area in half of patients. The most commonly affected areas are the terminal ileum followed by the colon. Forty-four percent of patients with have multiple discontinuous regions of intestine with both small and large intestine affected

NEC totalis is the most fulminant form of NEC with necrosis of at least 75% of the intestine.

Bell Classification

Stage I – clinical findings suspicious for NEC but no definitive criteria

Stage II – definitive NEC without indication for surgical intervention (e.g. abdominal radiograph with with pneumatosis intestinalis)

Stage III – evidence of pneumoperitoneum (i.e. free air) or irreversible tissue damage with perforation

Key history points

The presentation can vary but a common scenario is a consult in a premature baby who was previously tolerating enteral feeds and develops physiologic instability with apnea, bradycardia, lethargy, temperature instability, hypoglycemia and shock who became distended, developed green emesis, abdominal distention and/or bloody stools.

Key physical exam findings

The exam may be noteworthy for unstable vital signs, poor perfusion, decreased capillary refill and abdominal distension. Abdominal wall discoloration (i.e. erythema or a bluish discoloration) can indicate perforation.

The inguinal rings and scrotum should be evaluated for a bluish discoloration as meconium can travel to these areas through a patent processus vaginalis.

Differential diagnosis

A similar condition, spontaneous intestinal perforation (SIP) also occurs in premature infants although the babies are usually not as ill appearing, generally present much earlier in life and usually have not been fed.

Imaging

Flat and right side up lateral decubitus or cross table lateral abdominal radiographs are obtained to look for pneumatosis, portal venous gas or free air. Serial radiographs may show a fixed loop.

Indications for surgery

In the absence of an indication for surgery, infants are treated with supportive care such as intravenous fluid resuscitation, NPO with gastric decompression and broad spectrum antibiotics. Serial evaluation includes abdominal exams, two view abdominal radiographs, WBC and platelets and blood gases.

Evidence of intestinal necrosis or perforation is an indication for surgical intervention. Progression of the disease (e.g. abnormal blood work, thrombocytopenia, neutropenia and metabolic acidosis), a fixed loop indicating necrotic bowel and abdominal compartment syndrome are also indications for surgery. If abdominal compartment syndrome is present there may be a need to place a silo or leave the abdomen open.

The procedure may be either exploratory laparotomy or peritoneal drain placement depending on the weight and condition of the baby. Peritoneal drain placement may be appropriate in infants less than 1000 g or those with poor physiologic reserve and inability to handle the stress of surgery

Operative consent points

The goal of laparotomy is to resect necrotic bowel, preserve intestinal length. The consent should include exploratory laparotomy, bowel resection, likely ostomy and possible temporary abdominal wall closure (i.e. silo). The discussion should also include the possibility of short gut syndrome, that the infant may get sicker before getting better and blood product transfusions may be required.

A second look operation 48 to 72 hr later may be needed. if applicable given the clinical condition, a senior or attending should discuss the possibility of NEC totalis.

Complications

Complications include abdominal compartment syndrome, sepsis, death, short gut syndrome, intestinal stricture (common in the ileum and colon although this is more common after medical management).

Postoperative care

Overall survival depends on weight and age of infant, severity of NEC and amount of remaining bowel. NEC may be a risk factor in abnormal neurodevelopment.

When to call next level

This consult should prompt an early assessment and quick call to senior even though not all the workup is done - especially if there is evidence of free air on radiograph.

Author: Grace Mak

Thyroglossal Duct Cyst

Problem: Midline neck cyst

Likely Diagnosis: Thyroglossal duct cyst

The thyroid gland descends from the foramen cecum at the base of the tongue to the lower neck. Failure of this tract to regress can lead to thyroglossal duct cysts. It is the most common neck mass in children and occurs in seven percent of the population.

Key history points

The classic presentation is a midline neck mass. This may be asymptomatic or symptomatic (i.e. the infected mass or draining sinus seen in 25%). If infected, patients may have bad taste in mouth from draining pus and the mass may be enlarged, erythematous or tender.

While it is usually midline, it may also be just lateral of midline (40%), suprahyoid or suprasternal.

Most patients present in first five years of life.

Key physical exam findings

The midline neck mass moves up and down with swallowing.

Differential diagnosis

Alternative diagnoses include dermoid or branchial cleft cysts, ectopic thyroid and lymphadenopathy.

Imaging

An ultrasound of the neck (not just the mass) should be obtained if you cannot feel a norma thyroid. It is important to confirm that the mass you are palpating is not an ectopic thyroid which may be patients only thyroid tissue.

Indications for surgery

Existence of the mass is an indication for surgery due to the risk of infection and malignancy seen in one percent.

Preoperative preparation

In general, laboratory studies are not needed. Thyroid function tests may be obtained if you suspect hypothyroidism or ectopic thyroid. This is usually only an issue in the neonate.

If the cyst is infected antibiotics are administered and the abscess aspirated or drained if necessary. Excision is planned after the infection has cleared.

Operative consent points

For the procedure the neck is extended and a transverse cervical incision is made over the cyst. Once the cyst is exposed the dissection proceeds superiorly toward the hyoid. The center of the hyoid is removed including the track which may travel one centimeter on either side. The duct is not skeletonized superior to the hyoid as it may have many branches. The fistula is ligated near the base of the tongue with absorbable suture. Anesthesia may depress the base of the tongue to assist in dissection.

Complications

The Sistrunk procedure has potential risks of bleeding, infection and those related to anesthesia. There is a 10 % chance of recurrence and infection. The risk increases with an incomplete excision, intraoperative rupture, preoperative infection, multicystic lesions and reoperation.

Postoperative care

Without complications the prognosis is excellent.

When to call the next level

In general, a complete history and physical exam is completed before you call your senior. If there is any evidence of airway compromise, wheezing or stridor, call the upper level immediately.

Author: Diana Diesen

Thyroid Disease

Problem: Neck mass

Likely diagnosis: Thyroid disease

Thyroid nodules are much less common in children than adults but thyroid nodules are more likely to be malignant in children referred for evaluation (five versus 22 to 26%). When children present with thyroid cancer they are more likely to have extrathyroidal extension, regional lymph node involvement and distant metastasis.

Thyroid canceris divided into differentiated papillary (PTC) or follicular (FTC), medullary and anaplastic (unusual in children).Graves disease is an autoimmune disorder affecting the thyroid gland. It can result in hyperthyroidism, goiter, orbitopathy and pretibial myxedema.

Key history points

Patients may present with the chief complaint of a nodule - usually asymptomatic neck mass. An autonomous functioning nodule may be associated with hyperthyroid symptoms.

Patients with Graves disease present with hyperthyroidism symptoms – tachycardia, palpitations, anxiety, oil hair, sweating, enlarged protuberant eyes and/or pretibial edema.

Cancer is more common in adolescents. In the case of medullary carcinoma the age at presentation varies by type of RET mutation. MEN2B (M918T) patients may have thyroid cancer as early as infancy. MEN2A patient may develop thyroid cancer as young children (C634 mutations) or later in adolescence or adulthood.

Note the characteristics of mass (size, duration, change over time), signs of hyperthyroidism or hypothyroidism, history of radiation or chemotherapy exposure, previous chemotherapy/alkylating agents, changes in voice, difficulty swallowing and compressive symptoms.

Important is a personal or family history of thyroid cancer, elevated calcium, adrenal tumors, PTEN hamartoma syndromes, Familial adenomatous polyposis, Gardner syndrome or Peutz-Jegher.

Key physical exam findings

Note the size, location and characteristics (e.g. fixed) of mass, cervical adenopathy, evidence of vocal cord paralysis which should prompt preoperatively laryngoscopy) and signs of hyperthyroidism.

Differential diagnosis

Alternative diagnoses include lymphadenopathy, lymphatic malformation, branchial anomalies, thyroglossal duct cyst, enlarged parathyroid (especially in renal patients).

Imaging

Computerized tomography or magnetic resonance imaging should be considered im patients with a large or fixed mass, vocal cord paralysis or bulky metastatic disease. In the case of medullary thyroid cancer further work up may be needed. Ultrasound (US) assists in FNA.

Indications for surgery

The indications for thyroidectomy in children include thyroid nodules, thyroid cancer, Graves disease and prophylactic thyroidectomy in cases of MEN syndrome to prevent the development of medullary thyroid cancer.

If thyroid stimulating hormone (TSH) is suppressed and a hyperfunctioning nodule present, no fine needle aspiration (FNA) is needed and lobectomy recommended.

Treatment of thyroid nodule is based on FNA

Nondiagnositic – rebiopsy in three months

Benign - repeat ultrasound in six to 12 months with repeat biopsy for enlarging or suspicious findings. Lobectomy if the nodule is larger that four cm or significant growth, compressive symptoms, cosmetic, family choice, suspicious findings on US

Indeterminate (e.g. follicular lesion of undetermined significance(FLUS), follicular neoplasm, suspicious for malignancy) - lobectomy with isthmus or total/near total thyroidectomy (TT). If there are greater than three vascular invasions or the tumor is greater than four cm the patient will need a completion thyroidectomy. Consider a total thyroidectomy if the patient is high risk (e.g. family history, radiation history, PTEN syndrome or bilateral disease at presentation). Suspicious for malignancy should lean more toward total thyroidectomy.

Malignant – total or near total thyroidectomy ·

A central lymph node neck dissection is suggested if there is a positive FNA or clinical evidence of extra thyroidal invasion or local/regional metastasis pre- or intraoperatively. A unilateral dissection may be considered depending on tumor focality or size.

A lateral neck dissection is suggested only if there is a positive FNA of lateral lymph nodes.

Medullary thyroid cancer

MEN2A – specific RET mutation identifies one as moderate or high risk and affects the timing of thyroidectomy. Always US neck and lymph nodes. Calcitonin and CEA levels are obtained pre- and postoperatively. A node dissection may be required depending on calcitonin level and US findings. Family genetic screening and planning should be performed. Patients should receive long term surveillance with calcitonin, CEA, screening for adrenal tumor/pheochromocytoma and hyperparathyroidism.

MEN 2B – RET testing with US thyroid and lymph nodes, calcitonin and CEA levels. Thyroidectomy is performed before age one year. Surveillance is done with serial calcitonin, CEA, screening for adrenal tumor/pheochromocytoma. Family planning is required.

For more information on diagnosis and treatment of children with thyroid nodules, refer to the American Thyroid Association guidelines.

Graves disease

Thyroidectomy indicated if unresponsive to thionamides/I¹³¹ treatment, inability to tolerate thionamides, age less than 10 years or family preference.

Preoperative preparation

All patients should have TSH, T3, T4, calcium, PTH, calcitonin and CEA (if suspicious for medullary) checked.

For Graves disease – TSH, T3, T4 and thyrotropin-receptor antibodies; if TSH suppressed obtain a thyroid nuclear scan. If a hyperfunctioning nodule present no FNA needed and surgery recommended; if TSH not suppressed obtain an US of thyroid and lymph nodes with US guided FNA biopsy of thyroid mass and abnormal lymph nodes. PTC may present as a diffuse thyroid enlargement especially if associated with palpable cervical lymph nodes.

Graves disease patients are preoperatively treated with iodine, with or without a beta blocker and corticosteroids if doing emergently. It is preferable for the patient to be euthyroid prior to surgery to minimize the risk of thyroid storm.

Operative consent points

Thyroidectomy carries the risks of recurrent laryngeal nerve injury (horse voice, impaired speech, aspiration, if bilateral acute airway obstruction possible tracheostomy), hypocalcemia (which if untreated can lead to cataracts, brittle bones, muscle weakness and muscle irritability), lifelong thyroid medications (all total thyroidectomies and up to one third of lobectomies), thoracic duct injury bleeding and neck hematoma. The risks increase if also doing node dissection.

A cervical incision is made through the platysma. The strap muscles are split. The gland is rotated medially while dissection stays on the gland. The middle thyroid vein is ligated followed by the superior vessels. The recurrent nerve and parathyroid glands are identified and preserved. The inferior vessels are ligated.

Complications

The overall complication rate is 8.7 to 13% with endocrine complications in 5.6 to 11% (hypoparathyroidism in 5 to 15%; permanent in one to 2.5%).

Hypocalcemia can result in numbness and tingling around mouth or in hands, muscle irritability, Chvostek sign, EKG changes and seizures.

Nerve injury occurs in one ot six percent (recurrent laryngeal nerve, spinal accessory nerve, Horner’s syndrome, hoarse, weak or whispery voice, difficulty swallowing, coughing when drinking (signs of aspiration), acute airway obstruction or respiratory distress if bilateral. There is an increased risk if the patient is less than 10 years of age.

When to call the next level

Preoperatively, call when the consult is finished. Postoperatively, call for an expanding neck mass or hematoma, difficulty breathing/stridor (hematoma or nerve injury), whispery voice (concern for laryngeal nerve injury, scope vocal cords) and signs hypocalcemia (muscle irritability, Chvostek’s sign, EKG changes, seizures).

Author: Diana Diesen

Trauma Resuscitation and Initial Evaluation

Problem: Injured child

Likely diagnosis: Pediatric trauma

A pediatric trauma patient differs markedly from an adult trauma patient. Even between different ages, children differ in their injury patterns when it comes to trauma. One needs to be aware of these significant differences in order to best approach, examine and manage pediatric trauma patients in a time sensitive manner in order to optimize outcomes and survival.

In particular, the nonverbal child (i.e. babies, toddlers and developmentally delayed children) can be challenging to assess accurately. Not only do pediatric patients come in a variety of sizes, pediatric ongoing anatomical development plays a role in organ or tissue injury, systemic compensation to injury and healing that can be unseen in adults. As children become teenagers, their body development and size begins to be more similar to that of an adult.

Variation in care for pediatric trauma patients occurs between hospitals that treat adults and children compared to children only institutions.

Key history points

The level of response is determined by institution. Learn which level of response signifies the sickest patients (e.g. trauma red, trauma level 1) and which should be more stable. Be aware that any patient’s clinical status may deteriorate quickly.

Unless a pediatric patient in the trauma bay is unresponsive or with a significantly depressed Glascow coma scale, he or she may present with inconsolable crying, anxiousness and unwillingness to converse even if older in age or unable to be a reliable historian. Thus, the history should be obtained by witnesses if possible and physical exam is the key to diagnosis while knowing the injury patterns to be aware of for particular age groups and mechanisms of injury.

Was the trauma witnessed? What was the state of the child at the scene of the trauma? This may be different than the state of the child in the trauma bay now surrounded by more providers and undergoing intravenous catheter placement, in depth physical exam, wound bandage removal, placement of monitoring devices and separation from their caregiver.

For motor vehicle accidents was the child properly restrained based on the child’s size (five point restraint, car seat, booster seat, lap belt with shoulder strap or unrestrained). Improper restraints or seats put children at risk for head, intra-abdominal and cervical vascular injuries. Was the child wearing a helmet?

Ask EMS for the AMPLE history before they leave. Allergies, Meds, Past medical and surgical history, Last meal, Events/mechanism of injury.

Key physical exam findings

Use the Glasgow coma scale as modified for pediatric patients based on age groups.

The Broselow tape is a universal multicolored tape that is used to provide best estimation of medication doses, appropriate sized medical equipment and the weight based on what color the end length of the child falls under when the tape is placed along the length of the child.

The seat belt sign is a pattern of bruising across the neck, chest and abdomen corresponding to the straps of a car seat belt due to force exerted during crash. Have higher suspicion of neck vascular and intra-abdominal injuries. The handlebar sign is circular bruising to the epigastric area in the pattern of the end of the bicycle handlebar. Have a high suspicion for duodenal hematoma (that can progress to luminal obstruction and feeding intolerance) or pancreatic injury.

After stabilization, external findings, including those of nonaccidental trauma, should be documented according to hospital policy in a time sensitive manner. Evidence of retinal hemorrhages decreases with increasing age of the child.

Imaging

Focused assessment with sonography in trauma (FAST) has a low sensitivity (33%) for hollow viscous injury in children. Injury patterns more common in children such as solid organ injury (accounts for about 75% of all intra-abdominal injuries in children) after blunt abdominal trauma are not as readily diagnosed by ultrasound. FAST has a limited role in hemodynamically stable child.

There is a growing body of evidence that supports limiting radiation exposure in children due to the increased risk of radiation induced malignancy. The radiation dosing and or computerized tomography (CT) scan resolution should be appropriately adjusted for the child’s size and the diagnostic information needed. Liver and splenic injury CT grading systems are the same as for adults. Free fluid in the abdomen with no evidence of solid organ injury is concerning for hollow viscous perforation versus mesenteric tear injury causing bleeding. Utilize cystogram contrast formulas based on patient age for appropriate amount of contrast to detect bladder injuries.

Cardiac and aortic injuries are not as common in pediatric trauma as in adult trauma and thus thoracic imaging is less frequently needed.

Criteria for imaging of the head for diagnosing traumatic head injury may be dictated by institutionally derived algorithms based on verbal ability or age of the child.

Magnetic resonance imaging is gaining popularity for use in diagnosing head and intra-abdominal trauma especially in stable children suspected of nonaccidental trauma.

Preoperative preparation

Trauma labs are useful markers in raising suspicion for intra-abdominal trauma in children who require further work up. Abnormalities in the following do not necessarily correlate with grade of injury or clinically significant injury.

Aspartate transaminase (AST) and Alanine transaminase (ALT) - elevated threshold levels that would likely prompt further evaluation with imaging.

Lipase - any elevation is concerning for pancreatic injury.

Microscopic hematuria may indicate urinary tract injury.

At some institutions, the lab thresholds for suspicion of intra abdominal injury are lowered for nonaccidental trauma patients compared to accidental trauma patients. (example AST or ALT greater than 80 IU/L)

Indications for surgery

When a clinically significant injury requires surgery in a child, the technique for repair usually does not differ from adults. However, compared to adult trauma surgeons caring for adults with similar injuries, children cared for by pediatric trauma surgeons are less likely to be transfused blood products, less likely to undergo surgical operative intervention and less likely to be routinely full body CT scanned.

Studies have shown that even for higher grade solid injuries to the liver, kidney and spleen, salvage rates greater than 75% have been demonstrated with nonoperative management. Pancreatic injuries are more likely to fail nonoperative management.

Nonoperative management

The longstanding trend with pediatric trauma surgeons compared to adult trauma surgeons is to manage, especially solid organ injuries, nonoperatively.

Liver and Spleen

Recommended guidelines for resource use in isolated liver and spleen injuries

• no further imaging needed after the initial imaging that diagnosed the injury
• hospital stay in days is equal to one day plus the injury grade number (grades 1 through 4; example, a grade 3 liver injury expected on average to stay four days in the hospital for monitoring of persistent symptoms or new symptoms)
• activity restriction in terms of weeks is equal to two weeks plus the injury grade number (example, a grade 1 splenic injury should have activity restrictions for three weeks as a measure to avoid rebleeding from the injury)

Renal

For injury grades 3 and higher an ultrasound and functional scan at three to six months after the injury with biannual to annual blood pressure checks and labs for first several years after injury in order to diagnose Page kidney hypertension phenomenon.

Complications

Those more specifically related to pediatric trauma patients are pressure ulcers from prolonged placement on backboard or ill fitting cervical collars, delayed solid organ hemorrhage after initial nonoperative management (suspect in patients with persistent unchanged symptoms from the time of trauma even in the setting of normal vital signs).

Though outcomes for nonoperative management remain excellent, evidence based guidelines for follow up in pediatric trauma for most injuries is lacking.

When to call next level

Call your senior for any pediatric trauma patient with any vital sign instability, GCS less than 13 or worsening clinical status.

Author: Kathryn Bernabe

Omphalocele

Problem: Abdominal wall defect

Likely diagnosis: Omphalocele

Umbilical omphalocele is characterized by the failure of the embryo’s flat disk lateral folds to form the pleuroperitoneal canal in the midline and there is a failure of the viscera to return to the abdominal cavity following physiologic midgut herniation during the tenth week of gestation. As a result, the omphalocele includes a protective membranous sac composed of amniotic epithelium lined by peritoneum with the intervening space filled by Wharton’s jelly. Failure of the cephalic folds to meet in the midline will cause en epigastric omphalocele (Pentalogy of Cantrell) and failure of the caudal folds to fuse will cause a hypogastric omphalocele (cloacal exstrophy). Together with gastroschisis, omphalocele represents the most frequently encountered abdominal wall defects requiring neonatal intensive care. The incidence of omphalocele has remained constant over the years with roughly one to three per 10000 live births.

The stomach, small bowel, colon and liver are frequently contained in the omphalocele sac. Associated anomalies occur in 50 to 70 % of infants with omphalocele and cardiac defects are observed in 30 to 50 %. These include ventricular and atrial septal defects, ectopia cordis, tricuspid atresia and coarctation of the aorta. Karyotype abnormalities occur in 30 % of cases with trisomy 13, 18 and 21 being most common. Many syndromes and associations include abdominal wall defects. Two of the most common ones are Beckwith-Wiedemann syndrome (congenital abdominal wall defect, macroglossia, hypoglycemia, and propensity for development of abdominal tumors) and Pentalogy of Cantrell (epigastric omphalocele, diaphragmatic defect, pericardial defect, sternal cleft, and cardiac defect).

Key history points

Many times the diagnosis is implied with the prenatal ultrasound.

Key physical exam findings

Assess if membrane is intact. If not, then there is a need for prompt surgical intervention.

Differential diagnosis

Other diagnosis include

Umbilical cord hernia is different from omphalocele because there is no significant lateral fold defect. It is a simple failure of the midgut to return to the peritoneal cavity at 10 to 12 weeks. Therefore, the abdominal wall defect is smaller (less than five cm) and there is only midgut contents in the sac. Primary reduction is achievable in most cases.

Gastroschisis is characterized by prenatal evisceration through a defect in the anterior abdominal wall - almost always located just to the right of the umbilicus. This right sided predilection is theorized to be due to abnormal embryonic regression of the right umbilical vein. Importantly, the eviscerated abdominal contents do not have a protective membrane and are in direct contact with the amniotic fluid. The involved intestine is edematous, sometimes foreshortened and almost always nonrotated. Seven to 10 % of neonates with gastroschisis will have an associated intestinal atresia. Unlike omphalocele, gastroschisis is not associated with karyotype abnormalities.

Prune belly syndrome consists of a deficiency or absence of the abdominal wall musculature, a constellation of ureteral, bladder and urethral anomalies and bilateral cryptorchidism. Prune\ belly syndrome is distinguished from other abdominal wall defects by complete containment of the abdominal viscera within a continuous layer of skin with a normal umbilicus. In most cases, the lack of abdominal musculature does not cause significant functional impairment.

Imaging

The sensitivity and specificity of prenatal ultrasound in identifying abdominal wall defects is 60 to 75 % and 95%, respectively. Once a fetus with an abdominal wall defect is identified, directed ultrasounds should be performed to look for associated anomalies and malformations.

Indications for surgery

Definitive intervention depends on the size of the omphalocele. Primary closure of small omphaloceles may be carried out in the immediate neonatal period. Giant omphaloceles, however, are managed by allowing epithelialization of the sac with topical application of silver sulfadiazine, serial reductions with a custom made splint and elective repair at six to 24 months. Ruptured omphaloceles have very poor prognosis and require silo placement with delayed primary closure.

Preoperative preparation

Omphalocele presents with varying degrees of severity - nonetheless the presence of a neonatologist and pediatric surgeon should be considered mandatory in all cases whenever possible. Initial stabilization includes airway control, placement of an orogastric tube to facilitate visceral decompression and fluid resuscitation. Close blood glucose monitoring is also necessary given the incidence of Beckwith-Wiedemann syndrome. After stabilization, the surgeon must assess the viability of the membranous sac, size of the defect and decide between primary versus staged reduction. These patients also require postnatal karyotype analysis, echocardiography, renal ultrasound and detailed physical exam to assess for musculoskeletal defects prior to any operative intervention.

Operative consent points

The discussion should include chances of bleeding, infection, abdominal organ injury and abdominal compartment syndrome.

Complications

Complications of omphalocele primary closure include increased intra-abdominal pressure with the potential for abdominal compartment syndrome, wound infection, dehiscence and postoperative umbilical hernia. Other complications maybe related to associated anomalies such as prematurity, respiratory failure, hypoglycemia and hyperbilirubinemia.

Postoperative care

The management of omphalocele following reduction focuses on nutritional balance and early recognition of comorbidities. Most surgeons and neonatologists recommend 48 hours of antibiotics following reduction or establishment of silo protection. Enteral nutrition is established early given that the bowel is rarely involved.

The outcomes in omphalocele without associated anomalies are generally quite good with little to no long term morbidity. Most of the deaths in omphalocele occur due to the associated comorbidities. Other long term complications include bowel obstruction, abdominal wall hernias and an abnormal cosmetic appearance of the umbilicus.

When to call the next level

Any consult for omphalocele should be seen promptly and discussed with senior resident as soon as you get the consult.

Author: Matias Bruzoni

Vascular Access

Problem: Need for long term vascular access

All central lines are catheters that are inserted via head, neck or groin veins. The desired location of the tip will be at or near the cavoatrial junction. Central lines may be intended for short (less than four weeks) or long term (more than four weeks). The difference in the insertion is that short term catheters have no tunneling from the skin access to the venous access whereas long term catheters usually have an intentional tunneling of the catheter with a subcutaneous cuff to hold the catheter in place and decrease the infection risk. Also, subcutaneous infusion ports are a tunneled catheter that has an access port completely buried under the skin to minimize infection risks.

Central venous catheters deliver fluid or medications into a high flow site that allows rapid mixing of the agent delivered with central venous blood. This leads to quick mixture and limits the agent’s exposure to the vessel wall which may lead to irritation of the vein. They are also commonly used for patients who need frequent blood draws. It is important to note that, in general, a central line will be longer than a peripherally inserted catheter and should not be considered the first line for rapid infusion (e.g. trauma resuscitation).

Key history points

A consult for a central line usually arises from the service caring for the patient. This may result from lack of reliable peripheral venous access and/or need for central access for therapy (e.g. chemotherapy, parenteral alimentation, hemodialysis). Central venous catheters are inserted in all pediatric age ranges and, therefore, catheters inserted need to be representative of all sizes needed.

The important issues depend on the need for central venous access and include

• the reason for the central line
• whether the child previously had a central line
• if so, the location and number of vessels previously accessed
• problems with access in the past
• history of bleeding or easy bruising
• prior surgical history that may lead to altered venous anatomy

Anticoagulation medicines (including aspirin and nonsteroidal anti-inflammatory agents) should be noted.

Key physical exam findings

The general examination should include a focused assessment of central veins to try to identify the best vessel to be used. If there are scars near vessels with dilated distal veins it suggests that there may be stenosis or obstruction/occlusion of that vessel. Prominent venous collateral at the shoulder indicate subclavian thrombosis, Knowledge of previous central access in patient’s past is essential. Laboratory studies and imaging

A complete blood count and differential is essential. Neutropenia is associated with an increased risk of catheter sepsis. Thrombocytopenia will require correction. INR/PTT and bleeding studies are obtained as needed. An ultrasound (US) is suggested in all patients with multiple previous catheters. When equivocal, magnetic resonance venography may be required.

Indications for surgery

In general, if there is a good rationale for need for central venous catheter that should suffice – so long as other options have been considered. Common issues that preclude insertion of a central venous catheter include: functioning peripheral venous catheter for a patient only needing short term vascular access, bleeding issues that may preclude safe insertion and mediastinal masses which may make passage of a wire or catheter difficult.

Preoperative preparation

Prophylactic intravenous antibiotics may be given within one hour prior to insertion. The skin should be prepared with a chlorhexidine based prep in the operating room.

Operative consent points

The exact vein used may vary due to venous anatomy and prior line insertions. Therefore, do not commit on laterality or vein. The catheter requested should be listed. The risks of catheter insertion include bleeding and injury to surrounding structures (e.g. pneumothorax with the subclavian approach). Common issues after line placement include bleeding, line dysfunction or dislodgement, thrombosis (btoh catheter and vein) and infection.

If the child has diagnosis of cancer the oncology team may need to perform other procedures such as a lumbar puncture, bone marrow aspirate or biopsy. Make certain communication regarding additional procedures occurs with the referring team.

If the child has renal failure the dialysis catheter will be very large in diameter and should be placed in the internal jugular vein to avoid subclavian vein stenosis that would have implications for creation of upper extremity arteriovenous fistula or graft in the future.

Complications

Bleeding is common but can be addressed by direct pressure to the vein. Infection may be treatable and the line maintained. Notable exceptions include fungus and Pseudomonas which are difficult to eradicate without the line being removed. Occlusion of the catheter may occur due to thrombus formation. Patients having central lines for a prolonged period of time may outgrow their catheter where the tip is no longer central. Catheters may also break and those that are external may be maintained with catheter repair kits. Lines may be dislodged if they are pulled on and/or there is breakage of the catheter with resultant embolization of the catheter into the heart.

Postoperative care

A radiograph is obtained in the operating room and/or soon after surgery. Common postoperative issues include pain. Attention should be made as to the patient’s primary diagnosis to try to avoid some medications in certain patients (e.g. antipyretics such as acetaminophen and ibuprofen are often not used in cancer patients to avoid masking an infection). The instructions for the care for the catheter are institution dependent regarding dressing care, flushing and family teaching as well as who helps the family care for the line.

When to call the next level

Newborns, patients with coagulopathy, congenital heart disease or multiple previous catheters require special attention and should bve discussed with a senior resident or attending early in the consultation process.

Author: Kenneth Gow

Perspectives and Commentary

To comment on this handbook please contact the editors at NaT@eapsa.org.