Introduction

Welcome to the APSA Quality and Safety committee Congenital Diaphragmatic Hernia (CDH) Toolkit. This page is intended to help anyone who is interested in quality improvement (QI) for the management of patients with CDH.

Available toolkit projects are listed below. Many of the approaches described are evidence-based - some are not. These approaches have not been approved by APSA.

Preoperative Guidelines/Strategies

The neonatology and pediatric surgery departments at Children’s Memorial Hermann Hospital / University of Texas Health Sciences Center at Houston (UT Houston) developed an evidence-based guideline focused on the initial stabilization and management of CDH patients.

Protocol:

• UT Houston CDH Management (From Birth to Surgery)

Resources:

• UT Houston Methodology for the Development of the CDH Management Guidelines

Stakeholders: Maternal Fetal Medicine (MFM), Neonatology, Pediatric Surgery, Cardiology, Pharmacy.

Challenges & solutions:

Links to published data: [1][2][3]

Submitted by: Matt Harding, MD

Additional implementers: Tim Jancelewicz, MD, MA, Yigit Guner, MD

The initial medical management of CDH and pulmonary hypertension was standardized at Children’s Hospital Los Angeles (CHLA) focusing on clinical criteria and parameters for timing of repair.

Protocol:

• CHLA CDH Consensus Guidelines

Resources:

Stakeholders: neonatologists, pediatric surgeons, anesthesiologists, ECMO providers, nurses.

Challenges and solutions: The major challenge was communication amongst a large group of providers. The neonatologists selected a small group of faculty, based on experience and interest, to manage patients, prior to and during ECMO, in order to improve communication and standardize management of pulmonary hypertension.

Links to published data:

Submitted by: Juan Carlos Pelayo, MD

Additional implementers: Neonatologists: Rachel Chapman, Phillipe Fredrick; Pediatric Surgeons: David Bliss, MD, Chris Gayer, MD

ECLS and High Risk Algorithms

The Fetal Diagnosis and Treatment program at Hassenfeld Children’s Hospital at NYU Langone developed a high-level workflow algorithm to coordinate the multiple teams and decision-making that occurs during an expected high-risk CDH delivery with ECMO immediately available.

Protocol:

• NYU Langone High Risk CDH Delivery Plan

Resources:

Stakeholders: MFM, Neonatology, NICU Nursing, Pediatric Surgery, ECMO/Perfusion, OR Staffing, Pharmacy

Challenges and solutions: EXIT-to-ECMO for high-risk CDH has largely been abandoned due to a lack of significant benefit over an ECMO-on-standby approach. Institutions may accomplish a C-section with ECMO-on-standby approach in a variety of different ways, driven by physical plant, clinical service line, and staffing considerations. After several cases where this approach was deployed, there was a broad desire to create a codified approach to this multidisciplinary, high-risk plan effectively involving separate maternal and infant treatment teams co-localized to a single location. The provided workflow outlines our institution’s approach to providing a rapid assessment in the OR of the newborn and making the determination as to whether they should be cannulated or transported to the NICU for further care. Following implementation, team satisfaction and coordination across services were significantly streamlined.

Links to published data: [4][5]

Submitted by: Jason C. Fisher, MD

Surgical Strategies

Children’s Hospital Los Angeles (CHLA) developed a QI project to standardize the management of CDH given that the surgeons offering CDH repair during ECMO had different thresholds and time ranges before an operation was offered. In order to do so, CDH patients were divided into early versus delayed repair cohorts and differential protocols were applied based on the groups.

Protocol:

• CHLA CDH Surgical Management Protocol

Resources:

• CHLA CDH Patient Cohorts

Stakeholders: neonatologists, pediatric surgeons, anesthesiologists, ECMO providers, nurses.

Challenges and solutions: Surgeons that offered CDH repair during ECMO had different thresholds and time range before an operation was offered. We decided to divide our group into early versus delayed repair. Half of our group defined early repair as < 48hr on ECMO and late repair >48hr on ECMO.

Submitted by: Juan Carlos Pelayo, MD

Additional implementers: Neonatologists: Rachel Chapman, Phillipe Fredrick; Pediatric Surgeons: David Bliss, Chris Gayer, MD

Postoperative Strategies

At the University of Virginia, an opioid reduction strategy was implemented in the Neonatal Intensive Care (NICU) for patients undergoing open CDH repair. The intervention was centered on a previous experience implementing an opioid reduction strategy for neonates undergoing elective abdominal surgery. The intervention was a multi-modal approach based on: 1. Standing IV acetaminophen administration (based on patient’s dosing weight) for at least 48 hours post-operatively, 2. Postoperative pain management education for the neonatal nurse practitioner and nursing teams 3. Directly addressing the postoperative pain management strategy during the NICU provider signout following each CDH operation. Anesthesia, surgery and NICU representatives participated in these signouts. Using this protocol, postoperative opioid use was successfully reduced by 97%, while maintaining similar neonatal pain scores. The intervention also reduced the postoperative intubation time by approximately 100 hours.

• Protocol:
  • UVA CDH ERAS Protocol
• Resources:
  • UVA Stakeholder Meeting Goals & Notes
    
    • Initial stakeholder meetings which included pediatric surgery, pediatric anesthesiology, neonatal intensivist teams and NICU nursing team to discuss the feasibility and potential challenges of opioid reduction strategy in the post-surgical NICU population including patients with complex pathology such as CDH.
  • UVA NICU CDH ERAS IV Tylenol Order Set
    
    • Postoperative order set which included 48 hours of standing IV acetaminophen and minimization of automatic opioid infusions. Worked with our inpatient pharmacy group to approve automatic postoperative order set (1-year approval process).
    • Post-operative pain management education sessions including clinical benefits and safety profile of reduced opioid use given to NICU team. Course also highlighted strategies to achieve appropriate pain control utilizing non-opioid pain management options, and reduction of opioid infusions.
  • UVA HSR Protocol/IRB Submission
  Stakeholders: pediatric surgery team, neonatal intensivist team specifically including the nurse practitioners, the bedside nurse, and the pharmacology team. Pediatric anesthesia teams were also involved in early meetings. Challenges & solutions:
  • IV Acetaminophen approved by the pharmacology team. The initial concern of IV acetaminophen approval was centered on the price of the medication. Specifically, that IV acetaminophen could only be ordered on an individual basis at our institution. We were able to address this by working closely with pharmacology team and eventually coming an agreeement to reuse a single bottle of IV acetaminophen (1,000 mg bottle) for multiple post-operative doses given the size of the neonates.
  • Buy-in from the NICU team that postoperative pain was being appropriately managed.
  • The education intervention was a key aspect of the overall quality improvement initiative as it involved the full NICU team (from the onset) and provided direct support to the clinical advantages of reduced opioid use. Additionally, the education intervention was essentially a cost-neutral intervention aside from the necessary time on all parties.

Links to published data: [6][7][8][9][10]

Submitted by:Jeffrey Gander, MD, David Grabski, MD, Eugene McGahren, MD

Recent Research

For recent research pertaining to the management of patients with CDH, please refer to references: [3][11][12][13][14]

Additional Resources

• Video: APSA TEC Talk 2020: Protocols for the Management of CDH

Toolkit Curators

Nam Nguyen MD, Kirk Reichard MD, "Migration Editors": Raquel Gonzalez, MD, Kristen Kaiser, MD

References