Congenital Gastric Anomalies

Jonathan Vacek, MD, Mary E Fallat, MD
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Introduction

The literature on congenital gastric anomalies is limited to scattered case reports and small series. Deciding upon the appropriate course of management for these patients is largely informed by other surgical processes with similar pathophysiology. It was our aim to provide an overview of congenital gastric anomalies and highlight how their management can be linked to general surgical principles.

What are the types of congenital gastric anomalies?

The gastric anomalies under consideration for this topic are as follows:

  • congenital gastric outlet obstruction
    • prepyloric antral diaphragm – a thin distal antral membrane of gastric tissue covering the pyloric channel
    • pyloric atresia – nonpatent pyloric channel of varying severity
  • congenital microgastria – an underdeveloped or atretic stomach resembling tubular foregut structures [1]
  • congenital gastric diverticulum – a true diverticulum of the stomach wall [2]
  • positional defect of the stomach
    • gastric inversion – the pylorus is positioned above the gastroesophageal junction (only two cases reported [3][4])
    • situs inversus of the stomach – right sided stomach which can be isolated or associated with either situs inversus abdominus or totalis
  • dysplasia of gastric musculature
    • hypoplasia or aplasia of gastric smooth muscle in a localized or diffuse pattern
    • congenital hourglass stomach – hypertrophy of smooth muscle in the gastric wall causing two distinct chambers (only three patients have been described in the literature[5][6]), the noncongenital type can be acquired from chronic peptic ulcer disease or malignancy [2]. A symptomatic congenital hourglass stomach can be treated by resecting the stomach proximal to the lesion and reconstruction with either a Billroth procedure or a Roux-en-y loop.
  • angiodysplasia of the stomach – an arteriovenous malformation causing ectasia of the capillary bed (only the nonsyndromic angiodysplasia will be reviewed as syndromic vascular malformations are discussed elsewhere)
  • gastric heterotopic pancreas – Ormarsson defines this as pancreatic tissue found outside its normal localization and without any anatomical or vascular connection with the pancreas [7]
  • gastric duplication cysts – a cystic structure containing three layers resembling the normal gastric wall [8] the cyst may or may not have a communication with the lumen of an adjacent structure [8]

see also Peptic Ulcer Disease, Spontaneous Gastric Perforation and Gastric Tumors

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Introduction

The literature on congenital gastric anomalies is limited to scattered case reports and small series. Deciding upon the appropriate course of management for these patients is largely informed by other surgical processes with similar pathophysiology. It was our aim to provide an overview of congenital gastric anomalies and highlight how their management can be linked to general surgical principles.

What are the types of congenital gastric anomalies?

The gastric anomalies under consideration for this topic are as follows:

  • congenital gastric outlet obstruction
    • prepyloric antral diaphragm – a thin distal antral membrane of gastric tissue covering the pyloric channel
    • pyloric atresia – nonpatent pyloric channel of varying severity
  • congenital microgastria – an underdeveloped or atretic stomach resembling tubular foregut structures [1]
  • congenital gastric diverticulum – a true diverticulum of the stomach wall [2]
  • positional defect of the stomach
    • gastric inversion – the pylorus is positioned above the gastroesophageal junction (only two cases reported [3][4])
    • situs inversus of the stomach – right sided stomach which can be isolated or associated with either situs inversus abdominus or totalis
  • dysplasia of gastric musculature
    • hypoplasia or aplasia of gastric smooth muscle in a localized or diffuse pattern
    • congenital hourglass stomach – hypertrophy of smooth muscle in the gastric wall causing two distinct chambers (only three patients have been described in the literature[5][6]), the noncongenital type can be acquired from chronic peptic ulcer disease or malignancy [2]. A symptomatic congenital hourglass stomach can be treated by resecting the stomach proximal to the lesion and reconstruction with either a Billroth procedure or a Roux-en-y loop.
  • angiodysplasia of the stomach – an arteriovenous malformation causing ectasia of the capillary bed (only the nonsyndromic angiodysplasia will be reviewed as syndromic vascular malformations are discussed elsewhere)
  • gastric heterotopic pancreas – Ormarsson defines this as pancreatic tissue found outside its normal localization and without any anatomical or vascular connection with the pancreas [7]
  • gastric duplication cysts – a cystic structure containing three layers resembling the normal gastric wall [8] the cyst may or may not have a communication with the lumen of an adjacent structure [8]

see also Peptic Ulcer Disease, Spontaneous Gastric Perforation and Gastric Tumors

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Last updated: November 2, 2020