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NeoReviews Vol.6 No.11 2005 e493
© 2005 American Academy of Pediatrics

Gastroschisis

Embryology, Pathogenesis, Epidemiology

Shilpi Chabra, MD^*
Christine A. Gleason, MD

^* Assistant Professor of Pediatrics, University of Washington, Seattle, Wash
Division Head and Professor of Pediatrics, Department of Pediatrics, University of Washington, Seattle, Wash

Abbreviations: ICBDMS: International Clearinghouse of Birth Defects Monitoring Systems • ICD-9: International Classification of Diseases, 9th Revision • ICD-9-CM: International Classification of Diseases, 9th Revision, Clinical Modification

The first 300 words of the full text of this article appear below.

	Objectives

 
After completing this article, readers should be able to:

1. Describe normal embryology and various theories contributing to derangements in development leading to gastroschisis.
   
2. Delineate several theories regarding the pathogenesis of gastroschisis.
   
3. Explain the environmental and other risk factors linked to gastroschisis.
   
4. Describe the prevalence of gastroschisis in developed countries and various theories explaining it.
   

	Introduction

 
Gastroschisis is a congenital anterior abdominal wall defect, adjacent and usually to the right of the umbilical cord insertion. It occurs as a small, full-thickness periumbilical cleft either immediately adjacent to the umbilicus or separated from it by a strip of skin. This results in herniation of the abdominal contents into the amniotic sac, usually just the small intestine, but sometimes also the stomach, colon, and ovaries (Figure). The abdominal wall defect is relatively small compared with the size of the eviscerated bowel, which often develops walls that are matted and thickened with a fibrous peel. Gastroschisis has no covering sac and no associated syndromes. This differentiates it from an omphalocele, which usually is covered by a membranous sac and more frequently is associated with other structural and chromosomal anomalies (Table 1). In addition, although gastroschisis may be associated with gastrointestinal anomalies such as intestinal atresia, stenosis, and malrotation, it has a much better prognosis than omphalocele.

View larger version (100K): [in this window] [in a new window]   Figure. Gastroschisis, resulting in herniation of the abdominal contents into the amniotic sac.

Gastroschisis Omphalocele Incidence 1 in 10,000 (now increasing) 1 in 5,000 Defect Location Right paraumbilical Central Covering Sac Absent Present (unless sac ruptured) Description Free intestinal loops Firm mass including bowel, liver, etc Associated With Prematurity 50% to 60% 10% to 20% Necrotizing Enterocolitis Common (18%) Uncommon Common Associated Anomalies Gastrointestinal (10% to 25%) Intestinal atresia Malrotation Trisomy syndromes (30%) Cardiac defects (20%) Beckwith-Weidemann syndrome Cryptorchidism (31%) Bladder extrophy Prognosis Excellent for small defect Varies with associated anomalies Mortality 5% to 10% Varies with associated anomalies (80% with cardiac defect)

	Historical Perspective

 
The term gastroschisis is derived from the Greek word laproschisis, meaning "bellycleft." It was used in the 19th and early 20th centuries by teratologists to designate all abdominal wall defects. No clear distinctions were made between abdominal wall defects until 1953, when Moore and Stokes classified them based on their appearance at birth. They suggested that the term gastroschisis be reserved for those cases in which the defect is . . . [Full Text of this Article]