Ventral Abdominal Wall Defects

Sara A. Mansfield; Tim Jancelewicz

doi:10.1542/pir.2018-0253

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Sara A. Mansfield, MD, MS*
Tim Jancelewicz, MD, MA, MS*

^*Division of Pediatric Surgery, Le Bonheur Children’s Hospital, University of Tennessee Health Science Center, Memphis, TN

AUTHOR DISCLOSURE
Drs Mansfield and Jancelewicz have disclosed no financial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/investigative use of a commercial product/device.

Practice Gap

Clinicians should be aware of the strategies for prenatal and postnatal management of infants with omphalocele and gastroschisis.

Objectives

After completing this article, readers should be able to:

Recognize the implications and differences in management between gastroschisis and omphalocele.
Review the initial stabilization and management of infants with omphalocele and gastroschisis.
Review the surgical management options and rationale for infants with ventral abdominal wall defects.

Introduction

Abdominal wall defects represent a wide spectrum of congenital anomalies. These can range from lethal limb-body wall syndrome to benign umbilical cord hernias. Gastroschisis and omphalocele are the 2 most common defects and are the focus of this review. Although both of these diseases affect the umbilical area, they differ widely in their underlying pathogenesis, genetics, and associated disorders. Consequently, their management techniques and outcomes are quite different. This article reviews the pathogenesis, genetics, diagnostics, and outcomes for each disease, followed by an in-depth review of recent management updates.

Omphalocele

Pathogenesis

Omphalocele (or exomphalos) is a herniation of the abdominal viscera through a midline abdominal wall defect (Fig 1). This defect is located at the base of the umbilical stalk, and herniated viscera are covered by a 3-layer membrane of peritoneum, Wharton jelly, and amnion. This contrasts with umbilical cord hernias, which are covered by intact skin and contain only a small protrusion of abdominal contents, and gastroschisis, which has no covering at all and occurs to the right of midline (Table). Omphaloceles are present in approximately 1 per 1,100 pregnancies. However, there is a high rate of spontaneous abortion, making the incidence of omphalocele per live birth approximately 1 per 4,000 to 1 per 6,000. (1) …