Anemia of Prematurity - Portal Neonatal

Omphalocele and Gastroschisis
Last Updated: June 25, 2003
Synonyms and related keywords: abdominal wall defects, exomphalos, exumbilication
AUTHOR INFORMATION Section 1 of 10
Author: James G Glasser, MD, Clinical Associate Professor, Department of Pediatric Surgery,
Palmetto Health Alliance Children's Hospital
James G Glasser, MD, is a member of the following medical societies: American College of
Surgeons, American Pediatric Surgical Association, Christian Medical and Dental Society, South
Carolina Medical Association, and Southern Medical Association
Editor(s): David N Sheftel, MD, Director, Division of Neonatology, Lutheran General Children's
Hospital of Park Ridge, Clinical Associate Professor, Department of Pediatrics, Chicago Medical
School; Robert Konop, PharmD, Director, Clinical Account Management, Ancillary Care
Management; Brian S Carter, MD, Associate Director, Associate Professor, Department of
Pediatrics, Division of Neonatology, Vanderbilt University Medical Center and Gateway Medical
Center; Carol L Wagner, MD, Associate Professor, Department of Pediatrics, Division of
Neonatology, Medical University of South Carolina; and Neil N Finer, MD, Director, Division of
Neonatology, Professor, Department of Pediatrics, University of California at San Diego
INTRODUCTION Section 2 of 10
Background: Gastroschisis and omphalocele are among the most frequently encountered congenital
anomalies in pediatric surgery. Combined incidence of these anomalies is 1 in 2000 births, which
means, for example, that a pediatric surgeon will see 2 such babies for every 1 born with esophageal
atresia or tracheoesophageal fistula. Although specialists such as neonatologists and pediatric
surgeons are responsible for the care of these babies, this topic is included in a text of general
pediatrics to bring about a more thorough understanding of these anomalies and enable physicians to
care for these children more empathetically.
Many babies have correctable lesions and simply require routine pediatric care. For others, the
abdominal wall defect is part of a larger constellation of unresolved problems, and further care by
specialists is necessary. All of these children, however, require general management by pediatricians
who have knowledge of their particular anomalies and their past surgical histories. For example,
physicians should know if an associated malrotation was corrected (to prevent midgut volvulus) and
whether an abnormally located appendix was removed (to prevent occurrence of atypical
appendicitis).
Pathophysiology:
Embryology
Fetal growth and definition of form are orchestrated by processes that are specific regarding time and
location. Growth spurts often are followed by delays. Cellular differentiation, proliferation, migration,
and deposition are involved in the formation of new tissue. Initially, the embryo is flat within the
umbilical ring, which is defined histologically by the junction of cylindrical epithelium of the epiblast
(ectoderm) and the cuboidal epithelium of the amnion. Two layers comprise the embryo: the epiblast
(ectoderm), which becomes either neuroectoderm or surface epithelium, and the hypoblast, which
becomes the inner epithelium of gut-derived organs. Formation of a third germ layer (mesoblast)
occurs along with the change in the embryo's shape. Elongation of the embryonic disc and
longitudinal and lateral enfolding create a cylinder with a recognizable body plan.