Unusual congenital abdominal wall defect and review

Abstract

Abdominal wall defects distant from the umbilicus are very unusual. We describe a patient with a congenital defect adjacent and external to the lateral border of the recti muscles, rounded in shape, bilateral and symmetrical, but affecting different structures in each side, musculocutaneous agenesis in the left side and only muscle agenesis in the right one. The morphologic characteristics of the defect and the absence of associated anomalies, suggest that this anomaly may be the result of an event acting during phenogenesis. We consider that this defect is different from other structural abnormalities of the anterior abdominal wall which occur much earlier at certain periods of the embryogenesis. Though the etiology is speculative, we could postulate a disruptive origin of the defect of unknown cause.

Pitfalls in prenatal diagnosis of unusual congenital abdominal wall defects

“…Similar skin-covered abdominal wall defects have been reported and referred to as body wall dysplasia [9][10][11]. Two of our patients had small associated omphalocele sacs, which were easily managed.…”

Development of gastroschisis: Review of hypotheses, a novel hypothesis, and implications for research

“…In another scenario, the primary intestinal loop may herniate normally into the umbilical cord, with another part of the gut tube herniating through an unclosed portion of the body wall. The more lateral ventral wall defects [Melikoğlu et al, 1998; Fraser and Crabbe, 2002; Carrera et al, 2003; Ameh et al, 2004] that are not adjacent to the umbilical cord have been proposed to be due to an abnormality in the migration of the lateral plate mesoderm (specifically the parietal layer). These lateral ventral wall defects allow the internal organs to either bulge, covered by a thin membrane or normal skin, or eviscerate.…”

Left-sided Gastroschisis: Is it the Same Pathology as on the Right-Side?

“…Of the twenty cases of left-sided gastroschisis described in the literature, many diff er quite considerably from the classic right-sided form [2,9,13,15,19,22] , i. e. the defect is distant from the umbilicus, in the fl ank or the hypochondrium and most of the time the defect is large. The defects bear a greater resemblance to certain forms of aplasia of the fl ank [3] than to true gastroschisis. Other cases described are associated with a number of extra-intestinal congenital anomalies, including bilateral multicystic dysplastic kidney [14] , bladder pseudo-exstrophy or cardiac and vascular anomalies as in two out of the three cases described by Suver et al [18] .…”