Congenital Absence of the Abdominal Muscles and Genitourinary Malformation

Abstract

The congenital anomaly of absence of the abdominal muscles is rarely seen and is constantly associated, when seen, with a variety of genitourinary anomalies, which always include hypertrophied bladder and usually include dilated and hypertrophied ureters, cystic degeneration of the renal tissues and cryptorchidism. These urinary anomalies are often associated with urethral obstruction of some type, and some authorities, notably Housden, have expressed the belief that urethral obstruction is present for at least an early part of fetal life in all cases of absence of abdominal muscles and causes secondarily dilatation of the bladder, pressure reabsorption of the anlage of the abdominal musculature, dilatation of the ureters, pressure dilatation of the renal tubules with cystic degeneration and nondescent of the testicles. However, there are few cases of absence of abdominal muscles reported in which an organic obstruction of the urethra was demonstrated\p=m-\infact, I believe only 2, 1 by Housden1 and 1 by Lichtenstein.2 It is difficult to understand the cases of gross urethral obstruction, with the same pressure anomalies of the genitourinary tract but with an entirely intact group of abdominal muscles. The cases in this report represent these two types : first, absence of abdominal muscles with a normal urethra and second, normal abdominal muscles with an obstructed urethra. REPORT OF CASESCase 1.-E. N., born June 10, 1939, the first child of Italian parents, was delivered without complications. His birth weight was 2,865 Gm., and his appear¬ ance was unusual in that the full, large abdomen was covered with thin skin which showed the pattern of many intestinal loops and one central longitudinal fold containing the umbilicus. Peristaltic motion could be watched in the intestinal loops. The costal margin was tilted forward and twisted slightly, so that it was prominent on the left. The head showed much molding, overriding of the parietal 1. Housden, L. G.: Congenital Absence of the Abdominal Muscles, Arch.

The prune belly anomaly: heterogeneity and superficial X-linkage mimicry.

“…The triad of deficient abdominal musculature, urinary tract and renal anomalies, and cryptorchidism has been recognised for many years (Parker, 1895;Howard, 1940;Roberts, 1956;Nunn and Stephens, 1961;Boume and Cerny, 1967;Williams and Burkholder, 1967;Texter and Murphy, 1968;Burke et al, 1969;Burkholder et al, 1970;Afifi et al, 1972;Palmer and Tesluk, 1974;Welch and Kearney, 1974). More than 250 patients with this constellation of features, referred to as the prune belly anomaly (PBA), have been described (Ives, 1974).…”

Complicated airway management in a child with prune‐belly syndrome

“…Prune‐belly syndrome is characterized by absent abdominal wall musculature with wrinkled overlying skin, urinary tract dilatation and cryptorchidism (1,2). Inadequate fetal urinary output secondary to urethral obstruction and the resultant oligohydramnios may lead to congenital pulmonary hypoplasia and Potter’s facies, characterized by micrognathia, malformed ears and nose (2,3).…”