Congenital Absence of the Abdominal Muscles: A Clinicopathologic Correlation

Abstract

Patients with congenital absence of the abdominal musculature consistently have gross urinary tract abnormalities and, if they are males, undescended testes. Anomalies affecting the cardiovascular, gastrointestinal, and musculoskeletal systems are frequent but inconsistent findings. Collagen deposits replacing the smooth muscle of the urinary tract, dysplasia of the renal parenchyma, and failure of muscles of the abdomen to develop are the salient histologic features. Renal failure and sepsis are the most common causes of death. Inefficient emptying of the urinary tract makes these patients prone to urinary infection, and an impaired cough mechanism can lead to pneumonia. The etiology of this anomaly is only speculative at this time.