Congenital Absence of Abdominal Musculature: Urologic Aspects

The developmental defect of absence of the abdominal musculature is part of a syndrome of multiple anomalies amongst which disorders of the urinary tract present the greatest threat to life. The condition is due to congenital dysplasia of the musculature of the abdominal wall and urinary tract. This leads to impaired emptying and upper tract dilation with kidney insufficiency. Some infants are doomed at birth by the severity of their renal damage. Others may be saved by urinary tract drainage. We suggest circumventing the highest point of obstruction first to preserve renal parenchyma, using “loop” or “double barrel” cutaneous ureterostomy as a preliminary measure. Later restoration of continuity can be established after correction of any impediment to bladder outflow. Longitudinal plication of attenuated abdominal wall structures has been successful in two cases. This reconstruction is important in regard to respiratory ventilation, bowel function, locomotion, and aiding voiding.