Case Study

Triad syndrome
(Eagle-Barrett or prune belly syndrome)

by Michael E. Speer, MD

Case presentation

This infant was born by elective cesarean because of oligohydramnios at 354/7 weeks’ gestation. Following a short trial of blow-by oxygen, the infant was intubated because of marked increased work of breathing. Over the next several hours the infant’s respiratory status improved and he was able to be extubated and then weaned to room air. A renal ultrasound showed small kidneys without hydronephrosis and massively dilated ureters. A voiding cystourethrography (VCUG) showed reflux on the right, no reflux on the left, enlargement of the urinary bladder, and dilation of the posterior urethra. A Foley catheter was placed for bladder drainage and amoxicillin was started for prophylaxis. The serum creatinine and BUN values slowly increased from birth, requiring the institution of peritoneal dialysis.

Denouement

This infant’s findings are characteristic of Triad Syndrome (hypogonadism, absence of abdominal wall muscle, dilated ureters/renal failure) also known as Eagle-Barrett or prune belly syndrome. The reported incidence is 1:40,000, and 95% of the affected infants are male. Pulmonary hypoplasia is a frequent finding as is acute and chronic renal failure secondary to in utero development of either renal dysgenesis or hydronephrosis. Far fewer cases may have congenital cardiac malformations and/or intestinal malrotation. Some also have feeding difficulties secondary to impaired peristalsis due to abdominal wall laxity. These children frequently require chronic dialysis and renal transplantation later in life. They have a functional bladder outlet obstruction that accounts for some of the findings regarding the bladder and ureters. Although in utero urinary diversion procedures have been attempted, they have had very limited success.