The urinary tract anomalies associated with agenesis of the abdominal walls

Posterior Urethral Valve Masquerading as the Prune Belly Syndrome

“…On voiding, the region of the bladder neck opens into an elongated trigonourethral canal and contrast medium may fill a utriculus masculinus. There may be some hold-up at the level of the pelvic floor, whilst distally there may be an anterior urethral diverticulum or megalourethra (Cremin, 1971 ;Shrom etal., 1981). Among infants with a posterior urethral valve, several clinical features may be present which suggest the prune belly syndrome.…”

Coexisting Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) and Prune Belly Syndromes

“…The observed abnormality of the abdominal wall is highly characteristic and proved to be combined both with undescended testicles and with significant anomalies of the urinary system. Dysplastic kidneys, dilated renal pelves and ureters, a large urinary bladder with a persistent urachus, vesicoureteric reflux, and dilatation of the bladder neck and the prostatic segment of the urethra with a bulging posterior wall are typical components of this syndrome (CREMIN 1971). Moreover, oligohydramnios, microcephaly and malrotation of the bowel have been associated findings also in some previous cases, and various pulmonary complications as well as urinary infection and renal failure have been frequent causes of early death (ALFORD et colI.…”

Inheritance of Kidney and Urinary Tract Diseases

“…The clinical course varies from neonatal death (20% of infants) to long-term survival without evidence of significant renal impairment. Megalourethra has been noted [277], as well as dilatation of the prostatic urethra (presumably due to lack of muscular support of the prostate), megacystis, megaureters, renal hypoplasia, and hydronephrosis. Decreased spermatogenesis and absence of spermatogonia have also been described [278].…”