British Jorirtiol of Urology (1976). 48, 76
0
Short Case Report
Posterior Urethral Valves in Siblings AMIUJK FAMASand DONALI) G . SWNW Drpartnlent Of SUrgew, DiL'iSiOtI O/' Ur(J/Ogy. UC'LA School of M ~ d i c i n e .Los Angi4i,s, Califi,rniu 90024,
Documentation of the familial occurrence of hypospadias, cryptorchidism, vesico-ureteric reflux and congenital uretero-pelvic junction obstruction has prompted the establishment by the American Academy of Pediatrics of a national registry of families in whom more than one sibling has a congenital genitourinary anomaly. We recently treated 2 brothers with posterior urethral valves causing severe damage to their upper urinary tracts. To our knowledge, no similar anomaly has been reported among siblings in the English literature.
USA,
Case Report A 7-day-old boy had urinary retention with overflow dribbling. Intravenous urogram a n d voiding cystourethrogram revealed a hydronephrotic right kidney, a non-functioning left kidney associated with posterior urcthral valves, mega-ureters, a n d VeSiCO-Ureteric reflux (Fig. I). H e underwent fulguration of the valve, left nephro-ureterectomy and urinary reconstruction. Aware of the disease in their youngest child, the parents brought in his older brother, age 2 years, for urological evaluation. He was reported to be without symptoms. Urinalysis, however, revealed pyuria and radiographic studies demonstrated disease that was identical, although mirror-imaged, t o that of his brother. A non-functioning right kidney was demonstrated on IVP with massive hydronephrosis. A voiding cysto-urethrograrn revealed posterior urethral valves with bilateral ureteral reflux and mega-ureters (Fig. 2). He also underwent fulguration of the posterior urethral valve, right nephrourcterectoniy, and subsequent urinary reconstruction.
~ alld
i1. voiding ~ , cysto-urethrograt~lshowing urctcrlc reflux
posterior urcthral valves.
Comment Siblings of children with congenital genitourinary anomalies should be scrutinised closely to detect similar anomalies before irreversible damage may occur. That the younger infant's anomaly triggered discovery of his brother's similar asymptomatic problem may have prevented further serious renal dam age.
Fig. 2. Voiding cysto-urethrogram showing posterior urethral valves associated with ureterlc reflux.
76
0
Short Case Report
Posterior Urethral Valves in Siblings AMIUJK FAMASand DONALI) G . SWNW Drpartnlent Of SUrgew, DiL'iSiOtI O/' Ur(J/Ogy. UC'LA School of M ~ d i c i n e .Los Angi4i,s, Califi,rniu 90024,
Documentation of the familial occurrence of hypospadias, cryptorchidism, vesico-ureteric reflux and congenital uretero-pelvic junction obstruction has prompted the establishment by the American Academy of Pediatrics of a national registry of families in whom more than one sibling has a congenital genitourinary anomaly. We recently treated 2 brothers with posterior urethral valves causing severe damage to their upper urinary tracts. To our knowledge, no similar anomaly has been reported among siblings in the English literature.
USA,
Case Report A 7-day-old boy had urinary retention with overflow dribbling. Intravenous urogram a n d voiding cystourethrogram revealed a hydronephrotic right kidney, a non-functioning left kidney associated with posterior urcthral valves, mega-ureters, a n d VeSiCO-Ureteric reflux (Fig. I). H e underwent fulguration of the valve, left nephro-ureterectomy and urinary reconstruction. Aware of the disease in their youngest child, the parents brought in his older brother, age 2 years, for urological evaluation. He was reported to be without symptoms. Urinalysis, however, revealed pyuria and radiographic studies demonstrated disease that was identical, although mirror-imaged, t o that of his brother. A non-functioning right kidney was demonstrated on IVP with massive hydronephrosis. A voiding cysto-urethrograrn revealed posterior urethral valves with bilateral ureteral reflux and mega-ureters (Fig. 2). He also underwent fulguration of the posterior urethral valve, right nephrourcterectoniy, and subsequent urinary reconstruction.
~ alld
i1. voiding ~ , cysto-urethrograt~lshowing urctcrlc reflux
posterior urcthral valves.
Comment Siblings of children with congenital genitourinary anomalies should be scrutinised closely to detect similar anomalies before irreversible damage may occur. That the younger infant's anomaly triggered discovery of his brother's similar asymptomatic problem may have prevented further serious renal dam age.
Fig. 2. Voiding cysto-urethrogram showing posterior urethral valves associated with ureterlc reflux.
76
