Males with Down's syndrome and nonneurogenic neurogenic bladder.
OBJECTIVE
We assessed the relationship of trisomy 21 with the most severe dysfunctional elimination syndrome, nonneurogenic neurogenic bladder or the Hinman-Allen syndrome.


METHODS
We retrospectively reviewed our experience with children with Down's syndrome (trisomy 21) in a 10-year period and identified a subset who presented for the evaluation and treatment of urinary tract infections associated with severe disorders of urinary and fecal elimination.


RESULTS
Four males 9 months, 14 years, 18 years and 21 years old met the criteria for review. All patients underwent radiological and urodynamic evaluation, and were diagnosed with hydronephrosis and prostatic urethral dilatation with pelvic floor spasticity. Renal function studies showed a creatinine of 0.7 mg./dl. in the 9-month-old, 1.2 mg./dl. in the 14-year-old, 1.9 mg./dl. in the 18-year-old and 2.2 mg./dl. in the 21-year-old patient. Three patients underwent surgical treatment to protect the upper urinary tract, including bladder augmentation cystoplasty and an appendiceal Mitrofanoff stoma in 2, and vesicostomy in 1. The remaining patient was treated conservatively with a behavioral modification program that included timed voiding and a bowel regimen.


CONCLUSIONS
Boys with trisomy 21 may be at risk for the Hinman-Allen syndrome. Surgical intervention and clean intermittent catheterization for renal preservation and continence can be performed in this population despite intellectual impairment. Further evaluation is necessary to determine whether this relationship is more common than appreciated and whether this syndrome occurs in females with trisomy 21.
Handel
Liann N
LN
Department of Surgery, Brown University Medical School, Rhode Island Hospital, Providence, Rhode Island, USA.
Barqawi
Albaha
A
Checa
Giancarlo
G
Furness
Peter D
PD
Koyle
Martin A
MA
D000293:Adolescent; D000328:Adult; D002648:Child; D004314:Down Syndrome / Q000150:complications*; D006801:Humans; D008297:Male; D012189:Retrospective Studies; D001750:Urinary Bladder, Neurogenic / Q000209:etiology*