title: Urinary reconstruction in vertebral, anorectal, cardiac, trachea-esophageal, renal abnormalities and limb defects association with chronic renal failure and penile duplication.
abstract: Various urological complications in VATER association require careful management. A 15-year-old boy with VATER association, including a hypoplastic lower urinary tract and diphallia, presented with chronic kidney disease and incontinence after a right loop ureterostomy. In order to acquire urinary continence without renal function impairment, an ileocecal reservoir with umbilical catheterizable stoma was created as a urinary reconstruction. The ectopic posterior penis was resected for cosmetic reasons, and the stump of the hypoplastic urethra was opened at the perineal skin. Clean intermittent self-catheterization through the umbilicus using disabled bilateral limbs was then achieved. This report describes the management of VATER association in a patient with complicated urological anomalies.
authors.affiliation: Department of Nephrology, Toho University Faculty of Medicine, Tokyo, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Muramatsu
authors.forename: Seiichiro
authors.initials: S
authors.lastname: Shishido
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Nihei
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hamasaki
authors.forename: Yoji
authors.initials: Y
authors.lastname: Hyodo
authors.forename: Takeshi
authors.initials: T
authors.lastname: Kawamura
authors.forename: Atsushi
authors.initials: A
authors.lastname: Aikawa
mesh_terms: D000293:Adolescent; D001006:Anus, Imperforate / Q000601:surgery*; D004947:Esophagus / Q000002:abnormalities* / Q000601:surgery; D006330:Heart Defects, Congenital / Q000601:surgery*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000601:surgery*; D008297:Male; D010409:Penile Diseases / Q000150:complications / Q000601:surgery*; D011884:Radius / Q000002:abnormalities* / Q000601:surgery; D019651:Plastic Surgery Procedures / Q000379:methods*; D013131:Spine / Q000002:abnormalities* / Q000601:surgery; D014132:Trachea / Q000002:abnormalities* / Q000601:surgery; D014551:Urinary Tract / Q000601:surgery*; D014570:Urologic Diseases / Q000150:complications / Q000601:surgery*
keywords: VATER association; chronic kidney disease; diphallia; lower urinary tract dysfunction; urinary reconstruction