Reduction cystoplasty in the prune belly syndrome: a long-term followup. 1994

T P Bukowski, and A D Perlmutter
Department of Pediatric Urology, Children's Hospital of Michigan, Detroit.

The role of reduction cystoplasty in the management of severe prune belly syndrome is controversial. From 1973 to 1990, 11 boys with severe prune belly syndrome underwent comprehensive urinary tract reconstruction, including overlapping reduction cystoplasty. Followup ranged from 1.5 to 18 years and 6 boys are now older than 18 years. Nine had sepsis or progressive renal failure as neonates; 7 had upper tract diversion as an initial procedure, of whom 2 have chronic renal failure. Currently 9 boys are voiding, including 1 who could not void preoperatively, with less than 50 cc residual after double or triple voiding. One boy underwent transplantation to an ileal conduit and 1 continues on intermittent catheterization. In the 9 cases in which bladder volume data were available average reduction in volume at operation was 52%. At an average of 7.7 years later (range 1.5 to 14) bladder volumes corrected for age were essentially unchanged or greater than corrected preoperative volumes. Since reflux was corrected in 7 of 9 boys it cannot account for the redevelopment of large bladder capacities (range 740 to 2,300 cc). In cases of the prune belly syndrome reduction cystoplasty has helped to improve voiding and minimize infection during early childhood but it does not seem to decrease bladder capacity or improve voiding dynamics in the long-term.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D011535 Prune Belly Syndrome A syndrome characterized by abdominal wall musculature deficiency, cryptorchism, and urinary tract abnormalities. The syndrome derives its name from its characteristic distended abdomen with wrinkled skin. Abdominal Muscle Deficiency Syndrome,Congenital Absence of the Abdominal Muscles,Eagle-Barrett Syndrome,Obrinsky Syndrome,Prune-Belly Syndrome,Eagle Barrett Syndrome,Prune Belly Syndromes,Prune-Belly Syndromes,Syndrome, Eagle-Barrett,Syndrome, Obrinsky,Syndrome, Prune Belly,Syndrome, Prune-Belly,Syndromes, Prune Belly,Syndromes, Prune-Belly
D001743 Urinary Bladder A musculomembranous sac along the URINARY TRACT. URINE flows from the KIDNEYS into the bladder via the ureters (URETER), and is held there until URINATION. Bladder,Bladder Detrusor Muscle,Detrusor Urinae,Bladder Detrusor Muscles,Bladder, Urinary,Detrusor Muscle, Bladder,Detrusor Muscles, Bladder
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D005500 Follow-Up Studies Studies in which individuals or populations are followed to assess the outcome of exposures, procedures, or effects of a characteristic, e.g., occurrence of disease. Followup Studies,Follow Up Studies,Follow-Up Study,Followup Study,Studies, Follow-Up,Studies, Followup,Study, Follow-Up,Study, Followup
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012720 Severity of Illness Index Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder. Illness Index Severities,Illness Index Severity

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