Urological manifestations associated with chronic intestinal pseudo-obstructions in children

Purpose: Chronic intestinal pseudo-obstruction syndrome is a rare pathological condition characterized by chronic intestinal occlusion without any anatomical or mechanical lesion. Recent progress in the treatment of these children has led to improved survival and development of associated urological complications. We define urological manifestations of chronic intestinal pseudo-obstruction to optimize evaluation and treatment. Materials and Methods: We evaluated retrospectively the charts of 20 girls and 19 boys with a mean age of 11.2 years (range 3 days to 26 years) diagnosed with chronic intestinal pseudo-obstruction from January 1975 to February 2000. Urological evaluation included prenatal and postpartum ultrasonography, serum renal function assessment, voiding cystourethrography and urodynamics. Results: Urological involvement was present in 27 of the 39 children (69%). Megacystis in 26 children (96.3%) was isolated in 12 (46.2%) and associated with ureterohydronephrosis in 14 (53.8%), including 4 with vesicoureteral reflux. One patient presented with a complex urinary anomaly. Prenatal diagnosis of megacystis was made in 16 patients (59%), including 4 with neonatal urinary retention and 12 with urinary tract infection. At followup 24 patients had 1 or greater febrile urinary tract infections while 3 had afebrile infections. In the 14 patients with megacystis and ureterohydronephrosis, 3 or greater febrile urinary tract infections developed while 10 patients with isolated megacystis had only 1 febrile urinary tract infection. Twelve patients underwent 17 urodynamic studies, all of whom initially presented with hypocontractile detrusor, increased age corrected capacity (194% 44%) and compliance (42 ml./cm. H20, range 13 to 214). Bladder proprioception was also diminished. Presently voiding is achieved by abdominal pressure in 14 casesand intermittent catheterization in 10. Antibioprophylaxis was given to 17 patients, at one point in time. Renal failure (1 iatrogenic, 1 complex anomaly) developed in 2 patients. Conclusions: The presence of megacystis on prenatal ultrasound should alert the physician to the possibility of chronic intestinal pseudo-obstruction. Bladder adynamia represents the dominant urological complication. Urological evaluation and management are required to ensure adequate bladder emptying to reduce the risk of urinary tract infection and preserve long-term renal function.