The urological manifestations of hollow visceral myopathy in children

Purpose: Hollow visceral myopathy is a rare clinical entity characterized by impaired intestinal function in the absence of mechanical occlusion. It can affect the smooth muscle of the whole or segments of the gastrointestinal tract and occasionally the urinary tract. We examined the urological manifestations of hollow visceral myopathy and management in the pediatric population. Materials and Methods: We reviewed the records of 14 male patients 1 day to 2 years old (mean age 4.6 months) and 10 female patients 1 day to 5 years old (mean age 9.4 months) at presentation to our institution with hollow visceral myopathy. In all patients genitourinary tract ultrasound, voiding cystourethrography and serum creatinine measurement were done at presentation. Results: All patients had gastrointestinal obstructive symptoms at presentation and 11 (46%) had urological symptoms, including urinary retention in 2, urinary tract infection in 3, and a prenatal diagnosis of megacystis and hydroureteronephrosis in 6. Overall 22 patients (92%) had urological abnormalities, all had poor bladder emptying and recurrent urinary tract infections, and 13 had megacystis associated with bilateral hydroureteronephrosis in 9 and unilateral hydroureteronephrosis in 2. There were 9 deaths from extensive gastrointestinal involvement and sepsis. Of the surviving 15 patients 13 have urological abnormalities, including 8 who perform and tolerate clean intermittent catheterization via the urethra and are well. Of the 4 male infants who did not tolerate clean intermittent catheterization appendicovesicostomy was done in 1, a Casale tube was placed in 1 and vesicostomy was performed in 2. The remaining female patient has day and night wetting. Conclusions: Urological abnormalities are common in hollow visceral myopathy and they can contribute to presenting symptoms. Clean intermittent catheterization via the urethra to aid in bladder emptying and decrease the frequency of urinary tract infections is the mainstay of treatment but surgery to construct an alternative catheterizable channel or vesicostomy may be required in intolerant patients.