Yuri Reinberg

Prognosis for Patients with Prenatally Diagnosed Posterior Urethral Valves

Children in whom posterior urethral valves are diagnosed shortly after birth are at higher risk for renal failure than children in whom posterior urethral valves are diagnosed later in life. The influence of prenatal diagnosis of posterior urethral valves on clinical outcome has not been established. We collected data on children with posterior urethral valves treated since birth at our hospital between 1975 and 1990. The clinical outcomes for 8 patients diagnosed prenatally and 15 diagnosed neonatally were compared. Of the 8 patients in the prenatal group 5 (64%) had renal failure compared to 5 of 15 (33%) in the neonatal group (p greater than 0.05). Nadir creatinine of more than 1.2 mg./dl. correlated with the development of renal failure in all patients in the neonatal and prenatal groups. There was 1 death in the prenatal group. In our experience prenatal diagnosis of posterior urethral valves has grave implications, including a 64% incidence of progressive renal failure and a 64% incidence of transient pulmonary failure. Oligohydramnios and postnatal pulmonary insufficiency are predictive of progressive renal failure. Earlier diagnosis and treatment of children with posterior urethral valves did not improve the clinical prognosis.

Seromuscular colocystoplasty lined with urothelium : experience with 16 patients

OBJECTIVES Report of initial clinical experience with seromuscular colocystoplasty lined with urothelium (SCLU), a new operation to increase bladder capacity and compliance without incorporating intestinal mucosa into the urinary tract. We have previously reported the technique and results of this procedure in a canine model. METHODS Sixteen patients underwent SCLU at two institutions. Their charts were reviewed retrospectively. The patients have been observed for a mean of 12 months. RESULTS Postoperatively, the bladder capacity increased an average of 2.4-fold from a mean of 139 mL (+/- 23.7 SEM) to 335 mL (+/- 38.9 SEM) in 14 patients (P < 0.001). Two patients required reaugmentation. Preoperative and postoperative bladder end filling pressures were available in 13 patients and decreased from a mean of 51.6 cm H2O (+/- 4.2 SEM) to 27.7 cm H2O (+/- 2.4 SEM) (P < 0.001). Of the 16 patients, 13 were incontinent preoperatively; 5 had renal insufficiency. Three patients have undergone renal transplantation following SCLU. After SCLU, 13 patients were dry day and night, 1 incontinent at night only, and 2 remained incontinent. Ten patients have undergone postoperative bladder biopsy. Seven demonstrate urothelium covering the augmented portion of the bladder, 2 have regrowth of colonic mucosa, and 1 shows a mixture of colonic mucosa and urothelium. CONCLUSIONS The results presented suggest that SCLU is a viable alternative to the more conventional forms of bladder augmentation in selected patients.

The Outcome of Renal Transplantation in Children with Posterior Urethral Valves

The effect of vesical dysfunction on the survival and function of renal transplants was evaluated by a retrospective study in which 18 children with posterior urethral valves and 18 children with vesicoureteral reflux were randomly matched with 36 children used as controls. There was no statistically significant difference in patient survival among the 3 groups. Five years after transplantation 50 per cent of the grafts in children with posterior urethral valves were functioning, while 73 and 75 per cent, respectively, of the grafts were functioning in children with vesicoureteral reflux and in the control group. Renal function during the 5 years was significantly better in children in the control group and in those with vesicoureteral reflux than in children with posterior urethral valves. We believe that the presence of an abnormal bladder may alter graft survival and adversely affect the function of the transplanted kidney.

Sacral Neuromodulation for the Dysfunctional Elimination Syndrome: A Single Center Experience With 20 Children

PURPOSE Recent advances in neuromodulation have demonstrated promise in treating children with the dysfunctional elimination syndrome refractory to medical management. Sacral nerve stimulation with the InterStim implantable device has been used in adults for management of chronic urinary complaints. However, there are few data regarding the usefulness of sacral nerve stimulation in children. We report our experience with sacral nerve stimulation for severe dysfunctional elimination syndrome. MATERIALS AND METHODS A total of 20 patients 8 to 17 years old with the dysfunctional elimination syndrome refractory to maximum medical treatment underwent sacral nerve stimulation at our institution. Patients were followed prospectively for a median of 27 months after the procedure. RESULTS Urinary incontinence, urgency and frequency, nocturnal enuresis and constipation were improved or resolved in 88% (14 of 16), 69% (9 of 13), 89% (8 of 9), 69% (11 of 16) and 71% (12 of 17) of the patients, respectively. Urinary retention requiring intermittent catheterization persisted in 75% of the patients (3 of 4) despite sacral nerve stimulation. Complications requiring operative treatment occurred in 20% of the patients (4 of 20). Following marked symptomatic improvement 2 devices were explanted at 20 and 19 months following placement, and both patients have remained symptom-free. CONCLUSIONS Sacral nerve stimulation is effective in the majority of our patients, and should be considered in children with severe dysfunctional elimination syndrome refractory to maximum medical treatment.

Early bladder outlet obstruction in fetal lambs induces renal dysplasia and the prune-belly syndrome

A model of posterior urethral valves in fetal lambs was developed in order to evaluate the effect of intrauterine urinary obstruction on the developing kidney. Complete urethral obstruction was induced in five fetal lambs at 43 to 45 days of gestation. Two control fetal lambs underwent sham operations. At full term (140 days), two of the five experimental lambs and both control lambs were available for postmortem examination. Results of gross and histological examination of the control lambs were normal. In contrast, the kidneys of the experimental lambs were markedly asymmetrical in size. Histological examination of the kidneys in experimental lambs showed cystic dilatation of the collecting ducts and occasional cystic dilatation of Bowmans spaces, features compatible with obstruction. Also noted were peripheral cortical cysts and primitive tubules lined with cuboidal epithelium and surrounded by fibromuscular collarettes, characteristic of renal dysplasia. One of the infant lambs had many characteristics of the prune-belly syndrome, including a wrinkled, markedly distended abdomen, deficient abdominal wall musculature, flared chest wall, limb deformities, and undescended testes. These results suggest that early in utero urethral obstruction (at the beginning of the second third of gestation) causes renal dysplasia. The results also support the hypothesis that the prune-belly syndrome results from abdominal distention that occurs early in gestation.

Influence of Intestinal Segment and Configuration on the Outcome of Augmentation Enterocystoplasty

The clinical and urodynamic results of enterocystoplasty were compared in 3 groups of patients in which the intact ileocecal segment (10 patients), tubular sigmoid segment (16) or sigmoid cup-patch segment (8) was used. In all 3 groups the upper urinary tract and renal function improved or remained stable, and the functional bladder capacity increased. All patients experienced involuntary, volume-dependent vesical contractions of comparable intensity 3 months postoperatively. After a mean followup of 19.4 +/- 8.9 months only 12 per cent of the patients with a cup-patch configuration demonstrated volume-dependent contractions, compared to all patients with ileocecal cystoplasty and 94 per cent with sigmoid tubular cystoplasty. The intensity of these contractions was significantly less in the cup-patch configuration group compared to the other groups. Patients with cup-patch cystoplasty achieved a significantly higher rate of urinary continence than those in the other 2 groups. It appears that the sigmoid cup-patch configuration may provide a lower pressure system and better continence compared to the intact ileocecal or tubular sigmoid segment, although the latter techniques have specific applications when confronted with a wide gap between the ureters and bladder or when dilated ureters require anastomosis to the bowel.

Perforation of the Gastric Segment of an Augmented Bladder Secondary to Peptic Ulcer Disease

Gastrocystoplasty consists of bladder augmentation using part of the gastric body. We describe a child in whom peptic ulcer disease developed in the gastric portion of the bladder, with subsequent perforation of the ulcer. Chronic renal failure, hypercalcemia and oliguria are potential ulcerogenic conditions commonly present in patients with gastrocystoplasty. Perforation of the ulcer is a potentially fatal complication. Periodic cystoscopy and prophylactic treatment with hydrogen blockers and alkalization of the urine should be considered in patients with those risk factors.

Therapeutic Efficacy Of Extended Release Oxybutynin Chloride, And Immediate Release And Long Acting Tolterodine Tartrate In Children With Diurnal Urinary Incontinence

PURPOSE We compare the tolerability and efficacy of extended release oxybutynin chloride, and immediate release and long acting tolterodine tartrate in children with nonneurogenic diurnal urinary incontinence and symptoms of overactive bladder. MATERIALS AND METHODS Children with a history of diurnal urinary incontinence were arbitrarily assigned to extended release oxybutynin, immediate release tolterodine or long acting tolterodine. The dose was titrated until effective (onset of complete diurnal urinary continence), maximal recommended dosage was achieved or bothersome anticholinergic side effects developed. An independent observer recorded the dose used, anticholinergic side effects and efficacy of therapy (incidence of urinary frequency, urgency, posturing and urinary incontinence). RESULTS The study included 86 girls and 46 boys. There were no statistically significant differences among the 3 treatment groups regarding the presence of peripheral or central nervous system anticholinergic side effects. Extended release oxybutynin and long acting tolterodine were significantly more effective at reducing daytime urinary incontinence than immediate release tolterodine (p <0.01 and 0 <0.05, respectively). Extended release oxybutynin was significantly more effective then long acting tolterodine for complete resolution of diurnal incontinence (p <0.05). CONCLUSIONS Extended release oxybutynin and long acting tolterodine are more effective than immediate release tolterodine in decreasing diurnal urinary incontinence. Extended release oxybutynin chloride is more effective than either immediate or long acting tolterodine for control of daytime urinary incontinence and urinary frequency.

Single Center Experience With Endoscopic Management of Vesicoureteral Reflux in Children

PURPOSE Dx/HA copolymer was approved by the Food and Drug Administration in 2001 for the treatment of VUR in children. Published results have varied widely, prompting us to report our single center experience with 300 consecutive patients. MATERIALS AND METHODS All patients undergoing Dx/HA injection (300 children, median age 5.4 years) at our institution were eligible for this study. We examined several parameters to determine which variables influence the success of Dx/HA injection, including preoperative reflux grade, bladder diverticula, neurogenic bladder, ureteral duplication, perioperative urinary tract infection, dysfunctional voiding, laterality of reflux and amount of Dx/HA injected. RESULTS A total of 225 patients (75%) underwent postoperative studies, and, thus, were eligible for study inclusion. Reflux was cured in 144 patients (64%) and improved in 44 (20%). A total of 20 patients had ureteral duplication anomalies. Cure rates in this subgroup were significantly decreased compared to nonduplicated cases (40% vs 66%). Injection in low grade (I to II) reflux was significantly more successful than in high grade (III to IV) reflux (72% vs 54%). A total of 10 patients (8.3%) with unilateral reflux had development of de novo contralateral reflux. Of the patients who failed initial injection 10 (4%) underwent repeat injection, of whom 5 (50%) were cured after the second injection. Two patients (0.9%) had development of temporary ureteral obstruction. CONCLUSIONS We report a large short-term single center experience with endoscopically injected Dx/HA. Although our cure rate of 64% was less than published rates for open surgery, the minimal morbidity and low complication rate of endoscopic treatment make it an attractive first line therapy for patients with VUR. Longer followup is required to better evaluate this technique.

Successful Treatment of Giggle Incontinence With Methylphenidate

AbstractPurpose: Giggle incontinence, the second most common type of childhood enuresis unrelated to disease, is notoriously difficult to treat. However, the association of laughter or emotion precipitated alteration of muscle tone is suggestive of a functional relationship to cataplexy, a part of the narcoleptic syndrome complex that may respond to stimulant medication.Materials and Methods: Two boys and 5 girls (mean age 10.9 years) with giggle incontinence, a positive family history of giggle incontinence (4 patients) and no evidence of urological disease were treated with methylphenidate for 1 to 5 years.Results: All patients responded positively with complete cessation of enuresis to varying dose schedules of methylphenidate.Conclusions: These results suggest that giggle incontinence is a centrally mediated and likely hereditary disorder that may share a common pathophysiological basis with the narcolepsy/cataplexy syndrome.