[Urethral duplication: long-term results for a rare urethral anomaly]. / Urethralduplikatur : Langzeitergebnisse einer seltenen Urethralfehlbildung.

INTRODUCTION: Urethral duplication is a rare anomaly that can occur in various forms. Urethral duplication must be diagnosed and adequately treated. Only if clinical complaints develop should surgical management be considered. PATIENTS AND METHODS: The aim of this study was to retrospectively analyze patients with congenital urethral duplication: age at the time of diagnosis, type of urethral duplication, diagnostic and therapeutic measures as well as clinical aftercare were evaluated. RESULTS: This homogeneous and selected cohort included nine male children. The average follow-up was 6.2 years. Seven children were symptomatic (77.7%). We found a double urethra with epispadias in four boys and with hypospadias in three boys and urethral duplication with perineal/anal meatus in one patient and normotopia in another patient. In two children without symptoms, the urethral duplication was an incidental finding during diagnostic work-up for other anomalies. Seven children had additional urological anomalies: one fusiform megalourethra, three urethral valves with hypospadias, and two epispadias. Two children without symptoms were managed conservatively. An open operation joining the urethras was performed in three children, and four boys underwent endoscopic treatment of the urethral duplication. Long-term results revealed normal continence in all of the children. One child exhibited recurrent urinary tract infections and late stricture, which was opened endoscopically in a second session. CONCLUSION: A surgical approach is only indicated in the presence of clinical complaints. There is no surgical gold standard. The decision is ultimately reached on an individual basis depending on the findings and anatomy in view of the rarity and variability of the clinical picture. The functional result is of foremost importance for the urethra that requires reconstruction, but the cosmetic aspect should be respected to the same extent. We present the results of our experience in treating children with this urological condition. At the same time larger (multicenter) studies are needed, which will make it possible to formulate guidelines for this rare anomaly.

Variety of congenital urethral lesions in boys with lower urinary tract symptoms and the results of endoscopic treatment.

OBJECTIVES: We described various types of congenital urethral anomalies seen in boys with LUTS such as refractory enuresis. Their urethrograpic and endoscopic finding were reviewed and the effect of trans-urethral incision (TUI) was analyzed. PATIENTS AND METHODS: We evaluated 67 boys with lower urinary tract symptoms (LUTS, mean: 9 years old), in a period of three and a half years. A voiding cystourethrogram (VCUG) was performed in 37 patients and if we suspected a urethral abnormality, endoscopy was performed. Congenital urethral obstruction was diagnosed from VCUG and endoscopic findings and classified into Types 1, 3 and 4 posterior urethral valves (PUV) according to Douglas Stephens' description. Trans-urethral incision (TUI) was carried out for congenital urethral obstruction and the effect was judged three months later. RESULTS: On VCUG, 17 patients (45.8%) had an abnormal urethral configuration. On endoscopy, nine patients (24.3%) were diagnosed as having PUV. The effect of TUI on PUV excluding Type 3 was 80%, while that on Type 3 was 25%. DISCUSSIONS: The incidence of PUV compared to bulbar urethral narrowing was significantly different from that described in previous Japanese reports, but similar to other countries. The reason is thought to be the lack of standardized interpretations of VCUG images and endoscopic findings, resulting in the overestimation of the bulbar urethral lesion. CONCLUSION: The incidence of PUV in Japanese boys with LUTS was higher than had ever been described. The improvement rate by TUI was high in PUV excluding Type 3, but low in Type 3. The ring like strictures at the bulbar urethra may be less important than has previously been thought.

Mild oliguria in preterm infants who later developed periventricular leukomalacia.

The aim of this study is to determine whether or not renal involvement was present during the early neonatal period in preterm infants with PVL. We conducted a case-control study. The following items were evaluated; urine output, serum levels of sodium (Na), potassium (K), chloride (Cl), urea nitrogen (UN), and creatinine (Cr). The factors that could influence the urine output were also compared between the PVL and the control group. The mean urine output during the first 24h in the PVL group was 19.8ml/kg/day, and was significantly lower than in the control group (28.8ml/kg/day, p<0.05). The mean UN and Cr were not significantly different between the two groups. The minimal serum Na and Cl levels in the PVL group were significantly lower (128.3 and 94.3mEq/l) than those in the control group (134.8 and 100.7mEq/l, p<0.01 each). The maximal serum K level was significantly higher in the PVL group (6.47mEq/l) as compared to the control group (5.57mEq/l, p<0.05). There were no differences in any postnatal variables between the two groups. The preterm infants who later developed PVL had mild but significant oliguria during the first 24h of life. This suggests that preterm infants with PVL will have renal involvement immediately after birth.

The practice of urethral dilation for voiding dysfunction among fellows of the Section on Urology of the American Academy of Pediatrics.

PURPOSE: Review of the literature reveals little scientific evidence to prove or disprove the efficacy of urethral dilation for children with dysfunctional voiding and recurrent urinary tract infections. We ascertain the current practice of urethral dilation among pediatric urologists. MATERIALS AND METHODS: An anonymous questionnaire regarding the practice of urethral dilation was mailed to all fellows in the Section on Urology of the American Academy of Pediatrics. The questionnaire contained 14 questions and included biographical information. RESULTS: The questionnaire response rate was 64%. When asked how one would treat a typical female child with classic voiding dysfunction, 87% responded with timed voiding and relaxation techniques with or without anticholinergics. Only 2.5% would proceed directly to cytoscopy and urethral dilation. For patients who did not respond to initial treatment, 67% would proceed with either urodynamics or biofeedback and only 10% would then perform urethral dilation. Of responders 61% do not practice urethral dilation under any circumstance, 36% will use it when all other means of therapy have failed, while only 2% use urethral dilation regularly and 2% find it helpful and will occasionally use it. Among physicians who perform urethral dilation 63% believed that less than half of the patients experienced long-term improvement. Overall, there was no consensus as to mechanism of action of urethral dilation. CONCLUSIONS: Urethral dilation is rarely used as primary treatment for pediatric voiding dysfunction, is usually reserved until all other treatments have failed and appears to have long-term efficacy in less than 50% of patients. To define better the need for this treatment modality and its efficacy, a randomized prospective trial is mandatory.

Congenital urethrocutaneous fistula.

BACKGROUND: A 3-year-old boy visited our hospital for aberrance of urination. He had a fistula on his ventral penile shaft. Our diagnosis was congenital urethrocutaneous fistula. METHODS/RESULTS: We performed one-stage repair transverse preputial onlay island flap urethroplasty. Postoperatively, the patient was voiding comfortably with no recurrence of fistula. CONCLUSIONS: Congenital urethrocutaneous fistula is rare. Eighteen cases of congenital urethrocutaneous fistula have been reported previously. We consider the etiology of congenital urethrocutaneous fistula as a deficiency of the urethral plate and fusion of urethral folds.

The nonneurogenic neurogenic bladder of early infancy.

PURPOSE: The nonneurogenic neurogenic bladder has traditionally been believed to represent a disorder of older children. We report on patients in whom congenital dysfunctional voiding may be the underlying cause of nonneurogenic neurogenic bladder in early infancy. MATERIALS AND METHODS: We evaluated 5 male and 2 female patients newborn to 30 months old with dysfunctional neurogenic bladder and no evidence of neurological pathology or anatomical outflow obstruction. Presentation included prenatal hydronephrosis in 3 cases, urosepsis in 2 and failure to thrive in 2. Radiological and urodynamic investigations revealed thick walled, poorly compliant bladders in 5 patients and incomplete bladder emptying bordering on urinary retention in 2. There was significant upper tract pathology in all cases, including azotemia in 4, reflux in 4 and hydroureteronephrosis in 6. Due to poor bladder function, manifested by incomplete emptying or high storage pressures, all patients were initially treated with cutaneous vesicostomy. RESULTS: Bladder appearance and function subsequently improved in 3 patients and vesicostomy was reversed. Three patients with persistently thickened bladders, including 2 with renal failure, underwent bladder augmentation. The remaining patient had improved bladder storage function but requires intermittent catheterization. CONCLUSIONS: The nonneurogenic neurogenic bladder represents a rare, severe form of dysfunctional voiding that may be present even in the neonatal period.

Uropathies diagnosed in the neonatal period: symptomatology and course.

Forty infants with a uropathy diagnosed during the first two months of life were studied. Presenting signs were urinary tract infection in one-half of the cases, disorders of micturition, pelvic or abdominal wall malformations, abdomino-pelvic mass, and macroscopic hematuria. Obstructive uropathy was observed in 17 children and vesicoureteric reflux in 29. We noted a high incidence of extrarenal malformations (14 of the 40 cases) in this series of uropathies diagnosed during the neonatal period. Despite early diagnosis, the course was not favorable in 11 cases with congenital anomalies of the renal parenchyma.