Ectopia of both moieties of ureteral duplication anomalies.

The upper pole ureter of a duplication anomaly can be orthotopic or associated with an ectopic insertion, a ureterocele, or even ureteropelvic junction obstruction. The lower pole ureter may terminate orthotopically or laterally on the trigone, resulting in varying grades of vesicoureteral reflux. It can also be associated with lower pole ureteropelvic junction obstruction. In this report, we describe 3 patients with ureteral duplication anomalies in whom both upper and lower ureters terminate ectopically outside the bladder. The mode of presentation, the diagnosis, and the management of this rare anomaly are described. In addition, a hypothesis is advanced to help understand the embryologic pathophysiology.

Incidence and natural history of contralateral vesicoureteral reflux in patients presenting with unilateral disease.

PURPOSE: The new onset of contralateral vesicoureteral reflux after the surgical correction of unilateral disease has been well documented and it is attributed to the surgical procedure. However, to our knowledge the spontaneous development of contralateral reflux in patients treated nonoperatively for unilateral disease has not been documented previously. We evaluated the incidence and natural history of contralateral reflux in children presenting with initially unilateral disease. MATERIALS AND METHODS: We reviewed the records of 130 girls and 13 boys with a mean age of 29.1 months presenting with primary unilateral reflux between 1983 and 1996. The grade of and time to new onset contralateral reflux were noted. Whereas the initial radiological diagnosis relied on contrast voiding cystourethrography, followup evaluation included contrast and radionuclide voiding cystourethrography. The upper tracts were assessed by ultrasound and/or excretory urography, and cortical renal scans were performed selectively. RESULTS: Patients were followed a mean of 32.2 months (range 4.6 to 147). Of the 143 patients 135 presented with a febrile urinary tract infection, and in 14 reflux was associated with a ureteral duplication anomaly. At presentation reflux was grade I or II in 110 patients, grade III in 26 and grade IV or V in 6. Followup voiding cystourethrography in the 143 patients revealed new onset contralateral vesicoureteral reflux in 47 (33%). Mean time to the appearance of new onset reflux was 17.6 months (range 0.6 to 91.8). Reflux was grades I to III in 22, 21 and 4 patients, respectively. Contralateral reflux resolved spontaneously in 19 cases (40%), persists in an additional 16 (34%) and was surgically corrected at ipsilateral ureteroneocystostomy in the remaining 12 (26%). Contralateral renal scarring was noted in 3 cases (6%). CONCLUSIONS: New onset contralateral vesicoureteral reflux developed in a third of the patients initially diagnosed with unilateral disease while they were followed nonoperatively. This finding suggests that contralateral reflux after the surgical correction of unilateral disease may not always be associated with the surgical procedure. It may be a manifestation of the natural history of unilateral reflux.

Spontaneous resolution of an in utero perirenal urinoma associated with posterior urethral valves.

Ultrasound imaging of a 26-week-gestation fetus demonstrated a large, nonemptying bladder. At 27 weeks, a distended, thick-walled bladder, left hydronephrosis, and a perirenal urinoma were present, without ascites. Observation was undertaken, as the amniotic fluid volume was normal. At 29 weeks, the left perirenal fluid collection persisted but, at 30 weeks, was absent. After delivery at 36 weeks, no ultrasound evidence for perirenal urinoma or ascites was present. Isotope renal scan showed preserved renal function bilaterally. This case illustrates that in utero urinomas associated with posterior urethral valves can resolve spontaneously, with preservation of renal function.

Initial experience with home therapeutic electrical stimulation for continence in the myelomeningocele population.

PURPOSE: In an attempt to enhance bladder and bowel continence 29 patients underwent long duration, low intensity transcutaneous therapeutic electrical stimulation. This technology has previously been shown to be effective for inducing regenerative hypertrophy of disuse atrophied skeletal muscle, and it has been associated with improved continence in the myelomeningocele population. Our objectives were to enhance urinary and fecal continence, and evaluate the mechanisms by which therapeutic electrical stimulation influences continence. MATERIALS AND METHODS: A total of 29 children with lumbar or sacral myelomeningocele underwent therapeutic electrical stimulation at home during sleep using a commercially available stimulator with a safety sensing circuit and 2 sets of electrodes placed on the skin at bedtime. Left and right side electrode placement was alternated on 6 of 7 consecutive nights. In 9 boys and 2 girls with more than 9 months of followup initial and subsequent cystometrography, urethral pressure profile, electromyography, voiding cystourethrography and renal ultrasound studies were evaluated. RESULTS: In the 11 children there was no radiographic evidence of urinary tract or musculoskeletal deterioration during treatment. Mean actual bladder capacity increased from 133 to 196 ml. (p < 0.05). Mean bladder capacity, as a percent of maximum predicted bladder capacity for a normal age matched child without myelomeningocele, also significantly increased from 59 before to 76% after 9 months of therapeutic electrical stimulation (p < 0.05). No significant change was noted in urethral pressure profile. A subjective improvement in the sensation of pelvic fullness was also observed. CONCLUSIONS: When administered at home by parents while the child sleeps, therapeutic electrical stimulation is safe for bladder and bowel continence in the myelomeningocele population. It seems to increase significantly bladder capacity, does not appear to change urethral pressure profile and results in a subjectively improved sensation of pelvic fullness, enhancing urinary and fecal continence. The most beneficial effect of therapeutic electrical stimulation seems to be on the bladder wall and less so on the striated pelvic floor musculature with subjective enhancement of pelvic fullness also contributing positively.

Ten years of experience with isolated urachal anomalies in children.

PURPOSE: The embryological and anatomical features of urachal anomalies have been well defined. Because of the variable clinical presentation, uniform guidelines for evaluation and treatment are lacking. In an attempt to establish a cost-effective approach leading to a favorable outcome, we reviewed the experience with urachal anomalies at a single institution in a 10-year period. MATERIALS AND METHODS: We reviewed the medical records and radiological studies of 12 boys and 9 girls newborn to 17 years old at presentation who were treated for a urachal anomaly. Diagnostic evaluation included voiding cystourethrography in 14 cases, ultrasound in 8, sinography in 7 and computerized tomography in 4. RESULTS: The 4 variants of urachal anomalies included a urachal sinus in 9 patients (43%), urachal cyst in 9 (43%), patent urachus in 2 (10%) and urachal diverticulum in 1 (4%). Treatment involved initial excision in 13 cases (61%) and secondary excision in 8 (39%). Staphylococcus aureus was the predominant organism recovered. CONCLUSIONS: Because of the variable presentation, the diagnosis of a urachal anomaly can be difficult. Our experience suggests that a cost-effective diagnostic approach can be developed based on the initial clinical evaluation. Whereas ultrasound is indicated when a urachal cyst or abscess is suspected on the initial study, sinography is the study of choice in the remainder of cases. A voiding cystourethrogram may not be required in view of the fact that none of the patients studied had an additional associated urinary tract anomaly.

Bilateral complete ureteral obstruction and renal insufficiency secondary to granulomatous disease.

Chronic granulomatous disease is a rare disorder of neutrophil function that impairs the ability of phagocytes to react to infection and produces inflammatory lesions that may involve many organ systems. A young man recently presented with bilateral mid ureteral obstruction and renal insufficiency secondary to inflammation due to chronic granulomatous disease. To our knowledge, we believe this to be the only case of complete bilateral ureteral obstruction due to chronic granulomatous disease reported to date. A review of the disease, its pathogenesis and potential involvement of the urinary tract is presented.