Clinical outcomes after increasing bladder outlet resistance without augmentation cystoplasty in neurogenic bladder.

INTRODUCTION: Patients with neurogenic bladder (NGB) and urinary incontinence (UI) due to low bladder outlet resistance may require bladder neck procedures (BNPs) to achieve continence. These patients may also have reduced bladder capacity and or elevated detrusor storage pressures that require augmentation cystoplasty (AC). AC is not without complications that include risks for bladder rupture, urolithiasis, urinary tract infections and metabolic issues. Avoidance of AC would be helpful in patients with neurogenic urinary incontinence that have safe bladder parameters in the setting of low bladder outlet resistance. OBJECTIVE: To determine if pre-operative urodynamics could select children with NGBs and UI for isolated BNPs without AC. Additionally we sought to determine the safety of BNPs without AC and future need of AC with long-term follow-up. STUDY DESIGN: This is an IRB-approved retrospective analysis of all patients undergoing BNPs for management of neurogenic UI over a 17-year period. We separated these BNP patients into two groups: No AC + BNP (Group 1) vs. AC + BNP (Group 2). Our primary analyses focused on postoperative outcomes for patients in Group 1. Outcomes assessed included additional surgical procedures, urodynamic changes, development of CKD, new hydronephrosis (HDN) and vesicoureteral reflux (VUR). Secondary analysis included the timeline for the development of any bladder deterioration that necessitated AC in Group 1. RESULTS: 93 patients underwent BNP at a mean age of 10.8 years. Thirty did not have AC at the time of surgery (Group 1). These children had larger (p < 0.001) and more compliant (p < 0.001) bladders than Group 2 having simultaneous augmentation. At 6 years mean follow-up in Group 1 patients, three developed new reflux and three had new hydronephrosis. Nine (30%) had additional continence procedures. Twelve required (40%) AC at a mean of 23 months after the initial BNP. No patients had AC after 5 years. Detrusor end filling pressure increased 14.8 cm H2O (p = 0.028) and expected bladder capacity decreased 26.1% (p = 0.005) after isolated BNP. DISCUSSION: We found that from our cohort of patients who had normal bladder compliance and normal/near normal expected capacity preoperatively 40% required subsequent AC. We were unable to find pre-operative clinical parameters which predicted failure or conversion to AC. We found that 43.3% of our BNP without AC patients had no subsequent invasive procedures with mean 6-year follow-up. We found that none of our patients developed any degree of CKD. Finally, we found that the majority of patients that converted to AC after their BNP did so within the first 2 years after their initial BNP and no patients required augmentation 5 years post their initial BNP. This data validates that these patients require very strict follow up, particularly in the first 5 years after surgery. CONCLUSIONS: BNP without AC is safe in only a few selected patients with NGB. Despite preoperative selection, there are significant changes in bladder dynamics and 40% required subsequent augmentation. Bladder deterioration occurs early and generally in the first 2 years. Since there are no apparent reliable pre-operative variables predicting the need for subsequent AC, parents should be counseled regarding vigilant post-operative follow-up.

Prevalence of upper urinary tract anomalies in hospitalized premature infants with urinary tract infection.

OBJECTIVE: The 2011 American Academy of Pediatrics (AAP) guidelines address imaging after initial febrile urinary tract infection (UTI) in infants >2 months of age. We sought to determine the frequency of upper urinary tract anomalies (hydronephrosis and vesicoureteral reflux (VUR)) in hospitalized premature infants with UTI. STUDY DESIGN: We retrospectively reviewed the electronic medical records of neonatal intensive care unit (NICU) admissions at a tertiary care children's hospital between 1 January 2006 and 31 December 2010. We queried the records for UTI, renal ultrasound (US) and voiding cystourethrogram (VCUG). RESULT: We identified 3518 unique admissions. UTI occurred in 118 infants (3%). Sixty-nine (60%) had a normal US. Renal dilation was predominantly renal pelvic dilation (12%) and isolated caliectasis (22%). VUR was identified in 15 (14%) infants evaluated with a VCUG. VUR was identified in nine (12%) infants without and in seven (16%) with an abnormality on US. Reflux was identified in 7% of male and 38% of female infants with a UTI. CONCLUSION: Anatomic abnormalities of the upper urinary tract are uncommon in premature infants with a UTI that occurs during neonatal hospitalization. In concordance with the AAP guidelines, a VCUG may not be required in all NICU infants under age 2 months after a single UTI.

Management of the neonatal ureterocele.

Ureteroceles are potentially complex anomalies that have historically been managed with open reconstructive operations. In the last decade, endoscopic management has been proposed as an initial approach in patients with ureteroceles. With antenatal diagnosis, intervention can be performed on an outpatient basis prior to the development of any infections. Recent peer-reviewed studies help to define the appropriate technique, the patient population best managed with incision, and long-term outcomes.

Congenital short colon with imperforate anus (pouch colon). Report of a case.

We report a case of a persistent cloaca and pseudoexstrophy associated with congenital pouch colon in a native-born American female child. This unusual anomaly occurs in two clinical settings. It has been reported in India as an isolated anomaly occurring primarily in males. Pouch colon also occurs in female patients with pseudoexstrophy or closed cloacal exstrophy. The typical anatomic features of this anomaly are discussed.

Delayed presentation of posterior urethral valves: a not so benign condition.

PURPOSE: Posterior urethral valves are usually detected during infancy by prenatal sonography. Rarely they may be diagnosed during later childhood, adolescence or even adulthood. Less is known about presentation and outcome in these older patients. We reviewed our experience at 4 institutions with the late presentation of posterior urethral valves. MATERIALS AND METHODS: A 13-year retrospective review revealed the late presentation of posterior urethral valves in 47 patients 5 to 35 years old (mean age 8). Data collected included presenting symptomatology, radiographic findings and renal function. Statistical analysis determined the relationships among presenting symptoms, patient age at diagnosis and renal function. RESULTS: The most common presenting symptoms were diurnal enuresis in 60% of the cases, urinary tract infection in 40% and voiding pain in 13%. Other presenting symptoms in less than 10% of the cases included poor stream, gross hematuria and proteinuria. At diagnosis hydronephrosis and vesicoureteral reflux were present in 40 and 33% of the patients, respectively, while serum creatinine was elevated in 35% and end stage renal disease had developed in 10%. The severity of presenting signs and symptoms was significantly associated with renal impairment, while patient age at diagnosis was not. CONCLUSIONS: Posterior urethral valves is not merely a disease of infancy. Voiding cystourethrography should be considered in boys older than 5 years who have voiding complaints, especially in association with diurnal enuresis or urinary tract infection. Patients who present late with posterior urethral valves are at risk for progression to end stage renal disease.

Circumcision and pediatric disorders of the penis.

Circumcision is a commonly performed procedure, but medical indications remain controversial. Most disorders of the penis in childhood can be diagnosed and managed by the primary care pediatrician. However, some require early recognition and surgical intervention, and prompt referral to a pediatric surgeon or urologist will optimize outcome. This article discusses the recognition and initial management of these problems, particularly those most commonly seen or followed in the outpatient setting. In addition, the authors review the issues of circumcision.

Cystic dysplasia of the rete testis: a benign congenital lesion associated with ipsilateral urological anomalies.

PURPOSE: Cystic dysplasia of the rete testis is a benign congenital lesion that can mimic testicular cancer. We report 6 cases, review the literature, discuss the embryological etiology and make management recommendations. MATERIALS AND METHODS: The records and pathology reports of 6 boys presenting with cystic dysplasia of the rete testis at 5 institutions were reviewed, as was the relevant literature. RESULTS: Of the 6 cases 5 presented as scrotal masses in previously healthy boys and 1 as an abdominal mass in a newborn with multiple congenital anomalies. One patient had been followed from birth for a multicystic dysplastic kidney and 4 were found to have an ipsilateral absent kidney during evaluation. Development of the contralateral side was normal in most cases. CONCLUSIONS: Cystic dysplasia of the rete testis is an unusual, benign congenital lesion that can mimic testicular cancer in presentation. The presence of ipsilateral renal anomalies, particularly renal agenesis, can suggest cystic dysplasia of the rete testis in the differential diagnosis preoperatively. Even if cystic dysplasia of the rete testis is suspected, we recommend inguinal exploration and early control of the spermatic cord in the event that neoplasia is identified. If possible, the goal of preserving as much normal testicular parenchyma as possible is desirable. Long-term followup for possible recurrence is recommended, particularly after local excision.

Prenatal intervention for hydronephrosis.

PURPOSE: The widespread use of prenatal ultrasound results in an increased recognition of fetal hydronephrosis and technological advances now make fetal intervention possible. However, efficacy is unknown, and there have been errors in diagnosis, and associated morbidity and mortality. This review focuses on the current status of prenatal diagnosis and management of hydronephrosis. MATERIALS AND METHODS: The relevant literature on prenatal physiology, prenatal diagnosis, experimental obstruction and clinical series of prenatal intervention was reviewed. RESULTS: Prenatal ultrasound is a poor discriminator of physiological hydronephrosis, obstruction, renal dysplasia and reflux. Persistent early onset oligohydramnios is the best predictor of poor neonatal outcome. New minimally invasive techniques may aid diagnostically but they may not improve outcome. Dysplasia is often present by the time hydronephrosis is detected and it is not reversible in experimental models. Prenatal intervention is technically feasible but the survival rate is only 47%, and catheter placement and open fetal surgery have significant fetal and maternal risks. Complications occur in up to 45% of fetuses. CONCLUSIONS: Prenatal intervention for hydronephrosis remains an experimental technique. The most important question is whether prenatal therapy for obstructive uropathy improves survival and decreases long-term morbidity and mortality in affected fetuses.

Pediatric endopyelotomy: the Washington University experience.

PURPOSE: Endopyelotomy has gained acceptance as minimally invasive therapy for ureteropelvic junction obstruction in adults. Its role in the treatment of pediatric ureteropelvic junction obstruction remains controversial. We report our experience with antegrade endopyelotomy for treating pediatric ureteropelvic junction obstruction. MATERIALS AND METHODS: A total of 17 patients 3 months to 17 years old underwent endopyelotomy as primary treatment for ureteropelvic junction obstruction (8) and after failed open pyeloplasty with secondary endopyelotomy performed a mean of 12 weeks after open pyeloplasty (9). Standard antegrade percutaneous techniques were used. Electrosurgical incision of the ureteropelvic junction at a posterolateral orientation was done in each case. Internal ureteral stents remained in place for 4 to 6 weeks postoperatively. RESULTS: In 5 of the 8 patients (62%) treated primarily the outcome was successful at a mean followup of 38 months (range 25 to 53). Failures occurred at 6 weeks, 3 months. In all 9 patients treated secondarily outcomes were successful at a mean followup of 59 months (range 16 to 110). CONCLUSIONS: Endopyelotomy as primary treatment of pediatric ureteropelvic junction obstruction remains controversial but it may be appropriate in select cases. On the other hand, endopyelotomy is safe and effective for pediatric patients in whom open pyeloplasty fails.

10-year experience with prenatal intervention for hydronephrosis.

PURPOSE: We evaluated clinical outcomes after attempted prenatal intervention in fetuses with hydronephrosis. MATERIALS AND METHODS: We retrospectively reviewed the histories of 10 fetuses considered for prenatal intervention between 1984 and 1993. One female and 8 male fetuses had bilateral hydroureteronephrosis and profound or progressive oligohydramnios, and 1 male fetus had massive progressive unilateral hydronephrosis. RESULTS: Shunt placement was not recommended and it was refused by the parents in 1 case each. Amniotic shunting was attempted in 8 fetuses between 22 and 28 weeks of gestation. Shunts were successfully placed with decreased hydronephrosis and increased amniotic fluid volume in 4 cases, while shunt placement was not technically possible in the remainder. Two shunts that retracted intra-abdominally at birth required laparotomy for retrieval. Postnatally all patients with shunts had compromised renal function. Of the 4 patients in whom attempts were unsuccessful 3 had mildly diminished renal function and 1 died of nonrenal causes (intraabdominal sepsis) on day 16 of life. No patient with a functioning shunt had postnatal pulmonary problems, whereas 3 without successful intervention had mild respiratory compromise. CONCLUSIONS: No definite advantage was noted in the small number of fetuses that underwent successful shunting. Successful shunt placement did not prevent renal insufficiency. The relief of oligohydraminos may benefit pulmonary function in some patients. The high technical failure and complication rates of in utero intervention should be considered before proceeding.

Buccal mucosal urethral replacement.

Graft substances, such as skin and bladder mucosa, have been previously used for urethral replacement when local epithelial tissue was not available. However, these substances have been associated with meatal prolapse, stricture and fistula formation. We have used buccal mucosa as a tissue for urethral substitution in these situations during the last 8 years. We review our clinical experience in 18 urethral reconstructions performed for urethral replacement in 4 cases of exstrophy/epispadias, 12 complex hypospadias repairs and 2 cases of complex bulbar urethral strictures. There have been 5 cases of meatal stenosis (2 requiring operative revision) but none of meatal eversion. There has also been 1 urethrocutaneous fistula and 1 mid graft stricture. Mean followup was 27 months and minimum followup was 6 months. Histological examination of the buccal mucosal graft compared to grafts of skin showed that the full thickness of the dermis or lamina propria is thinnest while the native vascular supply is greatest in the buccal mucosa. These 2 factors are associated with improved graft take and may explain the encouraging clinical results.

Characterization of a fibroblast cell from the urinary bladder wall.

For the first time we report on the growth, culture, and matrix production characteristics of a cell type isolated from the lamina propria of the urinary bladder wall. A fibroblastlike cell was identified as distinct from bladder detrusor smooth muscle cells and urothelium based on morphology, growth characteristics, and immunohistochemical staining. Characterization of extracellular matrix synthesis by this cell type using 35S-methionine metabolic labeling demonstrated that these cells are capable of secreting components of the surrounding connective tissue, including several fibrillar collagens, a basement membrane collagen, and fibronectin.

Developmental changes in normal fetal bovine whole bladder physiology.

In the present study, normal and experimentally altered whole fetal bovine bladders have been used in vitro to study developmental changes in compliance and capacity. For the first time, we have attempted to define the relative contributions of the detrusor and mucosal layers to bladder compliance. Fetal bovine bladder compliance increases with fetal development. Elimination of the active component of smooth muscle tension improves compliance and increases capacity more than 60% in younger fetuses but only 35% in older fetuses. Active smooth muscle tension as evaluated by whole bladder cystometry is highest in the youngest fetuses and decreases with fetal age. Surgical removal of the detrusor layer (smooth muscle and connective tissue) also increases compliance and capacity substantially in all fetal bladders. These observations show that both smooth muscle and connective tissue are important in the function of the developing fetal bladder. Changes in both of these bladder wall components probably occur during development and are responsible for the physiologic changes observed.

Comparative studies on the ontogeny and autonomic responses of the fetal calf bladder at different stages of development: involvement of nitric oxide on field stimulated relaxation.

This initial study correlates the passive length-tension relationship, contractile and relaxant responses to field stimulation and contractile responses to specific autonomic agonists and antagonists with gestational age. Fetal bovine bladders were separated into three groups based on the head-rump length (FL): 30 to 45 cm. (early gestation), 50 to 65 cm. (middle gestation) and 70 to 85 cm. (late gestation). Each bladder was separated into upper and lower bladder segments; longitudinal strips of smooth muscle were isolated and placed in individual muscle baths. Passive length-tension studies demonstrated that compliance was greatest in the bladder of late gestation and lowest in the bladder of early gestation period. Field stimulation (FS) elicited frequency-dependent contractile responses in all strips. In the upper bladder, the maximal response and maximal rate of tension generation to FS was lowest in the youngest fetuses and increased in proportion to the gestational age. In the lower bladders, there were no gestational age-related differences in the maximal response or maximal rate of tension generation in response to field stimulation. The maximal response of the upper bladder to bethanechol increased significantly from the youngest gestational age to mid-gestation, with no further changes between mid- and late gestation. The maximal response to field stimulation and bethanechol were equal between upper and lower bladder segments for the youngest gestational bladders, whereas for the oldest gestational ages, the maximal response of the upper bladder to FS and bethanechol were significantly greater than the responses of the lower bladder. In the presence of maximal precontraction with bethanechol, FS induced a rapid and marked decrease in tension. The magnitude of the relaxation was substantially greater for the strips of lower bladder than for the strips of upper bladder at late gestation. In lower bladders, the magnitude of the field stimulated relaxation was greater in the strips from the older fetuses than in the strips from younger fetuses. In all strips, field stimulated relaxations were completely inhibited by pretreatment with L-NAME (an inhibitor of nitric oxide synthesis), indicating that the FS-induced relaxation was due to nitric oxide. In addition to nitric oxide-induced relaxation, beta adrenergic stimulation also induced a significant relaxation of the isolated strips. In summary, these data suggest that, in the tubular shaped fetal bovine bladder, there were distinct differences in the autonomic responses between the upper bladder segment and the lower bladder segment in the late gestation period.