Urological results after fetal myelomeningocele repair in pre-MOMS trial patients at the Children's Hospital of Philadelphia.

INTRODUCTION: Myelomeningocele patients deal with multiple medical issues, including lower extremity neurological deficits, bowel and bladder incontinence and the sequelae of hydrocephalus secondary to a Chiari II malformation. In utero intervention holds the promise of reversing some of the sequelae and improving outcome. MATERIAL AND METHODS: Between 1998 and 2003 (preceding the formal Management of Myelomeningocele Study, MOMS), an initial group of 58 patients underwent in utero repair of their myelomeningocele between 21 and 25 weeks' gestation. Long-term (5-year) follow-up has occurred in this cohort of patients. Previous reports have documented decreased incidence of ventriculoperitoneal shunting and neuromotor functioning, showing improved outcomes compared with historical controls. RESULTS: Overall, 4 fetal deaths occurred, while the majority of patients returned for follow-up for up to 5 years after closure. Phone follow-up has also been conducted for those who could not return. To date, 10 patients (18.5%) have successfully toilet-trained, while 2 patients have bowel continence and 1 has bladder continence but requires enemas; 2 patients who successfully toilet-trained developed spinal dermoid cysts requiring surgical resection. DISCUSSION: Historically, in utero repair of myelomeningocele patients yields a greater percentage of patients who have achieved continence compared with those undergoing postnatal repair. The MOMS trial will compare contemporary urological outcomes of those patients undergoing either prenatal or postnatal repair in a randomized fashion. The results of this trial showed a decreased need for ventriculoperitoneal shunting in those patients who underwent in utero repair as well as an improvement in lower extremity function.

Conservative nonsurgical management of spina bifida.

The nonsurgical management of patients with spina bifida is predicated on maintaining a compliant bladder of adequate size or correcting detrusor sphincter dyssynergy that can lead to progressive bladder damage and ultimately upper tract changes. Pharmacologic management, targeted at the detrusor and/or external sphincter, can be done. Neuromodulation using transcutaneous approaches with interferential electrostimulation, sacral (S2-S3) via digital transcutaneous electrical nerve stimulation, and percutaneous tibial nerve stimulation all have shown varied successes.

Transient asynchronous testicular growth in adolescent males with a varicocele.

PURPOSE: We assessed the testicular growth of adolescent males followed nonsurgically for the presence of left varicocele. MATERIALS AND METHODS: We retrospectively reviewed the charts of adolescent males with a diagnosis of unilateral left varicocele and ultrasound testis volume measurements seen during a 10-year period. A total of 161 boys underwent at least 2 testicular ultrasounds as part of the evaluation for left varicocele. Patients were excluded from study for a history of inguinal/scrotal pathology or endocrinopathy that could affect testicular size. Sonographic testicular volume was calculated using the Lambert volume (length x width x height x 0.71). The resulting volumes were compared to previously published criteria for surgical repair (15%, 20% and 2 cc size differentials). RESULTS: Of the 71 boys with 3 followup ultrasounds 38 (54%) initially had a 15% or greater volume differential. After nonsurgical followup with ultrasounds for 2 years 60 boys (85%) had testicular volume differentials in the normal range (less than 15%). Of the patients 71% were spared potential surgery by size criteria and 50% were spared surgery by the same 15% volume differential criteria. CONCLUSIONS: Adolescent males with unilateral left varicocele often demonstrate asynchronous testicular growth that usually equalizes in time. Therefore, sonographic testicular size measurement at a single point during adolescence is insufficient to determine the need for varicocelectomy. When contemplating varicocelectomy we recommend at least 2, and preferably 3, testicular volume measurements 1 year apart to establish accurately decreased left testicular volume compared to a normal right testis.

Long-term outcomes in children treated by prenatal vesicoamniotic shunting for lower urinary tract obstruction.

OBJECTIVE: Limited information is available about long-term outcomes in children treated prenatally for lower urinary tract obstruction. Our aim was to evaluate outcomes in children treated in utero with vesicoamniotic shunts. METHODS: Clinical outcomes in 20 pregnancies with a singleton male fetus, oligo/anhydramnios, and lower urinary tract obstruction were studied using chart review and phone and written clinical questionnaire for parents, pediatricians, and urologists. RESULTS: Overall 1-year survival was 91%. Two neonatal deaths occurred from pulmonary hypoplasia. Mean gestational age at delivery was 34.6 weeks, mean days from shunting to delivery were 84.4, and mean birth weight was 2,574 g. Prenatal urinary prognosis was good in 13, borderline in 2, and poor in 3 of the survivors. Mean age at follow-up was 5.83 years. Posterior urethral valves were confirmed in 7 males, urethral atresia in 4, and prune belly syndrome in 7. Eight children had acceptable renal function, 4 had mild insufficiency, and 6 required dialysis and eventual renal transplant. Eleven children had normal bladder function with spontaneous voiding, 6 required catheterization, and 1 child still had a vesicostomy. Height and weight were below the 25th percentile in 9 children. Persistent respiratory problems were present in 8, musculoskeletal problems in 9, and frequent urinary tract infections were reported in 9. Health-related quality of life results in our group with lower urinary tract obstruction were similar to those in a healthy child population. CONCLUSION: Male children who underwent prenatal bladder shunting were neurodevelopmentally normal. Although one third of the surviving babies required dialysis and transplantation, the majority have acceptable renal and bladder function and report satisfactory quality of life. LEVEL OF EVIDENCE: III.

Bladder Function After Fetal Surgery for Myelomeningocele.

BACKGROUND: A substudy of the Management of Myelomeningocele Study evaluating urological outcomes was conducted. METHODS: Pregnant women diagnosed with fetal myelomeningocele were randomly assigned to either prenatal or standard postnatal surgical repair. The substudy included patients randomly assigned after April 18, 2005. The primary outcome was defined in their children as death or the need for clean intermittent catheterization (CIC) by 30 months of age characterized by prespecified criteria. Secondary outcomes included bladder and kidney abnormalities observed by urodynamics and renal/bladder ultrasound at 12 and 30 months, which were analyzed as repeated measures. RESULTS: Of the 115 women enrolled in the substudy, the primary outcome occurred in 52% of children in the prenatal surgery group and 66% in the postnatal surgery group (relative risk [RR]: 0.78; 95% confidence interval [CI]: 0.57-1.07). Actual rates of CIC use were 38% and 51% in the prenatal and postnatal surgery groups, respectively (RR: 0.74; 95% CI: 0.48-1.12). Prenatal surgery resulted in less trabeculation (RR: 0.39; 95% CI: 0.19-0.79) and fewer cases of open bladder neck on urodynamics (RR: 0.61; 95% CI: 0.40-0.92) after adjustment by child's gender and lesion level. The difference in trabeculation was confirmed by ultrasound. CONCLUSIONS: Prenatal surgery did not significantly reduce the need for CIC by 30 months of age but was associated with less bladder trabeculation and open bladder neck. The implications of these findings are unclear now, but support the need for long-term urologic follow-up of patients with myelomeningocele regardless of type of surgical repair.

Prenatal management of urogenital disorders.

Antenatal sonography has increased the detection of urogenital anomalies markedly, including conditions that lead to significant morbidity and mortality. Prenatal intervention is feasible to arrest and sometimes reverse the sequelae of bladder-outlet obstruction, but not necessarily renal damage. Myelomeningoceles, the most severe form of spina bifida,can be corrected in utero, with improvements in hydrocephalus and a decreased incidence of ventricular shunting postnatally. Medical therapy to prevent virilization associated with congenital adrenal hyperplasia has been successful, with improved ability to detect its presence prenatally. As techniques evolve to correct underlying disease processes,it becomes important to assess the therapies critically, particularly with long-term outcome data.

Initial management of complex urological disorders: bladder exstrophy.

Bladder exstrophy is a rare condition associated with lifelong issues including infection,renal insufficiency, incontinence, and fertility. Despite its seriousness, many affected children lead normal lives. With new operative techniques, children require fewer surgeries and have more hope for continence. With close follow-up, no child should develop renal insufficiency. Families should be reassured that a normal life and parenthood with normal sexual function are possible.

Neuropathic bladder in the neonate.

The management of infants born with myelomeningocele depends on understanding how their bladder stores and empties urine. Storage at low pressure with effective emptying periodically throughout the day is the goal. Intervention is designed to impact on one or both of these processes so that infants can remain infection-free and at the same time allow for appropriate renal growth over time. Urodynamic evaluation plays an important role, so that neonates can be stratified according to their risk. Most patients require intermittent catheterization and pharmacotherapy to achieve these goals at some point in their lives.

How does acute urolithiasis present after renal denervation?

PURPOSE: To present our experience with the acute presentation of urolithiasis after laparoscopic renal denervation and nephropexy for intractable renal pain in the pediatric patient. PATIENTS AND METHODS: Five patients aged 8 to 16 years (mean age 11.4 years) with a history of laparoscopic renal denervation presented with acute abdominal symptoms; obstructing urolithiasis was diagnosed. RESULTS: There were a total of 12 episodes of acute urolithiasis in this cohort. All five patients presented with periumbilical and epigastric discomfort when the stone was on the denervated side. During the episodes, of the five patients, three (60%) also presented with vomiting; four (80%) had at least one episode of gross hematuria; and five (100%) had microscopic hematuria during episodes on the denervated side. None of the patients presented with flank discomfort on the denervated side. CONCLUSIONS: Stone-related pain in patients undergoing laparoscopic renal denervation and nephropexy is not a typical presentation, and one needs to be wary of vague abdominal pain in this population.

Prenatal management of urogenital disorders.

Antenatal sonography has markedly increased the detection of urogenital anomalies, including those conditions that lead to significant morbidity and mortality. Prenatal intervention is feasible to arrest and sometimes reverse the sequelae of bladder outlet obstruction but not necessarily renal damage. Myelomeningoceles, the most severe form of spina bifida, can be corrected in utero, with improvements in hydrocephalus seen along with a decreased incidence of ventricular shunting postnatally. Medical therapy to prevent virilization associated with congenital adrenal hyperplasia has been successful, with improved ability to detect its presence prenatally now possible. As further techniques evolve to correct underlying disease processes, it becomes important to critically assess the therapies, particularly with long-term outcome data.

Quantifying demographic, urological and behavioral characteristics of children with lower urinary tract symptoms.

OBJECTIVE: This project examines the voiding and behavioral characteristics of children referred to a specialty voiding clinic, including the impact of incontinence on the child and family. PATIENTS AND METHODS: A total of 351 new patients (aged 5-17 years) referred to our specialty voiding clinic completed background information, including demographics and medical history, a standardized voiding questionnaire, school history, and questions about child and family quality of life, prior to their first appointment. RESULTS: Patients are primarily female (53%) and Caucasian (70%) with a mean age of 9.5 years (range 5-17; SD=3.5). Of the patients, 25% were diagnosed with a mental or behavioral health problem. Mean urological symptom score was 12 (range 0-29). Higher symptom scores are associated with younger age, ethnic minority status, a mental health diagnosis, being on psychotropic medications, and a poor child and family quality of life. Families of children who are wet day and night reported a poorer quality of life as compared to the families of children who were daytime wetters or bedwetters only. CONCLUSION: Symptom scores are associated with type of incontinence, social and quality of life variables. Collecting this baseline data will enable ongoing monitoring of progress for these complex patients.

Cryptorchid testis histopathology in myelomeningocele patients.

PURPOSE: Cryptorchidism occurs in 25% of boys with myelomeningocele (MMC) compared to 3% of the general population. Testicular biopsy histopathology correlates with future sperm counts. We studied testicular histology in boys with cryptorchidism and MMC to investigate if the MMC influences histological findings. MATERIALS AND METHODS: The study group consisted of six patients with MMC and undescended testis (UDT) who underwent orchiopexy and bilateral testis biopsy. Twelve testicular biopsies from six patients were compared to 40 biopsies from 20 UDT-only controls. Total germ cell count per tubule (TGC/T) and the percentage of adult dark spermatogonia (%Ad) in undescended and contralateral descended testes from the patients were compared with controls. RESULTS: In the study group, two had total absence of germ cells (TGC/T=0) and three had severely reduced germ cells (TGC/T<0.2). Four had total absence of Ad spermatogonia and the remaining two had severely reduced Ad spermatogonia (%Ad=5). The mean TGC/T and %Ad in patients with UDT and MMC were conspicuously lower than controls. The differences did not reach statistical significance (P=0.09-0.29). CONCLUSION: These results suggest that patients with both MMC and UDT have a more severe reduction in total number and more severely delayed maturation of germ cells than do patients with UDT alone. With only six patients in this study, there was not the power to detect statistical significance. In addition to the reproductive problems due to erection and ejaculatory dysfunction in patients with MMC, this severe testicular histopathology may increase the risk of subfertility.