Age and factors associated with self-clean intermittent catheterization in patients with spina bifida.

PURPOSE: The purpose of this study is twofold: 1) to determine the age when a child with spina bifida (SB) will most likely transition from caregiver clean intermittent catheterization (CIC) to self-CIC, and 2) to identify factors associated with self-CIC in children older than that age. METHODS: This is a retrospective, single-institution cohort study of individuals with SB. Data were collected prospectively as part of the National Spina Bifida Patient Registry. For Aim 1, we identified all individuals who perform self-CIC and who had a documented transition from caregiver-CIC. We then determined the age of transition to self-CIC. For Aim 2, we compared individuals over age 10 years (age cutoff determined by Aim 1) who use self-CIC to those who use caregiver-CIC to determine what variables were associated with self-CIC. RESULTS: From our SB population, 206 individuals used self-CIC. Of these, 64 patients had documented ages of transition from caregiver- to self-CIC. 46 (71.9%) and 56 (87.5%) patients had transitioned to self-CIC by 10 and 14 years, respectively. For Aim 2, we used age 10 as a cutoff, based on the findings from Aim 1, and found that 287/696 patients were ⩾ 10 years and using CIC. Factors independently associated with lower likelihood of self-CIC were thoracic spinal lesions (odds ratio (OR) 0.45) and Medicaid insurance (OR 0.24). CONCLUSIONS: The ages at self-CIC transition vary, although most patients transition by age 10. Thoracic-level spinal lesions and Medicaid insurance are associated with lower odds of self-CIC.

Isolated intraspinal germinoma in a child with congenital adrenal hyperplasia.

STUDY DESIGN: Case report. OBJECTIVE: To report an intramedullary germinoma associated with congenital adrenal hyperplasia (CAH). METHOD: We report a 14-year-old white male with CAH who was found to have an intramedullary germinoma of the conus medullaris. Presentation and evaluation were for progressive urinary incontinence. A brief review of relevant literature was presented. RESULTS: Cases where germinomas have occurred simultaneously with CAH have been identified intracranially, but to our knowledge, this is the first case of CAH and intramedullary spinal cord germinoma occurring simultaneously. CONCLUSION: Although rare, primary intramedullary germinomas do occur. The association between CAH and spinal cord germinoma should be considered by clinicians.

Synthesis and biological evaluation of tropane-like 1-[2-[bis(4-fluorophenyl)methoxy]ethyl]-4-(3-phenylpropyl)piperazine (GBR 12909) analogues.

We have prepared azabicyclo[3.2.1] derivatives (C-3-substituted tropanes) that bind with high affinity to the dopamine transporter and inhibit dopamine reuptake. Within the series, 3-[2-[bis-(4-fluorophenyl)methoxy]ethylidene]-8-methyl-8-azabicyclo[3.2.1]octane (8) was found to have the highest affinity and selectivity for the dopamine transporter. These azabicyclo[3.2.1] (bridged piperidine) series of compounds differ from the well-known benztropines by a 2-carbon spacer between C-3 and a diarylmethoxy moiety. Interestingly, these new compounds demonstrated a much lower affinity for the muscarinic-1 site, at least a 100-fold decrease compared to benztropine. Replacing N-methyl with N-phenylpropyl in two of the compounds resulted in a 3-10-fold increase in binding affinity for the dopamine transporter. However, those compounds lost selectivity for the dopamine transporter over the serotonin transporter. Replacement of the ether oxygen in the diarylmethoxy moiety with a nitrogen atom gave relatively inactive amines, indicating the important role which is played by the ether oxygen in transporter binding. Reduction of the C-3 double bond in 8 gave 3 alpha-substituted tropanes, as shown by X-ray crystallographic analyses of 11, 12, and 19. The 3 alpha-substituted tropanes had lower affinity and less selectivity than the comparable unsaturated ligands.

Accuracy of renal-bladder ultrasonography as a screening method to suggest posterior urethral valves.

PURPOSE: We determine the usefulness of ultrasonography as a screening modality to support the diagnosis of posterior urethral valves. MATERIALS AND METHODS: A retrospective chart analysis was performed of 93 consecutive boys with newly diagnosed posterior urethral valves from August 1985 through February 1999. Of the 93 boys 65, including 62 who underwent renal and bladder ultrasonography, had preoperative renal sonography available and constitute our study population. Patient age range at presentation was newborn to 13 years, with 33 of the 65 boys presenting as infants (age 1 year or younger). Presentation included fetal hydronephrosis in 13 cases, urinary tract infection in 18, voiding dysfunction in 9, abdominal mass in 6, renal insufficiency in 5, failure to thrive in 4, hematuria in 1 and other in 9. RESULTS: Preoperative sonography identified bilateral hydronephrosis in 48 boys (74%), unilateral hydronephrosis in 9 (14%) and no hydronephrosis in 8 (12%). Bladder sonography revealed an abnormal bladder (thickened, trabeculated and/or significantly distended) in 54 boys (87%). An abnormal bladder was seen in 43 of the 46 boys with bilateral hydronephrosis, 6 of 8 boys with unilateral hydronephrosis and 5 of 8 with normal upper tracts. Only 3 of the 62 boys (5%) had a normal upper tract and bladder. The sensitivity of renal ultrasonography in suspecting posterior urethral valves was 88%, while sensitivity of renal-bladder ultrasonography was 95%. When stratifying for age younger than 4 years and 4 or older, the sensitivity of predicting posterior urethral valves with renal-bladder ultrasonography was 87% and 98%, respectively. CONCLUSIONS: While voiding cystourethrography remains the imaging gold standard for the diagnosis of posterior urethral valves, renal-bladder ultrasonography is an appropriate screening study for a boy suspected to have the condition. Renal-bladder ultrasonography may prove particularly useful in the evaluation of the boy with significant voiding dysfunction before deciding on a more invasive study, such as voiding cystourethrography.

Urological outcome of patients with cervical and upper thoracic myelomeningocele.

PURPOSE: We determined the incidence of voiding disorders and upper tract deterioration in children with cervical and upper thoracic level myelomeningocele. MATERIALS AND METHODS: We retrospectively reviewed the neurosurgical and urological presentation, evaluation and treatment of 11 patients of whom 8 had cervical and 3 had upper thoracic myelomeningocele. There were 7 female and 4 male patients 10 months to 39 years old (mean age 12 years). All patients were ambulatory and 5 of 11 (45%) had hydrocephalus. Although a voiding history was obtained by a neurosurgeon in all patients, only 9 had undergone a formal urological evaluation, including video urodynamic assessment in 5. RESULTS: No patient had an increased post-void residual and none had ever been placed on intermittent catheterization. Tethered spinal cords from caudal fixation (thickened filum terminale and a lipomyelomeningocele) were present in 2 patients with urinary incontinence of whom 1 had recurrent cystitis. Imaging studies of the upper tracts were normal in all 9 patients and video urodynamics were normal in 3 of 5. No patient has required urological operative intervention. CONCLUSIONS: To our knowledge, this study represents the largest reported series of patients with cervical and upper thoracic myelomeningocele who have undergone urological evaluation. Our experience suggests that patients with cervical and upper thoracic myelomeningocele have a low risk of voiding disorders and upper tract deterioration unless other conditions, such as a congenital tethered cord, are present. Additional reports of children with cervical and upper thoracic myelomeningocele are necessary to confirm these findings.

Pediatric inflammatory bladder tumors: myofibroblastic and eosinophilic subtypes.

PURPOSE: Benign bladder tumors are rare in children. A number of descriptive terms have previously been used to describe inflammatory tumors with myofibroblastic proliferation or eosinophilic infiltration. We present our experience with these tumors and review the literature. MATERIALS AND METHODS: We retrospectively reviewed the records of all children presenting with a benign focal bladder mass during the last 5 years, including 2 girls and 3 boys 2 to 12 years old (mean age 7). RESULTS: Presenting symptomatology included irritative voiding symptoms, suprapubic pain and hematuria. All bladder masses were diagnosed by ultrasonography and all patients underwent transurethral resection. Three children who had an inflammatory bladder tumor with myofibroblastic features required open tumor excision with preservation of the bladder. In the 2 remaining children urine culture was positive preoperatively and the diagnosis was an inflammatory bladder tumor with eosinophilic infiltration. Transurethral resection of the mass was performed without partial cystectomy. CONCLUSIONS: Inflammatory myofibroblastic and eosinophilic tumors represent forms of focal cystitis with a tumefactive component. Differentiation from the malignant bladder neoplasms of childhood has important consequences for therapy. Management of these benign but aggressive inflammatory lesions involves local control by endoscopic or open surgery with bladder preservation. We encourage refined use of the terminology for inflammatory myofibroblastic and eosinophilic tumors.

Tunica vaginalis onlay urethroplasty as a salvage repair.

PURPOSE: We review our experience with tunica vaginalis onlay urethroplasty for urethral repair after multiple previous hypospadias repairs. MATERIALS AND METHODS: From January 1992 through December 1997 we repaired the urethra in 10 boys and 1 man using a tunica vaginalis onlay flap. The tunica vaginalis was placed as a patch on the urethra that was proximal to the glans or brought out to the urethral meatus. RESULTS: No intraoperative complications or difficulties were associated with harvesting the tunica vaginalis. Of the 4 children and 1 adult in whom the onlay flap was brought out to the urethral meatus meatal stenosis developed in 3 (60%), while 2 void without problems. In 6 children the onlay flap was placed proximal to the glans and in 1 who was lost to followup 2 discontinuous patches were placed. In 3 of the 5 remaining boys (60%) stricture developed, while 2 void without problems. CONCLUSIONS: Tunica vaginalis onlay urethroplasty results in a substantial complication rate. We did not confirm any advantage of its use.

Renal transplantation in children with posterior urethral valves revisited: a 10-year followup.

PURPOSE: Renal transplantation is safe and effective for end stage renal disease in children with posterior urethral valves. We previously reported our 5-year post-transplantation results in boys with posterior urethral valves and matched controls. Graft survival was similar. However, we were concerned about elevated serum creatinine and the potential detrimental effects of hostile bladder dynamics in these children. We performed this study to determine whether our concern would be substantiated. MATERIALS AND METHODS: We retrospectively analyzed the clinical records and computerized transplantation database in 268 boys younger than 19 years who underwent renal transplantation from May 1968 through November 1988. The 18 children with posterior urethral valves were compared to a nonobstructed cohort of 18 boys in regard to age, number of transplants, donor type and immunosuppression. All children had at least 10 years of followup (range 10 to 19). RESULTS: The 10-year post-transplant patient survival in the posterior urethral valves and control groups was 94 and 100%, while 10-year graft survival was 54 and 41%, respectively. There was no statistically significant difference in graft survival when comparing immunosuppression type, donor source and pre-transplant proximal urinary diversion. The 10-year mean serum creatinine was 2.3 and 2.0 mg./dl. in the posterior urethral valve and control groups, respectively (not statistically significant). CONCLUSIONS: Our renal transplantation results in children with posterior urethral valves are comparable to those in children with nonobstructive end stage renal disease. The 10-year graft survival was better but not statistically significant in the posterior urethral valve group, while serum creatinine was similar. Our concern regarding renal transplantation in children with posterior urethral valves was not substantiated.

Outcome and cost analysis of operative versus nonoperative management of neonatal multicystic dysplastic kidneys.

PURPOSE: We determine whether nephrectomy or observation is the more appropriate treatment of neonates with multicystic dysplastic kidney in terms of medical care and cost-effectiveness. MATERIALS AND METHODS: We retrospectively reviewed our 10-year clinical experience with 17 female and 32 male neonates presenting with multicystic dysplastic kidney who were followed with serial renal ultrasound. The literature also was reviewed. RESULTS: Nephrectomy was performed in 12 patients (24%) for various reasons, of which family request at concomitant surgery was the most common (7). No kidney was removed due to hypertension or tumor. In the remaining 37 patients followup continues (mean 42 months) with involution developing in 9 multicystic dysplastic kidneys (24%) and 9 patients (24%) lost to followup. The total cost of 1-hour outpatient simple nephrectomy was estimated at $5,000 to $7,000 and, when performed as a concomitant procedure, it cost $2,000 to $5,000, the equivalent charges incurred for 17 to 28 serial ultrasound studies performed by a radiologist. Our review of the literature revealed that children with multicystic dysplastic kidney are at minimal risk for hypertension, pain and infection. The most important reason to perform screening renal ultrasound in this condition is to detect earlier stage Wilms tumor (3 to 10-fold the general pediatric population risk of 1/10,000 cases). With a maximum risk of 0.1% for Wilms tumor controversy exists as to whether any screening program is necessary. When screening is instituted, options include monthly parental abdominal palpation versus serial renal ultrasound. Because Wilms tumor has a rapid growth rate, when screening renal ultrasound is instituted, it must be performed no less than every 3 months until age 8 years (total of 32 studies) to screen effectively for early stage tumors. CONCLUSIONS: The risks associated with multicystic dysplastic kidney are slight. Early nephrectomy is more cost-effective than observation in neonates with multicystic dysplastic kidney only when observation involves screening with ultrasonography every 3 months until age 8 years. Extensive parental counseling should be provided on the slight but definite risks of this condition.

Hematuria. An integrated medical and surgical approach.

The need to perform a detailed work-up of microscopic hematuria is based on the following set of questions: Does the history or physical examination findings suggest systemic or renal disease? Is the patient able to acidify and concentrate urine? Is proteinuria present? Do other family members have hematuria or other renal problems? Does the microscopic analysis show casts, crystals, or WBCs? Are the RBCs eumorphic or dysmorphic? Using this scheme of questions, most children do not require laboratory tests or radiographic studies. In the case of gross or macroscopic hematuria, the initial evaluation may require only a urine culture, urine calcium-to-creatinine ratio, and renal and bladder sonography or a very detailed evaluation for renal parenchymal disease, stones, tumors, or anatomic abnormalities. In these instances, consultation with a pediatric nephrologist, urologist, or both is necessary.

Critical analysis of the clinical presentation of acute scrotum: a 9-year experience at a single institution.

PURPOSE: We assessed the significance of the clinical presentation of boys who underwent surgical exploration for acute scrotum. MATERIALS AND METHODS: We retrospectively analyzed the records of 115 consecutive boys who underwent surgical exploration for acute scrotum between October 1986 and January 1996. We divided the children into group 1-83 with spermatic cord torsion a mean of 14.4 years old, group 2-27 with torsion of a testicular appendage a mean of 9.4 years old and group 3-5 with epididymo-orchitis a mean of 14.1 years old. Particular attention was given to nausea and vomiting, patient age and duration of pain. RESULTS: Nausea and vomiting occurred in 69 and 60% of the boys in group 1, 8 and 4% in group 2 and none in group 3. Nausea and vomiting had positive predictive values of 96 and 98%, respectively, for spermatic cord torsion. Only 6 of the 83 boys (7%) with spermatic cord torsion were younger than 11 years, whereas 15 of the 27 (56%) with torsion of a testicular appendage were younger than 11 years. Of the 83 boys with spermatic cord torsion the testes were salvaged in 51 (61%) and the duration of pain was 40 minutes to 12 hours (mean 4 hours). The testes were not salvaged in any patient with greater than 12 hours of pain. CONCLUSIONS: We believe that any boy 11 years old or older with scrotal pain less than 12 hours in duration that is associated with nausea or vomiting should be considered to have torsion of the spermatic cord. In this day of cost-effective medical management it is not necessary to perform imaging in this subset of boys before surgical exploration.

Two-stage Fowler-Stephens orchiopexy with laparoscopic clipping of the spermatic vessels.

PURPOSE: We report the outcome of a staged approach to the intra-abdominal testicle and provide baseline data on operative time, postoperative course and testicular survival. MATERIALS AND METHODS: We retrospectively reviewed the records of 441 boys (547 undescended testes). There were 105 boys (24%) with 124 nonpalpable testes (23%). All patients underwent laparoscopy. Ligation of spermatic vessels was performed as stage 1 on 20 abdominal testes (4%) in 18 boys (4%). Stage 2 orchiopexy was done using an open technique. RESULTS: Two-stage orchiopexy in 18 boys included a bilateral procedure in 2, of which 1 was asynchronous and 1 was synchronous. Average operative time was 55 minutes for stage 1 and 67 minutes for stage 2. Stage 1 and 2 procedures were performed on an outpatient basis in 18 and 17 (94%) boys, respectively. There were no complications after stage 1 and 1 wound infection developed after stage 2. One testis with no vas deferens was determined to be nonviable at stage 2. The remaining 19 testes (95%) were considered viable at a followup of 6 months or greater. Viability was based on testicular size and consistency similar to those of the contralateral testis. CONCLUSIONS: Laparoscopic ligation of spermatic vessels as a stage 1 procedure is a natural extension of laparoscopy. A staged approach provides adequate viability of the intra-abdominal testis.

Contralateral vesicoureteral reflux after simple and tapered unilateral ureteroneocystostomy revisited.

PURPOSE: We reviewed our experience with contralateral vesicoureteral reflux following unilateral ureteroneocystostomy. MATERIALS AND METHODS: We retrospectively identified 88 children who underwent unilateral ureteroneocystostomy from 1986 through 1995, including standard repair in 69 and tapered repair in 19. Cystoscopy was performed in all cases immediately before repair. RESULTS: Grades IV to V vesicoureteral reflux was identified preoperatively in 34% of the patients, including 13 (19%) and 14 (74%) who underwent standard and tapered repair, respectively. Renal duplication was noted in 24% of the cases, including 18 standard (26%) and 3 tapered (16%) repairs. An abnormal contralateral nonrefluxing ureteral orifice was present in 8 of the 53 standard (15%) and 3 of the 14 (21%) tapered cases. Ipsilateral reflux was corrected in all children undergoing standard repair but it persisted in 4 (21%) in the tapered repair group. Postoperatively contralateral vesicoureteral reflux developed in 1 child (1.4%) in the standard and 1 (5.3%) in the tapered repair group. CONCLUSIONS: Contralateral vesicoureteral reflux is rare and does not appear to be influenced by preoperative reflux grade, a duplicated system or the endoscopic appearance of the ureteral orifice.

3'-Chloro-3 alpha-(diphenylmethoxy)tropane but not 4'-chloro-3 alpha-(diphenylmethoxy)tropane produces a cocaine-like behavioral profile.

A series of 2'- and 3'-substituted and 3',3"-disubstituted 3 alpha-(diphenylmethoxy)tropane analogs were designed and synthesized as novel probes for the dopamine transporter. All the analogs were evaluated for displacement of [3H]WIN 35,428 binding at the dopamine transporter and for inhibition of [3H]dopamine uptake in rat caudate putamen. Compounds were observed to monophasically displace [3H]WIN 35,428 binding to the dopamine transporter with affinities of 21.6-1836 nM (Ki). Generally, meta-substituted compounds were more potent than benztropine and equipotent to or slightly less potent than their previously reported para-substituted homologs in inhibiting [3H]WIN 35,428 binding. However, these same meta-substituted analogs were typically less potent than the 4'-substituted analogs in inhibiting [3H]dopamine uptake. Ortho-substituted analogs were generally less potent in both binding and inhibition of uptake at the dopamine transporter than either benztropine or other aryl-substituted homologs. The analogs were also tested for binding at norepinephrine and serotonin transporters as well as muscarinic m1 receptors. None of the compounds in the present study bound with high affinity to either the norepinephrine or serotonin transporters, but all bound to muscarinic m1 receptors with high affinity (K1 = 0.41-2.52 nM). Interestingly, 3'-chloro-3 alpha-(diphenylmethoxy)tropane (5c) produced effects like cocaine in animals trained to discriminate 10 mg/kg cocaine from saline, unlike its 4'-Cl homolog and all of the previously evaluated benztropine analogs. Further evaluation of compound 5c and the other benztropine analogs will undoubtedly prove useful in the elucidation of the role of the dopamine transporter in the reinforcing effects of cocaine and the ultimate identification of a cocaine-abuse treatment.