Time to therapy and safety of testicular tissue cryopreservation in children undergoing gonadotoxic treatment or hematopoietic stem cell transplant.

BACKGROUND: With the use of multimodal treatments and hematopoietic stem cell transplant, the majority of children diagnosed with malignancies and hematologic diseases are now surviving into adulthood. Due to the gonadotoxic effects and potential for future infertility associated with many of these treatments, fertility counseling with sperm cryopreservation prior to starting therapy is the standard of care for post-pubertal males. Unfortunately, the options are limited for pre-pubertal patients or those unable to provide a specimen. Testicular tissue cryopreservation (TTC) is an investigational method to surgically obtain germ cells from testicular tissue and potentially restore future spermatogenesis. While TTC has been shown to be safe, little is reported on the time to treatment following the procedure to ensure adequate wound healing and avoid delays in definitive therapy. OBJECTIVES: The primary outcome was the time to initiation of treatment following TTC. Secondary outcomes were complication rates, delays in treatment due to TTC, and presence of germ cells. METHODS: We conducted a single-institution retrospective cohort study of patients undergoing TTC between 2017 and 2023. Patients at significant risk for treatment related infertility based on established criteria were eligible for TTC. Patients were excluded if they received their oncology or hematology care elsewhere. All patients were enrolled in an IRB approved research protocol with 75% of the tissue submitted for cryopreservation and 25% for research purposes. Time to therapy was defined as the first receipt of gonadotoxic treatment following TTC. RESULTS: A total of 122 patients (53 = malignant, 69 = non-malignant) underwent TTC with a median age of 5.9 years (IQR 2.3-9.35). Germ cells were identified in 115 (94%) specimens. A total of 109 (89%) patients underwent concomitant procedures. The median time to initiation of therapy was 5 (IQR 1.0-7.0) and 7 days (IQR 6.0-13.0) for malignant and non-malignant disease, respectively. The 30-day surgical complication rate was 2.5% and was similar between malignant vs non-malignant diagnoses (p = 0.58). All surgical complications were managed non-operatively. No patients had a delay in definitive treatment due to concern for wound healing or complications. DISCUSSION: Our surgical complication rates are similar to previous studies and are not affected by the time to treatment following TTC. Limitations of the study are its retrospective design, single institution, and short-term follow up. CONCLUSION: TTC can be performed safely, efficiently, and in conjunction with other necessary procedures without resulting in delays of definitive treatment. TTC affords the opportunity for fertility preservation in children who have no other options.

Distinguishing characteristics of pediatric patients with primary hyperoxaluria type 1 in PEDSnet.

BACKGROUND: Primary hyperoxaluria type 1 (PH1) is an autosomal recessive inborn error of metabolism that causes oxalate deposition, leading to recurrent calcium oxalate kidney stones, chronic kidney disease and systemic oxalosis, which produces a broad range of serious life-threatening complications. Patients with PH1 have delayed diagnosis due to the rarity of the disease and the overlap with early-onset kidney stone disease not due to primary hyperoxaluria. OBJECTIVE: The objective of this study was to determine the clinical features of individuals <21 years of age with PH1 that precede its diagnosis. We hypothesized that a parsimonious set of features could be identified that differentiate patients with PH1 from patients with non-primary hyperoxaluria-associated causes of early-onset kidney stone disease. STUDY DESIGN: We determined the association between clinical characteristics and PH1 diagnosis in a case-control study conducted between 2009 and 2021 in PEDSnet, a clinical research network of eight US pediatric health systems. Each patient with genetically confirmed PH1 was matched by sex and PEDSnet institution to up to 4 control patients with kidney stones without PH of any type. We obtained patient characteristics and diagnostic test results occurring before to less than 6 months after study entrance from a centralized database query and from manual chart review. Differences were examined using standardized differences and multivariable regression. RESULTS: The study sample included 37 patients with PH1 and 147 controls. Patients with PH1 were younger at diagnosis (median age of 3 vs 13.5 years); 75 % of children with PH1 were less than 8 years-old. Patients with PH1 were more likely to have combinations of nephrocalcinosis on ultrasound or CT (43 % vs 3 %), lower eGFR at diagnosis (median = 52 mL/min/1.73 m2 vs 114 mL/min/1.73 m2), and have normal mobility. Patients with PH1 had higher proportion of calcium oxalate monohydrate kidney stones than controls (median = 100 % vs 10 %). There were no differences in diagnosis of failure to thrive, stone size, or echocardiography results. CONCLUSIONS: Children with PH1 are characterized by presentation before adolescence, nephrocalcinosis, decreased eGFR at diagnosis, and calcium oxalate monohydrate stone composition. If externally validated, these characteristics could facilitate earlier diagnosis and treatment of children with PH1.

The real world experience of pediatric primary hyperoxaluria patients in the PEDSnet clinical research network.

The rarity of primary hyperoxaluria (PH) challenges our understanding of the disease. The purpose of our study was to describe the course of clinical care in a United States cohort of PH pediatric patients, highlighting health service utilization. We performed a retrospective cohort study of PH patients < 18 years old in the PEDSnet clinical research network from 2009 to 2021. Outcomes queried included diagnostic imaging and testing related to known organ involvement of PH, surgical and medical interventions specific to PH-related renal disease, and select PH-related hospital service utilization. Outcomes were evaluated relative to cohort entrance date (CED), defined as date of first PH-related diagnostic code. Thirty-three patients were identified: 23 with PH type 1; 4 with PH type 2; 6 with PH type 3. Median age at CED was 5.0 years (IQR 1.4, 9.3 years) with the majority being non-Hispanic white (73%) males (70%). Median follow-up between CED and most recent encounter was 5.1 years (IQR 1.2, 6.8). Nephrology and Urology were the most common specialties involved in care, with low utilization of other sub-specialties (12%-36%). Most patients (82%) had diagnostic imaging used to evaluate kidney stones; 11 (33%) had studies of extra-renal involvement. Stone surgery was performed in 15 (46%) patients. Four patients (12%) required dialysis, begun in all prior to CED; four patients required renal or renal/liver transplant.    Conclusion: In this large cohort of U.S. PH children, patients required heavy health care utilization with room for improvement in involving multi-disciplinary specialists. What is Known: • Primary hyperoxaluria (PH) is rare with significant implications on patient health. Typical involvement includes the kidneys; however, extra-renal manifestations occur. • Most large population studies describe clinical manifestations and involve registries. What is New: • We report the clinical journey, particularly related to diagnostic studies, interventions, multispecialty involvement, and hospital utilization, of a large cohort of PH pediatric patients in the PEDSnet clinical research network. • There are missed opportunities, particularly in that of specialty care, that could help in the diagnosis, treatment, and even prevention of known clinical manifestations.

Reoperative Anorectal Procedures in Patients with Anorectal Malformations - Is Bladder Function Affected?

BACKGROUND: Anorectal malformations (ARM) are associated with neurogenic bladder. The traditional surgical ARM repair is a posterior sagittal anorectoplasty (PSARP), which is believed to have a minimal effect on bladder dynamics. However, little is known about the effects of reoperative PSARP (rPSARP) on bladder function. We hypothesized that a high rate of bladder dysfunction existed in this cohort. METHODS: We performed a retrospective review of ARM patients undergoing rPSARP at a single institution from 2008 to 2015. Only patients with Urology follow-up were included in our analysis. Data was collected regarding original level of ARM, coexisting spinal anomalies and indications for reoperation. We assessed urodynamic variables and bladder management (voiding, CIC or diverted) before and after rPSARP. RESULTS: A total of 172 patients were identified, of which 85 met inclusion criteria with a median follow-up of 23.9 months (IQR, 5.9-43.8 months). Thirty-six patients had spinal cord anomalies. Indications for rPSARP included mislocation (n = 42), posterior urethral diverticulum (PUD; n = 16), stricture (n = 19) and rectal prolapse (n = 8). Within 1 year following rPSARP, 11 patients (12.9%) had a negative change in bladder management, defined as need for beginning intermittent catheterization or undergoing urinary diversion, which increased to 16 patients (18.8%) at last follow-up. Postoperative bladder management changed in rPSARP patients with mislocation (p < 0.0001) and stricture (p 0.005) but not for rectal prolapse (p 0.143). CONCLUSIONS: Patients who undergo rPSARP warrant especially close attention for bladder dysfunction as we observed a negative postoperative change in bladder management in 18.8% of our series. LEVEL OF EVIDENCE: Level IV.

Kidney function outcomes in patients after complete primary repair of bladder exstrophy and penopubic epispadias: Results from the international bladder exstrophy consortium.

INTRODUCTION: Historically, repair of bladder exstrophy (BE) is associated with compromise to the upper tracts; the single stage repair of BE was considered to exacerbate risks of kidney impairment. OBJECTIVE: We aim to evaluate the risk of upper urinary tract deterioration or chronic kidney disease after the complete primary repair of exstrophy (CPRE). STUDY DESIGN: As part of the U.S.-India Multi-institutional Bladder Exstrophy Collaboration, we prospectively performed data collection on all patients managed at the Civil Hospital, Ahmedabad from 2010 to 2020. All patients who underwent primary or redo BE or primary penopubic epispadias (PE) repair using CPRE were included. Data on annual VCUG and DMSA, serum creatinine and cystatin-C, urinary albumin, and creatinine were aggregated. RESULTS: 72/104 patients who underwent CPRE at a median age of 1.7 years (IQR: 1.1-4.6) were included: 43(60%) patients with primary BE, 17(24%) with redo BE, and 12(17%) with primary PE. At a median follow-up of 4 years (IQR: 3-6), the overall median eGFR was 105 for BE, and 128 ml/min for PE. 14(19%) patients had eGFR<90, and 22(31%) had microalbuminuria. 21(30%) patients had kidney scarring in DMSA and 31(44%) had VUR. Multivariate analysis showed that neither kidney scarring nor VUR could predict the presence of eGFR<90 or microalbuminuria. Of 72 patients, 2 (3%) patients had dry intervals >3 h, 9 (13%) patients have dry intervals of 1-3 h and 44 (61%) patients had dry intervals <1 h during follow-up. We found that kidney function outcomes (i.e., eGFR and microalbuminuria) were not associated with continence status (p = 0.3). DISCUSSION: In this series, we report a 5% incidence of CKD stage 2 or above that was not impacted by continence status. Furthermore, a 40% incidence of VUR and a 30% incidence of kidney scarring during follow-up was observed within this cohort, neither of which had a significant impact on renal function deterioration (i.e, decline in eGFR), but underscores the need for close kidney surveillance in children that have undergone bladder exstrophy repair. CONCLUSIONS: Modern CPRE technique for the repair of BE may increase the risk of kidney scarring in the intermediate-term follow-up, however, this finding does not correlate with low eGFR and presence of albuminuria inpatients. Therefore, close follow-up with serial kidney function measurements is warranted and necessary after CPRE.

International Pediatric Multidisciplinary Management Using Telemedicine to Promote Equitable Care.

Objective: To evaluate the use of telemedicine as a collaboration tool between a pediatrician and subspecialists looking to address challenges, such as the lack of health care specialists, which are present in the Dominican Republic. Study design: During this 6-year study, 65 patients were evaluated by a medical team consisting of a local pediatrician and 17 subspecialists from a leading academic medical center in the Unites States. Patient's age ranged from 2 months to 16 years of age (mean 8 years old). The most common reasons for referral were masses or malignancies, vascular malformations, urogenital anomalies, stuttering, and cochlear implant programming. Results: A total of 39 out of 65 cases (60%) carried an initial diagnosis. Of the 65 cases, a change in medical management occurred in 92.31% of cases (60 cases). There was no change in medical diagnosis or treatment in 5 of 65 cases (8%). Conclusion: This protocol exhibited high patient satisfaction with the technology and platform and direct patient savings from transportation costs. It also demonstrated the importance of thorough diagnosis in providing appropriate treatment and solutions. Telemedicine use in comparable practices should be studied further to aid in the development of policies for the diagnosis and management of chronic illnesses that require referrals to subspecialists.

Outcomes according to channel type for continent catheterizable channels in patients undergoing simultaneous urinary and fecal reconstruction.

INTRODUCTION: When creating a continent catheterizable channel (CCC) the choice of bowel segment used as the conduit should be tempered with the morbidity associated with it. The split-appendix technique allows the creation of both a urinary and fecal CCC without the need for a bowel anastomosis. However, there is concern that by splitting the appendix there is compromise to its blood supply and may affect outcomes. We aim to compare what affect the bowel segment used for urinary and fecal CCCs has on perioperative and long-term outcomes in patients undergoing simultaneous urinary and fecal reconstruction. METHODS: A retrospective review was performed analyzing all patients that underwent simultaneous continent catheterizable urinary and fecal CCC between the years 2010-2016. Patient demographics, channel characteristics, perioperative complications and clinical success rate were analyzed. RESULTS: A total of 106 patients were identified that had simultaneous fecal and urinary CCC created at time of reconstruction. For urinary CCC, there were 64 patients (60.4%) that underwent a split-appendix technique, 27 patients (25.4%) underwent a Monti, and 15 patients (14.2%) had the appendix used only for the urinary channel. Those patients undergoing a split-appendix technique had median operative time of 447 min compared to 619 min when a Monti channel was created. The median length of hospital stay was 9 days for the split-appendix technique compared to 12 and 13 day median hospital stay when the appendix was used only for the urinary channel or a Monti was created, respectively. There was no difference seen in revision free survival of the channel following surgery of any of the channels with median follow-up of 44.5 months. However, there were more subfascial revisions of urinary CCC in those that underwent a Monti (5 patients, 18.5%) compared to other bowel segments (0 patients). CONCLUSIONS: Utilizing a split-appendix approach for creation of urinary and fecal CCCs does not affect 30-day complications or long-term revision rates compared to other established techniques. This technique minimizes the potential surgical morbidity of a bowel anastomosis and provide shorter operative times, when feasible, at time of simultaneous creation of fecal and urinary CCC.

Does a Multidisciplinary Pediatric Stone Center Improve Outcomes?

INTRODUCTION: Urolithiasis is becoming more prevalent in children in the United States. A multidisciplinary pediatric stone center was initiated in 2014 to address this growing public health issue. The purpose of this manuscript is to assess the development of the stone center and its clinical outcomes. METHODS: A retrospective cohort study was performed to assess clinical outcomes. Inclusion criteria included patients younger than 21 years of age with more than 6 months of followup. Data abstracted from the medical record included patient demographics, medications, imaging, metabolic evaluations, surgical procedures, and emergency department visits. The number of surgical procedures, emergency department visits, and computerized tomography scans were compared before and after the first visit to the stone center. RESULTS: A total of 353 patients were evaluated in the stone center during the study period, 264 (98 male, 166 female) of whom met inclusion criteria. The mean age was 14.5 years, and the mean followup was 1.1 years. Of all patients 60% had a metabolic abnormality. Prior to the first visit 104 patients underwent surgery. Surgical procedures decreased from 39% to 17% during the year before and at any time after the first visit (p <0.0001). Emergency department visits per year decreased from 1.4 to 0.6 before and after the first visit (p <0.0001). Computerized tomography scan use decreased from 32% to 24% (p=0.3). CONCLUSIONS: A multidisciplinary stone center can be a feasible option to coordinate care and improve clinical outcomes. In our series the number of surgical procedures and emergency department visits decreased after enrollment.

A fast-growing, painless, solid scrotal mass.

The history and physical examination of an adolescent male are critical to early detection of scrotal abnormalities. Although rare, paratesticular rhabdomyosarcoma grows quickly and can be life-threatening. This article focuses on the importance of prompt recognition, diagnosis, and appropriate management of paratesticular rhabdomyosarcoma in an adolescent.

End stage renal disease and kidney transplant in patients with anorectal malformation: is there an alternative route?

BACKGROUND/PURPOSE: Mortality from end stage renal disease (ESRD) in patients with anorectal malformation (ARM) is reported to be between 2.5 and 6%. The risk differs depending on the type of ARM (6.4% "high" vs. 1.1% "low"). The purpose of this study was to review the characteristics of the ARM patients who received a kidney transplant (KT) to potentially identify if any modifiable factors existed that may have prevented ESRD. METHODS: The Colorectal Center and the kidney transplant databases at Cincinnati Children's Hospital were queried to identify patients with ARM and a KT. Data obtained included: gender, type of ARM, associated characteristics, urological status at birth, surgical and medical management, age at KT, and possible interventions that could have prevented or delayed the KT. RESULTS: 20 patients with ARM who underwent KT were identified. 16 were females, thirteen of whom had a cloaca; the average common channel length in these patients was 5 cm, ranging from 2 to 8 cm. Nine cloaca patients had hydrocolpos and 5 of them were not drained at birth. Eleven patients presented with renal failure at birth. Five patients with cloaca had common channel atresia/stenosis, 2 male patients had severe urethral atresia, 2 patients had absent bladders, and 1 patient had bilateral blind ureters. At birth, 6 patients had single functional kidneys, 4 had bilateral hydronephrosis with megaureters, 3 patients had bilateral dysplastic kidneys, 1 patient had a single kidney with hydronephrosis, and 1 patient had a single hypoplastic kidney. The average age at KT was 10.9 years (range 2-21 years.); in 3 patients, the KT was performed before the repair of the ARM. One patient died after the KT and one patient had rejection following the KT and is currently on hemodialysis. CONCLUSION: Patients with cloaca and recto-bladderneck fistula are the type of ARM with the highest risk for ESRD. Kidney failure at birth, common channel/urethral atresia, hydrocolpos, single kidney, and ectopic ureters represent high risk for future KT. Even when missed opportunities were identified, the impact of interventions to prevent or delay the onset of ESRD could not be proven.

Endoscopic treatment with dextranomer/hyaluronic acid for persistent incontinence after continent urinary reconstruction.

PURPOSE: To determine the applicability and long-term outcome of endoscopic injection of dextranomer/hyaluronic acid (Dx/HA) to correct incontinence in patients who had previously undergone continent urinary reconstruction. PATIENTS AND METHODS: A retrospective cohort study was performed of all patients who underwent Dx/HA injection at our institution from January 2001 to June 2011. All patients had adequate bladder capacity and compliance on maximized medical therapy before injection. "Success" was defined as either "continence" (daytime dry interval >3 hours) or "improvement" (daytime dry interval >2 hours). RESULTS: A total of 22 children (16 females and 6 males) had Dx/HA injections for persistent incontinence from either bladder neck (7), Mitrofanoff (10), or both (5). Median age at injection was 13 years (range 4-21). Children underwent an average of 1.6 injection sessions per patient with an average of 2.6 mL of Dx/HA per session. At a median follow-up of 72 months (range 4-104), 19 (86.4%) patients had successful results (16 continent, 3 improved). For those incontinent from bladder neck, 42% became continent after 1, 75% after 2, and 83% after 3 injections, with a success rate of 91% (10 continent, 1 improved). For those incontinent from Mitrofanoff, 20% became continent after 1, and 73% after 2 injections, with a success rate of 86% (11 continent, 2 improved). CONCLUSIONS: Endoscopic injection of Dx/HA to correct residual incontinence in selected children after urinary reconstruction appears to be safe and effective, achieving a dry interval in more than two-thirds of patients.

Unilateral ureteral reimplantation and management of contralateral low grade or resolved vesicoureteral reflux.

PURPOSE: New contralateral vesicoureteral reflux after unilateral ureteral reimplantation is well described in the literature. Management of high grade vesicoureteral reflux with resolved contralateral reflux is less extensively studied. Most surgeons perform a bilateral procedure in these cases. We report the results when an open procedure was deferred on the contralateral side. MATERIALS AND METHODS: A retrospective cohort study was performed of all patients with unilateral vesicoureteral reflux undergoing ureteral reimplantation performed by 1 surgeon between 2003 and 2012. Resolved or low grade contralateral reflux was observed if the kidney was normal. Dextranomer/hyaluronic acid copolymer injection was also offered for persistent grade I contralateral reflux. Outcomes were abstracted from the medical record and compared to those in patients undergoing bilateral ureteral reimplantation. RESULTS: Of 78 patients undergoing unilateral ureteral reimplantation 15 met inclusion criteria. Median age was 5.5 years, and median followup was 7.6 years. Extravesical detrusorrhaphy was performed in all cases. The 15 study patients initially had contralateral reflux that either resolved (8) or persisted as grade I (7). Six patients had results on 2 cystograms that were negative for contralateral reflux before ureteral reimplantation. Length of stay was 1 day less and costs were 59% lower for patients undergoing unilateral vs bilateral ureteral reimplantation. Postoperatively 2 of 15 patients (13%) had an afebrile urinary tract infection. All 15 patients had normal contralateral kidneys on postoperative ultrasound. CONCLUSIONS: Observation of contralateral resolved or low grade vesicoureteral reflux at unilateral ureteral reimplantation is feasible, with minimal morbidity and a shorter hospital stay compared to performance of bilateral ureteral reimplantation. This approach appears to be a reasonable option to discuss with parents during preoperative counseling.

Low incidence of urinary tract infections following renal transplantation in children with bladder augmentation.

PURPOSE: Children with end-stage renal disease and bladder dysfunction may require augmentation cystoplasty before kidney transplantation. Previous reports have suggested unacceptable urinary tract infection rates in these immunosuppressed patients. We reviewed our experience in this population. MATERIALS AND METHODS: We retrospectively studied patients undergoing augmentation cystoplasty and subsequent renal transplantation by a single surgeon between 1989 and 2007. This cohort was compared with a control group on clean intermittent catheterization who had undergone transplantation without augmentation. Patient demographics, etiology of renal failure, surgical details, surgical/allograft outcomes and occurrence of urinary tract infection were analyzed. RESULTS: The augmented group included 17 patients with a median age at reconstruction of 6.4 years. Stomach was used in 15 patients and colon in 2. Median time between reconstruction and transplantation was 1.2 years. Median followup after transplantation was 7.7 years. The control group included 17 patients with a median age at transplantation of 10.9 years. Median followup in the controls was 6.1 years. All ureteral reimplantations were antirefluxing. Patients on clean intermittent catheterization were maintained on oral antibiotic suppression and/or gentamicin bladder irrigations. In the augmented group 35 episodes of urinary tract infection were noted, and the number of documented infections per patient-year of followup was 0.22, compared to 32 episodes of urinary tract infection and 0.28 infections per patient-year of followup in the controls. No allograft was lost to infectious complications. CONCLUSIONS: In our series there was no increase in urinary tract infection rate following renal transplantation in patients with augmented bladders compared to controls. This finding may be due to the use of gastric augmentation, antirefluxing reimplantation and gentamicin irrigations.

Progression of renal insufficiency in children and adolescents with neuropathic bladder is not accelerated by lower urinary tract reconstruction.

PURPOSE: Children with chronic renal insufficiency and neuropathic bladder resistant to medical management may require lower urinary tract reconstruction before renal transplantation. A low pressure urinary reservoir optimizes the chance of graft survival and may slow native kidney death. We evaluated whether the renal deterioration rate is affected by augmentation cystoplasty. MATERIALS AND METHODS: We performed a retrospective cohort study in children who presented to our institution with chronic renal insufficiency and neuropathic bladders from 2005 to 2009. Chronic renal insufficiency was defined as a glomerular filtration rate of less than 60 ml per minute. As a surrogate for renal function change, we used the inverse creatinine trend with respect to time to determine the progression rate of renal insufficiency before and after augmentation. RESULTS: A total of 11 patients with a mean glomerular filtration rate of 34 ml per minute per 1.73 m(2), mean bladder capacity 168 ml and mean compliance 3.5 ml/cm H(2)O met study inclusion criteria. Bladder augmentation or replacement was done at a mean age of 9.7 years with a resultant mean capacity of 486 ml and compliance of 14.7 ml/cm H(2)O. Mean followup was 4 years before and 1.9 years after augmentation. There was no statistically significant difference between the preoperative and postoperative slopes of inverse creatinine in 8 of 11 patients (73%). Two of the 3 patients (18%) with different preoperative and postoperative slopes had improving renal function after surgery. There was no statistically significant difference in slopes across all patients. CONCLUSIONS: In our series bladder augmentation did not appear to hasten progression to end stage renal disease in patients with severe chronic renal insufficiency and neuropathic bladder.

Risk factors for urinary tract infection after dextranomer/hyaluronic acid endoscopic injection.

PURPOSE: Endoscopic injection of dextranomer/hyaluronic acid is an option for primary vesicoureteral reflux. Few groups have assessed the rate of urinary tract infection after dextranomer/hyaluronic acid injection. We reviewed our experience with dextranomer/hyaluronic acid injection, and determined the incidence of and risk factors for postoperative urinary tract infection. MATERIALS AND METHODS: A retrospective cohort study was performed of all children with primary vesicoureteral reflux treated with dextranomer/hyaluronic acid from 2002 to 2007 at a single institution. Patient demographics and clinical outcomes were abstracted from the medical record. Risk factors for postoperative urinary tract infection, including female gender, preoperative vesicoureteral reflux grade, recurrent urinary tract infection, bladder dysfunction, nephropathy and persistent vesicoureteral reflux after surgery, were analyzed in a multivariate logistic regression model. RESULTS: We treated 311 children, of whom 87% were female and 13% were male (464 renal units), during the study period. Mode of presentation was urinary tract infection in 85% of cases. Mean followup was 2.6 years. Postoperatively urinary tract infection developed in 40 patients (13%) and febrile urinary tract infection developed in 11 (3.5%). Of patients with urinary tract infection 26 had initially negative postoperative voiding cystourethrogram, of whom 16 underwent repeat voiding cystourethrogram and 9 showed recurrent vesicoureteral reflux. Five of these 9 patients had clinical pyelonephritis. Of assessed risk factors only preoperative recurrent urinary tract infection (OR 2.2, p = 0.03) and bladder dysfunction (OR 3.3, p = 0.001) were independent predictors of post-injection urinary tract infection. CONCLUSIONS: In our series urinary tract infection after dextranomer/hyaluronic acid injection was rare. Patients with recurrent urinary tract infections and bladder dysfunction preoperatively are at increased risk for urinary tract infection after treatment. Patients with febrile urinary tract infection after dextranomer/hyaluronic acid injection are at high risk for recurrent vesicoureteral reflux.

End cutaneous ureterostomy for the management of severe hydronephrosis.

PURPOSE: Although rarely indicated, surgical treatment of severe megaureter can pose a formidable technical challenge, especially in the small infant. We present our experience and outcomes with end cutaneous ureterostomy as a temporizing adjunct to future ureteral reimplantation. MATERIALS AND METHODS: We performed a retrospective cohort study of patients who underwent end cutaneous ureterostomy between 1993 and 2005. Patient demographics, surgical details and outcomes were recorded. RESULTS: A total of 29 patients (22 males, 7 females) underwent diversion of 34 renal units. Primary megaureter was diagnosed in 15 patients (17 renal units). Secondary megaureter was found in 10 patients (12 renal units). Postoperative megaureter was diagnosed in 4 patients (5 renal units). Mean patient age at time of diversion was 3.2 months for those with primary megaureter and 1.4 years overall. Bilateral diversion or diversion of a solitary functioning kidney was performed in 14 patients (48%), of whom 4 had renal insufficiency. Nine patients (31%) had a febrile urinary tract infection while awaiting undiversion, with no evidence of renal scarring on followup. Undiversion was performed in 12 patients (13 renal units) with primary megaureter at a mean age of 18 months. Overall, undiversion was performed in 21 patients (23 renal units), and ureteral tailoring was required in only 5 renal units (22%). Mean followup after undiversion was 4.2 years for primary megaureter and 3.9 years overall. CONCLUSIONS: End cutaneous ureterostomy is a safe and effective procedure to temporize massive hydronephrosis while awaiting definitive ureteral reimplantation.

Incontinence following bladder neck reconstruction--is there a role for endoscopic management?

PURPOSE: Little has been reported concerning the efficacy of endoscopic injection of dextranomer/hyaluronic acid for the treatment of residual incontinence following bladder neck reconstruction. We present the experience of 2 institutions using endoscopic submucosal injection of dextranomer/hyaluronic acid to correct incontinence in patients who had previously undergone bladder neck reconstruction with or without concomitant enterocystoplasty. MATERIALS AND METHODS: A retrospective chart review was performed with patient demographics, indications for treatment and outcomes recorded. All patients had adequate bladder capacity and compliance on maximized medical therapy before injection. Continence was defined as at least a 3-hour daytime dry interval, while improvement was defined as an increase in the daytime dry interval to at least 2 hours. RESULTS: A total of 14 patients (10 females and 4 males) underwent 21 injections. At a median followup of 17 months 10 patients had successful results (6 continent, 4 improved). CONCLUSIONS: Endoscopic injection of dextranomer/hyaluronic acid to correct incontinence following bladder neck reconstruction appears safe and can increase the daytime dry interval in more than 70% of carefully selected patients. Continued followup is necessary to evaluate the long-term effectiveness of this treatment.

The use of perioperative antibiotics in tonsillectomy: does it decrease morbidity?

OBJECTIVE: To assess the efficacy of perioperative antibiotics in decreasing post-operative morbidity among patients undergoing tonsillectomy or adenotonsillectomy. DESIGN: Meta-analysis based on a structured search of the literature, using MEDLINE and the Cochrane database. SUBJECTS: Only articles, which included both treatment and control groups, were included in the final analysis. Studies were limited to ones that involved human subjects, including both children and adults. Studies using steroids and topical antibiotics were excluded. OUTCOMES: Articles were abstracted for patient factors, elements of study design, methods of patient assignment to treatment and control groups, and clinical outcomes. The primary outcome, time required for return to normal oral intake, was analyzed in the final meta-analysis. METHODS: Four hundred and twenty-eight articles were initially identified. There were 23 potentially appropriate articles. Of these, 18 were able to be located in full text form and in English. Seven of these studies directly studied the efficacy of perioperative antibiotics (versus no antibiotics) in decreasing post-operative morbidity. Four studies had sufficient information to calculate effects estimates (xi) and standard deviations (Si) for the primary outcome. Three studies either did not report the outcome of interest or did not report a measure of stability (e.g. p-value or confidence interval). The data available from the first four studies were combined in a quantitative meta-analysis. Statistical analyses were performed using STATA for Windows software. RESULTS: The pooled estimate indicated that the antibiotic group returned to normal oral intake, on average, 1 day sooner than the controls. This difference was found to be statistically significant with a 95% confidence interval of 0.5-1.6 days. An additional assessment of three qualitative reports also suggested the use of perioperative antibiotics for adenotonsillectomy was associated with less post-operative pain. However, studies varied in terms of study quality, sample size, outcome examined, measure used and antibiotic administered. Definitive conclusions regarding the effect of perioperative antibiotics on other outcomes including bleeding, halitosis, fever, activity level and nausea and vomiting could not be drawn due to the small numbers of studies. CONCLUSIONS: In this meta-analysis, the use of perioperative antibiotics in patients who have had tonsillectomy or adenotonsillectomy appears to be associated with a 1-day reduction in the time required for return to normal oral intake. For other potentially important outcomes, such as post operative pain or bleeding, sufficient data were not available to make any definitive conclusions regarding the effect of perioperative antibiotics.

Perforation of Malone antegrade continence enema: diagnosis and management.

PURPOSE: Severe bowel dysfunction often accompanies neurogenic bladder, and Malone antegrade continence enema (MACE) procedures can lead to improvement in the quality of life of these patients. However, complications such as catheter false passage with subsequent intraperitoneal instillation of irrigation can lead to significant morbidity. We present our experience with the diagnosis and management of this condition. MATERIALS AND METHODS: The records of all patients undergoing MACE procedures at pediatric institutions from 1989 to 2002 were retrospectively reviewed. The records of patients diagnosed with a perforation were assessed for presentation, initial imaging studies, management and outcomes. RESULTS: Of 187 consecutive patients treated with MACE procedures we identified 6 females and 1 male (3.7%). Mean patient age at initial surgery was 11.3 years. Of the 7 patients presented within 3 months of the initial surgery, 6 presented with abdominal pain after irrigation and 4 reported traumatic catheterization. Six patients had extravasation of contrast material on imaging studies. Two patients presented with peritonitis and underwent immediate laparotomy. In 5 patients endoscopy was performed with catheter placement which was then maintained for 6 weeks. After a mean followup of 4.7 years 4 patients have complete continence, 2 have a MACE button in place and 1 has mild fecal leakage. CONCLUSIONS: MACE procedures have a low incidence of conduit false passage and perforation. Prompt diagnosis and early intervention are crucial to management. Endoscopic evaluation with catheter placement can be helpful in preserving continence and decreasing morbidity.

Ureteroscopy is safe and effective in prepubertal children.

PURPOSE: We present our experience performing ureteroscopy in prepubertal children. MATERIALS AND METHODS: We performed a retrospective cohort study of children 12 years or younger who underwent ureteroscopy at 2 institutions between 1993 and 2003. Patient demographics, indications for surgery, operative technique, surgical outcomes and complications were recorded. RESULTS: A total of 39 boys and 32 girls underwent 34 flexible and 47 rigid ureteroscopies. Retrograde access was obtained in 77 procedures, while 4 were performed via an antegrade approach. Mean age was 7.5 years (range 1 to 12) and mean followup was 2.2 years (0.5 to 10). Endoscopic lithotripsy was performed in 65 cases, with a success rate of 98%. A ureteral stent was placed postoperatively in 55 patients. Endoscopic incision was performed for a ureteral stricture in 3 patients and for ureteropelvic junction obstruction in 6. An obstructing fibroepithelial polyp was found in 2 patients, 1 underwent removal of a foreign body and 4 did not have intraluminal pathology. Dilation of the ureteral orifice was required in 23 cases (30%). There were no intraoperative ureteral injuries. One patient had development of a ureteral stricture (complication rate 1.3%) that was successfully treated endoscopically. CONCLUSIONS: Rigid and flexible ureteroscopy can be performed safely and effectively in prepubertal children. Successful outcomes can be obtained for calculi that are similar to those in the adult population. Ureteroscopic treatment can be effective in selected children with intraluminal obstruction. To our knowledge this is the largest series in the literature to document outcomes of ureteroscopy in this age group.