Male epispadias repair: Outcomes at three sites prior to the establishment of a multi-institutional collaboration.

INTRODUCTION: Epispadias, which occurs on the more mild end of the Bladder Exstrophy Epispadias Complex (BEEC) spectrum, presents still with a wide range of severity in boys, from mild glanular epispadias to penopubic epispadias with severe urethral and bladder neck defects. Surgical management ranges from isolated epispadias repair to epispadias repair with bladder neck reconstruction (BNR) with or without pelvic osteotomies. OBJECTIVES: We aimed to evaluate outcomes in epispadias treated at three institutions prior to formation of a formal collaboration. In addition, we sought to delineate outcomes based on anatomic severity at time of diagnosis, and initial procedure performed in cases of penopubic epispadias. METHODS: IRB approved databases were retrospectively queried at three institutions for patients who underwent repair of epispadias between 1/1993 and 1/2013. Degree of epispadias, age and technique at initial repair, and self-reported continence status at last follow-up were recorded. Continence was categorized as: wet, intermediate (dry 2-3 h), or dry, while also distinguishing those who void and those who require clean intermittent catheterization (CIC). Those not seen since 1/1/2015, younger than 10 years at last follow up, or in whom continence data were not recorded were excluded. RESULTS: A total of 48 boys were identified; 36 met inclusion criteria. The epispadias cohort consisted of 8 glanular epispadias (GE) (22%); 8 penile epispadias (PE) (22%), and 20 penopubic epispadias (PPE) (56%) with a median follow-up of 11.3 years (3.2-26.2 years). Overall, 33 of 36 (92%) boys void per urethra. Within the group that voids, 19/33 (58%) are completely dry, while 6/33 (18%) are wet. Among patients who underwent initial epispadias repair without concurrent or subsequent bladder neck reconstruction, continence rates were: GE 63% (5/8); PE 75% (6/8); PPE 71% (5/7). Among the 9 boys with PPE who underwent initial epispadias repair with concurrent BNR, 22% (2/9) were dry with no further surgeries. Overall, 8/20 (40%) of boys with PPE void with complete dryness. DISCUSSION: This multi-center retrospective review of continence in epispadias demonstrates that even some boys with glanular and penile epispadias can have challenges with continence, and boys with penopubic epispadias may remain wet despite careful preoperative assessment of bladder neck functionality and concurrent BNR. CONCLUSION: Continence outcomes in boys with all degrees of epispadias can be variable. Even boys with more distal defects may have significant bladder neck deficiency. And those with the most severe form of epispadias may require bladder neck reconstruction to achieve continence.

Comorbidity of Obsessive-Compulsive Symptomatology and Bowel and Bladder Dysfunction in a Tertiary Children's Hospital: A Case Series.

OBJECTIVE: To improve understanding of co-occurring obsessive-compulsive disorder (OCD) and bowel and bladder dysfunction (BBD) in an outpatient pediatric urology clinic setting. To provide a series of clinical care recommendations to enhance the identification and integrated medical and behavioral health treatment of these disorders. METHODS: Authors participated in a series of discussions to define the patient population and specific aims for the study. Efforts were made to select index patients of diverse background despite limited sample availability. Three patients were ultimately selected for the study. RESULTS: The case studies highlight the connection between urinary issues and underlying OCD symptoms. All patients were initially seen for medical treatment of urological symptoms, then referred for psychological evaluation, and were diagnosed with and received treatment for OCD. Similarities among the cases, including experience of phantom urinary incontinence, stressors present that contributed to symptoms, and obsessive fears of incontinence that led to compulsive behavior, were discussed. Differences between the cases, including patient age, sex, and compulsive behavior demonstrated (ie, urinary frequency, excessive wiping) were also reviewed. CONCLUSION: Access to a behavioral health provider can enhance the identification of comorbid psychological disorders and can reduce duration of symptoms through appropriate psychological treatment. Authors provide recommendations for screening measures and reinforce importance of including basic education on psychiatric diagnoses and impact on urological symptoms in urology and pediatric fellowship training programs. Future research exploring prevalence of comorbid psychiatric conditions and efficacy of treatment interventions in a pediatric urology population would be beneficial.

The urological management of children with spinal cord injury.

BACKGROUND: The urologic management of children with spinal cord injury (SCI) differs from that of the adult insofar as the care involves a developing organ system and will be ongoing for years. Preservation of renal function as well as prevention of urinary tract infection in concert with both bladder and bowel continence are the essential guiding principles. METHODS: This is a non-systematic review of the literature and represents expert opinion where data are non-existent. This review focuses on special considerations in children with spinal cord injuries. RESULTS: SCI in children is less frequent than in adults and affects mainly older children and teenagers. The etiology of SCI in children is usually motor vehicle accidents. The cervical spine is often injured. The urologic evaluation is similar to that for adults but may involve more frequent radiologic and urodynamic assessment to monitor renal function as the child grows. CONCLUSIONS: Treatment may be divided into medical vs. surgical and mirrors the approach to children who have a neurogenic bladder due to spina bifida. Bladder management should be associated with bowel management to achieve the goals of continence and social acceptability.

Pelvic floor laxity: A not so rare but unrecognized form of daytime urinary incontinence in peripubertal and adolescent girls.

INTRODUCTION: Stress urinary incontinence (SUI) is common among older multiparous females but rarely reported in active, young girls. OBJECTIVES: Our hypothesis is: physically active adolescent females develop pelvic floor laxity demonstrable on upright VCUG. Our objectives are to (1) increase awareness of SUI in young females, (2) test our hypothesis with an upright VCUG, and (3) report effectiveness of step-wise management. STUDY DESIGN: A retrospective review was performed of nulliparous girls with only SUI seen from 2000 to 2015, who were evaluated with upright voiding cystourethrography (VCUG) (bladder descent defined as ≥2 cm drop of bladder neck below pubic ramus at capacity). Data collection included level of physical activity, physical examination, BMI and Z-scores, urodynamics, management, and treatment response. Standard urotherapy (SUT) (timed voiding, proper diet, adequate fluids, bowel management) and biofeedback therapy (BFT) was initiated. Fisher exact test was used to calculate 'p' values. RESULTS: Thirty-three females (median age 15.1 years, range 5.5-20.3) were identified who underwent an upright VCUG; 20 had bladder neck descent (Fig.). Of these 20, 15 (75%) were involved in strenuous activity, whereas only three of 13 (23%) without descent engaged in intense athletics. No differences were noted in median BMI and Z-score with strenuous activity (21.1 (15.2-26.7) and 0.31 (-0.9-1.94)), respectively, versus patients without (21.3 (15.8-33.5) and 0.62 (-0.0-2.38)). Average follow-up for all was 16.6 months (range 0.4-102.2). Of 20 demonstrating bladder neck descent, three did not complete therapy and were lost to follow-up. Only six of these 17 became dry. Of the remaining 11, eight underwent surgery: Burch colposuspension (5), fascial sling (2), Coaptite to the bladder neck (1), and an artificial urinary sphincter (1). This latter girl had a failed Burch colposuspension 1 year previously. All surgical patients are dry. Of 13 without bladder descent on VCUG, five did not complete therapy and were lost to follow-up. The remaining eight were managed non-surgically; seven were fully dry at last follow-up. Overall, 13 of 25 (52%) achieved dryness. SUT and BFT were more effective in those without, than in those with bladder descent (87.5% vs. 35.3%, p = 0.0302, Fisher exact test). DISCUSSION AND CONCLUSIONS: Physically active, nulligravid girls with SUI can be efficaciously diagnosed on upright VCUG. They should be considered for non-surgical therapy but will likely require bladder neck elevating surgery. Non-surgical therapy works for those with minimal bladder descent on cystography.

The Megameatus, Intact Prepuce Variant of Hypospadias: Use of the Inframeatal Vascularized Flap for Surgical Correction.

INTRODUCTION: The megameatus intact prepuce (MIP) variant of hypospadias is a rare variant of hypospadias that is diagnosed either early at the time of circumcision or later as the foreskin is retracted. The true incidence of the anomaly is difficult to determine precisely as some patient never come to medical attention but is felt to under 5% of all cases of hypospadias. The purposes of this study are to review the embryology and clinical findings of MIP and then, in light of a personal experience, present a series of patients evaluated for MIP who were treated with a modification of the Mathieu technique. MATERIALS AND METHODS: A PubMed search of all articles in the MIP variant of hypospadias was carried out followed by an exhaustive review of the literature. The charts of all patients evaluated and treated at Boston Children's Hospital by MC between 2007 and 2017 were reviewed retrospectively. The patients were divided into two groups: those who underwent the standard procedure and those who underwent a repair using a modification of the Mathieu procedure using an inframeatal flap. RESULTS: The embryologic explanation of the MIP variant is not clear but failure of the distal, glanular portion of the urethra to tubularize results in spectrum of abnormality characterized by a deep glanular groove and an abnormal opening of the urethra anywhere from the mid-glans to a subcoronal location. Surgical repair is complicated by a wide distal urethra which may be injured if not properly identified. Overall good outcomes were noted with one patient experiencing a urethra cutaneous fistula in the first group and one patient having a mild glans dehiscence in the second. CONCLUSION: The MIP variant of hypospadias is a rare variant of hypospadias that presents as a spectrum of urethral anomaly. Surgical repair may not always be necessary but if surgical repair is carried out, the Mathieu technique modification may offer better anatomic delineation of the urethra and will provide an extra layer of tissue to cover the reconstructed urethra. Low complication rates should be expected with adequate functional outcome such as a normal urinary stream. In addition, criteria for selecting patients for surgical repair are provided.

The Efficacy of Congenital Penile Curvature Repair in Preadolescent Males: Early Outcomes.

OBJECTIVE: To characterize the spectrum of congenital penile curvature without hypospadias evaluated at our institution, and to assess the efficacy of surgical repair in prepubescent boys. METHODS: The study group included 82 boys with a diagnosis of congenital penile curvature without hypospadias who elected for surgical repair from 2008 to 2010. We retrospectively reviewed clinical and operative characteristics and recorded surgical outcomes during the length of patients' follow-up at our institution. RESULTS: Of the patients electing surgery, 32 of 82 (39%) underwent penile degloving alone for class I (skin) chordee. Ten of 82 patients (12.2%) required further excision of class II (dysgenic fascial) chordee, and 40 of 82 (48.8%) patients required some combination of techniques to correct varying degrees of class III (dysgenic fascial) chordee. A total of 22.2% of patients returning for follow-up with corporal disproportion had persistent penile curvature compared with 14.3% of boys with fascial chordee and 11.5% of boys with skin chordee. Those children treated with tunical plications and incisions alone fared worse than those children treated with some combination of the Nesbit or modified Nesbit procedure. CONCLUSION: Our study shows not only that the proportion of patients with a hyposplastic urethra may be smaller than previously reported but also that corporal disproportion may be more prevalent. Attention should be paid to this later group as plication without the use of Nesbit techniques may not be enough to ensure an appropriate repair.

Management of Proximal Hypospadias with 2-Stage Repair: 20-Year Experience.

PURPOSE: We describe our experience with 2-stage proximal hypospadias repair. We report outcomes, and patient and procedure characteristics associated with surgical complications. MATERIALS AND METHODS: We retrospectively studied patients with proximal hypospadias who underwent staged repair between January 1993 and December 2012. Demographics, preoperative management and operative technique were reviewed. Complications included glans dehiscence, fistula, meatal stenosis, nonmeatal stricture, urethrocele/diverticula and residual chordee. Cox proportional hazards model was used to evaluate the associations between time to surgery for complications and patient and procedure level factors. RESULTS: A total of 134 patients were included. Median patient age was 8.8 months at first stage surgery and 17.1 months at second stage surgery, and median time between surgeries was 8 months. Median followup was 3.8 years. Complications were seen in 71 patients (53%), with the most common being fistula (39 patients, 29.1%). Reoperation was performed in 66 patients (49%). Median time from urethroplasty to surgery for complication was 14.9 months. Use of preoperative testosterone decreased risk of undergoing surgery for complication by 27% (HR 0.73, 95% CI 0.55-0.98, p = 0.04). In addition, patients identified as Hispanic were at increased risk for undergoing surgery for complications (HR 2.40, 95% CI 1.28-4.53, p = 0.01). CONCLUSIONS: We review the largest cohort of patients undergoing 2-stage hypospadias repair at a single institution. Complications and reoperation rates were approximately 50% in the setting of complex genital reconstruction.

The surgical management of male epispadias in the new millennium.

Male epispadias is a rare and challenging urologic condition. As part of the epispadias-exstrophy complex of genitourinary anomalies, it covers a broad spectrum with the mildest forms being the rarest. The anatomic classification into glanular, penile, and penopubic is based on the location of the urethral meatus. However, this classification fails to include some of the suprapubic malformations that may be present such as pubic diastasis, bladder neck abnormalities, and vesicoureteral reflux. Careful preoperative evaluation should allow the identification of these anomalies and will help achieve the goals of surgical reconstruction. These goals include cosmetic and functional reconstruction of the penis and urethra as well as, when needed, creation of a continence mechanism that will allow for normal bladder function, storage, and evacuation of urine. This review will focus on: 1) the preoperative evaluation and management of male patients with epispadias and 2) surgical management based on the anatomic classification.

Evaluation of a novel gel-based ureteral stent with biofilm-resistant characteristics.

PURPOSE: Current ureteral stents, while effective at maintaining a ureteral lumen, provide a substrate for bacterial growth. This propensity for biofilm formation may be a nidus for bacterial growth leading to infection and a reason for early removal of a stent before it is clinically indicated. A newly devised stent, composed of a highly hydrated, partially hydrolyzed polyacrylonitrile polymer, is believed to have bacterial resistant properties. The objective of this study is to evaluate the biofilm growth and bacterial resistant properties of this novel stent. MATERIALS AND METHODS: Multiple 1 cm sections of the pAguaMedicina™ Pediatric Ureteral Stent (pAMS) (Q Urological, Natick, MA) and the conventional polymer stent (SS) (Boston Scientific, Natick, MA) were incubated for 3 days in the 3 different growth media. Afterward, J96 human pathogenic Escherichia coli was added. At 3, 6, 9, 12, and 15 days following bacterial inoculation, the stent segments were washed, sonicated, and analyzed for bacterial growth. Scanning electron microscopy (SEM) imaging was performed to assess biofilm formation. RESULTS: pAMS demonstrated significant reductions (43-71 %) in bacterial counts when compared to standard stents in all conditions tested. SEM imaging demonstrated biofilm formation on both types of stents in all media, with a relative reduction in apparent cell debris and bacteria on the pAMS. CONCLUSIONS: In this study, the gel-based stent shows a demonstrable reduction in bacterial counts and biofilm formation. The use of the pAMS may reduce the risk of infection associated with stent usage.

Incidental capture of rarely diagnosed pediatric tumor: an infant boy with clear cell sarcoma of the kidney.

Clear cell sarcoma of the kidney (CCSK) is an uncommon neoplasm that accounts for less than 5% of all pediatric renal tumors. Compared with Wilms' tumor, CCSK has a higher rate of relapse, greater propensity for bone metastasis, and poorer overall survival. We present the case of a 19-month-old boy with a large renal mass diagnosed incidentally by ultrasonography during surveillance for vesicoureteral reflux. This report describes the rare occurrence of an incidental radiologic capture of CCSK and provides a brief review of disease pathology.

The impact of electrocautery method on post-operative bleeding complications after non-newborn circumcision and revision circumcision.

PURPOSE: We evaluated post-operative bleeding complications in non-newborns following use of monopolar versus bipolar electrocautery for circumcision or revision circumcision. MATERIALS AND METHODS: We retrospectively reviewed sequentially performed cases of circumcision and revision circumcision performed by nine pediatric urologists at our institution from 2005 to 2010. In order to incorporate both the monopolar and bipolar electrocautery experience for a single surgeon employing bipolar technique, sequential cases from 2002 to 2010 were reviewed. Variables assessed included age, procedure, method of electrocautery, skin approximation and dressing, and bleeding complications. RESULTS: 1810 patients that underwent either circumcision or revision circumcision were reviewed. Complete data was available for 1617 patients. Age at operation was a mean 3.7 ± 4.9 yrs and median 1.5 yrs. Return for bleeding complication for all surgeons, was 2/336 (0.6%) for bipolar and 28/1281 (2.2%) for monopolar (p = 0.0545). For the single surgeon using bipolar technique, returns were 2/336 (0.6%) for bipolar and 5/309 (1.6%) for monopolar (p = 0.2133). Returns per procedure type were 1/200 (0.5%) bipolar and 24/844 (2.8%) monopolar for primary circumcision (p = 0.0513), and 1/136 (0.7%) bipolar and 4/437 (0.9%) monopolar (p = 0.84) for revision. Four of 1617 (0.2%) patients returned to the operating room [4/1281 (0.3%) monopolar (p = 0.31)]. There was no difference in return to the operating room for circumcision versus revision. CONCLUSION: Return for bleeding complications after circumcision and revision circumcision occurred more frequently after monopolar electrocautery compared to bipolar. However, there was no significant difference between the two electrocautery methods. Either form of electrocautery appears to be effective for this common pediatric urologic procedure.

Bladder diverticula in children.

This review of bladder diverticula in children includes: historical aspects of the condition, its presentation and diagnosis, as well as management options. The purpose of the review is to provide a comprehensive and in-depth understanding of a relatively infrequent but clinically challenging urologic abnormality that may affect not only the bladder but also the upper urinary tract and kidney.

Variations in management of mild prenatal hydronephrosis among maternal-fetal medicine obstetricians, and pediatric urologists and radiologists.

PURPOSE: There are no current guidelines for diagnosing and managing mild prenatal hydronephrosis. Variations in physician approach make it difficult to analyze outcomes and establish optimal management. We determined the variability of diagnostic approach and management regarding prenatal hydronephrosis among maternal-fetal medicine obstetricians, pediatric urologists and pediatric radiologists. MATERIALS AND METHODS: Online surveys were sent to mailing lists for national societies for each specialty. Participants were surveyed regarding criteria for diagnosing mild prenatal hydronephrosis and recommendations for postnatal management, including use of antibiotic prophylaxis, followup scheduling and type of followup imaging. RESULTS: A total of 308 maternal-fetal medicine obstetricians, 126 pediatric urologists and 112 pediatric radiologists responded. Pediatric urologists and radiologists were divided between Society for Fetal Urology criteria and use of anteroposterior pelvic diameter for diagnosis, while maternal-fetal medicine obstetricians preferred using the latter. For postnatal evaluation radiologists preferred using personal criteria, while urologists preferred using anteroposterior pelvic diameter or Society for Fetal Urology grading system. There was wide variation in the use of antibiotic prophylaxis among pediatric urologists. Regarding the use of voiding cystourethrography/radionuclide cystography in patients with prenatal hydronephrosis, neither urologists nor radiologists were consistent in their recommendations. Finally, there was no agreement on length of followup for mild prenatal hydronephrosis. CONCLUSIONS: We observed a lack of uniformity regarding grading criteria in diagnosing hydronephrosis prenatally and postnatally among maternal-fetal medicine obstetricians, pediatric urologists and pediatric radiologists. There was also a lack of agreement on the management of mild intermittent prenatal hydronephrosis, resulting in these cases being managed inconsistently. A unified set of guidelines for diagnosis, evaluation and management of mild intermittent prenatal hydronephrosis would allow more effective evaluation of outcomes.

Robot-assisted laparoscopic excision of symptomatic retrovesical cysts in boys and young adults.

PURPOSE: We review our surgical experience with the management of retrovesical cystic anomalies using robot-assisted laparoscopic techniques. MATERIALS AND METHODS: We retrospectively reviewed the presentation, diagnosis and treatment of 6 patients 28 months to 22 years old with retrovesical cystic anomalies who underwent robot-assisted laparoscopic excision at our hospital between January 2006 and November 2010. RESULTS: Presenting signs and symptoms included urinary retention, lower urinary tract symptoms, abdominal pain and repeated epididymitis. Associated anomalies consisted of hypospadias, vesicoureteral reflux, renal agenesis, 5alpha-reductase deficiency, premature adrenarche and cryptorchidism. Cystic anomalies ranged from 3 to 6 cm long. The final diagnoses were prostatic utricular cyst, müllerian duct cyst and seminal vesicle cyst. Ectopic insertion of vas into the cyst was found in 4 cases, requiring ligation of the affected vas in 3. Mean ± SD operative time including cystoscopy was 198 ± 23.8 minutes, and estimated blood loss ranged from 5 to 10 ml. Mean ± SD hospital stay was 1.33 ± 0.52 days. All patients had resumed their regular activities within 2 weeks postoperatively. De novo contralateral epididymitis developed 2 months postoperatively in 1 patient. Otherwise, there was no recurrence of cystic mass or presenting signs or symptoms during followup of 3 to 56 months. CONCLUSIONS: In the management of retrovesical cystic anomalies robot-assisted laparoscopic excision affords a natural extension of conventional laparoscopy with the additional advantages of 3-dimensional vision and ease of instrument control.