Travis Groth

Advanced Transitional Cell Carcinoma of the Bladder in a 16-Year-Old Girl With Hinman Syndrome

The present report describes a 16-year-old girl with a history of Hinman syndrome who was found to have invasive transitional cell carcinoma of the bladder. Bladder cancer is rare in children. Typically, the tumors are of low grade and stage. We present the first case of a teenage girl diagnosed with Hinman syndrome who developed an invasive bladder transitional cell carcinoma. The patient with a neurogenic bladder who has undergone bladder augmentation is at increased risk for bladder cancer. This risk may now have to be extended out to the non-neurogenic neurogenic bladder population if further cases are identified.

Short-term outcomes of the multi-institutional bladder exstrophy consortium: Successes and complications in the first two years of collaboration☆

INTRODUCTION/BACKGROUND Bladder exstrophy is a rare diagnosis that presents major reconstructive challenges. To increase experience and proficiency in the care of bladder exstrophy (BE), the Multi-Institutional BE Consortium (MIBEC) was formed, with a focus on refining technical aspects of complete primary repair of bladder exstrophy (CPRE) and subsequent care. OBJECTIVE Outcome measures included successful CPRE (absence of dehiscence), complications, and integrated points of technique and care over the short-term. STUDY DESIGN Boston Childrens Hospital, Childrens Hospital of Philadelphia and Childrens Hospital of Wisconsin alternately served as the host, with observation, commentary and critique by visiting collaborating surgeons. CPRE with bilateral iliac osteotomy was performed at 1-3 months of age. High-definition video capture of the surgery allowed local and distant broadcast to facilitate real-time observation and teaching, and recording of all procedures. RESULTS From February 2013 to February 2015, MIBEC participating surgeons performed CPRE on 27 consecutive patients (22 classic BE, five epispadias). There were no dehiscences in 27 patients (0%, 95% CI 0-12.5%). Thirteen girls and 14 boys underwent CPRE at a median age of 2.3 months (range 0.1-51.6). One boy had a hypospadiac urethral meatus at CPRE completion. Hydronephrosis of mild or moderate grade was present postoperatively in eight girls and two boys. Additional results, per gender, are presented in the Summary table below. DISCUSSION Absence of dehiscence in this cohort was comparable or compared favorably with the literature. However, several girls had significant obstructive complications following CPRE. The rate of bladder outlet obstruction (BOO) in girls was increased compared with published reports. A low complication rate was noted in the boys following CPRE, which was comparable to reports in the literature, and early signs of continence and spontaneous voiding were noted in some boys and girls. Limitations included variation in patient age at presentation, thereby introducing a wide age range at CPRE. Outcome data were limited by short follow-up regarding voiding with continence. CONCLUSION This collaborative effort proved beneficial regarding significantly increased surgeon exposure to CPRE, refinement of CPRE technique, surgeon learning and expertise. Technical refinement of CPRE is ongoing.

Urinary Proteome Analysis in Patients With Stable SFU Grade 4 Ureteropelvic Junction Obstruction Differs From Normal

OBJECTIVE To evaluate and analyze the urinary proteome in infants with stable grade 4 ureteropelvic junction obstruction (UPJO) and compare to age-matched normal controls. METHODS Bladder urine specimens were obtained from 21 healthy infants with normal maternal/fetal ultrasound and 25 infants with grade 4 unilateral UPJO. All patients had >40% ipsilateral individual kidney function by renal scanning and the anteroposterior (AP) diameter of the hydronephrotic kidney ranged from 1.6-3.9 cms at presentation. Over a 5-year follow-up period, the disease progressed in 7 infants (28%), resolved in 4 (16%), and remains stable in the majority (56%). The urinary specimens were prepared using standard methods and subjected to LC/MS/MS analysis. The normalized data were annotated utilizing the Ingenuity Pathways Analysis (IPA; www.Ingenuity.com) knowledge platform. RESULTS In the stable UPJO group, the urinary proteomes obtained in infancy differed significantly from the age-matched controls. Analysis revealed important differences in a number of biologic functions including inflammation, apoptosis, tubular injury and fibrosis, and reactive oxygen species response. CONCLUSION The urinary proteomes from the bladder in patients with stable grade 4 UPJO (by imaging criteria) are significantly different at birth and during the first year of life and seem to indicate the presence of an ongoing active renal response to UPJO. The imminent discovery of surrogate urinary biomarkers may result in reconsideration of the watchful waiting strategy during this critical period of renal maturation and development in infancy.

Urolithiasis in Patients on High Dose Felbamate

PURPOSE We report 4 cases of felbamate urolithiasis. We identified only 1 prior case report of a felbamate stone. Felbamate is an antiepileptic drug used to treat refractory seizures and has minor side effects when given in recommended doses. We analyzed the characteristics, evaluation, treatment and outcomes in this challenging group of patients. MATERIALS AND METHODS Following institutional review board approval, we conducted a retrospective chart review of all patients who presented with a diagnosis of urolithiasis, were on felbamate and had stone analysis consistent with a felbamate origin. RESULTS All 4 patients had refractory seizures and 3 had severe developmental delay. Presentation ranged from an incidental finding to gross hematuria to agitation and pain. Stones were not visible on plain x-ray except in 1 case involving mixed stone composition. Decrease or cessation of the drug has not been feasible in 2 patients, and 3 patients have had recurrent stones. Initial stone analysis did not correctly identify the stone composition as felbamate in 2 cases, suggesting that the origin of these stones may not always be recognized. CONCLUSIONS We report the occurrence of felbamate stones in a series of patients on high dose felbamate therapy. Accurate diagnosis is made more difficult by the clinical complexity of the patient population (including severe developmental delay), the radiolucent nature of the stones and the possibility of inaccurate analysis of stone composition.

Intravesical Botulinum Toxin for Persistent Autonomic Dysreflexia in a Pediatric Patient

Introduction. We present a novel case of persistent autonomic dysreflexia in a pediatric spinal cord injury patient treated successfully with intravesical botulinum toxin. Study Design. A retrospective chart review of one patient seen at the Childrens Hospital of Wisconsin from 2006 to 2012 was performed. Results. A pediatric spinal cord injury patient with known neurogenic bladder presented with severe hypertension consistent with autonomic dysreflexia. His symptoms and hypertension did not improve with conservative measures, and he necessitated ICU admission and antihypertensive drips. He was taken to the operating room for intravesical botulinum toxin for refractory bladder spasms. Following this, his symptoms abated, and he was weaned off IV antihypertensives and returned to his baseline state. His symptoms were improved for greater than six months. Conclusions. There are few treatment options for the management of refractory autonomic dysreflexia. Intravesical botulinum toxin has never been reported for this use. Dedicated research is warranted to assess its efficacy, as it was used successfully to abort autonomic dysreflexia in this patient.

Clinical pathway for early discharge after complete primary repair of exstrophy and epispadias by using a spica cast

INTRODUCTION/OBJECTIVE Secure closure of the pubic diastasis during bladder exstrophy and epispadias repair decreases the abdominal wall tension at the time of reconstruction. Pelvic osteotomies are routinely performed at the time of abdominal wall and bladder reconstruction in order to more easily facilitate pubic symphyseal diastasis approximation. Postoperative pelvic immobilization is performed by methods that include modified Bucks traction, modified Bryants traction, and spica casting. People undergoing closure often require inpatient hospitalization for 2-8 weeks because of the pelvic immobilization. The present study examined the findings from a clinical pathway for early discharge after complete primary repair of exstrophy (CPRE) and proximal epispadias repair with spica casting. METHODS The present study is a retrospective review of patients that underwent pelvic osteotomies with spica casting at the time of CPRE or proximal epispadias repair from November 2006 to March 2013. All patients had anterior innominate osteotomies and spica cast pelvic immobilization. RESULTS Pelvic osteotomies and spica cast pelvic immobilization were performed on 17 children. The median postoperative stay was 6.0 days and the subdivided results are in Table. No children experienced an abdominal or orthopedic complication. A few children required minor cast adjustments to relieve pressure. After cast removal, no skin breakdown, pressure necrosis, or nerve palsy were found. The median length of casting without pinning was 31 (26-48) days. DISCUSSION The use of spica cast pelvic immobilization after exstrophy and epispadias repair is safe and allows for earlier discharge when compared to other methods of pelvic immobilization. However, although the family appreciates early discharge and additional bonding, the priority is the success of the closure. The present findings demonstrate, and are corroborated by other spica cast publications, that spica casting is as effective as modified Bryants traction or modified Bucks traction. The success rates for CPRE with spica casting are similar to published staged repairs and have the benefit of allowing for bladder cycling, which potentially enables better bladder growth and development. If success and complication rates are comparable amongst the different pelvic immobilization groups, then variables including hospital length of stay and cost become appropriate comparisons. CONCLUSION The shortened discharge time, along with a significant decrease in acuity of care leads to significant decreases in hospital costs. Additional hospital stay when using modified Bryants traction or modified Bucks traction with external fixation will accrue significant hospital costs.

Gas analysis using Raman spectroscopy demonstrates the presence of intraperitoneal air (nitrogen and oxygen) in a cohort of children undergoing pediatric laparoscopic surgery.

Clinically significant gas embolism during laparoscopy is a rare but potentially catastrophic event. Case reports suggest that air, in addition to the insufflation gas, may be present. We studied the effects of equipment design and flushing techniques on the composition of gas present under experimental and routine pediatric surgical conditions. Concentrations of nitrogen (N2), oxygen (O2), and carbon dioxide (CO2) were measured by Raman spectroscopy in gas delivered to and retrieved from a mock peritoneum during simulated laparoscopy. We then analyzed the composition of insufflated and recovered gases during elective laparoscopic procedures conducted with CO2-preflushed and unflushed tubing to determine the presence of significant (10%) quantities of air. In vitro, CO2 was not detected at the distal end of insufflator tubing until after delivery of approximately 0.2 L of gas, and N2 persisted until >0.4 L was delivered, with 40% ± 8% (mean ± SD, range 33%–49%) recovered from the mock peritoneum at the termination of initial insufflation. In clinical studies, preflushing reduced the initial concentration of N2 from 78% ± 0.5% to 23% ± 15%, but >10% air was detected in all subsequent samples, regardless of insufflation technique. Laparoscopic equipment and practice routinely permit delivery of air to the insufflated cavity. Purging the equipment with CO2 reduces but does not eliminate air (N2, O2) within the peritoneal cavity during laparoscopy. Thus, when vascular injury occurs, embolized gases will contain variable quantities of N2, O2, and CO2. As the initial insufflation volume diminishes and approaches the volume of the insufflation tubing, which occurs in infants and young pediatric patients, the concentration of N2 will approximate that of room air in an unflushed system. Small insufflation volumes containing high N2 concentrations can contribute to catastrophic air emboli in neonates and small pediatric patients.

Antibiotic Prophylaxis with Trimethoprim-Sulfamethoxazole versus No Treatment after Mid-to-Distal Hypospadias Repair: A Prospective, Randomized Study

Purpose To evaluate the impact of prophylactic antibiotics after distal hypospadias repair on postoperative bacteriuria, symptomatic urinary tract infection, and postoperative complications in a prospective, randomized trial. Materials and Methods Consecutive patients aged 6 months to 2 years were enrolled at our institution between June 2013 and May 2017. Consenting patients were randomized to antibiotic prophylaxis with trimethoprim-sulfamethoxazole versus no antibiotic. Patients had catheterized urine samples obtained at surgery and 6–10 days postoperatively. The primary outcome was bacteriuria and pyuria at postoperative urine collection. Secondary outcomes included symptomatic urinary tract infection and postoperative complications. Results 70 patients consented to the study, of which 35 were randomized to receive antibiotics compared to 32 who did not. Demographics, severity of hypospadias, and type of repair were similar between the groups. Patients in the treatment group had significantly less pyuria (18%) and bacteriuria (11%) present at stent removal compared to the nontreatment group (55% and 63%; p=0.01 and p < 0.001, resp.). No patient had a symptomatic urinary tract infection. There were 11 postoperative complications. Conclusions Routine antibiotic prophylaxis appears to significantly decrease bacteriuria and pyuria in the immediate postoperative period; however, no difference was observed in symptomatic urinary tract infection or postoperative complications. Clinical Trial Registration Number NCT02593903.

Postoperative Immobilization and Pain Management After Repair of Bladder Exstrophy

Surgical repair of bladder exstrophy is an ongoing challenge for pediatric urologists. Postoperative immobilization is a mainstay of care to decrease tension on the repair site and is often utilized in conjunction with pelvic osteotomies performed in the same operative setting by pediatric orthopedic surgeons. Multiple pelvic immobilization techniques have been developed in conjunction with repair techniques including special techniques for neonates. The most commonly utilized techniques for pelvic immobilization are Buck’s and Bryant’s traction and spica casting. A multimodal pain management approach is critical with pelvic immobilization to minimize postoperative pain and anxiety associated with reconstructive surgery at a young age.

The value of interventional radiology in re-establishment of lost access to catheterizable channels to the bladder and bowel

OBJECTIVE The efficacy of interventional radiology (IR) procedures in regaining lost access to continent catheterizable channels in pediatric urology patients is uninvestigated. This paper assesses this efficacy, as well as prevention of surgical revision of these channels as a result of IR intervention. METHODS A retrospective chart analysis was performed over 8 years for children presenting with lost access to the bladder or bowel that could not be regained by a pediatric urologist. Rates of successful re-establishment of access in IR and the need for future surgical revision were calculated. RESULTS Twenty pediatric patients underwent 32 attempts to re-establish lost access in IR. IR was successful in 78.1% (25/32) of episodes for 15/20 patients. No intervention required general anesthesia. Thirty percent (6/20) were able to avoid surgical revision. Another 45% (9/20) had access re-established in IR but later had surgery related to their channel (endoscopic, percutaneous, or open). Only three patients required open revision. The five patients in whom IR access failed, did require surgery. CONCLUSION Image-guided re-establishment of access to continent catheterizable channels in children is efficacious. It can diffuse an emergency situation and delay or obviate the need for surgical correction. Additionally, a general anesthetic is not necessary.