Cystinuria Complicated by Anuria From Bilateral Obstructing Stones Requiring Bilateral Mini Percutaneous Nephrolithotomy in a 22-Month-Old.

Pediatric nephrolithiasis is increasing in incidence and presents differently compared to adults. We report a case of nephrolithiasis in a pediatric patient, presenting with complaints of emesis, anuria, hematuria, and abdominal distension, leading to a diagnosis of bilateral obstructing cystine stones requiring bilateral percutaneous nephrolithotomy. Pediatric patients with anuria should be evaluated for bilateral nephrolithiasis as an etiology. Calculous anuria requires prompt recognition of the pathologic process and relief of the obstruction with close follow-up and supportive care until definitive stone management. Bilateral percutaneous nephrolithotomy can provide definitive surgical intervention without significant morbidity.

Design and Implementation of an Emergency Undocking Curriculum for Robotic Surgery.

INTRODUCTION: Current training for robotic surgery crisis management, specifically emergency robotic undocking protocol (ERUP), remains limited to anecdotal experience. A curriculum to impart the skills and knowledge necessary to recognize and complete a successful ERUP was developed using an education approach then evaluated. METHODS: Baseline knowledge and confidence regarding ERUP were established for 5 robotic teams before completing 2 full-immersion simulations separated by an online self-paced learning module. In each simulation, teams operated on a perfused hydrogel model and were tasked to dissect a retroperitoneal tumor abutting a major vessel. During vascular pedicle ligation, a major vascular bleed and nonrecoverable robotic fault were remotely induced, necessitating ERUP with open conversion. After the simulation, participants completed surgery task load index (cognitive load assessment) and realism surveys. Weighted checklists scored participants' actions during each simulation. Surgical metrics including estimated blood loss, time to control bleeding, and undocking time were recorded. Curriculum retention was assessed by repeating the exercise at 6 months. RESULTS: Participants experienced high levels of cognitive demand and agreed that the simulation's realism and stress mimicked live surgery. Longitudinal analysis showed significant knowledge (+37.5 points, p = 0.004) and confidence (+15.3 points, p < 0.001) improvements from baseline to completion. Between simulations, checklist errors, undocking time, and estimated blood loss decreased (38⇾17, -40 seconds, and -500 mL, respectively), whereas action scores increased significantly (+27 points, p = 0.008). At 6 months, insignificant changes from curriculum completion were seen in knowledge (-4.8 points, p = 0.36) and confidence (+3.7 points, p = 0.1). CONCLUSIONS: This simulation-based curriculum successfully improves operative team's confidence, knowledge, and skills required to manage robotic crisis events.

Antibiogram Directed Prophylaxis for Transrectal Prostate Biopsy: An Application of Recommendations in the Setting of High Fluoroquinolone Escherichia coli Resistance.

INTRODUCTION: We investigated local resistance patterns to guide antibiotic use for the prevention of infections associated with transrectal prostate biopsy with ultrasound guidance. METHODS: Per the AUA (American Urological Association) recommendations (2014 and 2016) for transrectal prostate biopsy with ultrasound guidance prophylaxis, local antibiogram resistance was reviewed. Rates of Escherichia coli fluoroquinolone resistance were between 20% and 28%. Thus, the antibiotics chosen were a single dose of oral ciprofloxacin and intramuscular ceftriaxone at least 30 minutes before transrectal prostate biopsy with ultrasound guidance. Data were reviewed retrospectively between July 2012 and December 2015. There was no standard prophylaxis before protocol implementation in August 2014. Univariable analyses were performed with appropriate testing followed by multivariable logistic regression. RESULTS: Of 2,351 biopsies 799 were performed in patients in the protocol group. Before protocol implementation 26 different antibiotic regimens were used. The protocol group had significantly more cases of chronic kidney disease, a history of cancer, larger prostate volume and greater number of cores during transrectal prostate biopsy with ultrasound guidance. The overall hospital admission rate after transrectal prostate biopsy with ultrasound guidance was 1.35% for the nonprotocol group and 0.4% for the protocol group (p = 0.026). The incidence of organisms with antimicrobial resistance in blood and urine decreased from 20.7% (23 cases) in the nonprotocol group and 7.1% (4) in the protocol group (p=0.030). All positive blood cultures occurred in the nonprotocol group and all were ciprofloxacin resistant E. coli. On multivariable logistic regression those patients requiring hospitalization were 12.9 (95% CI 2.81-58.96) times more likely to have resistant organisms cultured (p=0.001). CONCLUSIONS: The transrectal prostate biopsy with ultrasound guidance antibiotic prophylaxis protocol decreased unwanted variation among practitioners, which is ultimately associated withimproved quality. Antibiogram directed prophylaxis where there is high fluoroquinolone resistance maintains low infection and hospital admission rates after transrectal prostate biopsy with ultrasound guidance.

Can a simple question predict prolonged uroflow lag times in children?

OBJECTIVE: It is our experience that some children with bowel and bladder dysfunction (BBD) who have bladder neck dysfunction had a tendency to complain of dizziness when we subjected the patients with dizziness to tilt-table testing to confirm the diagnosis of autonomic dysfunction. From these findings we postulated that patients who complain of dizziness are likely to have prolonged lag times that are a proxy for bladder neck dysfunction (BND). STUDY DESIGN: This was an Institute Review Board-approved study in which we enrolled new patients in a sequential fashion who were referred to our outpatient urology offices for evaluation of BBD over a 3-month period. All patients were asked if they frequently experienced dizziness when they rapidly stood from a sitting or lying down position or when they raised their head rapidly. An analysis of the following parameters was undertaken: prevalence of dizziness, bladder neck dysfunction, and comorbid psychiatric problems. Uroflowmetry findings were analyzed as well. RESULTS: Tilt-table group: In the tilt-table group the median age of the patients was 14.5 (4.5-18) years for the five males and five females who were tested. All males and four out of five females experienced a blood pressure drops of 20 mmHg or more on table tilting and three experienced syncope. All patients had prolonged lag times, with an average lag time of 16.5 s. All these patients were able to tolerate alpha-blockers once they were adequately hydrated and salt loaded. Alpha-blocker dosing was increased gradually. In the questionnaire group, 212 patients were initially enrolled: 125 girls and 87 boys. Eleven of 12 males and eleven of 16 females had prolonged lag times when dizziness was present. Sensitivity was 92% and 69%, specificity was 80% and 91% respectively for male and females. Analysis of the psychiatric history revealed a statistically significant association with dizziness and neuropsychiatric problems in the patients and family members. CONCLUSION: We have been able to show a high degree of sensitivity in male patients and a high degree of specificity in female patients that a simple question, "Do you get dizzy on rising in the morning or with rapid standing?", is a reliable marker for BND in children. We have also seen that there is an association between dizziness and psychiatric problems in patients and in family members. It appears from our results and the available literature that autonomic dysfunction is commonly associated with BND and dizziness can be a simple proxy question to identify this problem.

Abdominopelvic ultrasound: a cost-effective way to diagnose solitary kidney.

PURPOSE: Solitary kidneys are detected on approximately 1 of 1,500 prenatal ultrasounds and during evaluation for other urological complaints. Although renal scintigraphy is currently the gold standard for confirming the diagnosis and ruling out renal ectopia, scintigraphy is associated with radiation exposure, placement of an intravenous line and sedation. We hypothesize that ultrasonography alone is sufficient to detect solitary kidneys and that confirmatory renal scintigraphy is unnecessary. MATERIALS AND METHODS: We reviewed the records of children with a solitary kidney who underwent ultrasound and nuclear scintigraphy at our institution from 2001 to 2010. Radiological findings were compared to assess the accuracy of ultrasound in diagnosing solitary kidneys. Costs were calculated based on 2011 Medicare global reimbursement. RESULTS: A total of 25 children met the inclusion criteria of undergoing ultrasound and renal scintigraphy (dimercapto-succinic acid or mercaptoacetyltriglycine scan). The majority of cases were male (16, 64%) and left sided (17, 68%). Median age was 9 days (range 1 day to 11.6 years) at first ultrasound and 4.4 months (3 weeks to 12 years) at first renal scintigraphy. In 24 patients ultrasound correctly diagnosed a solitary kidney as confirmed by nuclear scan. In 1 patient ultrasound suggested a pelvic kidney but repeat ultrasound was negative, as was dimercapto-succinic acid scan. The diagnostic accuracy of ultrasound was 96%. Medicare reimbursement for dimercapto-succinic acid scan (CPT 78700) is $460 to $720 ($222 plus $240 for radiotracer plus $260 for anesthesia, if used). CONCLUSIONS: Our findings suggest that ultrasonography alone is sufficient to make the diagnosis of solitary kidney. Omitting routine renal scintigraphy saves approximately $460 to $720 per case, and avoids radiation and discomfort without sacrificing diagnostic accuracy.

Tailored sutureless meatoplasty: a new technique for correcting meatal stenosis.

OBJECTIVE: Standard practice for meatoplasty includes the use of sutures to evert the mucosa. We present a novel technique that is equivalent to standard meatoplasty without the need for suturing. MATERIALS AND METHODS: A retrospective chart review was performed of patients who presented with primary meatal stenosis in 2007-2009. One group underwent a tailored, sutureless meatoplasty performed by one surgeon. The second group underwent a standard meatoplasty with mucosal advancement and suturing of the mucosal edges by another surgeon. Data were collected regarding demographics, presenting symptoms, symptom resolution, and postoperative complications. These were then compared between the two groups. Preoperative and postoperative uroflowmetry data were collected for the sutureless group to demonstrate objective improvement. RESULTS: Of 85 patients, aged 2-17 years, 60 underwent the tailored, sutureless procedure while 25 had a standard meatoplasty. The majority of patients presented with a deviated and/or narrowed stream of urine. There was no statistical difference between the two groups in regard to symptom resolution. Uroflowmetry data analyzed for 20 patients in the sutureless group demonstrated a significant improvement in Q(max) and flow pattern; there was no significant change in % post-void residual. No patient had a complication in either group, or presented with recurrence. CONCLUSIONS: Tailored sutureless meatoplasty is comparable to meatoplasty with mucosal advancement in resolving symptoms, improving uroflowmetry parameters, and complication rate, but is more efficient and cost effective.

Familial testicular torsion.

PURPOSE: Isolated reported cases of familial torsion yield modest information on incidence, genetics or clinical features. We present what is to our knowledge the largest series of familial torsion, including the first 3 generation case and a review of the literature. MATERIALS AND METHODS: Since 2006, we have questioned the presence of a positive family history in all patients with torsion at the initial consultation. We compiled data on familial relationship, laterality, age and outcomes of the proband and affected relatives. We collected previously published cases to better understand clinical features and genetics. RESULTS: Eight of 70 boys (11.4%) with torsion had affected family members. Another 2 families were included from a historical perspective. One relative was affected in 7 families, 2 were affected in 2 and 3 were affected in 1. First degree relatives were most commonly affected. In 1 family torsion occurred in 3 consecutive generations. Despite a family history 50% of patients experienced testicular loss. Brothers were affected in each of the 10 previously reported cases. In 3 families fathers were also affected. There were 3 sets of monozygotic twins. We noted laterality concordance 5 times and discordance 6 times. Age at torsion in probands was adolescence except in 2 with neonatal torsion. No clear inheritance mode was found. CONCLUSIONS: Familial torsion occurs in about 10% of probands and can affect multiple relatives and generations. A positive family history may be useful for torsion diagnosis and management. Relatives of affected patients need education on the signs and symptoms of torsion, and the importance of early presentation to improve outcome.

A conservative approach to testicular rupture in adolescent boys.

PURPOSE: Management for blunt trauma with breach of the renal capsule or bladder (extraperitoneal) has largely become nonsurgical since a conservative approach proved to be effective and safe. Currently the recommendation for managing testicular rupture is surgical exploration and débridement or orchiectomy. We report outcomes in boys diagnosed with testicular rupture and treated without surgical intervention. MATERIALS AND METHODS: In the last year we conservatively treated 7 consecutive boys with delayed presentation of testicular rupture after blunt scrotal trauma. Patients were treated with scrotal support, antibiotics to prevent abscess, rest, analgesics and serial ultrasound. We report clinical information and outcomes. RESULTS: The 7 boys were 11 to 14 years old and presented 1 to 5 days after injury. Trauma was to the left testis in 3 cases and to the right testis in 4. Patients presented with mild to moderate pain and similar scrotal swelling. Ultrasound findings consistently revealed hematocele and increased echogenicity. Blood flow was present in the injured portion of the testes in 3 cases and to the remainder of the affected testicle in 6 of the 7 boys. In the remaining boy an adequate waveform was not seen in either testicle, which the radiologist thought was secondary to prepubertal status. Other findings included scrotal edema, irregular contour and seminiferous tubule extrusion. Followup was greater than 6 months in all cases. Five boys were seen at the office and the 2 remaining had telephone followup. In all cases hematocele resolved, testicular size stabilized without atrophy and echogenicity normalized in the 5 patients with followup ultrasound. One patient required surgical repair of hydrocele 4 months after trauma but no other patient needed surgical exploration. No abscess or infection developed. CONCLUSIONS: A conservative approach in a select group of adolescent boys with testicular rupture can result in resolution of the fracture and maintenance of testicular architectural integrity.

Dynamic regulation of sphingosine-1-phosphate homeostasis during development of mouse metanephric kidney.

Branching morphogenesis of the metanephric kidney is critically dependent on the delicate orchestration of diverse cellular processes including proliferation, apoptosis, migration, and differentiation. Sphingosine-1-phosphate (S1P) is a potent lipid mediator influencing many of these cellular events. We report increased expression and activity of both sphingosine kinases and S1P phosphatases during development of the mouse metanephric kidney from induction at embryonic day 11.5 to maturity. Sphingosine kinase activity exceeded S1P phosphatase activity in embryonic kidneys, resulting in a net accumulation of S1P, while kinase and phosphatase activities were similar in adult tissue, resulting in reduced S1P content. Sphingosine kinase expression was greater in the metanephric mesenchyme than in the ureteric bud, while the S1P phosphatase SPP2 was expressed at greater levels in the ureteric bud. Treatment of cultured embryonic kidneys with sphingosine kinase inhibitors resulted in a dose-dependent reduction of ureteric bud tip numbers and increased apoptosis. Exogenous S1P rescued kidneys from apoptosis induced by kinase inhibitors. Ureteric bud tip number was unaffected by exogenous S1P in kidneys treated with N,N-dimethylsphingosine, although tip number increased in those treated with d,l-threo-dihydrosphingosine. S1P1 and S1P2 were the predominant S1P receptors expressed in the embryonic kidney. S1P1 expression increased during renal development while expression of S1P2 decreased, and both receptors were expressed predominantly in the metanephric mesenchyme. These results demonstrate dynamic regulation of S1P homeostasis during renal morphogenesis and suggest that differential expression of S1P metabolic enzymes and receptors provides a novel mechanism contributing to the regulation of kidney development.