Giant Congenital Melanocytic Nevus of the Male External Genitalia: A Pediatric Case Report of Diagnosis, Evaluation and Management.

Congenital melanocytic nevi are present at birth or develop within the first few months of life. Giant congenital melanocytic nevi are a rare variant and may involve the external genitalia with a confluent "bathing trunk" distribution. Rapid growth of proliferative nodules of melanocytic cells may cause disfigurement and anatomical distortion resulting in psychological distress and loss of functionality. We report the case of a neglected 17-year-old nonverbal male who received a resection of a Giant Congenital Melanocytic Nevi (GMN) engulfing the penis and scrotum with final resected dimensions of 36.0×20.0×8.0 cm.

Role of progressive urethral dilation and primary valve ablation in the long-term renal outcomes of small, preterm infants with posterior urethral valve.

INTRODUCTION: PUV patients managed with primary vesicostomy instead of primary valve ablation (PVA) historically are preterm, low-birth-weight (LBW) infants with inadequate urethral size. We previously described progressive urethral dilation (PUD) as an effective method of enhancing the likelihood of PVA in these infants, allowing equal access to PVA as an initial management method. OBJECTIVE: We aim to characterize renal outcomes in patients managed with PUD + PVA and compare this to outcomes with PVA alone. We also re-examine the effect of LBW and gestational age on renal outcomes in PUV with a cohort treated uniformly by PVA. METHODS: We performed retrospective review of 78 neonates with PUV treated with PVA prior to 10 weeks of age with >1 year of follow up. Before valve ablation, boys either underwent PUD (serial upsizing of a smaller bore urethral catheter to an 8Fr catheter; PUD + PVA) or non-dilation (smaller bore catheter was maintained; PVA-only). PUD + PVA versus PVA-only was compared using chi-square and t-test. Logistic regression was performed to assess the effect of PUD, preterm (<37 weeks), LBW (<2.5 kg), and other predictors on the final outcomes of CKD3+ and ESRD. RESULTS: 31 of 78 patients underwent PUD + PVA. Mean follow up was 5.2 years (SD 3.4), with no significant difference between PUD + PVA and PVA-only. The PUD + PVA group included significantly lower gestational age infants with lower birth weight and ablation weight. There was no significant effect of PUD on final CKD3+ or ESRD outcome on univariable or multivariable analysis. When adjusted for other variables, only Cr nadir >0.5 remained an independent predictor of CKD3+ (OR 41.2; p < 0.001) and ESRD (OR 18.9; p = 0.015). DISCUSSION: We previously demonstrated that PUD is an effective means to achieve PVA in small neonates who might otherwise require vesicostomy. The data herein demonstrates no significant effect of PUD on renal outcomes. In this unique cohort of newborns treated with PVA, only creatinine nadir and not gestational age or an independent predictor of outcomes. CONCLUSION: In small preterm infants who would have been excluded from PVA due to limited urethral size, PUD + PVA confers the same renal outcomes as PVA alone in larger infants. This novel data will assist in the risk-benefit analysis of using PUD before PVA in newborns diagnosed with PUV. When primary intervention is uniformly PVA, preterm birth and LBW are not independent predictors of renal outcomes.

Sutureless circumcision using monopolar diathermy and 2-octyl cyanoacrylate is safe and effective in a cohort of 634 post-neonatal prepubescent boys.

BACKGROUND: Circumcision is the most commonly performed surgical procedure in the world, with one-third of males circumcised globally. Post-neonatal, prepubescent sutured circumcision demonstrates complication rates ranging from 1.7% to 9.1%. We have previously reported that 2-octyl cyanoacrylate (2-OCA, Dermabond, Ethicon) primary circumcision (PC) and circumcision revision (CR) in prepubescent children demonstrated superior cosmesis, shorter operating room (OR) times and cost savings. OBJECTIVE: The aim of our study is to evaluate complication and reoperation rates with a scalpel-free and suture-less technique for primary circumcision (PC) and circumcision revision (CR) using 2-OCA. METHODS: Following IRB approval, we conducted a retrospective review of all boys at our institution who underwent PC or CR using 2-OCA and monopolar diathermy between January 2014 and January 2021. All procedures were performed by a single surgeon. The technique is outlined in the figure below. No aligning sutures or instruments were used in this process. Patients that required sutures or compressive dressings based on age or associated anomalies were excluded from analysis. We obtained all returns to our system within 30 days of the procedure and returns to the OR during the study period using the REDCap database. RESULTS: Of 1107 procedures performed during the study period, 634 procedures (479 PC and 155 CR) met inclusion criteria. Median age was 12 months (range 3 months-10.4 years) with minimum follow up of 1 year. There were 3 patients (0.47%) that returned to system within 30 days for surgical site bleeding, and one patient (0.15%) required surgical intervention within 30 days. Nine patients required reoperation after 30 days, five (0.8%) for iatrogenic phimosis, 3 (0.5%) for redundant prepuce and 1 for keloid formation. We observed an overall complication rate of 1.3% (6/634) and reoperation rate of 1.6% (10/634). DISCUSSION: Since FDA approval in 1998, 2-OCA has been widely adopted as a replacement for sutures in tension-free wounds. However, it has yet to gain widespread use for circumcision given concerns for wound dehiscence and surgical site bleeding. This study demonstrates that a scalpel-free and suture-less technique demonstrates complication and reoperation rates are lower than reported circumcision using scalpel and sutures. Limitations include retrospective design, single surgeon experience, and REDCAP database only identifying patients with complications that required a return to system. CONCLUSION: Our technique for suture-less circumcision using 2-OCA offers superior cosmesis, shorter OR times, cost savings, and a lower complication rate (1.3%) compared to sutured circumcision (>1.7%) reported in post-neonatal prepubescent boys.

Parapelvic cyst: A rare cause of ureteropelvic junction obstruction in a pediatric patient managed with robotic cyst decortication.

INTRODUCTION: We report a case of a right parapelvic renal cyst causing intermittent ureteropelvic junction obstruction (UPJO). DIAGNOSTIC EVALUATION: A 13-year-old male was referred for right flank pain. Stone protocol CT revealed renal pelvis dilation with punctate stones. Due to concern for intermittently obstructive calculi, he underwent ureteroscopy, which was unremarkable. A diuretic renogram showed symmetric uptake with partial emptying on the right with pain after diuretic administration. In office, we potentiated a Dietl's crisis with ultrasound obtained before and after fluid intake. Comparison of ultrasounds revealed a parapelvic cyst causing calyceal dilation. He was counseled for robotic cyst decortication and possible pyeloplasty. SURGICAL CONSIDERATIONS: A robotic cyst decortication was performed. Once decorticated, the cyst base was fulgurated and pararenal fat was interposed into the cyst base. Console time was 70 min with minimal blood loss. The patient was discharged post-operative day 1. Follow-up renal ultrasound at 4 months demonstrated resolution of hydronephrosis and parapelvic cyst. CONCLUSION: Parapelvic renal cysts causing intermittent UPJO is a rare entity that may be missed on a diuretic renogram. Clinical suspicion and appropriate imaging with ultrasound or magnetic resonance imaging are useful. Robotic cyst decortication is a technically feasible approach to treat this condition.

Inflammatory Myofibroblastic Tumor Presenting as Gross Hematuria in a Pediatric Patient With VACTERL Syndrome Following Bladder Augmentation.

Inflammatory myofibroblastic tumors (IMT) are rare and poorly understood inflammatory neoplasms. Most commonly occurring in the liver and gastrointestinal tract, cases of bladder involvement have been rarely reported. Bladder IMT generally presents with gross hematuria and can be differentiated from other bladder tumors by expression of anaplastic lymphoma kinase. We report the occurrence of an Bladder IMT detected following lower urinary tract reconstruction with bladder augmentation.

A 22-Year-Old Retained Ureteral Stent: One of the Oldest Removed Using a Multimodal Endourologic Approach.

Background: Ureteral stent encrustation poses a distinct challenge to urologists. The purpose of our study is to present a patient with one of the oldest retained ureteral stents reported in the literature, effectively treated at our institution with a multimodal endourologic approach. Case Presentation: After IRB approval and patient's consent, we present the case of a 47-year-old man who was referred to our institution for gross hematuria and a right retained ureteral stent, incidentally found on imaging. This patient had a history of traumatic stab wound 22 years prior, requiring an exploratory laparotomy and a ureteral stent insertion. Preoperative CT scan revealed a fragmented and heavily encrusted right ureteral stent. The patient was effectively treated with a multimodal endourologic approach, including a cystolitholapaxy, a right retrograde flexible ureteroscopy (URS), and a prone split-leg right percutaneous nephrolithotomy combined with a right retrograde URS. The patient was rendered stone and stent free. Conclusion: To our knowledge, this 22-year-old retained stent is one of the oldest reported in the literature. As observed in our patient, multimodal endourologic techniques are safe and effective in patients with retained ureteral stents to render then stent and stone free.