1. Delayed recognition of bladder exstrophy and persistent cloaca: an OEIS variant Authors: Itunu O. Arojo MD1, Lynn L. Woo MD 1, 2. 1. Case Western Reserve University School of Medicine, Cleveland, OH; University Hospitals Cleveland , Medical Center, Urology Institute, Cleveland, OH; 2. Rainbow Babies and Children Hospital, Cleveland, OH. Infant is a term female born to an otherwise healthy 31 year-old G3P2 diagnosed with an omphalocele containing the bladder on prenatal imaging (image 1A, B). On the 22 week growth scan, she was noted to have a widened pubic diastasis, possible bifid clitoris, dilated recto-sigmoid colon, and mild polyhydramnios which subsequently normalized by third trimester. At birth, an omphalocele was observed with asymmetric labia and clitoral tissue. There was an imperforate anus, and a single mucosal-lined opening in the perineum through which urine and meconium drained intermittently. Drops of urine were also seen emanating from a second, more anteriorly-located orifice within the clitoral tissue (Image2). Endoscopy confirmed the presence of a cloacal malformation with what appeared to be an intact urethra/bladder neck complex, vaginal duplication, and a fistulous tract to the rectum. A small feeding tube placed into the clitoral orifice could be seen entering the bladder separately on cystoscopy, which also appeared to show an intact bladder. Cystogram showed a smooth-walled bladder without evidence of reflux, however the infant did not void, and opacification of the urethra and common channel could not be achieved (Image 3). Spinal ultrasound was normal. On DOL 4, the omphalocele ruptured, revealing a true exstrophied bladder (Image 4). The infant remained stable and was discharged home on DOL 14, with plastic wrap covering the exposed bladder plate and diverting colostomy/mucus fistula. Cloacal repair and closure of bladder exstrophy is tentatively planned for when the infant is 6 months old. Images: Image 1A/B: Prenatal MRI demonstrating abdominal wall defect. Image 2: fistulous opening in clitoris draining urine (Black arrow) Image 3A/B: Cystogram Image 4: Red rubber catheter through the open bladder and out through the cloaca. The feeding tube traverses the clitoral tract and exists through the exstrophied bladder. 2. TITLE: Survival in a functionally anephric neonate with autosomal recessive polycystic kidney disorder (ARPKD) Sarah L. Hecht MD*, Vijaya M. Vemulakonda, MD Division of Urology, Children’s Hospital of Colorado *Presenting author: [email protected] INTRODUCTION: The functionally anephric fetus traditionally has been considered nonviable. Some centers have begun offering aggressive perinatal care for patients with bilateral renal agenesis with rare case reports of survival past the neonatal period(1,2). We present a patient with prenatally diagnosed ARPKD who successfully underwent postnatal nephrectomy and neonatal dialysis as a bridge to kidney transplant. CASE: A 27-year-old G2P1 mother presented to our fetal care center after 26-week prenatal ultrasound revealed enlarged echogenic kidneys and new anhydramnios, consistent with ARPKD. Fetal MRI confirmed enlarged, hyperintense kidneys (Figure 1). Neonatal respiratory survival was deemed likely based on fetal lung volumes. Parents elected to pursue any available medical interventions. The child was born at 36 weeks. She was anuric as expected. She underwent left nephrectomy on day of life 2 to create space for peritoneal dialysis. A peritoneal dialysis catheter and temporary hemodialysis line were placed at that time. Her course was complicated by bacteremia, line thrombosis, bacterial peritonitis, feeding intolerance, and difficulty achieving therapeutic peritoneal dialysis volumes requiring intermittent hemodialysis. At 10 months of age, she is tolerating goal enteric feeds and is on a stable home peritoneal dialysis regimen. CONCLUSION: We report survival in a functionally anephric neonate with ARPKD managed with unilateral nephrectomy and peritoneal dialysis. Pulmonary survivors eligible for future kidney transplant may be candidates for aggressive perinatal management in a condition previously considered universally fatal. Efforts are underway to further study who is a candidate for such intervention, and whether offering such care is ethical(3,4). 3. Benign infantile hemangioma presenting as solid testicular mass Victor Kucherov, MD(1) (presenter, main contact), Kudakwashe Chikwava, MD(2), Steven Blumer, MD (3), T. Ernesto Figueroa, MD(4) 1. Department of Urology, Thomas Jefferson University Hospital, Philadelphia, PA. E-mail: [email protected] 2. Department of Pathology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE. E- mail: [email protected] 3. Department of Radiology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE. E-mail: [email protected] 4. Division of Pediatric Urology, Nemours/Alfred I. duPont Hospital for Children, Wilmington, DE. E-mail: [email protected] A 3-month old boy presented with right scrotal swelling and concern by his pediatrician for right hydrocele. Examination revealed an enlarged and firm right testicle concerning for solid mass; exam was otherwise benign without signs of lymphadenopathy or intra-abdominal mass. Work- up with scrotal ultrasound (US) identified a 4 cm exophytic right testicular mass with numerous satellite lesions throughout the testicle concerning for a malignant process. Further evaluation with computed tomography (CT) of the chest, abdomen, and pelvis did not reveal any metastatic disease. Tumor markers including alpha fetoprotein (AFP) and human chorionic gonadotropin (hCG) were within normal limits. Given concern for malignancy right radical orchiectomy was performed. Gross pathologic evaluation revealed a pink/tan lobulated mass displacing the testicle inferiorly. Microscopic examination found the mass to be arising from the tunica albuginea and visceral tunica vaginalis with focal extension into the distal spermatic cord and testicular parenchyma. The cells formed numerous capillary beds. Immunohistochemistry was negative for germ cell and sex-cord stromal markers. Final diagnosis with benign infantile hemangioma. Literature review found this to be a rare tumor without association to neoplasms of other body sites. Post-operatively he was followed with scrotal US which did not reveal interval appearance of any other masses. 4. Unusual Presentation of Fusiform Megalourethra and Anterior Urethral Valves as a Transient Fetal Cystic Perineal Mass Lauren E. Corona* and John M. Park University of Michigan, Department of Urology, Ann Arbor, Michigan *Presenter email: [email protected] Case Report: A 28 year old G1P1 was referred with a 22 week 46 XY fetus showing a perineal midline fluid filled structure, confirmed by ultrasound and MRI. Moderately dilated right pelvis was the only other abnormality. A follow up ultrasound repeated at 25 weeks showed resolution of this cystic structure and hydronephrosis. Male infant was born at 41 weeks. Neonatal exam findings included bilateral non-palpable intraabdominal testes (confirmed by ultrasound), penoscrotal transposition, large phallus with dorsal curvature, ventral subcoronal urethrocutaneous fistula (along with patent orthotopic urethral meatus), partially formed prepuce, and imperforate anus. The newborn ultrasound showed an unremarkable bladder and mild right hydronephrosis. He underwent colostomy and mucous fistula creation on day of life (DOL) 1. He subsequently stopped voiding, and ultrasound on DOL 2 showed severe bilateral hydronephrosis with marked bladder distension. Unsuccessful attempts at catheterization led to bedside suprapubic tube (SPT) placement. Voiding cystourethrogram performed via SPT showed right renal duplication, grade 4 lower pole reflux, non-trabeculated large capacity bladder and obstruction in the anterior urethra. Diagnostic cystoscopy at 3 weeks of age revealed a massively dilated anterior urethra consistent with fusiform megalourethra and 3-French caliber anterior urethral valve near the bulbar urethral region. Successful direct vision internal urethrotomy (DVIU) was performed 1 week later. Thereafter, the patient voided normally per urethra. He was placed on prophylactic antibiotics for right lower pole reflux. He awaits urethral and phallic reconstruction along with orchidopexy 7. Difficult decisions: fetal intervention in twin gestation Authors: Bridget L. Findlay, M.D., Patricio C. Gargollo, M.D., Candace F. Granberg, M.D. Affiliation: Mayo Clinic, Rochester, MN Presenter Name and Contact: Bridget L. Findlay, M.D.; [email protected] A 36 year old G4P2103 woman with monochorionic diamniotic twin gestation was found to have antenatal bladder outlet obstruction (Figure 1) and oligohydramnios of Twin B on 20 week ultrasound. Bilateral kidneys also appeared to be echogenic (Figure 2). Twin A did not have any abnormalities. There was no history of congenital abnormalities with her other three children and no family history of urologic abnormalities or kidney disease. Given the implications of bladder outlet obstruction, the patient opted for two rounds of vesicocentesis at 20w5d and 21w1d for Twin B. Urine biochemistry was significant for a markedly elevated Beta 2 microglobulin (>20,000), indicating severe renal damage. Chromosomal FISH/microarray studies were negative. Vesicoamniotic shunt was placed at 21w6d (Figure 3). She was admitted at 24w6d for premature rupture of membranes (PROM) and ultimately