Genitourinary and perineal vascular anomalies in children: A Seattle children's experience.

AIMS: We sought to evaluate the initial presentation and diagnosis, to compare presenting symptoms of these anomalies with respect to location, and describe the management course and review the treatment outcomes of vascular anomalies (VA) in genitourinary and perineal area. We described changes in the evaluation and management approach over time. METHODS: After IRB approval, we reviewed the Vascular Anomalies Program 3,780 patient database from 1998-2013, identifying 85 patients with GUP VA. Demographics, presentation, anatomic location, imaging modalities and treatments were reviewed. For the 76 patients that were managed at our institution, we analyzed data for management approach change over time, duration of follow-up, complications, and treatment outcomes. Using Chi-squared test, we analyzed the treatment eras for any difference in presenting symptoms, or management approach. RESULTS: From the VAP database, we identified 85 patients with GUP VA. Vascular tumors (VT) were more common than vascular malformations (VM) (62/85, 72.9% vs. 23/85, 27.1%). The 62 patients with VT were subdivided into infantile hemangioma (IH) (49, 79%) and congenital hemangioma (CH) (13, 20.9%). The 23 patients with VM were subdivided into simple (18, 78.2%) and complex (2, 8.7%), and one patient had Klippel-Trenaunay syndrome. Overall, genital VA were more common than perineal (43/85, 54.2% vs. 24/85, 28.2%). None of our patients had urinary involvement. Ultrasound was used in 34% (29/85) of VA patients, the majority were VM (18/23, 78%). Majority of patients were symptomatic with ulceration, pain and bleeding (54/85, 63.5%). Malformations were more complex, presenting as extensive lesions (6/23, 26.1%), which included pelvic extension in 5 (21.7%). Seventy-six patients were managed at our institution. Treatment was utilized more frequently than observation overall (46/76, 60% vs. 30/76, 40%), and more frequently for VM than VT (12/18, 63% vs. 34/58, 57%). After introduction of oral propranolol for treatment of IH in 2010, fewer patients presented with ulceration (p = 0.213), and more were treated with propranolol over procedural therapy (p = 0.157). Overall, complete treatment of these lesions was achieved in 82.9% (63/76), with low complication rate (6/76, 7.9%). CONCLUSIONS: Evaluation and management of VA is best performed with expert multidisciplinary team at tertiary care center. Imaging studies are mostly used in evaluation of VM. Symptomatic VA involving the perineum and genitalia require therapeutic management. With the introduction of propranolol for treatment of IH, oral therapies were used more often with excellent outcomes. A multidisciplinary approach is essential to treatment innovation, patient-centered therapies and improving therapeutic outcomes.

Successful pregnancy in patients with exstrophy-epispadias complex: A University of Washington experience.

INTRODUCTION: With advances in genitourinary reconstructive surgery, women with exstrophy-epispadias complex (EEC) have improved health and quality of life, and may reach reproductive age and consider pregnancy. Despite literature suggesting impaired fertility and higher risk with pregnancy, childbirth is possible. Medical comorbidities, including müllerian anomalies, contribute to increased risk of obstetric and urologic complications during pregnancy. OBJECTIVES: We reviewed our experience with EEC patients who achieved pregnancy to investigate (1) urological characteristics of women who achieved pregnancy; (2) pregnancy management, complications, and delivery; and (3) neonatal outcomes. We developed recommendations for managing pregnancy in women with EEC. STUDY DESIGN/RESULTS: This was a retrospective chart review of 36 female patients with EEC seen at our institution between 1996 and 2013. Female patients less than 18 years, and patients who did not have documented pregnancy were excluded. This resulted in a total of 12 patients with 22 pregnancies. All women with successful pregnancy had bladder exstrophy. The majority had undergone prior bladder augmentation (75%) and were on self-catheterization programs (92%). Thirty-six percent had symptomatic urinary tract infections (UTIs) during pregnancy. Five women had more than one pregnancy. There were four terminations of pregnancy. Of 18 desired pregnancies, there were four spontaneous abortions (SABs) (22%) and 16 live births (78%). The cesarean delivery (CD) rate was 100% (14/14), of which the majority were vertical (classical) uterine incisions with a paramedian skin incision. With the exception of one patient, there were no CD surgical complications. The mean gestational age at delivery was 36 weeks (Range 25 4/7 to 39 4/7 weeks) among eight pregnancies with known gestational age. There were no stillbirths, one neonatal death and no birth defects. DISCUSSION: Women with EEC can have successful pregnancies, though at increased risk for preterm delivery and SABs. In our cohort, the rate of SAB is similar to that described in prior studies. Symptomatic UTIs likely due to self-catheterization were common. Cesarean delivery using a paramedian skin incision and classical uterine incision were not associated with major complications in this cohort. Limitations include reliance on retrospective data and small sample size. The strength of this study is the longitudinal detailed management of pregnancies in EEC women by a single team over time. A multidisciplinary approach to providing a continuum of care from pediatrics through adolescence to adulthood optimizes successful transitions, reproductive health, and successful pregnancies. Based on our experience, an algorithm providing guidance for pregnancy management was developed.

Robot-assisted laparoscopic ureteral reimplantation: a single surgeon comparison to open surgery.

OBJECTIVE: The aim was to report a single surgeon's experience comparing open and robot-assisted laparoscopic extravesical ureteral reimplantation (RALUR) to treat vesicoureteral reflux (VUR). SUBJECTS AND METHODS: We retrospectively reviewed the outcomes of RALUR and open extravesical ureteral reimplantations consecutively performed by a single surgeon between January 2008 and December 2010 using the da Vinci(®) Surgical System. Both groups of patients were subjected to identical pre- and postoperative care protocols. RESULTS: During the defined study interval, 20 open and 20 RALUR procedures were completed by a single surgeon at our institution. Gender and VUR grade were similar in both cohorts. Operative times were longer in the RALUR group, but postoperative opioid use (morphine equivalents) was significantly lower in the RALUR group (RALUR: 0.14 mg/kg, open: 0.25 mg/kg, p = 0.021). There was no significant difference in estimated blood loss (EBL) or length of hospitalization (LOH). The overall rate of surgical complications was similar; however, the complications in the open group tended to be less severe than those occurring in the RALUR group. On follow-up, after a median of 52 months for open surgery and 39 months for RALUR, two children had developed a febrile urinary tract infection in both groups, of which one in the open group had persistent VUR. CONCLUSION: This single-surgeon experience of open and initial experience with RALUR performed with the same surgical technique on consecutive cohorts with identical post-surgical care protocol allows a comparative analysis of outcomes for a surgeon transitioning to RALUR. The RALUR reduces postoperative analgesic requirements while yielding similar clinical outcomes as the open technique.

Society for fetal urology recommendations for postnatal evaluation of prenatal hydronephrosis--will fewer voiding cystourethrograms lead to more urinary tract infections?

PURPOSE: There is no consensus on the extent and mode of postnatal imaging after a diagnosis of prenatal hydronephrosis. We validated the protocol of our practice, which parallels current Society for Fetal Urology (SFU) recommendations, in limiting voiding cystourethrogram, while examining its impact on the incidence of febrile urinary tract infections. A secondary goal was to examine predictors of postnatal intervention. MATERIALS AND METHODS: We evaluated a cohort of 117 infants with prenatal hydronephrosis and retrospectively reviewed outcomes. Excluded from study were 30 infants with anatomical abnormalities. Third trimester prenatal ultrasound was done to evaluate SFU grade, laterality and anteroposterior diameter. Cox proportional hazard model and chi-square analysis were used to assess predictors of resolution and surgical intervention. RESULTS: A total of 87 infants with a median followup of 33.5 months were included in analysis. Postnatal voiding cystourethrogram was done in 52 patients, of whom 7 had vesicoureteral reflux. In 6 infants (6.9%) a febrile urinary tract infection developed, which was diagnosed with a catheter specimen during followup. In 3 infants a urinary tract infection developed immediately after catheterization. Anteroposterior diameter 9 mm or greater and SFU grade 3 or greater independently predicted the need for postnatal intervention (p = 0.0014 and 0.001, respectively). CONCLUSIONS: With adherence to our protocol, voiding cystourethrogram was avoided in almost half of evaluated infants. No infant diagnosed with vesicoureteral reflux had a urinary tract infection. Catheterization was associated with a urinary tract infection in 50% of cases. An anteroposterior diameter of 9 mm or greater and a SFU grade of 3 or greater were associated with postnatal progression to surgery. Patients with a SFU grade of 4 progressed to surgical intervention at a faster rate than those with a grade of greater than 3.

Secondary vaginoplasty for disorders for sexual differentiation: is there a right time? Challenges with compliance and follow-up at a multidisciplinary center.

PURPOSE: We present the experience of a multidisciplinary center for disorders of sexual differentiation (DSD) in treating females requiring vaginoplasty. Specifically, we evaluate outcomes and compliance with follow-up protocols in patients undergoing secondary vaginoplasties. MATERIALS/METHODS: We retrospectively reviewed consecutive DSD patients who underwent feminizing genitoplasties in 2006-2010. A subset of patients were instructed in vaginal self-dilation at time of secondary vaginoplasty. Through follow-up visits and administered questionnaires we assessed outcomes, compliance and overall satisfaction. RESULTS: Twenty-two feminizing genitoplasties were completed during the study interval. There were no postoperative complications; average blood loss was 74 ml and mean length of stay was 3 days. Ten females underwent secondary vaginoplasty. The response rate to questionnaires was 5 of 9. Age of initiation for self-dilation ranged from 8 to 24 years. Initial compliance two months postoperatively was good, but diminished 12-24 months after surgery. Responses to the quality-of-life questionnaire were diverse, reflecting a range of patient ages and varied experiences. CONCLUSION: A multidisciplinary, comprehensive approach is necessary to care for patients with DSD due to psychosexual, medical and reconstructive concerns. A secondary vaginoplasty to facilitate menarche and psychosocial concerns is feasible with minimal complications, though compliance and toleration with regard to post-surgical dilation regimens is variable.

De Castro technique used to create neophallus: a case of aphallia.

We report on a rare case of aphallia in an X, Y-born male treated at our institution. The child underwent phalloplasty at 5 years of age using the De Castro phalloplasty technique and an abdominal skin flap. Although moderate distal necrosis was seen, the overall immediate and 12-month postoperative results were highly encouraging for our patient. Thus, this technique should be seen as a temporizing reconstructive option for patients with aphallia. Long-term follow-up is necessary to determine whether the neophallic size augments with axial growth and the need for additional intervention to facilitate onset of sexual activity.