Clitoral hoodplasty in females with disorders of sex development.

INTRODUCTION: The surgical management of girls with masculinized genitalia is gradually changing towards a more conservative approach. Reports on loss of clitoral sensitivity and related impairment of sexual function in women after feminizing genital surgery in childhood have been pivotal in this evolution. An exposed clitoral glans is occasionally seen at follow-up, and while patients may complain of aesthetics, no clitoral discomfort secondary to glans exposure has been reported. A technique has been developed to reconstruct the clitoral hood and the present study reports the preliminary results. OBJECTIVES: To report the novel technique and preliminary results of clitoral hood reconstruction to cover an exposed glans after previous clitoroplasty in patients with congenital adrenal hyperplasia (CAH). PATIENTS AND METHODS: Six female patients (mean age 13, range 4-21 years) with CAH sought medical help for clitoral concerns after previous reduction clitoroplasty. In two of the six patients, the main complaint was clitoral hypersensitivity or even pain. The clitoral glans was completely exposed in all patients, who were subsequently treated with a bi-laminar V-Y clitoral hoodplasty to create a mobile and natural-looking clitoral hood composed of an inner and outer preputial skin layer. RESULTS: Postoperative covering of the glans was complete in five patients, and partial in one. The two patients with pre-operative pain became asymptomatic; all six patients were pleased with the cosmetic postoperative results. CONCLUSION: Clitoral hoodplasty provides simple, yet effective, relief for women with cosmetic concerns or clitoral discomfort after previous feminizing surgery.

Barriers to transition in young adults with neurogenic bladder.

INTRODUCTION: 'Transition' not only involves finding an adult healthcare provider, but also includes the process of developing the patient's ability to care for him/herself. Recent literature states that 40% of young adults with special healthcare needs are receiving the tools needed for transition. Pediatric urologists treating patients with complex anomalies, such as spina bifida, often anticipate poor outcomes for patients who are ill equipped for transition to adult care. The goal of this study was to identify potential barriers for young adults with neurogenic bladder when transitioning to independent care. STUDY DESIGN: A prospective IRB-approved study was performed on all patients with neurogenic bladder referred to the transitional urology clinic. Reasons for missed appointments were tracked, and all patients were asked to complete the Transition Readiness Assessment Questionnaire (TRAQ) in private prior to an appointment. The TRAQ responses are scaled 1-5, with higher numbers corresponding to higher transition readiness of each individual skill. The mean score for each question was calculated across all patients, and the mean TRAQ score was calculated across all questions for each patient. To assess if certain subgroups were more prepared for transition, mean scores were compared between sexes, patients aged <19 and ≥19 years old, and between ambulatory and full-time wheelchair users with unpaired t-tests. RESULTS: A total of 73% (58/79) of patients referred to the transitional clinic came to their appointment. The most common reason for missed clinic appointments was related to health insurance coverage (47%). A total of 42 patients completed the TRAQ at a mean age of 19.5 years old; 90% (38/42) had spina bifida. Females, ambulatory patients, and those ≥19 years old had higher overall mean TRAQ scores, but these differences were not statistically significant. The highest TRAQ scores were related to taking and ordering medications, utilization of medical supplies, communication with healthcare providers, and assisting with household duties. The majority of the patients indicated 'I am learning to do this'. The lowest scores were in response to questions about health insurance coverage, payments for medications or medical equipment, financial help, and utilization of community services. Most patients responded 'I do not know how but I want to learn'. CONCLUSIONS: Young adults with neurogenic bladder needed the most guidance during transition to independent care, with management of health insurance and finances. Based on these findings, dedicated social work and nurse visits have been included into the transition process.

Impact and frequency of extra-genitourinary manifestations of prune belly syndrome.

INTRODUCTION: Prune belly syndrome (PBS) extra-genitourinary (extra-GU) manifestations are serious comorbidities beyond the genitourinary (GU) anomalies of this disease. We hypothesized an underestimation of the reported frequency and understated impact on quality of life (QOL) of extra-GU comorbidities in PBS survivors beyond the newborn period. To assess this, the frequencies of extra-GU manifestations of PBS in a contemporary cohort of living patients were compared to compiled frequencies from published literature. Second, the impact of extra-GU PBS manifestations on patient/family QOL was assessed via a non-validated open-ended survey. MATERIAL AND METHODS: From 2010 to 2013, PBS survivors were prospectively recruited locally or at three PBS Network National Conventions. The family/subject was asked to complete a detailed PBS questionnaire, non-validated QOL survey, and provide medical records for review. Clinical data were extracted from medical records for local patients. The frequencies of extra-GU manifestations were compared between the contemporary, living cohort and a published literature cohort derived from PubMed. RESULTS AND DISCUSSION: Seven of 706 published studies met criteria for frequencies tabulation of extra-GU PBS manifestations. This largest reported living PBS patient cohort (n = 65) was 99% male with mean age 10 years (1 month-45 years). The living PBS cohort had a statistically significantly higher incidence of gastrointestinal (63%), orthopedic (65%), and cardiopulmonary (49%) diagnoses compared to the compiled published cohort (n = 204). Eleven PBS males and 32 family members completed the QOL survey. Of these, 47% listed at least one non-GU problem (i.e. lung disease, skeletal problems, constipation) as negatively affecting their QOL; 42% listed at least one GU problem (i.e. self-catheterization, recurrent UTIs) as negatively affecting their QOL; 56% reported musculoskeletal surgery and 21% reported gastrointestinal surgery/medication as positively impacting their QOL. CONCLUSIONS: In this large contemporary series, surviving individuals with PBS had a significantly higher incidence of orthopedic, gastrointestinal, and cardiopulmonary diagnoses than previously reported in PBS publications. From the patient/family QOL perspective, non-GU PBS manifestations negatively impact their QOL and treatment of these non-GU conditions improves their lives. As urologic surgeons for these medically complex patients, it is extremely important to be aware of and prepare for the high incidence of non-GU PBS comorbidities directly impacting the medical and surgical treatment and QOL of PBS patients and their families.

Non-absorbable sutures are associated with lower recurrence rates in laparoscopic percutaneous inguinal hernia ligation.

INTRODUCTION: Laparoscopic hernia repair with percutaneous ligation of the patent processes vaginalis is a minimally invasive alternative to open inguinal herniorrhaphy in children. With the camera port concealed at the umbilicus, this technique offers an excellent cosmetic result. It is also faster than the traditional laparoscopic repair with no differences in complication rates or hospital stay. The goal of this study was to describe a series of consecutive patients, emphasizing the impact of suture materials (absorbable vs. non-absorbable) on hernia recurrences. METHODS: A retrospective review was performed of consecutive transperitoneal laparoscopic subcutaneous ligations of a symptomatic hernia and/or communicating hydrocele by 4 surgeons. Patients > Tanner 2 or with prior hernia repair were excluded. The success of the procedure and number of sutures used was compared between cases performed with absorbable vs. non-absorbable suture. Risk factors for surgical failure (age, weight, number of sutures used, suture type) were assessed with logistic regression. RESULTS: 94 patients underwent laparoscopic percutaneous hernia ligation at a mean age of 4.9 years. Outcomes in 85 (90%) patients with 97 hernia repairs at a mean of 8 months after surgery revealed 26% polyglactin vs 4% polyester recurrences (p = 0.004) which occurred at mean of 3.6 months after surgery, Table 1. Repairs performed with non-absorbable suture required only 1 suture more often than those performed with absorbable suture (76% vs 60%, p = 0.163). Logistic regression revealed suture type was an independent predictor for failure (p = 0.017). Weight (p = 0.249), age (p = 0.055), and number of sutures (p = 0.469) were not significantly associated with recurrent hernia. DISCUSSION: Our review of consecutive hernia repairs using the single port percutaneous ligation revealed a significantly higher recurrent hernia rate with absorbable (26%) versus non-absorbable (4%) suture. This finding remained significant in a logistic regression model irregardless of number of sutures placed, age, and weight. Though the authors acknowledge the drawback of the potential for learning curve to confound our data, we still feel these findings are clinically important as this analysis of outcomes has changed our surgical practice as now all providers involved perform this procedure with exclusively non-absorbable suture. We thus suggest that surgeons who perform this technique, especially those newly adopting it, use non-absorbable suture for optimal patient outcomes. CONCLUSIONS: Recurrent hernia after laparoscopic percutaneous hernia ligation was significantly lower in repairs performed with non-absorbable suture. Based on this data, we recommend the use of non-absorbable suture during laparoscopic ligation of inguinal hernias in children.

Robotic resection of choledochocele in an adult with intracorporeal hepaticojejunostomy and Roux-en-Y anastomosis: encouraging progress for robotic surgical treatment of biliary disease.

BACKGROUND: Robotic surgery offers three-dimensional visualization and precision of movement that could be of great value to hepatobiliary surgeons. Previous reports of robotic choledochocele resections in adults have detailed extracorporeal jejunojejunostomies. We describe a total robotic excision of a choledochal cyst with hepaticojejunostomy and intracorporeal Roux-en-Y anastomosis. METHODS: A 58-year-old woman underwent a robotic excision of a small choledochocele with hepaticojejunostomy and intracorporeal Roux-en-Y. RESULT: Port placement was determined via collaborative surgical discussion and previously reported robotic right hepatectomies. Total operative time was 386 min and total robot working time was 330 min. The hepaticojejunostomy was performed using 5-0 PDS suture with parachute-style back wall and running front wall sutures. The jejunojejunostomy was a stapled anastomosis. Estimated blood loss was less than 100 mL. The patient was ambulating and tolerating oral intake on post-operative day 1, and was discharged home on post-operative day 2. CONCLUSIONS: Robotic resection of choledochal cyst with intracorporeal Roux-en-Y anastomosis is feasible, with advantages over open surgery such as superior visualization, precision, and post-operative patient recovery.