Predictors of surgical complications in boys with hypospadias: data from an internationa registry.

Background: Complications are frequently reported after hypospadias repair and there is a need to understand the factors that influence their occurrence. Methods: Data from boys with hypospadias born between 2000 and 2020 were obtained from the International Disorders of Sex Development (I-DSD) Registry. Logistic regressions, fisher's exact tests and spearman's correlation tests were performed on the data to assess associations between clinical factors and complication rates. Results: Of the 551 eligible boys, data were available on 160 (29%). Within the cohort, the median (range) External Masculinization Score (EMS) was 6 (2, 9). All presented with one or more additional genital malformation and 61 (38%) presented with additional extragenital malformations. Disorders of androgen action, androgen synthesis and gonadal development were diagnosed in 28 (18%), 22 (14%) and 9 (6%) boys, respectively. The remaining 101 (62%) patients were diagnosed as having non-specific 46,XY Disorders of Sex Development. Eighty (50%) boys had evidence of abnormal biochemistry, and gene variants were identified in 42 (26%). Median age at first hypospadias surgery was 2 years (0, 9), and median length of follow-up was 5 years (0, 17). Postsurgical complications were noted in 102 (64%) boys. There were no significant associations with postsurgical complications. Conclusions: Boys with proximal hypospadias in the I-DSD Registry have high rates of additional comorbidities and a high risk of postoperative complications. No clinical factors were significantly associated with complication rates. High complication rates with no observable cause suggest the involvement of other factors which need investigation.

Non-invasive evaluation of botulinum-A toxin treatment efficacy in children with refractory overactive bladder.

OBJECTIVE: We aimed to evaluate the efficacy and safety of intravesical onabotulinum toxin A (onaBoNTA) injections for the treatment of children diagnosed with refractory overactive bladder (OAB) by using non-invasive methods. METHODS: A total of 31 pediatric patients with a mean age of 10.2 years received intravesical onaBoNTA injection at the dose of 10 U/kg (max: 200 U). Twenty-one patients who failed to respond to the first injection, received second injection 6 months after the first one. The patients were retrospectively evaluated after the 1st and the 2nd injections by means of standardized questionnaire forms and voiding diary records. RESULTS: In the 6-month follow-up, 10 patients (32.2%) were determined to have full response after the first injection. The number of patients with partial response and no response were found to be 15 (48.4%) and 6 (19.4%), respectively. The mean value of Dysfunctional Voiding and Incontinence Symptom Score (DVISS) of the patients with full response was 8.5 before the injection, which decreased to 1 at 6-month follow-up and to 0.5 at 12-month follow-up (p < 0.001). Twenty-one patients without full response after the first injection received a second injection 6 months after the first one. Full response was achieved in 9 (42.9%) of the 21 patients who had a second injection, but only partial response was achieved in 9 (42.9%). Three (14.3%) of the remaining patients did not respond to the second injection either. 1-year follow-up evaluations revealed that the rates of the full response, partial response, and no response were 61.3, 29, and 9.7%, respectively. CONCLUSIONS: Based on our results, onaBoNTA therapy is an effective and reliable second-line off-label therapy in the management of patients with non-neurogenic OAB that is refractory to medical therapy. Asking the patients/guardians to fill out a standardized questionnaire form before and after the therapy enables easy and non-invasive assessment of the response to the therapy.

Sacrococcygeal Teratoma associated with Trisomy 13.

Sacrococcygeal teratoma (SCT) is rarely associated with syndromes. We report a female newborn with a prenatal diagnosis of small sacrococcygeal teratoma and postnatally diagnosed as having trisomy 13. The sacrococcygeal teratoma was excised. It was reported as mature teratoma. The child succumbed to sepsis postoperatively.

[Spontaneous bile duct perforation: a rare cause of acute abdominal pain during childhood]. / Spontan koledok perforasyonu: Cocukluk çaginda nadir bir akut karin nedeni.

BACKGROUND: Spontaneous perforation of the bile duct (SPBD) is a rare cause of acute abdominal pain during childhood. Pancreatico-biliary malfunction has been postulated to contribute to its etiology. Factors related to diagnosis and treatment and difference from the other common causes of acute abdominal pain are emphasized. METHODS: Five patients (3 boys, 2 girls, mean age 4.6) were admitted with peritonitis and operated with initial diagnosis of perforated appendicitis. During laparotomy, SPBD was detected. Presentation, laboratory findings and operative technique of the patients were evaluated retrospectively. RESULTS: Common complaints were abdominal pain and bilious vomiting. Abdominal distention was present in all patients. Leukocytosis and mild hyperbilirubinemia were detected in 5, elevated serum transaminase levels in 4, hyperglycemia in 1 and constipation in 1 patient(s). Abdominal ultrasonography showed a large amount of free fluid. During laparotomy, sterile bile peritonitis was detected initially. After exploration, SPBD was seen. T-tube drainage of the bile duct was carried out. Patients were discharged after removal of the T-tubes. Pancreatico-biliary malfunction was detected in 4 of 5 patients. CONCLUSION: In patients with generalized peritonitis, elevated transaminase levels and hyperbilirubinemia, SPBD must be considered. Even though the T-tube drainage is the treatment of choice, Roux-en-Y hepatico-portoenterostomy may be mandatory in certain patients.