Sexual function in adult females after feminizing genitoplasty for congenital adrenal hyperplasia.

OBJECTIVE: To investigate sexual function and subjective attitudes towards surgery in adult women after feminizing genitoplasty (FG) for congenital adrenal hyperplasia (CAH). MATERIALS AND METHODS: We retrospectively reviewed the medical files of patients who underwent FG from 1996 to 2018. Of those, patients older than 16 years of age were asked to answer non-standardized questions aiming at the awareness of genital surgery and standardized questionnaire evaluating sexual function (GRISS). The anonymized answers were compared to a control group of 50 healthy females. Student's t-test, Pearson's χ2 test, Fisher's exact test and Spearman's rank correlation coefficient were performed. A P-value less than 0.05 was considered significant. RESULTS: Out of 106 patients who underwent FG, 64 were included, 55 successfully contacted and 32, aged 17-40 years (median 25.5), answered the questionnaires (58% response rate). In all, 11 patients underwent historical clitorectomy in early childhood by a pediatric gynecologist before 1996 and pull-through vaginoplasty at puberty. A total of 21 patients underwent modern one- or two-staged FG. There were statistically significant differences between the CAH group and controls as to the proportion living in committed partnerships (28% and 84%, respectively; p = 0.0000), having sexual intercourse (41% and 92%, respectively; p = 0.0000) and having their own children (13% and 36%, respectively; p = 0.0369). The median overall GRISS score was 4 (range 1-7) in the CAH group compared to 1 (range 1-9) in the control group, with infrequency and vaginismus representing the least favorable subscales (median 5). In all, 81% of the women operated on for CAH would recommend such an operation to any other individual with CAH and 66% of them suggested the surgery should be performed in early childhood. DISCUSSION: By this study we have contributed to the debate over suggestions that FG may cause adverse effects on sexual life of the patients in adulthood, beside already published effects on their mental well-being and LUTS. Despite possible selection bias due to only 58% response rate the comparison with control group shows only a moderate sexual dysfunction and more significant impact on long-term partnership that might be caused by other factors related to CAH beside FG itself. CONCLUSIONS: Partnership, offspring, sexual frequency and function were impaired in adult females with CAH after FG. However, the median GRISS score was below the pathological level of 5.

Lung Resection in Children with Necrotizing Pneumonia: Outcome and Follow-up.

INTRODUCTION: The number of patients with necrotizing pneumonia has increased in recent years. The aim of this study is to review the incidence, management, and outcome of pediatric necrotizing pneumonia requiring surgical therapy and to prove that lung resection results in favorable development of patients. We hypothesize that overall lung function in children after lung resection does not differ from that of the healthy population. MATERIALS AND METHODS: A retrospective tertiary referral center study with a prospective follow-up spirometric study of patients with necrotizing pneumonia managed between January 2010 and December 2019 was performed. RESULTS: The study cohort consisted of 1,295 patients admitted to the pediatric department for community-acquired pneumonia; 47 patients developed necrotizing pneumonia, 36 of whom underwent parenchymal lung resection. A 5-year rise in the occurrence of necrotizing pneumonia requiring resection was 77%, with a significant increase in the last 3 years (p < 0.05). The median age at the time of surgery was 32.5 (interquartile range [IQR]: 32.25) months. Streptococcus pneumoniae was the most prevalent pathogen (83%), although 53.3% of these patients were vaccinated against the agent. In 67% of patients, preresection procedures were performed: drainage of pneumothorax (17%), drainage of empyema (46%), drainage of empyema with use of alteplase (25%), and thoracoscopic decortication (12%). Surgical procedures included lobectomy (72.2%), wedge resection (13.9%), bilobectomy (8.3%), and pneumonectomy (5.6%). The postoperative complication was bronchopleural fistula in three patients. There were two (5.5%) postoperative deaths due to multiple organ failure. The follow-up spirometry was performed 43.3 (median, IQR 23.8-66.7) months after surgical intervention. Normal lung function was detected in 35 (64.8%) patients, restrictive pattern in 6 (11.1%) patients, obstructive pattern in 11 (20.4%) patients, and combined in 2 (3.7%) patients. CONCLUSION: The number of patients with necrotizing pneumonia requiring resection has increased significantly in the last 3 years (p < 0.05). Aggressive surgical treatment results in significant clinical improvement in most cases and favorable lung function outcome. Long-term follow-up showed normal spirometry in 64.8% of cases.

Lower Urinary Tract Symptoms in Adult Females after Feminizing Genitoplasty for Congenital Adrenal Hyperplasia.

PURPOSE: We investigated quality of life, long-term lower urinary tract symptoms, sexual function and subjective attitudes toward surgery in adult women after feminizing genitoplasty for congenital adrenal hyperplasia. MATERIALS AND METHODS: We retrospectively reviewed the medical files of all patients with congenital adrenal hyperplasia who underwent feminizing genitoplasty from 1996 to 2018 in our tertiary center. Of those, patients older than 16 years of age were asked to answer 1 nonvalidated and 3 standardized and validated questionnaires evaluating their current mental well-being (WHO-5 Well-Being Index), lower urinary tract symptoms (ICIQ-FLUTS) and sexual function (GRISS). The anonymized answers of this cross-sectional study were compared to a control group of 50 healthy females. Student's t-test, Pearson's χ2 test, Fisher's exact test and Spearman's rank correlation coefficient were performed. A p-value less than 0.05 was considered significant. RESULTS: Out of 106 patients who underwent feminizing genitoplasty, 64 patients were included and 32 patients, aged 17 to 40 years (median 25.5 years), answered the questionnaires (50% response rate). The difference between congenital adrenal hyperplasia and control group mental well-being was not statistically significant (WHO-5 median score 60 and 64, respectively; p=0.82). We found no significant difference in the lower urinary tract symptoms subscales of filling, voiding or incontinence, nor in the overall lower urinary tract symptoms score (ICIQ-FLUTS overall median score 3.5 and 3, respectively; p=0.43). CONCLUSIONS: We found in our group no abnormal mental well-being or prevalence of long-term symptoms of lower urinary tract dysfunction in adult female patients with congenital adrenal hyperplasia following feminizing genitoplasty.

Gastric tumors in children: single-center study with emphasis on treatment of repeated recurrence.

PURPOSE: Analysis of surgical management and survival of pediatric patients with gastric tumors treated at our institution. METHODS: A retrospective study of patients with primary gastric tumors treated between 1993 and 2018 was conducted. RESULTS: Eight patients, five girls and three boys, were diagnosed with gastric tumors at an average age of 10.4 years (1 day-15.4 years). Surgical management included Billroth type I procedure in five and tumor excision in three patients. Histology revealed gastrointestinal stromal tumor (GIST) in four patients and one of each of schwannoma, myofibroblastic tumor, hamartoma and teratoma. Microscopically clear margins were reported in six patients. Repeated local recurrence occurred in three patients (2 × GIST, 1 × myofibroblastic tumors) who consequently underwent three, four and six reoperations. One of these patients had liver metastases, which were managed with ligation of the hepatic arteries. This patient was also diagnosed with a lung hamartoma, which was treated with a lobectomy. Survival rate was 100% with a median follow-up of 8.6 years (7 months-25.5 years). CONCLUSIONS: Gastric tumors are rare in children and represent a management challenge. Repeated recurrence of GISTs and myofibroblastic tumors remains frequent even after complete resection and may necessitate multiple surgeries, therefore patients require a lifelong follow-up.