Reconstructive surgery for females with congenital adrenal hyperplasia due to 21-hydroxylase deficiency: a review from the Prince of Wales Hospital

Abstract

Objectives: To present the results of feminising genitoplasty done in female patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Design: Case series.Setting: A tertiary referral centre in Hong Kong.Patients: Female patients with congenital adrenal hyperplasia undergoing corrective surgery for virilisation between 1993 and 2012. Main outcome measures:The operative result was judged with a scoring system (1-3) for four areas: appearance of clitoris, labia and vagina, plus requirement for revision surgery.Results: A total of 23 female patients with congenital adrenal hyperplasia with a median age of 17.5 (range, 1.5-33.8) years were identified. Of these individuals, 17 presented in the neonatal period and early infancy, of which four had an additional salt-losing crisis. Six patients-including four migrants from mainland China-were late presenters at a median age of 2 (range, 0.5-14) years. Twenty-two patients had corrective surgery at a median age of 2 (range, 1-14) years. Clitoral reduction was performed in all, and further surgery in 21 patients. The additional surgery was flap vaginoplasty in 10 patients, a modified Passerini procedure in six, and a labial reconstruction in five; one patient with prominent clitoris was for observation only. Minor revision surgery (eg mucosal trimming) was required in three patients; a revision

The choice of feminizing genitoplasty tactic in girls with congenital adrenal hyperplasya

“…Сравнительные когорты зачастую включают различные хирургические методы, от некоторых из которых уже отказались в современной генитальной хирургии [17]. Большинство авторов сообщают о высоком уровне хороших результатов -от 79 до 95% в отдаленном периоде в среднем от 19 мес до 21 года [18][19][20][21][22][23]. Barbara Dobrowolska-Glazar в 2020 г. представила исследование 9 пациенток с ВДКН со средним периодом наблюдения 11 лет [24].…”