Osifo Osarumwense D, Amusan Taiwo I
Pediatric Surgery Unit, Department of Surgery, University of Benin Teaching Hospital, Benin City, Nigeria.
Afr J Reprod Health. 2009 Dec;13(4):129-36.
Congenital aberrations of female children's external genitalia are common worldwide with varied mode of presentation especially in regions with poor awareness. This prospective experience between July 2004 and June 2008 at two Nigerian healthcare facilities is on the mode of presentation and challenges of management of female children with ambiguous genitalia. Congenital adrenal hyperplasia (CAH) 19 (47.5%), female pseudohermaphroditism 20 (50%) and vaginal atresia 1 (2.5%) manifested as aberrations of external genitalia of 40 female children who presented between the ages of 3 months and 16 years (average 9 years). Cultural influence, lack of awareness, inadequate examination of external genitalia at birth and lack of diagnostic facilities resulted in late presentation and diagnosis with all the cases of CAH and pseudohermaphroditism raised as males. Five cases who developed female secondary sexual characteristics at puberty attempted suicide before presentation. Gender reassignment and feminizing genitoplasty were major challenges, but outcomes were encouraging.
女童先天性外生殖器畸形在全球范围内都很常见，其表现形式多样，尤其是在认知度较低的地区。2004年7月至2008年6月在尼日利亚两家医疗机构的这项前瞻性研究，关注的是生殖器模糊女童的表现形式及管理挑战。19例（47.5%）先天性肾上腺皮质增生症（CAH）、20例（50%）女性假两性畸形和1例（2.5%）阴道闭锁表现为40名年龄在3个月至16岁（平均9岁）女童的外生殖器畸形。文化影响、认知不足、出生时对外生殖器检查不充分以及缺乏诊断设施导致就诊和诊断延迟，所有CAH和假两性畸形病例最初都按男性抚养。5例在青春期出现女性第二性征的患者在就诊前企图自杀。性别重新分配和女性化生殖器整形是主要挑战，但结果令人鼓舞。
