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先天性肾上腺皮质增生症患儿会阴成形术术后长期随访结果
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Abstract:
目的：了解先天性肾上腺皮质增生症患儿接受会阴成形术后外生殖器外观的长期结果。研究方法：回顾性研究44名既往于我院诊断为先天性肾上腺皮质增生症患儿行生殖器女性化手术长期的外生殖器外观结果。研究结果：共有29例(65.9%)患者报告了外观问题，包括阴蒂肥大、大阴唇融合或褶皱、小阴唇冗长或缺失等。在计划一期手术的39例生殖器成形术中，有1例(2.6%)因术后尿道阴道瘘行修补手术，11例(25%)外生殖器外观明显异常达二次手术指征。结论：先天性肾上腺皮质增生症导致的生殖器性别模糊无法通过一次手术解决问题，部分患儿需要进一步治疗。
Objective: To discover the long-term outcome of external genital appearance after perineoplasty in children with congenital adrenal hyperplasia. Methods: A retrospective study was conducted on the long-term external genital appearance of 44 children who were diagnosed as congenital adrenal hyperplasia and had previously undergone genital feminization in our hospital. Results: A total of 29 patients (65.9%) reported appearance problems, including clitoral hypertrophy, labia majora wrinkle or fusion, and labia minora hypertrophy or deficiency. Among the 39 cases of patients who received genitoplasty scheduled for primary surgery, 1 case (2.6%) underwent repair surgery due to postoperative urethrovaginal fistula, and 11 cases (25%) had significant abnormal appearance of external genitalia indicating secondary surgery. Conclusion: Genital gender ambiguity caused by congenital adrenal hyperplasia cannot be resolved by a single operation, and some children need further treatment.
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