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神经保留阴蒂成形术是先天性肾上腺皮质增生症(CAH)患者的一种选择,对于青春期及成年女性患者,既往行阴蒂退缩或不完全复位后出现阴蒂疼痛时,可以选择该术式。

Nerve Sparing Clitoroplasty is an Option for Adolescent and Adult Female Patients with Congenital Adrenal Hyperplasia and Clitoral Pain following Prior Clitoral Recession or Incomplete Reduction.

机构信息

Comprehensive Center for Congenital Adrenal Hyperplasia and Institute for Pediatric Urology, Komansky Center for Children's Health, Department of Urology, New York-Presbyterian Hospital-Weill Cornell Medicine, New York, New York.

Comprehensive Center for Congenital Adrenal Hyperplasia and Institute for Pediatric Urology, Komansky Center for Children's Health, Department of Urology, New York-Presbyterian Hospital-Weill Cornell Medicine, New York, New York.

出版信息

J Urol. 2016 Apr;195(4 Pt 2):1270-3. doi: 10.1016/j.juro.2015.12.053. Epub 2016 Feb 28.

Abstract

PURPOSE

In the past many female patients with congenital adrenal hyperplasia and atypical genitalia were surgically treated with clitoral recession or incomplete reduction of erectile bodies. We report the results of repeat clitoral surgery performed for clitoral pain or enlargement using a nerve sparing reduction clitoroplasty technique.

MATERIALS AND METHODS

We identified 6 female patients with congenital adrenal hyperplasia who had undergone prior clitoral recession or incomplete reduction elsewhere. They then presented to our center with clitoral pain or enlargement, where they were treated with nerve sparing clitoroplasty between 2000 and 2010. We collected patient reported data relating to clitoral sensation and sexual function outcomes.

RESULTS

Mean ± SD age at evaluation for repeat clitoral surgery was 21 ± 7 years and mean age at clitoroplasty was 22 ± 8 years. Median postoperative followup was 9 months (IQR 32-6). All patients showed improvement with resolution of clitoral pain or enlargement.

CONCLUSIONS

Clitoral pain and enlargement upon arousal can be a major concern for women with clitoromegaly and congenital adrenal hyperplasia after clitoral recession is performed. Our series suggests that clitoral recession or incomplete reduction in childhood may be an inadequate initial solution in the congenital adrenal hyperplasia population due to the potential for future androgen elevation and the possibility of later symptom development. In addition we found that patients may be successfully treated with nerve sparing clitoroplasty, resulting in resolution of pain and ability to engage in sexual activity.

摘要

目的

过去,许多患有先天性肾上腺增生和非典型生殖器的女性患者接受了阴蒂退缩或勃起体不完全切除的手术治疗。我们报告了使用保留神经的阴蒂缩小成形术治疗阴蒂疼痛或增大的重复阴蒂手术的结果。

材料和方法

我们确定了 6 名在其他地方接受过阴蒂退缩或不完全切除的先天性肾上腺增生女性患者。她们因阴蒂疼痛或增大而在我们中心就诊,于 2000 年至 2010 年期间接受了保留神经的阴蒂成形术治疗。我们收集了与阴蒂感觉和性功能结果相关的患者报告数据。

结果

重复阴蒂手术评估时的平均年龄 ± 标准差为 21 ± 7 岁,阴蒂成形术的平均年龄为 22 ± 8 岁。术后中位随访时间为 9 个月(IQR 32-6)。所有患者的阴蒂疼痛或增大均得到改善。

结论

阴蒂增大和性唤起时的疼痛可能是接受过阴蒂退缩术的阴蒂肥大和先天性肾上腺增生女性的一个主要问题。我们的系列研究表明,由于未来雄激素升高的可能性和后期症状发展的可能性,儿童时期的阴蒂退缩或不完全切除可能是先天性肾上腺增生患者的初始解决方案不充分。此外,我们发现患者可以通过保留神经的阴蒂成形术成功治疗,从而缓解疼痛并能够进行性行为。

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