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一名46, XX性发育障碍卵睾体患者的诊断与手术决策:病例报告

Diagnosis and surgical decision-making of a 46, XX ovotesticular disorders of sex development patient: a case report.

作者信息

Zhao Hanxing, Chen Zhixing, Wang Baoyun, Zhang Zhenyu, Li Zhengyong

机构信息

Department of Burn and Plastic Surgery, West China Hospital, Sichuan University, Chengdu, China.

Department of Plastic Reconstructive and Aesthetic Surgery, West China Tianfu Hospital, Sichuan University, Chengdu, China.

出版信息

Front Surg. 2024 Dec 11;11:1502340. doi: 10.3389/fsurg.2024.1502340. eCollection 2024.

Abstract

BACKGROUND

Ovotesticular disorder of sex development is a rare form of disorder of sex development that manifests as ovotestis in individuals. The precise diagnosis and the choice of surgical procedures are still in conflict condition due to the rarity of the disease, diverse clinical presentations, and the lack of evidence-based medical studies on postoperative outcomes.

CASE PRESENTATION

We present a 46, XX ovotesticular disorder of sex development case, aged 19, with Prader stage IV virilization who underwent feminizing genitoplasty surgery. Our surgical strategy prioritized the patient's genitourinary function restoration and cosmetic reconstruction achieved satisfactory results. We attribute the success of the treatment to the systematic diagnostic process and individualized surgical planning.

CONCLUSION

The purpose of this article is to provide an evaluation protocol for the ovotesticular disorder of sex development, improving the diagnostic rate and providing some fresh ideas for surgical management.

摘要

背景

卵睾性发育障碍是性发育障碍的一种罕见形式,患者表现为卵睾。由于该疾病罕见、临床表现多样以及缺乏关于术后结果的循证医学研究,其精确诊断和手术方式的选择仍存在争议。

病例报告

我们报告一例19岁的46,XX卵睾性发育障碍患者,Prader分期为IV期男性化,接受了女性化生殖器成形术。我们的手术策略优先考虑恢复患者的泌尿生殖功能,美容重建取得了满意的效果。我们将治疗的成功归因于系统的诊断过程和个性化的手术规划。

结论

本文旨在提供一种卵睾性发育障碍的评估方案,提高诊断率,并为手术治疗提供一些新思路。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d737/11668777/2cc62853af44/fsurg-11-1502340-g001.jpg

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