Liu Wen,Liu Pei,Liang Haiyan,et al.Clinical characteristics of ovotesticular disorder of sex development (OT-DSD) with transverse gonad ectopia and a literature review[J].Journal of Clinical Pediatric Surgery,,():546-551.[doi:10.3760/cma.j.cn101785-20260324-00129]
Clinical characteristics of ovotesticular disorder of sex development (OT-DSD) with transverse gonad ectopia and a literature review
- Keywords:
- Ovotesticular Disorder of Sex Development; Ovotesticular Transverse Ectopia; Symptoms and Signs; Diagnosis; Urogenital Surgical Procedures; Treatment Outcome
- Abstract:
- Objective To summarize the clinical characteristics of ovotesticular disorder of sex development (OT-DSD) with transverse gonad ectopia. Methods For this case series,the authors retrospectively reviewed OT-DSD complicated by transverse ectopia (OTTE) hospitalized between January 2015 and January 2026.The following clinical data were collected:initial presentation,external masculinization score (EMS),assigned gender,sex hormone and stimulation tests,anti-Müllerian hormone (AMH) level,karyotype and gene testing,imaging findings,operative age,intraoperative findings,surgical interventions and pathological outcomes.In addition,PubMed and WANFANG were searched between January 1955 and December 2025 to identify published cases of OTTE,using terms ("ovotesticular disorders of sex development" AND "ectopia") and Chinese terms.All clinical and surgical data were extracted. Results Six children at our center had a 46,XX karyotype and all of them were SRY-negative.Five had bilateral ovotestes (5/6) assigned as boys and another one of ovotestis-ovary gonads was reared as a girl ultimately.Crossed gonads were ovotestes in all cases.Five cases showed a left ovotestis crossing to right side.The management of gonald was typically staged:first-stage gonadal biopsy by laparoscopy,followed by excision of heterologous gonadal tissue and gonadal fixation.Four children underwent staged repair except for female.Müllerian structures were preserved (n=5) and split along midline to lesson traction tension (n=1).The literature review identified four OOTE cases detected incidentally during surgical explorations.And 7 gonads were resected together with Müllerian remnants. Conclusion OTTE is latent clinically and difficult to recognize preoperatively.Due to a lack of standardized guidelines,its clinical management largely relies on multidisciplinary evaluations and individualized decision-making at experienced centers.
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Memo
收稿日期:2026-3-24。
通讯作者:张潍平,Email:zhangwp59616406@126.com