真两性畸形——附1例报告并文献复习

True hermaphroditism: one case report and review of the literature

目的总结对真两性畸形的诊断和治疗经验。方法结合文献,回顾性分析我院收治的1例真两性畸形患者的临床资料。结果本患者社会性别男性,原有先天性尿道下裂、双侧隐睾症,15岁时反复间断出现无痛性肉眼血尿,伴睾丸疼痛,喉结及阴茎、睾丸较同龄男性小,乳房较大。17岁时行盆腔探查,予子宫、阴道及右侧输卵管切除。病理诊断真两性畸形。术后1年睾丸酮水平正常,恢复良好。结论真两性畸形确诊必须依靠性腺组织学检查。性别决定以恢复性功能为目的。治疗必须切除与决定性别相抵触的性腺及所属内生殖道,并行相应外生殖器矫形术。对决定为男性者,术后要长期随诊睾丸,以防恶变。

ObjectiveTo summarize the experience of diagnosis and treatment of true hermaphroditism. MethodsTheclinical data of 1 patient with true hermaphroditism in our hospital was analyzed retrospectively in combination with relatedliterature.ResultsThe social sex of the patient was male,who was diagnosed as hypospadia and bilateral crytorchidism inchildhood.When the patient was 15 years old,painless gross hematuria occurred repeatedly with testis pain, smaller laryngealprotuberance, penis, testis and bigger breast than other boys at the same age. The patient underwent the hysterectomy,vaginectomy and right salpingectomy at the age of 17 years old. The confirmed diagnosis was maken by postoperativepathology examination. In the period of 1-year-follow-up, the patient recovered well with normal testosterone and sexualfunction.ConclusionsHistological examination of the gonad is important to the diagnosis of true hermaphroditism. Thepurpose of gender decision is to recover sexual function.The contradictory gonad and affiliated internal ductal structuresshould be removed. Genitalia orthopaedics is necessary too. The long-term follow-up to testis is important for the malepatients.