[Adolescent female reproductive system dysplasia: a clinical study of 356 cases].

J L Xu, Y P Wang, X Zhang, W Liu, X F Huang, J Q Li
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Abstract

Objective: To explore the age of onset and consultation, the main clinical manifestations, common types of combined malformations, the relationship of endometriosis, surgical prognosis and different types of proportion of adolescent female reproductive system dysplasia. Methods: The medical records of 356 patients (aged 10-19) with female reproductive system dysplasia in Women's Hospital, School of Medicine, Zhejiang University from January 2003 to August 2018 were collected and retrospectively analyzed. Results: (1) Among the 356 adolescent dysplasia patients, uterine dysplasia (23.6%, 84/356), oblique vaginal septum syndrome (OVSS; 22.5%, 80/356) and vaginal dysplasia (21.6%, 77/356) were the most frequent ones, followed by multi-sectional dysplasia (16.0%, 57/356), other types of developmental abnormalities like external genitaliaand urogenital fistula (13.5%, 48/356) and Mayer-Rokitansky-Küster-Hauser syndrome (MRKH syndrome; 2.8%, 10/356). (2) There were significant differences between the median age of onset and the age of consultation of patients with OVSS and other types of abnormalities except hymen atresia (both P<0.05). In contrast, there were no significant differences between the age of onset and the age of consultation of the patients of uterine dysplasia, vaginal dysplasia, hymen atresia, MRKH syndrome and multi-sectional dysplasia (all P>0.05). (3) The clinical manifestations were lack of specificity, and mainly abnormal finding was lower abdominal pain. (4) After admission, the majority of patients underwent comprehensive cardiopulmonary examination (71.3%, 254/356) and urinary system examination (63.5%, 226/356). Only 18.3% (65/356) of patients had completed abdominal organ examination, and 5.9% (21/356) skeletal system examination. About other systemic malformations, urological malformations were the most common (27.5%, 98/356), followed by anorectal malformation (0.6%, 2/356), heart malformations (0.3%, 1/356), and spinal malformations (0.3%, 1/356). 46.4% (84/181) of the surgical patients were diagnosed with combined endometriosis. Patients with obstructive genital tract malformations were more likely to combine with endometriosis than non-obstructive ones [50.3% (74/147) vs 29.4% (10/34); P<0.05]. However, there was no significant difference between the severity of endometriosis of those two kinds (P>0.05). (5) Totally 308 patients were followed up successfully with a median of 25.0 years old, and 20 cases were treated again; 12.0% (37/308) of them were suffering from menstrual disorder and 33.1% (102/308) of them with dysmenorrhea. Totally 130 patients had sexually active reported no sexual problems. Conclusions: Uterine dysplasia, OVSS and vaginal dysplasia are the most common syndromes in adolescent female reproductive system dysplasia along with frequent cases of coexisting urinary malformations and increasing risks of endometriosis. Meanwhile, the lack of specificity of clinical manifestations might delay the timely diagnosis and treatment after the onset of symptoms. Nonetheless, most patients could achieve good surgical outcomes.

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[青少年女性生殖系统发育不良:356 例临床研究]。
目的探讨青春期女性生殖系统发育不良的发病和就诊年龄、主要临床表现、合并畸形的常见类型、与子宫内膜异位症的关系、手术预后及不同类型的比例。研究方法收集浙江大学医学院附属女子医院2003年1月-2018年8月356例(10-19岁)女性生殖系统发育不良患者的病历资料,并进行回顾性分析。结果:(1)在356例青春期发育不良患者中,以子宫发育不良(23.6%,84/356)、阴道斜隔综合征(OVSS;22.5%,80/356)、阴道发育不良(21.6%,77/356)最为多见,其次为多节发育不良(16.0%,57/356)、其他类型的发育异常,如外生殖器和泌尿生殖器瘘(13.5%,48/356)以及 Mayer-Rokitansky-Küster-Hauser 综合征(MRKH 综合征;2.8%,10/356)。(2)除处女膜闭锁外,OVSS 患者的中位发病年龄和就诊年龄与其他异常类型患者存在明显差异(PP 均>0.05)。(3)临床表现缺乏特异性,主要异常发现为下腹痛。(4)入院后,大多数患者接受了全面的心肺检查(71.3%,254/356)和泌尿系统检查(63.5%,226/356)。只有 18.3%(65/356)的患者完成了腹部器官检查,5.9%(21/356)的患者完成了骨骼系统检查。关于其他系统畸形,最常见的是泌尿系统畸形(27.5%,98/356),其次是肛门直肠畸形(0.6%,2/356)、心脏畸形(0.3%,1/356)和脊柱畸形(0.3%,1/356)。46.4%(84/181)的手术患者被诊断为合并子宫内膜异位症。生殖道阻塞性畸形患者合并子宫内膜异位症的几率高于非阻塞性畸形患者[50.3%(74/147) vs 29.4%(10/34);PP>0.05]。(5) 共有 308 例患者成功接受了随访,中位年龄为 25.0 岁,其中 20 例再次接受了治疗;12.0%(37/308)的患者患有月经紊乱,33.1%(102/308)的患者患有痛经。共有 130 名有性生活的患者表示没有性问题。结论子宫发育不良、卵巢功能不全和阴道发育不良是青春期女性生殖系统发育不良中最常见的综合征,同时还经常合并泌尿系统畸形,并增加了子宫内膜异位症的风险。同时,由于临床表现缺乏特异性,可能会延误发病后的及时诊断和治疗。尽管如此,大多数患者都能获得良好的手术效果。
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