中华妇产科杂志2024,Vol.59Issue(5) :360-367.DOI:10.3760/cma.j.cn112141-20240209-00089

青春期女性生殖系统发育异常356例临床分析

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

徐嘉聆 王亚萍 张喜 刘武 黄秀峰 李娟清
中华妇产科杂志2024,Vol.59Issue(5) :360-367.DOI:10.3760/cma.j.cn112141-20240209-00089

青春期女性生殖系统发育异常356例临床分析

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

徐嘉聆 1王亚萍 1张喜 1刘武 1黄秀峰 1李娟清1
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作者信息

  • 1. 浙江大学医学院附属妇产科医院妇科,杭州 310006
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摘要

目的 探讨青春期女性生殖系统发育异常不同类型的构成比、发病及就诊年龄、主要临床表现、常见的合并畸形、合并子宫内膜异位症(内异症)、手术治疗及预后等情况.方法 回顾性分析2003年1月至2018年8月于浙江大学医学院附属妇产科医院住院的356例青春期(10~19岁)生殖系统发育异常患者的临床资料.结果 (1)356例青春期生殖系统发育异常患者中,子宫发育异常(23.6%,84/356)、阴道斜隔综合征(22.5%,80/356)及阴道发育异常(21.6%,77/356)常见,其次为多部位发育异常(16.0%,57/356)、其他类型发育异常(13.5%,48/356)及Mayer-Rokitansky-Küster-Hauser综合征(MRKH综合征;2.8%,10/356).(2)阴道斜隔综合征、非处女膜闭锁的其他类型发育异常患者的中位发病年龄与中位就诊年龄比较,差异均有统计学意义(P均<0.05),子宫发育异常、阴道发育异常、处女膜闭锁、MRKH综合征、多部位发育异常患者的中位发病年龄与中位就诊年龄比较,差异均无统计学意义(P均>0.05).(3)6种类型青春期生殖系统发育异常患者的临床表现缺乏特异性,以下腹痛为主.(4)大部分患者入院后完善了心肺检查(71.3%,254/356)及泌尿系统检查(63.5%,226/356),仅18.3%(65/356)完善了腹腔器官检查,5.9%(21/356)完善了骨骼系统检查;完善检查后发现,青春期生殖系统发育异常合并泌尿系统畸形常见(27.5%,98/356),其次发现了合并肛门闭锁(0.6%,2/356)、右位心(0.3%,1/356)及脊柱侧弯(0.3%,1/356).46.4%(84/181)的患者术中发现合并内异症,其中梗阻性生殖系统发育异常患者比非梗阻性生殖系统发育异常患者更易合并内异症[分别为50.3%(74/147)、29.4%(10/34);P<0.05],但两者合并中~重度内异症的比例比较,差异无统计学意义(P>0.05).(5)共随访308例患者,中位随访年龄为25.0岁,其中20例患者再次手术,12.0%(37/308)的患者存在月经异常,33.1%(102/308)伴痛经,130例已有性生活的患者均未诉性生活异常.结论 青春期女性生殖系统发育异常以子宫发育异常、阴道斜隔综合征及阴道发育异常常见,且合并泌尿系统畸形者多见,同时合并内异症的风险增加.其临床表现缺乏特异性,发病后难以得到及时诊疗,但大部分患者手术治疗效果较好.

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-Kiister-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.

关键词

生殖器,女(雌)性/先天畸形/青春期

Key words

Genitalia,female/Congenital abnormalities/Puberty

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出版年

2024
中华妇产科杂志
中华医学会

中华妇产科杂志

CSTPCDCSCD北大核心
影响因子:2.752
ISSN:0529-567X
参考文献量7
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