摘要
目的:子宫附腔畸形(accessory cavitated uterine malformation,ACUM)是一种先天性苗勒管发育异常,临床上多见于年轻女性,常发生进行性下腹痛,由于病灶较小、医师对此类疾病缺乏充分的认识,漏诊及误诊率高,常导致治疗延误。本研究旨在探讨子宫附腔畸形的临床特征、诊治方式及预后情况,总结相关经验,为临床诊治提供更多参考依据。方法:回顾性分析2023年5月至2025年11月在中南大学湘雅三医院妇科收治的16例ACUM患者的临床资料,包括年龄、临床表现、既往史、月经及生育史、肿瘤标志物、影像学检查、治疗方式、病理检查及预后情况等。采用Kappa检验进行2种辅助检查诊断的一致性检验。结果:16例患者确诊年龄为23~53(32.00±7.56)岁,病程为5个月~12年。临床表现为下腹痛16例,其中左下腹痛6例、右下腹痛3例、伴同侧盆腔痛2例;月经期疼痛10例,经期疼痛后改为非经期疼痛1例,非经期疼痛5例。16例患者均行妇科彩色超声检查,病灶位于子宫左前壁宫角下方肌层内12例,位于子宫右前壁宫角下方肌层内4例,结节呈低回声4例,混合回声12例,边界清晰13例,边界欠清晰3例,结节最大径为17~38(28.31±6.04) mm,囊内无回声区最大径为5~29(18.63±6.77) mm,囊壁内可探及类内膜回声12例。16例患者均行盆腔磁共振成像(magnetic resonance imaging,MRI)平扫+增强检查,结节病灶呈短T_(1)长T_(2)信号7例,等T_(1)稍短T_(2)信号5例,等T_(1)等T_(2)信号1例,长T_(1)短T_(2)信号2例,长T_(1)长T_(2)信号1例,其中结节内呈短T_(1)长T_(2)信号7例。16例患者首先考虑诊断为ACUM的妇科超声的符合率为81.25%,盆腔MRI的符合率为56.25%,2种检查方法的判断一致性强度较弱(Kappa值=0.186,P=0.375)。13例行癌抗原125(cancer antigen 125,CA125)检测,数值为12.90~91.80 U/mL,其中≤35 U/mL的10例,>35 U/mL的3例。15例患者采用腹腔镜下子宫病损切除术(其中6例同时行宫腔镜检查),1例因年纪大且无生育需求行腹腔镜下全子宫+双侧输卵管切除术。结合病理检查及临床、影像学资料,16例患者均诊断为ACUM,其中3例考虑合并局灶腺肌症。术后随访时长为2~28(13.50±8.12)个月,术后疼痛症状均消失15例,疼痛明显减轻1例,术后妊娠足月顺产1例。结论:ACUM是一种特殊的梗阻性疾病,容易与囊性腺肌病、子宫肌瘤囊性变等混淆,当临床上出现年轻女性进行性下腹痛,特别是偏侧性疼痛及伴随盆腔牵涉痛时,需高度怀疑ACUM,推荐采用三维妇科超声、盆腔MRI进行辅助诊断,首选腹腔镜下子宫病灶切除术来根治此类疾病,必要时行宫腔镜检查来鉴别诊断。
Objective:Accessory cavitated uterine malformation(ACUM)is a congenital Müllerian duct developmental anomaly.Clinically,it is commonly observed in young women presenting with progressive lower abdominal pain.Due to the small size of the lesions and insufficient awareness of this condition among clinicians,the rates of missed diagnosis and misdiagnosis are relatively high,often leading to delayed treatment.This study aims to explore the clinical characteristics,diagnostic and therapeutic approaches,and prognosis of ACUM,summarize relevant clinical experience,and provide references for clinical diagnosis and management.Methods:A retrospective analysis was conducted on the clinical data of 16 patients with ACUM who were admitted to the Department of Gynecology,the Third Xiangya Hospital of Central South University from May 2023 to November 2025.The collected data included age,clinical manifestations,medical history,menstrual and reproductive history,tumor markers,imaging findings,treatment methods,pathological results,and prognosis.The Kappa test was used to evaluate the diagnostic consistency between two auxiliary imaging modalities.Results:The age at diagnosis ranged from 23 to 53 years[(32.00±7.56)years],and the disease duration ranged from 5 months to 12 years.All 16 patients presented with lower abdominal pain,including left lower abdominal pain in 6 cases,right lower abdominal pain in 3 cases,and ipsilateral pelvic pain in 2 cases.Dysmenorrhea occurred in 10 patients,pain initially associated with menstruation that later became non-menstrual pain occurred in 1 patient,and non-menstrual pain occurred in 5 patients.All 16 patients underwent gynecologic color Doppler ultrasonography.Lesions were located within the myometrium beneath the uterine cornual region of the left anterior uterine wall in 12 cases and the right anterior uterine wall in 4 cases.The nodules showed hypoechoic signals in 4 cases and mixed echogenicity in 12 cases.Clear boundaries were observed in 13 cases,while indistinct boundaries were observed in 3 cases.The maximum diameter of the nodules ranged from 17 to 38 mm[(28.31±6.04)mm]and the maximum diameter of the anechoic area within the cyst ranged from 5 to 29 mm[(18.63±6.77)mm].Endometrium-like echoes within the cyst wall were detected in 12 cases.All 16 patients underwent pelvic magnetic resonance imaging(MRI)with plain and contrast-enhanced scans.The nodular lesions showed short T_(1) and long T_(2) signals in 7 cases,slightly shorter T_(2) signals with equal T_(1) values in 5 cases,equal T_(1) and T_(2) signals in 1 case,long T_(1) and short T_(2) signals in 2 cases,and long T_(1) and long T_(2) signals in 1 case.Among them,short T_(1) and long T_(2) signals were indicated within the nodules in 7 cases.The diagnostic coincidence rate for ACUM was 81.25%with gynecological ultrasonography and 56.25%with pelvic MRI.The agreement between the 2 diagnostic modalities was weak(Kappa=0.186,P=0.375).A total of 13 patients underwent cancer antigen 125(CA125)testing,with values ranging from 12.90 to 91.80 U/mL.Among them,10 cases had CA125≤35 U/mL and 3 cases had CA125>35 U/mL.A total of 15 patients underwent laparoscopic resection of uterine lesions(including hysteroscopy in 6 cases),while 1 patient underwent laparoscopic total hysterectomy with bilateral salpingectomy due to advanced age and no reproductive requirement.Based on pathological examination combined with clinical and imaging findings,all 16 patients were diagnosed with ACUM,including 3 cases suspected of concomitant focal adenomyosis.The postoperative follow-up duration ranged from 2 to 28 months[(13.50±8.12)months].Postoperative pain symptoms disappeared in 15 patients and were significantly relieved in 1 patient.1 patient achieved full-term vaginal delivery after surgery.Conclusion:ACUM is a special type of obstructive disease that can easily be confused with cystic adenomyosis or cystic degeneration of uterine fibroids.When young women present with progressive lower abdominal pain,especially unilateral pain accompanied by referred pelvic pain,ACUM should be highly suspected.Three-dimensional gynecological ultrasonography and pelvic MRI are recommended for auxiliary diagnosis.Laparoscopic resection of uterine lesions is the preferred treatment for radical management of this condition,and hysteroscopy may be performed when necessary for differential diagnosis.
作者
黄宗燕
陈佩琳
胡维维
吴诗莹
向慧敏
蒋建发
肖松舒
HUANG Zongyan;CHEN Peilin;HU Weiwei;WU Shiying;XIANG Huimin;JIANG Jianfa;XIAO Songshu(Department of Gynecology,Third Xiangya Hospital,Central South University,Changsha 410013;Department of Gynecology,Guangxi Hospital Division of the First Affiliated Hospital,Sun Yat-sen University,Nanning 530022;Xiangya School of Public Health,Central South University,Changsha 410013,China)
出处
《中南大学学报(医学版)》
北大核心
2026年第1期169-178,共10页
Journal of Central South University (Medical Science)
基金
国家卫生健康委员会医药卫生科技发展研究中心课题(WKZX2024GQ0202)
湖南省卫生健康科研课题重点项目(20257411)。
关键词
子宫附腔畸形
梗阻性疾病
临床特征
诊治方式
预后
accessory cavitated uterine malformation
obstructive disease
clinical characteristics
diagnosis and treatment method
prognosis
