[Clinical characteristics of accessory cavitated uterine malformation]
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Abstract
OBJECTIVES: 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 T1 and long T2 signals in 7 cases, slightly shorter T2 signals with equal T1 values in 5 cases, equal T1 and T2 signals in 1 case, long T1 and short T2 signals in 2 cases, and long T1 and long T2 signals in 1 case. Among them, short T1 and long T2 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.
CONCLUSIONS: 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.
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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，包括左下腹痛6例、右下腹痛3例、伴同侧盆腔痛(大腿内侧、臀部或腰部放射痛)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(包括月经前、月经干净后)疼痛5例。疼痛表现为间歇性或持续性胀痛，进行性加重，部分患者夜间为甚，伴有恶心、呕吐、肛门坠胀感。月经周期正常14例，周期缩短2例；月经持续时间正常16例；经量正常14例，经量偏多1例，经量偏少1例。既往顺产6例，剖宫产2例，顺产+剖宫产2例，未生育6例。既往考虑痛经16例，子宫腺肌症1例，左侧卵巢巧克力囊肿(直径约6 cm)1例，双肾小结石并左肾轻度积水1例，尿路感染1例( 表1 )。16例患者既往均采用药物治疗，包括甾体类止痛药、中药、复方短效避孕药、地诺孕素、促性腺激素释放激素激动剂(gonadotropin-releasing hormone agonist，GnRH-a)等，采用其中一种或多种药物治疗，疼痛可暂时缓解，当病情进行性加重后治疗效果不佳。
患者的临床特点
Table 1 Clinical characteristics of the patients
确诊
年龄/岁
G：怀孕次数；P：分娩次数。
14例患者行妇科三维超声检查，2例行妇科普通超声检查。宫腔形态均正常，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；形态规则、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，囊性无回声区周边可见环形肌层包绕，囊壁内可探及类内膜回声12例。超声首先考虑诊断为ACUM 13例，肌瘤囊性变1例，囊性腺肌瘤2例( 表2 )。超声典型图像见 图1 。
患者的辅助检查情况
Table 2 Condition of accessory examination of the patients
MRI：磁共振成像；DWI：弥散加权成像；ADC：表观弥散系数；ACUM：子宫附腔畸形。
三维妇科超声图像
Figure 1 Three-dimensional gynecological ultrasound image
A: Uterine cavity was normal in shape. A kind of circular hypoechoic nodule was found in the muscle layer below the uterine horn in the left anterior wall of the uterus, with clear boundaries. B: Surrounding ring thick wall showed hypoechoic, and the sac showed no echo. UT: Uterus; M: Mass.
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 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。弥散加权成像(diffusion weighted imaging，DWI)序列呈稍高/高信号12例，未见弥散受限4例，相应表观弥散系数(apparent diffusion coefficient，ADC)图呈稍低/低信号11例，稍高信号1例，未见弥散受限4例。MRI典型图像见 图2 。增强扫描未见明显强化12例，不均匀强化4例。MRI首先考虑诊断为ACUM 9例，肌瘤出血变性5例，囊性腺肌瘤并出血2例( 表2 )。
盆腔 MRI 图像
Figure 2 Pelvic MRI image
Uterine cavity was normal in shape, and a uterine tubercle was found in the left lateral wall of the uterus, which was not communicated with the normal uterine cavity. The tubercle showed long T 1 and short T 2 signals, and short T 1 and long T 2 signals inside. MRI: Magnetic resonance imaging.
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=0.186， P =0.375； 表3 )。
妇科超声、盆腔 MRI 对首先考虑诊断为 ACUM 的一致性比较
Table 3 Comparison of consistency between gynecological ultrasound and pelvic MRI in the initial consideration of ACUM
Kappa值=0.186， P =0.375。
13例患者术前行癌抗原125(cancer antigen 125，CA125)检测，数值为12.90~91.80 U/mL，≤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例行腹腔镜下左侧卵巢巧克力囊肿剥除术后左下腹疼痛未缓解，术后半年再次行腹腔镜下子宫病损切除术。1例患者因年纪较大且无生育需求行腹腔镜下全子宫+双侧输卵管切除术。
腹腔镜下表现：12例病灶位于子宫前壁圆韧带的左下方，4例病灶位于右下方，其中5例部分凸向阔韧带。外观均似肌瘤样隆起( 图3 A)，子宫及双侧附件的外观形态未见异常，盆腔中未见子宫内膜异位病灶。宫腔镜下表现：6例宫腔形态正常，双侧宫角及输卵管开口可见。
术中的肿块情况
Figure 3 Intraoperative mass
A: After injecting posterior pituitary extract around the mass, the mass turned white. The arrow indicates the left round ligament. B: Completely resected mass has a regular shape. C: Section of the mass revealed a cystic structure, with brown, chocolate-like fluid inside the cavity. D: Mass after evacuation of intracystic fluid shows endometrial tissue on the inner cyst wall.
子宫病损切除术：术中予稀释的垂体后叶素6 U穿刺注射入肿块周围的子宫肌层来促进子宫收缩、减少手术创面出血，超声刀切开子宫前壁肿块包膜，见肿块位于子宫肌层内，边界清晰，质地中等，双极、超声刀钝锐性分离肿块表面直至将整个肿块完整剥除( 图3 B)，创缘未达子宫内膜层，予1-0或2-0可吸收线分层连续缝合子宫肌层创缘，将切除的肿块装入标本袋中取出。术中出血量为5~30(13.67±7.85) mL。术后剖视见肿块均为囊性结构，内含褐色巧克力样液体，囊壁厚为0.4~0.5 cm(图 3 C、 3 D)。
16例患者的检材组织见形成良好的腔样结构及平滑肌壁，囊壁被覆子宫内膜组织，可见子宫内膜腺体及间质，结合临床及影像学资料均诊断为ACUM。其中3例平滑肌壁内见局灶异位的子宫内膜腺体及间质，考虑ACUM合并局灶腺肌症。
通过门诊、电话或微信进行术后随访，随访时长为2~28(13.50±8.12)个月。术后疼痛症状消失15例，疼痛明显减轻1例，术后复查妇科彩色超声均未见复发证据，截至随访结束术后妊娠者2例，其中1例行早期人工流产术，1例足月顺产，未发生子宫破裂等不良妊娠情况。
讨
ACUM的发病人群多为年轻未生育女性，平均年龄为21.9(14~39)岁，30岁以上女性不到10% [ 3 ] ，主要表现为月经初潮后不久出现进行性加重的周期性下腹痛，部分可表现为偏侧性疼痛，同时伴有同侧盆腔牵涉痛、性交痛等 [ 4 ] 。然而，也有文献 [ 5 ] 报道部分患者无明显临床症状，通过影像学检查及妇科双合诊检查时意外发现。本研究患者确诊时的平均年龄为32岁，较文献[ 3 ]报道的年龄偏大，但多数患者发病年龄较早，病程较长者可达10余年，少数患者病程较短，既往剖宫产术中未发现病灶，可能是当时囊腔较小，且未表现出临床症状而难以被发现。本研究大部分患者有下腹偏侧性疼痛，少数有同侧盆腔痛，多表现为月经期或月经前后出现进行性疼痛，与文献 [ 3 ] 报道相符。当出现进行性加重的偏侧下腹痛及盆腔疼痛时，可能是囊腔内的周期性产生的液体不断积聚，囊腔内压力增大并压迫周围的感觉运动神经导致 [ 2 ] 。另外，当合并同侧泌尿系梗阻、炎症或卵巢巧克力囊肿时，疼痛源于合并症，导致治疗效果不理想。在本研究中，病例1合并同侧肾小结石及轻度肾积水，病例3合并同侧卵巢巧克力囊肿，病例8出现腰部牵涉痛并怀疑尿路感染，予以对症治疗后疼痛仍未缓解。Jain等 [ 6 ] 的报道中也有类似合并肾结石的情况，但经文献检索未发现ACUM与肾结石之间有明确关联，肾结石很可能是偶然发现的。
ACUM病灶均为单侧化，解剖部位比较固定，位于子宫前外侧壁肌层、宫角和圆韧带止点下方及子宫动脉的上方，外形多为圆形，少数为椭圆形 [ 2 , 7 ] 。病灶一般为单个腔，极少数存在双腔 [ 8 ] 。据文献 [ 9 ] 报道，囊腔病灶的平均外腔直径为22.8(0.9~ 24.8) mm，平均内腔直径为14.1(12.2~16.1) mm。囊壁为平滑肌组织，囊内含褐色巧克力样液体。本研究患者的ACUM病灶多位于左侧，其解剖位置、形态外观、囊腔大小及囊内容物性质与上述文献报道大致相符。
由于ACUM的发病率尚不明确，医生对此类疾病缺乏充分的认识，常易误诊为囊性腺肌病、子宫肌瘤囊性变等，考虑病灶较小、无明确手术指征，往往导致治疗延误。2010年Acién等 [ 10 ] 提出了ACUM的诊断标准：1)孤立的附属空腔肿块与输卵管不相通；2)正常的子宫(子宫内膜腔)、输卵管和卵巢；3)经外科切除及病理检查证实空腔肿块；4)腔内衬有子宫内膜的腺体和间质；5)腔内含巧克力样液体；6)病理上无子宫腺肌病特点(若子宫切除)，尽管在附腔周围肌层可能有小的腺肌病灶。三维妇科超声和盆腔MRI检查在明确宫腔形态、病灶的解剖位置及特殊声像表现上具有重要作用。超声通常表现为子宫圆韧带下方肌层内有一个边界清晰的厚壁囊肿，与宫腔及输卵管不相通，腔内衬子宫内膜回声，囊内液呈磨砂玻璃样回声，多普勒显示囊腔周围有血流信号，腔内无血流 [ 2 , 9 ] 。MRI表现为圆形光滑的病灶，腔内容物在T 1 WI及T 2 WI上均呈高信号，脂肪抑制T 1 WI图像无信号抑制，在T 2 WI上被一个低信号环包围，增强扫描未见增强，腔内衬子宫内膜在T 2 WI呈高信号 [ 11 - 12 ] 。本研究中，盆腔MRI提示囊内容物为短T 1 长T 2 信号，提示血性内容物，而腔壁内膜显示欠清，容易误诊为肌瘤出血变性，相比之下超声对类内膜回声的识别度好，因此超声首先诊断为ACUM的符合率较高，但需要更大样本量予以支持。病理检查是明确诊断的重要指标，表现为宫腔内衬子宫内膜腺体和间质，周围的平滑肌纤维有序排列 [ 12 ] ，病灶周围偶可检测出局部小腺肌病灶，可能是由于附腔内长时间血液积聚导致囊壁张力较大而形成。本研究中3例患者平滑肌壁内见局灶异位的子宫内膜组织，加上CA125水平稍高，符合ACUM合并局灶腺肌症的诊断。另外，免疫组织化学染色中可见CD10、雌激素受体(estrogen receptor，ER)和孕激素受体(progesterone receptor，PR)均呈阳性 [ 13 ] ，符合子宫内膜的特点。
临床上常需与以下疾病进行鉴别。1)囊性腺肌病：多见于高龄多产的妇女，腺肌瘤通常与子宫其他部位的腺肌病灶相融合，常累及或靠近子宫内膜-肌层交界处，边缘不规则，形成的囊性成分含多个小的区域(1 cm)，残角子宫内含有内膜，与单角子宫腔不相通，多表现为周期性疼痛 [ 14 - 15 ] 。可采用三维超声、宫腔镜检查或子宫输卵管造影进行鉴别诊断。4)Robert子宫：不对称性纵隔子宫，另一半宫腔呈单角状，宫腔镜检查仅见一侧输卵管开口，腹腔镜下子宫轮廓外形正常或有小凹陷 (<1 cm)，月经初潮后因腔内积血闭锁，可出现进行性加重的痛经 [ 16 ] 。痛经症状与ACUM、II型残角子宫类似，通常为偏侧性，多从月经初潮后开始出现，采用三维超声、宫腔镜或输卵管造影可鉴别。5)宫角妊娠：ACUM病灶存在于一侧圆韧带下方，靠近宫角处，当腔内观察到子宫内膜的蜕膜变化时可能被误诊为宫角妊娠 [ 17 - 18 ] ，但宫角妊娠病灶存在血流信号，达到一定孕周后可观察到卵黄囊、胚芽及心管搏动等声像。6)卵巢巧克力囊肿：靠近阔韧带的卵巢巧克力囊肿可能在出现单侧出血内容物、周期性盆腔疼痛的情况下被误诊为ACUM，巧克力囊肿位于卵巢，容易通过卵巢血管或邻近卵泡的存在而被发现，且周围没有较厚的平滑肌组织环 [ 7 ] 。
药物治疗可选择GnRH-a、口服避孕药或孕激素等，疼痛症状可能不会完全缓解，通常在治疗结束时迅速复发 [ 19 ] 。本研究患者既往均采用药物保守治疗，但病情进行性加重或停药后治疗无效。硬化疗法通常是将硬化剂往腹腔内注射，引起囊壁炎症、破坏和纤维化，可能导致囊腔闭塞来达到治疗的目的 [ 2 ] 。硬化治疗可避免形成瘢痕子宫，对于想尽快妊娠或不想手术的患者可能是一种可行的选择，但仍存在复发风险，且无法获得病理结果。Naftalin等 [ 9 ] 报道了4例ACUM经阴道超声引导下的乙醇硬化治疗，1例治疗6个月后症状复发选择腹腔镜切除。目前手术治疗是首选方案，包括宫腔镜、腹腔镜和开腹手术。Pontrelli等 [ 20 ] 报道了宫腔镜下成功切除ACUM的案例，这可能是一种新的治疗方式的探索，手术时间短，创伤小，但如果附腔切除不全可能存在复发风险，没有缝合加固子宫肌层可能对年轻患者未来的妊娠结局产生影响。腹腔镜下切除ACUM比开腹手术创伤小，是目前最常见、最有效的治疗方法。术中要注意保护年轻患者的生育力，完整切除肿块，子宫圆韧带应尽可能保留，避免损伤子宫动脉，创面缝合充分，避免发生不良生育结局 [ 2 , 21 - 22 ] 。年纪较大且无生育需求的患者可选择子宫切除术。本研究多数采用腹腔镜下子宫病灶切除术，术中根据解剖层次小心完整地剥离出子宫附腔，可减少内膜残留导致病情复发，术后效果满意，1例患者足月妊娠顺产。术后发生子宫破裂等不良妊娠情况与创面深度、缝合情况及术后避孕时间等可能有一定关系，未来仍需更多病例予以支持佐证。
综上所述，ACUM是一种特殊的梗阻性疾病，容易与囊性腺肌病、子宫肌瘤囊性变等疾病混淆，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。
对象与方法
本研究已获得中南大学湘雅三医院伦理委员会批准(审批号：快25318)。
选取中南大学湘雅三医院妇科2023年5月至2025年11月收治的经临床、影像学资料和病理检查确诊为ACUM的16例患者作为研究对象。排除标准为临床资料大部分缺乏的患者。
回顾性收集16例患者的临床资料，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SPSS 27.0统计学软件进行统计与分析。计数资料以例(%)表示，符合正态分布的计量资料以均数±标准差表示，采用Kappa检验进行2种辅助检查诊断的一致性检验。 P <0.05为差异有统计学意义。
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- europepmc
- last seen: 2026-06-04T01:30:01.192114+00:00
- pmc
- last seen: 2026-05-13T20:22:03.195721+00:00
- pubmed
- last seen: 2026-06-04T00:30:14.435327+00:00
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Courtesy of the U.S. National Library of Medicine
Courtesy of the U.S. National Library of Medicine