血管内平滑肌瘤病子宫外超声表现

Analysis of ultrasonic manifestation of intravascular leiomyomatosis expanding beyond uterus

目的探讨血管内平滑肌瘤病子宫外的超声声像图特点,减少对血管内平滑肌瘤病的漏误诊。方法选取2014年7月1日至2018年7月1日在北京协和医院经术后病理证实的43例血管内平滑肌瘤病的住院患者,均伴有超声可见的静脉内占位。分析患者的临床资料及超声声像图特点,主要观察内容包括患者的临床症状,病变的大小、长度、累及范围、回声类型以及病变内部血流信号等特点。43例均为女性,其中27例既往曾行子宫肌瘤剔除术或全子宫切除术(7例子宫肌瘤剔除术后,20例子宫全切术后),另外16例既往无相关妇科手术史。结果临床症状:11例患者首发症状为呼吸困难和心悸,11例出现腹胀和腹痛,6例出现下肢水肿,5例发现盆腔包块,2例出现发作性晕厥,2例出现不规则阴道出血,1例出现大小便频繁,另有5例无任何症状。超声表现:43例患者中均可于下腔静脉或者髂静脉内见条索样低回声占位,其中术前超声正确诊断血管内平滑肌瘤病者42例。病灶长度不一,最长者从盆腔经由髂静脉到下腔静脉延伸至右心房,彩色多普勒血流成像示低回声病变与静脉管壁之间可见细条状血流信号。31例心脏受累的患者中,右心房内可见低回声团块,1例伴右心房、右心室同时受累。36例伴有盆腔或子宫肌层低回声或者混合回声病变。结论血管内平滑肌瘤病发病率较低,临床表现不具有特异性,当病变超出子宫时,超声声像图表现具有一定的特异性。熟悉血管平滑肌瘤子宫以外的超声表现,有助于提高超声对血管平滑肌瘤的诊断。

Objective Intravascular leiomyomatosis is a rare disease with low morbidity and can be easily misdiagnosed.This study focused on exploring the ultrasonic features of intravascular leiomyomatosis beyond uterus,with the purpose of improving the recognition and diagnosis of intravascular leiomyomatosis.Methods Forty-three cases of intravascular leiomyomatosis confirmed by postsurgical pathological examinations in Peking Union Medical College Hospital from July1,2014to July1,2018were selected,which all had venous involvement detected by ultrasound.Both clinical data and ultrasonic features of43cases of intravascular leiomyomatosis were retrospectively analyzed.The main observational items include clinical symptoms,size of lesion,length,extent and range of involvement,echo types and blood flow signals inside the lesion.Forty-three patients were all female,of which27patients had a history of hysteromyoma(7myomectomy and20hysterectomy)and16without any related gynecologic surgeries.Results The clinical symptoms of these cases varied,which manifested as dyspnea and palpitation(n=11),abdominal pain and distension(n=11),edema of lower extremity(n=6),pelvic lumps(n=5),paroxysmal syncope(n=2),irregular vaginal bleeding(n=2),frequent urination(n=1)and no clinical symptoms(n=5).Ultrasonography showed43patients all had visible masses at the site of the inferior vena cava or iliac vein detected in the lumen,of which42cases were correctly diagnosed with intravascular leiomyomatosis by ultrasound beforeoperation The length of lesion varied,and the longest one rooted from pelvic to right atrium via venoussystem in some patients.Color Doppler flowing imaging demonstrated a bundle of fine blood flow betweenthe lesion and venous wall.Of all the patients,31had right atrial involvement,and1had both right atrialand right ventricle involvement.Thirty-six had lumps of hypoechogenicity or mixed echogenicity rootedin pelvic or uterus.Conclusions Intravascular leiomyomatosis is a kind of rare disease with no specificclinical symptoms,while it can be presented with specific ultrasonic features when expanding beyond uterus.Therefore,the identification of the ultrasonic features outside the uterus can play a significant role in thepreoperative diagnosis of intravascular leiomyomatosis in ultrasound diagnosis.