OHVIRA综合征继发自发性膀胱破裂

文献信息

中文摘要

作为一种罕见的中肾管所致苗勒管畸形，OHVIRA综合征以双子宫、梗阻性半阴道及同侧肾异常三联征为特征。同样罕见的是自发性膀胱破裂（SRUB），该情况构成危及生命的外科急症。本病例呈现了OHVIRA综合征合并SRUB的前所未见的表现。一名19岁女性因在长期进行性痛经和近期排尿困难基础上叠加的急腹症就诊。影像学检查证实了OHVIRA综合征的诊断；然而，SRUB仅在术前膀胱镜检查过程中、恰在计划行阴道镜隔膜切除之前于术中才被发现。实施了急诊腹腔镜膀胱修补术以治疗腹腔内膀胱破裂。OHVIRA和SRUB的识别常被遗漏或显著延迟。为克服这一点，自月经初潮起即出现进行性痛经的青少年应接受单侧梗阻性苗勒管畸形（包括OHVIRA综合征）的检查。此外，新发的泌尿系统症状和提示腹膜炎的急性腹痛应提高对SRUB的怀疑。在微创手术时代，阴道镜隔膜成形术联合腹腔镜膀胱修补术应被视为主要、创伤最小且最有效的处理方式。

英文摘要

As a rare mesonephric duct-induced Mullerian anomaly, OHVIRA syndrome is characterised by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal anomaly. Equally rare is the spontaneous rupture of the urinary bladder (SRUB), a condition that constitutes a life-threatening surgical emergency. This case represents an unprecedented presentation of OHVIRA syndrome involving SRUB. A 19-year-old female presented with acute abdomen superimposed on a long-standing progressive dysmenorrhea and recent dysuria. Diagnosis of OHVIRA syndrome was confirmed by imaging; however, SRUB was only discovered intraoperatively during a pre-procedure cystoscopy, just prior to the planned vaginoscopic septal resection. An emergency laparoscopic bladder repair was performed to treat the intraperitoneal bladder rupture. Identification of both OHVIRA and SRUB is often missed or significantly delayed. To overcome this, adolescents presenting with progressive dysmenorrhea since menarche should be investigated for unilateral obstructive Mullerian anomalies including OHVIRA syndrome. Furthermore, a new onset of urinary symptoms and acute abdominal pain suggestive of peritonitis should raise the suspicion of SRUB. In the era of minimally invasive surgery, combined vaginoscopic septoplasty and laparoscopic bladder repair should be considered the primary, least traumatic and most effective management.