Bernardini F, Bonetti E, Pozzati F, La Fera E, Campolo F, Naldini A, Testa A C, Scambia G, Catena U
Facts Views Vis Obgyn. 2024 Dec;16(4):489-492. doi: 10.52054/FVVO.16.4.055.
Complete bicorporeal uterus, double cervix and obstructive longitudinal vaginal septum (classified as U3bC2V2 according to ESHRE/ESGE classification) is a rare congenital anomaly of the genital tract. This condition is typically associated with ipsilateral renal agenesis and is known as Herlyn-Werner-Wunderlich syndrome or OHVIRA (Obstructed HemiVagina and Ipsilateral Renal Anomaly) syndrome. The primary symptoms include dysmenorrhea and pelvic pain, which usually manifest after menarche due to haematocolpos in the obstructed hemivagina. Diagnosis is often challenging and frequently delayed. Early detection and surgical drainage of the haematocolpos are essential for symptom relief and prevention of complications. Various surgical approaches have been described, with vaginoplasty and septal resection being the recommended treatment.
To propose a step-by-step demonstration with narrated video footage of an integrated approach for one-stop diagnosis and ultrasound-guided endoscopic hymen-sparing treatment in a patient with OHVIRA syndrome.
We present the case of a 17-year-old virgo-intacta female who was referred to our institution due to dysmenorrhea, abnormal uterine bleeding and a right presumed ovarian endometrioma. A preoperative evaluation, including pelvic ultrasound and MRI, suspected a U3bC2V2 malformation, associated to a right haematocolpos and ipsilateral renal agenesis. The patient underwent a complete minimally invasive vaginoscopic resection of the obstructive longitudinal vaginal septum under transabdominal ultrasound guidance, using a 15Fr bipolar mini-resectoscope. The procedure successfully drained the haematocolpos and allowed visualisation of the right cervix, confirming the preoperative diagnosis.
The procedure was performed in our Digital Hysteroscopic Clinic (DHC) - CLASS Hysteroscopy -under general anaesthesia (with laryngeal mask), according to an ambulatory model of care. No complications occurred and the patient was discharged three hours after the procedure.
After 40 days, hysteroscopic office control revealed a normal vagina with double cervix and complete bicorporeal uterus (classified as U3bC2V0 according to ESHRE/ESGE classification), and the patient had complete relief of symptoms.
The combined approach of one-stop diagnosis and ultrasound-guided minimally invasive vaginoscopic resection of the obstructive longitudinal vaginal septum, using a 15Fr bipolar mini-resectoscope, has proven to be an effective strategy. This approach leads to optimal surgical results without complications.
完全双体子宫、双宫颈及梗阻性纵向阴道纵隔（根据ESHRE/ESGE分类为U3bC2V2）是一种罕见的先天性生殖道畸形。这种情况通常与同侧肾缺如相关，被称为赫林-韦纳-温德利希综合征或OHVIRA（梗阻性半阴道和同侧肾异常）综合征。主要症状包括痛经和盆腔疼痛，通常在初潮后因梗阻性半阴道积血而出现。诊断往往具有挑战性且经常延迟。早期发现并手术引流积血对于缓解症状和预防并发症至关重要。已经描述了各种手术方法，阴道成形术和纵隔切除术是推荐的治疗方法。
通过带旁白的视频片段逐步演示一种综合方法，用于OHVIRA综合征患者的一站式诊断和超声引导下保留处女膜的内镜治疗。
我们报告了一名17岁未破处女性的病例，该患者因痛经、异常子宫出血及右侧疑似卵巢子宫内膜异位瘤转诊至我院。术前评估，包括盆腔超声和MRI，怀疑为U3bC2V2畸形，伴有右侧积血和同侧肾缺如。患者在经腹超声引导下，使用15Fr双极微型切除镜，进行了完全微创阴道镜下梗阻性纵向阴道纵隔切除术。该手术成功引流了积血，并使右侧宫颈得以可视化，证实了术前诊断。
该手术在我们的数字宫腔镜诊所（DHC）——CLASS宫腔镜下，在全身麻醉（喉罩）下，按照门诊护理模式进行。未发生并发症，患者术后3小时出院。
40天后，宫腔镜门诊检查显示阴道正常，有双宫颈和完全双体子宫（根据ESHRE/ESGE分类为U3bC2V0），患者症状完全缓解。
使用15Fr双极微型切除镜进行一站式诊断和超声引导下微创阴道镜切除梗阻性纵向阴道纵隔的联合方法已被证明是一种有效的策略。这种方法可带来最佳手术效果且无并发症。
