Kapczuk Karina, Friebe Zbigniew, Iwaniec Kinga, Kędzia Witold
Division of Gynecology, Department of Perinatology and Gynecology, Poznań University of Medical Sciences, Poznań, Poland.
Division of Gynecology, Department of Perinatology and Gynecology, Poznań University of Medical Sciences, Poznań, Poland.
J Pediatr Adolesc Gynecol. 2018 Jun;31(3):252-257. doi: 10.1016/j.jpag.2017.09.013. Epub 2017 Oct 10.
To assess the clinical course of obstructive Müllerian anomalies found in girls after menarche.
A retrospective case series of adolescents who, between 2009 and 2016, were treated for vaginal or uterine obstructive malformations diagnosed after menarche.
Division of Gynecology, Poznań University of Medical Sciences, Poznań, Poland.
Twenty-two patients who, at the age range between 11.4 and 18.2 (median, 13.1) years, between 2 and 74 (median 7.5) months after menarche, underwent surgical repair of obstructive genital anomaly.
Müllerian defect type, presentation, radiologic findings, pre- and postoperative course.
Eighteen patients (18 of 22; 81.8%) were diagnosed with obstructed hemivagina ipsilateral renal anomaly syndrome. One patient (1 of 22; 4.5%) was diagnosed with uterus didelphys and unilateral cervical atresia. Three patients (3 of 22; 13.6%) had unicornuate uterus with a cavitated, noncommunicating rudimentary horn. The right side was affected in 13 patients (13 of 22; 59.1%), and the left side in 9 patients (9 of 22; 40.9%; P > .05). All but 1 patient had renal agenesis on the side of obstruction. Before repair of the obstructive genital anomaly, 4 patients underwent unnecessary surgeries for misdiagnosed ovarian cysts. Serious complications (pelvic inflammatory disease, vesicovaginal fistula) occurred in 2 patients with microperforated pyocolpos. Pelvic endometriosis was found in 4 of our patients.
Our case series suggests that obstructed hemivagina ipsilateral renal anomaly syndrome is the most common obstructive Müllerian anomaly diagnosed in adolescents after menarche. The differential diagnosis for unilateral kidney agenesis accompanied by dysmenorrhea in adolescent girls should include obstructive genital tract anomaly. Accurate diagnosis of an obstructive genital anomaly early after menarche might help prevent unnecessary surgeries and infection-related complications. Meanwhile, prompt surgical correction of an obstructive genital tract anomaly results in relief of symptoms and might reduce the risk of endometriosis.
评估初潮后女孩中发现的梗阻性苗勒管异常的临床病程。
一项回顾性病例系列研究，研究对象为2009年至2016年间因初潮后诊断出的阴道或子宫梗阻性畸形而接受治疗的青少年。
波兰波兹南医科大学妇科。
22例患者，年龄在11.4至18.2岁（中位数13.1岁）之间，初潮后2至74个月（中位数7.5个月），接受了梗阻性生殖器异常的手术修复。
苗勒管缺陷类型、临床表现、影像学检查结果、术前及术后病程。
18例患者（22例中的18例；81.8%）被诊断为梗阻性半阴道同侧肾异常综合征。1例患者（22例中的1例；4.5%）被诊断为双子宫和单侧宫颈闭锁。3例患者（22例中的3例；13.6%）为单角子宫伴空化、不连通的残角。右侧受累13例（22例中的13例；59.1%），左侧受累9例（22例中的9例；40.9%；P>.05）。除1例患者外，所有患者梗阻侧均有肾缺如。在梗阻性生殖器异常修复前，4例患者因误诊为卵巢囊肿而接受了不必要的手术。2例微穿孔积脓阴道患者发生了严重并发症（盆腔炎、膀胱阴道瘘）。4例患者发现盆腔子宫内膜异位症。
我们的病例系列表明，梗阻性半阴道同侧肾异常综合征是初潮后青少年中最常见的梗阻性苗勒管异常。青春期女孩单侧肾缺如伴痛经的鉴别诊断应包括梗阻性生殖道异常。初潮后早期准确诊断梗阻性生殖器异常可能有助于避免不必要的手术和感染相关并发症。同时，及时手术矫正梗阻性生殖道异常可缓解症状，并可能降低子宫内膜异位症的风险。
