Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome)
Management and outcome of patients with combined vaginal septum, bifid uterus, and ipsilateral renal agenesis (Herlyn-Werner-Wunderlich syndrome)
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DOI：
10.1016/j.jpedsurg.2006.01.021
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发表时间：
2006-05-01
影响因子：
2.4
通讯作者：
Laberge, Jean-Martin
中科院分区：
文献类型：
--
作者：
Gholoum, Suad;Puligandla, Pramod S.;Laberge, Jean-Martin
目的：Herlyn-Wemer-Wunderlich综合征（HWWS）是一种罕见的苗勒管畸形，包括子宫发育不良、半阴道隔和同侧肾发育不全。本研究的目的是评估HWWS.Methods的患者的自然史和结果：与伦理审查委员会批准，所有患者的子宫/阴道异常进行了审查，1982年和2004年之间。排除泄泻腔和/或肛门直肠异常的患者。提出的症状，术前调查，手术管理，和长期随访assessed.Results：80例患者确定与子宫/阴道异常，12 HWWS。就诊时的中位年龄为13岁。大多数患者（11/12）表现为腹痛和/或盆腔肿块。2例患者出现腹腔内脓肿。7例患者在就诊时表现为痛经，其中4例患者有痛经。症状持续时间范围为0.5至12个月。通过超声（n = 11）、计算机断层扫描（n = 3）和/或磁共振成像（n = 2）确诊。手术治疗包括阴道中隔切除术和阴道积血/子宫阴道积血引流。1例患者因输卵管积脓需要输卵管切除术。随访超声检查显示无复发性收集。中位随访时间为3年（2个月至16年）。11名患者在治疗后无症状。一名患者抱怨月经不规律。结论：这是迄今为止最大的审查HWWS在儿科患者之一。阴道中隔切除术后的长期结局良好。女性盆腔包块和同侧肾发育不全应怀疑此诊断。(c)2006年爱思唯尔公司All rights reserved.
Objective: Herlyn-Wemer-Wunderlich syndrome (HWWS) is a rare miillerian anomaly consisting of uterine didelphy, hemivaginal septum, and ipsilateral renal agenesis. The purpose of this study was to evaluate the natural history and outcome of patients with HWWS.Methods: With ethics review board approval, all patients with uterine/vaginal anomalies were reviewed between 1982 and 2004. Patients with cloacal and/or anorectal anomalies were excluded. Presenting symptoms, preoperative investigations, operative management, and long-term follow-up were assessed.Results: Of 80 patients identified with uterine/vaginal anomalies, 12 had HWWS. Median age at presentation was 13 years. Most patients (11/12) in this series presented with either abdominal pain and/or pelvic masses. Two patients had intra-abdominal abscesses. Seven patients were menstruating at presentation with 4 of these patients having dysmenorrhea. Symptom duration ranged from 0.5 to 12 months. Diagnosis was confirmed by ultrasound (n = 11), computed tomographic scan (n = 3), and/or magnetic resonance imaging (n = 2). Operative management included vaginal septectomy and drainage of the hematocolpos/hematometrocolpos. One patient required salpingectomy for pyosalpinx. Follow-up ultrasounds revealed no recurrent collections. Median follow-up was 3 years (2 months to 16 years). Eleven patients were asymptomatic after treatment. One patient complained of irregular menses.Conclusion: This is one of the largest reviews of HWWS in pediatric patients to date. Good long-term outcome occurs after vaginal septectomy. This diagnosis should be suspected in females with a pelvic mass and ipsilateral renal agenesis. (c) 2006 Elsevier Inc. All rights reserved.