Pitfalls in the diagnosis and management of obstructive uterovaginal duplication:: A series of 32 cases
Pitfalls in the diagnosis and management of obstructive uterovaginal duplication:: A series of 32 cases
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DOI：
10.1542/peds.2008-0219
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发表时间：
2008-10-01
期刊：
影响因子：
8
通讯作者：
Nihoul-Fekete, Claire
中科院分区：
文献类型：
--
作者：
Capito, Carmen;Echaieb, Anis;Nihoul-Fekete, Claire
目标。阻塞性子宫阴道重复畸形很少见，而且经常被误诊。本研究的目的是回顾我们机构中接受这种畸形治疗的所有患者，评估他们的长期结果，并讨论这种畸形的胚胎学起源。方法。从 1984 年到 2007 年，我们机构治疗了 32 名患有阻塞性子宫阴道复制的患者。我们将他们分为两组（青春期前 [n = 8] 和青春期 [n = 24]），并分析他们的临床和放射学表现及其治疗。重新联系了年龄 > 18 岁的患者 (n = 22)。 结果。对于青春期前组，诊断时的中位年龄为 6 个月。产后超声检查显示 6 例受试者同侧肾脏缺失，尽管 4 名患者产前诊断为同侧多囊性发育不良肾。在 3 例受试者中，这种肾脏异常与盆腔透明肿块有关，使我们能够在产前怀疑这一诊断。所有患者均经阴道入路治愈。对于青春期组，初潮后诊断的中位延迟时间为 9 个月。在紧急情况下接受治疗的患者 (n = 11) 中，有 9 例因腹部手术不当而误诊，其中包括 3 例梗阻半子宫的子宫切除术。就长期结果而言，5 名患者正在尝试生育，其中 4 名患者成功怀孕。一名患者在梗阻半子宫子宫切除术后因对侧峡部狭窄而导致不孕。结论。如果处理得当，阻塞性子宫阴道重复是一种良性病理。因此，青春期前后女孩腹痛的治疗应包括通过放射学盆腔和肾脏评估完成的系统腹部和妇科检查。手术治疗应通过阴道途径进行，以避免不孕。关于畸形的起源，产前发育不良的肾脏和产后肾脏缺失的高度相关性表明沃尔夫管和苗勒氏管发育异常，这与这种畸形的经典定义形成鲜明对比。
OBJECTIVES. Obstructive uterovaginal duplication is rare and frequently misdiagnosed. The aims of this study were to review all the patients managed for this malformation in our institution, evaluate their long-term outcomes, and discuss the embryologic origin of this malformation.METHODS. From 1984 to 2007, we treated 32 patients for obstructive uterovaginal duplication in our institution. We separated them in 2 groups (prepubertal [n = 8] and pubertal [n = 24]) and analyzed their clinical and radiologic presentations and their treatments. Patients > 18 years of age (n = 22) were recontacted.RESULTS. For the prepubertal group, the median age at diagnosis was 6 months. Postnatal ultrasound showed an absent ipsilateral kidney in 6 case subjects, although 4 patients had a prenatal diagnosis of ipsilateral multicystic dysplastic kidney. This renal anomaly was associated with a pelvic sonolucent mass in 3 case subjects, allowing us to prenatally suspect the diagnosis. All of the patients were cured by vaginal approach. For the pubertal group, the median delay of diagnosis after menarche was 9 months. Among patients managed in an emergency setting (n = 11), there were 9 misdiagnoses with inappropriate abdominal surgeries, including 3 hysterectomies of the obstructed hemiuterus. Concerning long-term results, 5 patients were attempting to have children, with successful pregnancies for 4 of them. One patient suffered from infertility attributable to contralateral isthmic stenosis after a hysterectomy of the obstructed hemiuterus.CONCLUSIONS. Obstructive uterovaginal duplication is a benign pathology when properly managed. Therefore, management of abdominal pain in peripubertal girls should include systematic abdominal and gynecologic examinations completed by radiologic pelvic and renal evaluation. Surgical treatment should be performed by vaginal approach to avoid infertility. Concerning the origin of the malformation, the high association of prenatal dysplastic kidneys and postnatal absent kidneys suggests anomalies of both wolffian and mullerian duct development, contrasting with the classic definition of this malformation.