Li Zheng, Yu Xin, Shen Jianxiong, Liang Jinqian
From the Department of Orthopaedic Surgery (ZL, XY, JS, JL), Peking Union Medical College Hospital, Peking Union Medical College, Beijing, China.
Medicine (Baltimore). 2014 Dec;93(28):e185. doi: 10.1097/MD.0000000000000185.
Herlyn-Werner-Wunderlich syndrome (HWWS) is a congenital Müllerian duct anomaly characterized by uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Little is reported about spinal deformity associated with this syndrome. This study presents a case of scoliosis occurring in the setting of HWWS and explores the possible association between the 2 diseases. A previously unreported scoliosis in HWWS is described. The patient is a 12-year-old Chinese female with scoliosis that underwent a posterior correction at thoracic 5-thoracic 12 (T5-T12) levels, using the Moss-SI (Johnson & Johnson, American) spinal system. At 24-month follow-up, the patient was clinically pain free and well balanced. Plain radiographs showed solid spine fusion with no loss of deformity correction. Six months after scoliosis correction surgery, the patient went to our clinics for the treatment of HWWS. She was performed a vaginal septum resection and detected with pyocolpos. Her follow-up was symptom free at the fourth postoperative month. The prevalence of scoliosis among patients with HWWS was 8.57% that is much higher than the incidence of congential scoliosis among general population (1/1000). To the best of our knowledge, this is the first report of HWWS with thoracic scoliosis. During surgery, surgeons and anesthesiologists must pay particular attention to the Müllerian duct anomaly and renal agenesis associated with HWWS. There is a potential association between congenital scoliosis and HWWS.
赫林-韦纳-温德利希综合征（HWWS）是一种先天性苗勒管异常疾病，其特征为双子宫、半阴道梗阻和同侧肾缺如。关于该综合征相关脊柱畸形的报道较少。本研究报告了1例HWWS合并脊柱侧弯的病例，并探讨这两种疾病之间可能存在的关联。文中描述了1例此前未报道过的HWWS合并脊柱侧弯病例。该患者为一名12岁中国女性，因脊柱侧弯接受了胸5至胸12（T5-T12）节段的后路矫正手术，采用了美国强生公司的Moss-SI脊柱系统。术后24个月随访时，患者临床无疼痛且平衡良好。X线平片显示脊柱融合牢固，畸形矫正无丢失。脊柱侧弯矫正手术后6个月，该患者因HWWS前来我院就诊。她接受了阴道纵隔切除术，并发现有阴道积脓。术后第4个月随访时她无症状。HWWS患者中脊柱侧弯的患病率为8.57%，远高于普通人群中先天性脊柱侧弯的发病率（1/1000）。据我们所知，这是首例HWWS合并胸段脊柱侧弯的报告。手术过程中，外科医生和麻醉医生必须特别注意与HWWS相关的苗勒管异常和肾缺如情况。先天性脊柱侧弯与HWWS之间可能存在关联。
