Wunderlich 综合征的临床分类诊治体会

Clinical experience in classification, diagnosis, and treatment for Wunderlich syndrome

目的：探讨Wunderlich综合征（ Wunderlich syndrome， WS）的临床特点和诊治方法。方法回顾性分析2008年9月至2014年2月收治的15例WS患者的临床特点及诊治方法。主要临床表现有突发性腰背部或腹部疼痛（15／15）、低血容量性休克（5／15）、肉眼血尿（4／15）和肾区叩击痛（15／15）。实验室检查异常：贫血（9／15）、凝血功能异常（5／15）。15例中5例危重患者，表现为中、重度低血容量性休克（收缩压≤90 mmHg，1 mmHg＝0．133 kPa）伴重度贫血（血红蛋白＜60 g／L）和凝血功能障碍。结果急性出血期彩色多普勒超声、增强CT检查对WS的确诊比例为11／15、15／15，诊断WS原发病因的确诊比例分别为4／15和10／15，增强CT均优于彩色多普勒超声检查（ P＜0．05）。5例同时行增强MRI，诊断结果与增强CT检查一致。根据急诊影像学病因诊断结果，危重患者采用急诊手术探查或肾动脉造影＋选择性动脉栓塞术，非危重患者采用保守治疗或急诊手术探查或肾动脉造影＋选择性动脉栓塞术。15例患者的原发病因为血管平滑肌脂肪瘤（8例）、肾细胞癌（3例）、肺癌肾转移瘤（1例）、肾囊肿（3例）。15例随访13～78个月，平均34个月，除1例危重患者死亡外，其他患者均获治愈或缓解。结论 WS的临床表现缺乏特异性。增强CT或MRI检查是确诊WS和诊断原发病因的主要方法，诊断价值优于超声。 WS治疗方法应依据病情危重程度和影像学病因诊断结果进行选择。

Objective To explore the clinical features, diagnosis and treatment of Wunderlich syndrome （WS）.Methods Fifteen patients with WS were included from September 2008 to February 2014, and their clinical features, diagnosis and treatment were retrospectively reviewed.The most common clinical manifestations were flank or abdominal pain （15/15）, hypovolemic shock （5/15）, gross hematuria （4/15） and percussion pain on kidney region （ 15/15 ） .Laboratory tests showed anemia （ 9/15 ） and coagulation abnormalities （5/15）.Five of 15 cases were critical patients with moderate to severe shock （ systolic blood pressure≤90 mmHg, 1 mmHg =0.133 kPa ） accompanied with severe anemia （ Hb 〈60 g/L ） and coagulation abnormalities.Results In the acute stage, the diagnostic ratios of ultrasonography and contrast enhanced CT for WS were 11/15 and 15/15, and cause determination ratios were 4/15 and 10/15, respectively.The latter was significantly higher than the former （ P〈0.05 ） .Contrast enhanced MRI was performed in 5 cases, and the results were identical to those of CT.According to the causes made by emergent imaging, critical patients underwent emergent operations or renal arteriography plus selective arterial embolization, and the other patients underwent conservative management, emergency operations, or renal arteriography plus selective arterial embolization, respectively.The causes of WS included angiomyolipoma （8 cases）, renal cell carcinoma （3 cases）, metastatic tumor of lung cancer （1 case） and renal cyst （3 cases） in this study.The mean follow-up period was 34 months.One critical patient died, and all the other patients were cured or relieved.Conclusions WS has no specific clinical features.Contrast enhanced CT or MRI is the main approach for diagnosis and cause determination, which is superior to ultrasonography.Treatments for WS vary according to severity classification and imaging diagnosis.