特发性门静脉高压症13例临床诊治分析

目的 通过对特发性门静脉高压症（idiopathic portal hypertension，IPH）患者的临床症状、影像学检查、手术治疗方式及预后情况描述，探讨特发性门静脉高压症的诊断、治疗及预后状况。方法 回顾性分析解放军总医院第五医学中心自2010年1月至2019年12月外科手术治疗的13例特发性门静脉高压症患者的临床资料，患者行胃镜检查发现食管胃底静脉曲张，5例出现上消化道出血，均无肝硬化。结果 13例均行脾切除+断流术+肝组织活检术，肝组织活检病理均可排除其他类型门静脉高压症，可提示特发性门静脉高压症。术后随访，患者食管胃底静脉曲张减轻，近期内出血风险明显降低，生活质量改善。1例在术后第4年出现黑便状况。结论 特发性门静脉高压症确诊主要依靠肝组织活检病理的排除性诊断，多普勒超声对特发性门静脉高压症具有一定的诊断价值。相对于内科药物治疗，外科脾切除加断流术能够更有效治疗IPH，且治疗效果要好于肝硬化型门静脉高压症。

Clinical diagnosis and treatment of 13 patients with idiopathic portal hypertension

Objective To investigate the diagnosis, treatment and prognosis of idiopathic portal hypertension (IPH) by reviewing the clinical symptoms, imaging examination, surgical treatment and prognosis of IPH.Methods The clinical data of 13 patients with IPH treated by surgery in the Fifth Medical Center of PLA General Hospital from Jan. 2010 to Dec. 2019 were analyzed retrospectively. Esophageal and gastric fundus varices were found by gastroscopy. Five patients had upper gastrointestinal bleeding, and none had liver cirrhosis. Results All of the 13 patients underwent splenectomy+pericardial devascularization+liver biopsy. Liver biopsy suggested idiopathic portal hypertension and excluded other types of portal hypertension. The esophageal and gastric varices were reduced, the risk of bleeding was significantly reduced, and life quality of the patients was improved in postoperative follow-up study. Only one patient developed black stool in the fourth year after operation.Conclusion The diagnosis of IPH mainly depends on the exclusion diagnosis of liver biopsy pathology, and Doppler ultrasound has certain value in diagnosis of IPH. Compared with medical treatment, the surgical treatment of splenectomy+pericardial devascularization is more effective for IPH, and the therapeutic effect is better than cirrhotic portal hypertension.