区域性门静脉高压症的诊断和治疗

Clinical diagnosis and treatment of regional portal hypertension

区域性门静脉高压症(RPH)是由脾静脉狭窄或闭塞引起的一种临床综合征,是一种罕见的、危及生命的上消化道出血疾病,却是门静脉高压症中唯一可以治愈的类型。胰源性疾病是RPH最常见的病因,孤立性胃底静脉曲张是其临床特征。对于肝功能正常及不明原因脾大的消化道出血患者,应考虑RPH的可能。RPH临床表现较为隐匿,与肝硬化所致的门静脉高压症在临床症状上有一定的相似性,由于对该疾病认识不足,临床易误诊。原发疾病多样性造成RPH临床表现多样化,使疾病诊断复杂化,对于临床表现不典型的患者,可选择辅助检查加以诊断。疾病诊断应以治疗原发病为主,同时遵循个体化原则。RPH的预后主要与原发疾病相关,良性病变预后好,恶性病变预后差。

Regional portal hypertension(RPH) is a clinical syndrome caused by stenosis or occlusion of the splenic vein. It is a rare yet life-threatening cause of upper gastrointestinal bleeding, but it is also the only type of portal hypertension that can be cured. Pancreatic diseases are the most common cause of RPH, and isolated gastric varices represent the characteristic clinical presentation. For patients with gastrointestinal bleeding with normal liver function and unexplained splenomegaly, the possibility of RPH should be considered. The clinical manifestations of RPH are relatively insidious, and are similar to those of portal hypertension caused by liver cirrhosis. Due to insufficient knowledge of RPH, it is more likely to be misdiagnosed clinically. The diversity of primary diseases results in the diversification of the clinical manifestations of RPH, which complicates the diagnosis of the disease.For patients with atypical clinical manifestations, specific auxiliary examinations can be applied to assist in the diagnosis. In terms of the treatment, the primary diseases should be attached the most importance to, and individualized therapies should be provided. The prognosis of RPH is mainly related to the condition of primary diseases, and benign lesions may herald a good prognosis yet malignant lesions a poor prognosis.