Cosme Jiménez A, Barrio Andrés J, Bujanda Fernández de Piérola L, Ojeda Pérez E, Poch Zapirain M, Orcolaga Alba R, Garmendia Larrañaga G, Gil Lasa I, Arenas Miravé J I
Gastroenterology Service, Hospital Nuestra Señora de Aránzazu San Sebastián, Guipúzcoa, Spain.
Rev Esp Enferm Dig. 2000 Jul;92(7):448-57.
To identify predisposing factors, clinical characteristics and effective treatment in patients with nonneoplastic cavernomatous transformation of the portal vein in our gastroenterology service.
We retrospectively reviewed the clinical records of 2,201 patients diagnosed as having portal hypertension (2,165 with cirrhosis and 36 with noncirrhotic portal vein hypertension) during the period from 1977 to 1998. The diagnosis of cavernomatous transformation was confirmed with angiographic or Doppler echographic studies, or both.
Thirteen patients (6 males, 7 females, age range 8 to 69 years) with cavernomatous transformation were found. Predisposing factors were omphalitis (1), echinococcal cyst (1), major abdominal surgery (3), liver cirrhosis (3), Sjögren syndrome (1), and no apparent cause (4). Eleven of the 13 patients had upper digestive tract bleeding from varices, 9 had splenomegaly, and 2 had cirrhotic decompensation. Splenectomy was done in 3 patients on admission, and in 5 patient shunts were used (splenorenal in 4, mesenteroatrial in 1) because of repeated bleeding.
Of the patients with noncirrhotic portal hypertension, 27.7% had nontumoral cavernomatous transformation of the portal vein. Previous abdominal surgery was the most frequent predisposing factor; the 2 cases of echinococcal liver disease and Sjögren disease were exceptional. Age younger than 30 years, bleeding esophageal varices and splenomegaly were the most frequent clinical features. Portosystemic shunt was the only effective treatment alternative in these patients.
确定我院胃肠病科门静脉非肿瘤性海绵样变患者的易感因素、临床特征及有效治疗方法。
我们回顾性分析了1977年至1998年间诊断为门静脉高压的2201例患者的临床记录（2165例肝硬化患者和36例非肝硬化门静脉高压患者）。门静脉海绵样变的诊断通过血管造影或多普勒超声检查，或两者同时进行得以证实。
发现13例门静脉海绵样变患者（6例男性，7例女性，年龄8至69岁）。易感因素包括脐炎（1例）、棘球蚴囊肿（1例）、腹部大手术（3例）、肝硬化（3例）、干燥综合征（1例）以及无明显病因（4例）。13例患者中有11例因静脉曲张出现上消化道出血，9例脾肿大，2例肝硬化失代偿。3例患者入院时接受了脾切除术，5例患者因反复出血接受了分流术（4例脾肾分流，1例肠系膜心房分流）。
在非肝硬化门静脉高压患者中，27.7%存在门静脉非肿瘤性海绵样变。既往腹部手术是最常见的易感因素；2例棘球蚴肝病和干燥综合征患者为例外。年龄小于30岁、食管静脉曲张出血和脾肿大是最常见的临床特征。门体分流术是这些患者唯一有效的治疗选择。
