Poddar U, Thapa B R, Puri P, Girish C S, Vaiphei K, Vasishta R K, Singh K
Department of Gastroenterology, Postgraduate Institute of Medical Education and Research, Chandigarh.
Indian J Gastroenterol. 2000 Jan-Mar;19(1):12-3.
Non-cirrhotic portal fibrosis (NCPF) is an infrequent cause of portal hypertension in children. We report 11 children with NCPF, from among 338 with portal hypertension, seen over 6.5 years.
The diagnosis was based on patent splenoportal axis on ultrasonography and/or splenoportal venography and liver biopsy showing no evidence of cirrhosis or other diagnosis, in children with portal hypertension. Those with variceal bleed were managed with endoscopic sclerotherapy and/or shunt surgery.
The median age was 11 years (range 5 to 14), and 8 were boys. Presentation was with variceal bleed in 6, lump in left upper abdomen in 5 (though all children had splenomegaly) and esophageal varices on endoscopy. The median spleen enlargement was 8.5 cm; 8 also had hepatomegaly. Hypersplenism was present in 7, and two had developed ascites after bleed. Of 6 children presenting with bleed, variceal obliteration was achieved on sclerotherapy (average 5.6 sessions) in 4 while two underwent shunt surgery for associated hypersplenism. After median follow up of 57.5 months (range 12-78) all are alive and well.
NCPF is an uncommon cause of portal hypertension in Indian children. Presentation with variceal bleed is less common than in adults; sclerotherapy is effective.
非肝硬化性门脉纤维化（NCPF）是儿童门静脉高压症的罕见病因。我们报告了在6.5年期间诊治的338例门静脉高压症患儿中的11例NCPF患儿。
诊断基于超声检查和/或脾门静脉造影显示脾门静脉轴通畅，以及肝活检显示无肝硬化证据或其他诊断，这些患儿患有门静脉高压症。有静脉曲张出血的患儿采用内镜硬化治疗和/或分流手术治疗。
中位年龄为11岁（范围5至14岁），8例为男孩。临床表现为6例静脉曲张出血，5例左上腹肿块（尽管所有患儿均有脾肿大）以及内镜检查发现食管静脉曲张。脾肿大的中位值为8.5 cm；8例还伴有肝肿大。7例存在脾功能亢进，2例出血后出现腹水。6例出血患儿中，4例经硬化治疗（平均5.6次）实现了静脉曲张闭塞，2例因合并脾功能亢进接受了分流手术。中位随访57.5个月（范围12 - 78个月）后，所有患儿均存活且状况良好。
NCPF是印度儿童门静脉高压症的罕见病因。静脉曲张出血的表现比成人少见；硬化治疗有效。
