Salihefendić Nizama, Licanin Zoran, Zildzić Muharem
Medicus A, Gracanica.
Med Arh. 2005;59(2):132-4.
Cavernous transformation of the portal vein occurs with long-standing portal vein thrombosis (PVT) because of the development and dilatation of multiple small vessels in and around the recanalising main portal vein. Thrombosis and occlusion of the portal vein leads to portal hypertension with enlarged spleen and the development of porto-systemic collaterals. The main clinical presentation is gastroesophageal variceal bleeding and hematologic abnormalities due to splenomegaly (hyperspleenismus-pancytopenia). We described the young patient with splenomegaly and extensive cavernous transformation of portal vein. The patient had thrombosis portal vein in early childhood and massive bleeding from large oesophageal varices at age 13. Full clinical evaluation is required because of abdominal pain. The liver is histologicaly and functionally normal. Diagnosis of cavernous transformation of the portal vein is confirmed by abdominal ultrasography, color Doppler ultrasonography and CT angiography. Oesophagogastroscopy reveals almost complete reduction of oesophageal varices, but confirms portal gastropathy as a source of patient's complaints. Natural course of PVT in this patient shows possibility of full reduction of oesophageal varices, but still the presence of different consequences of portal hypertension.
门静脉海绵样变是由于再通的门静脉主干内及周围多条小血管的形成和扩张，发生于长期门静脉血栓形成（PVT）时。门静脉血栓形成和闭塞导致门静脉高压，脾脏肿大以及门体侧支循环的形成。主要临床表现为胃食管静脉曲张破裂出血和脾肿大导致的血液学异常（脾功能亢进 - 全血细胞减少）。我们描述了一名患有脾肿大和广泛门静脉海绵样变的年轻患者。该患者在幼儿期发生门静脉血栓形成，13岁时因巨大食管静脉曲张出现大量出血。因腹痛需要进行全面的临床评估。肝脏在组织学和功能上均正常。门静脉海绵样变的诊断通过腹部超声、彩色多普勒超声和CT血管造影得以证实。食管胃镜检查显示食管静脉曲张几乎完全消退，但证实门静脉性胃病是患者症状的来源。该患者PVT的自然病程显示食管静脉曲张有可能完全消退，但门静脉高压的不同后果仍然存在。
