Leont'ev A F, Pugachev A G, Seniakovich V M, Tuĭchibekov Sh M, Chistova L V
Vestn Khir Im I I Grek. 1984 Aug;133(8):96-9.
A rare hereditary disease of the liver (fibrocholangiocystosis--congenital fibrosis of the liver) was surgically treated in 43 children. A conclusion is made that different palliative operations are inexpedient. Ilio-cavamesenterial anastomoses may be recommended for preventing relapses of gastroesophageal hemorrhages. These operations should be performed at the age of 5-10 years. After palliative operations recurrent hemorrhages were noted in 68,4%, lethality in 26,3%. After vascular anastomoses recurrent hemorrhages were found in 23,3%, lethality--in 7,7%.
43名儿童接受了一种罕见的肝脏遗传性疾病（纤维胆管囊肿病——先天性肝纤维化）的外科治疗。得出的结论是，不同的姑息性手术并不适宜。可推荐进行回肠-腔-肠系膜吻合术以预防胃食管出血复发。这些手术应在5至10岁时进行。姑息性手术后，68.4%的患者出现复发性出血，26.3%的患者死亡。血管吻合术后，23.3%的患者出现复发性出血，7.7%的患者死亡。
