Rintala R, Lindahl H, Pohjavuori M, Saxen H, Sariola H
Children's Hospital, University of Helsinki, Finland.
J Pediatr Surg. 1993 May;28(5):716-9. doi: 10.1016/0022-3468(93)90040-r.
We report surgical reversal of intractable total parenteral nutrition (TPN)-associated cholestasis refractory to conservative treatment in 9 premature infants. Indications for TPN were poorly tolerated enteral feedings in all patients. Five patients had undergone gastrointestinal operations; in addition, 7 of the 9 patients had had bacterial sepsis. The median duration of TPN was 28 days (range, 20 to 50 days). The median duration of preoperative full enteral nutrition after weaning from TPN was 34 days (range, 16 to 95 days). All patients had progressive conjugated hyperbilirubinemia, no excretion of Tc-labeled HIDA to the biliary tree and duodenum, and markedly elevated liver enzyme values. Intraoperative cholangiography showed normal biliary anatomy in all cases; in addition, 2 patients had gallbladder stones. Bile was hyperviscous in all patients and contained biliary sludge in 4. The biliary tree was irrigated and the liver biopsied in all patients. The gallbladder was removed from 2 patients who had stones in the gallbladder. Liver histology was consistent with TPN-associated cholestasis in all cases, and in addition, 4 cases showed significant destruction of intrahepatic bile ducts. One patient died 2 weeks postoperatively from intracerebral hemorrhage. Jaundice completely resolved in other patients within 2 weeks. HIDA-biligraphy performed 1 to 2 months postoperatively showed normal excretion of the radioactive marker to the biliary tree and duodenum in all cases. The functional abnormality in bile excretion and bile duct motility in TPN-associated cholestasis may be reversed by irrigation of the biliary tree. Surgical intervention should be considered when cholestasis is progressive and refractory to medical management.
我们报告了9例早产儿难治性全胃肠外营养（TPN）相关胆汁淤积经保守治疗无效后行手术逆转的情况。所有患者TPN的指征均为肠内喂养耐受性差。5例患者接受过胃肠道手术；此外，9例患者中有7例发生过细菌性败血症。TPN的中位持续时间为28天（范围20至50天）。从TPN撤机后术前完全肠内营养的中位持续时间为34天（范围16至95天）。所有患者均有进行性结合胆红素血症，锝标记的HIDA不能排泄至胆管树和十二指肠，且肝酶值显著升高。术中胆管造影显示所有病例胆管解剖结构正常；此外，2例患者有胆囊结石。所有患者胆汁黏稠，4例含有胆泥。所有患者均进行了胆管树冲洗和肝活检。2例胆囊有结石的患者切除了胆囊。所有病例肝脏组织学均符合TPN相关胆汁淤积，此外，4例显示肝内胆管有明显破坏。1例患者术后2周死于脑出血。其他患者黄疸在2周内完全消退。术后1至2个月进行的HIDA胆管造影显示所有病例放射性标记物向胆管树和十二指肠的排泄均正常。TPN相关胆汁淤积中胆汁排泄和胆管动力的功能异常可通过胆管树冲洗逆转。当胆汁淤积进展且药物治疗无效时，应考虑手术干预。
