Hepatopathy in two infants with short-bowel syndrome and cytomegalovirus infection

In children with short-bowel syndrome and the need for long-term parenteral nutrition, hepatic dysfunction is a multifactorial phenomenon that has not been completely understood. Alterations ill gut motility lead to intralumi nal stasis which is thought to be a major etiologic factor for bacterial ov ergrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastr oschisis and intestinal atresia. The intestinal lengths after resection wer e 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to i ntestinal shortness in the first patient and dilatation of the preatretic b owel segment with ineffective peristalsis in the second patient; Despite mu ltiple trials of enteral nutrition and medical therapy for gut decontaminat ion and stimulation of bowel motility, hepatopathy developed in both patien ts in a similar period of time and to about the same degree. At the age of 4 and 6 weeks, respectively, increasing bilirubin values were measured. Det erioration of liver function and thrombocytopenia at the age of 3 to 4 mont hs led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at th e age of 7 and 9 months, respectively. Additional hepatic injury secondary to CMV infection might have contributed to the rapid deterioration of liver disease. Screening for further hepatotoxic factors, especially infectious etiologies, is therefore recommended in children with short-bower syndrome. Liver transplantation should be considered early in cases of progressive h epatic dysfunction.