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Hepatopathy in two infants with short-bowel syndrome and cytomegalovirus infection
R Ensenauer1, R Hentschel, K Rückauer
1Universitäts-Kinderklinik Freiburg, Germany.
Insights
Hepatic dysfunction in children with short-bowel syndrome can be worsened by cytomegalovirus (CMV) infection. Early screening for infections and considering liver transplantation are crucial for managing progressive liver disease.
Area of Science:
- Pediatric Gastroenterology and Hepatology
- Intestinal Failure and Parenteral Nutrition
- Infectious Diseases in Immunocompromised Hosts
Background:
- Short-bowel syndrome (SBS) necessitates long-term parenteral nutrition (PN), increasing the risk of hepatic dysfunction in children.
- Gut stasis and bacterial overgrowth are recognized contributors to cholestasis in SBS, particularly with ileocecal valve absence.
- The multifactorial nature of PN-associated hepatopathy in SBS remains incompletely understood.
Observation:
- Two infants with SBS (post-gastroschisis/intestinal atresia) developed progressive hepatopathy despite optimized enteral nutrition and medical management.
- Both patients presented with elevated bilirubin, liver dysfunction, and thrombocytopenia around 3-4 months of age.
- Acute cytomegalovirus (CMV) infection was diagnosed and treated with ganciclovir, but both infants ultimately succumbed to acute liver failure.
Findings:
- CMV infection emerged as a significant factor, potentially exacerbating pre-existing liver injury in SBS patients on PN.
- The study highlights a critical interplay between intestinal failure, PN, and opportunistic infections in pediatric liver disease.
- Rapid deterioration and fatal outcomes underscore the severity of combined insults.
Implications:
- Routine screening for infectious etiologies, especially CMV, is recommended in pediatric SBS patients with worsening liver function.
- Early consideration of liver transplantation should be part of the management strategy for progressive hepatic dysfunction in this vulnerable population.
- Further research into the pathogenesis of hepatopathy in SBS, including infectious contributions, is warranted.
Abstract:
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 in gut motility lead to intraluminal stasis which is thought to be a major etiologic factor for bacterial overgrowth and subsequent cholestasis, especially when the ileocecal valve is absent. We report on two infants with short-bowel syndrome caused by gastroschisis and intestinal atresia. The intestinal lengths after resection were 18 and 55 cm. Long-term parenteral nutrition (PN) was obligatory due to intestinal shortness in the first patient and dilatation of the preatretic bowel segment with ineffective peristalsis in the second patient. Despite multiple trials of enteral nutrition and medical therapy for gut decontamination and stimulation of bowel motility, hepatopathy developed in both patients 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. Deterioration of liver function and thrombocytopenia at the age of 3 to 4 months led to the diagnosis of acute cytomegalovirus (CMV) infection. Treatment with ganciclovir followed. Both patients died of acute liver failure at the 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-bowel syndrome. Liver transplantation should be considered early in cases of progressive hepatic dysfunction.
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