Absence of Oesophageal Varices in Children with Splenomegaly and Advanced Intestinal Failure Associated Liver Disease

Abstract

Objectives of the study: Children with splenomegaly and portal hypertension due to primary liver disease demonstrate presence of gastro- oesophageal varices (GOV) on oesophago-gastroduodenoscopy (OGD). Intestinal failure – associated liver disease (IFALD) is a secondary liver disease developing in 40% to 60% of infants requiring long term parenteral nutrition for irreversible intestinal failure. A proportion of these children develop life threatening complications of cirrhosis and portal hypertension. Assessment of portal hypertension is important to determine the type of intestinal transplantation (ITx) offered as a life saving option. Intestinal transplantation is the only life saving option for these children. During intestinal transplant assessment within our programme, we assessed the children for the presence of GOV. Aim: To evaluate the incidence of GOV in children with IFALD referred for small bowel transplant assessment. Methods: Medical records of 122 patients who underwent assessment for small bowel transplant (SBtx) over the last 12 years (Sept 04 to Dec 16) were reviewed. Data detailing spleen size, splenomegaly, liver biopsy with staging of hepatic fibrosis and presence of GOV on oesophago-gastroduodenoscopy (OGD) was recorded. Results: 118/122 children assessed were included in the study as 4 children did not have ultrasound scan (USS). 98/118 (83 %) children had splenomegaly on USS. 54/98 (55.1%) children underwent OGD and oesophageal varices were present in 11 children (20.3%), of which only 10 had Grade 1 and one had Grade 3 varices. Of the 20 patients with a normal sized spleen, 15 underwent an OGD- no varices have been detected. 56 children had an OGD and a liver biopsy performed at the same time. 51/56 patients had fibrosis, 26/51 (50.9 %) were graded as mild fibrosis on liver biopsy, and 8/56 (14.2 %) of them had oesophageal varices (grade 1). Moderate to severe fibrosis was noted in 25/51 (49%) children and only 2/25 (8%) children had GOV, all Grade I. Summary and Conclusion: Children with IFALD, who have a normal size spleen did not have varices at OGD and therefore do not require endoscopy to stage their liver disease. In children with IFALD, splenomegaly is common and GOV are infrequent. Their presence does also not correlate with the degree of liver-fibrosis. In conclusion, USS evidence of splenomegaly alone is a poor predictor for GOV and additional tests such as platelet count, hepatic elastography may improve the assessment of portal hypertension in children with intestinal failure associated liver disease.