Significance of Endoscopic Small Bowel Biopsy in the Diagnosis of Intestinal Lymphangiectasia in Children

Ko, Jae Sung; Seo, Jeong Kee

Abstract

BACKGROUND/AIMS: Intestinal lymphangiectasia is a disease characterized by dilated lymphatics of the intestinal mucosa and excessive enteric loss of plasma proteins. Instead of multiple blind peroral jejunal biopsy, duodenal endoscopy and endoscopic small bowel biopsy were performed. We evaluated the significance of endoscopic small bowel biopsy and the usefulness of the other diagnostic methods in the diagnosis of intestinal lymphangiectasia in children.
METHODS
Fourteen children seen between August 1989 and August 1997 with clinically suggestive intestinal lymphangiectasia were analysed. The median age at onset of symptoms was 4 years. Primary intestinal lymphangiectasia occurred in 10 children and secondary intestinal lymphangiectasia occurred in 4 children, of whom two had Fontan operation, one had constrictive pericarditis, and one had Crohn's disease. Low fat, high protein diet with medium chain triglycerides was the mainstay of treatment.
RESULTS
1) Diarrhea was present in 14 patients, and edema in 11 patients. Hypocalcemic tetany occurred in 6 children and vomiting in 5 children. Dight children had ascites and three of these had chylous ascites. Growth retardation was present in four patients, chylothorax in one, and lymphedema in one. The initial serum albumin concentration was 1.8 g/dl, the serum calcium level 6.7 mg/dl, and the total lymphocyte count 623 /mm(3). 2) Dilated lymphatics in the small bowel mucosa was confirmed by endoscopic biopsy in 14 children(100%). The sensitivity of alpha1-antitrypsin clearance was 100%. Duodenal endoscopy showed scattered white spots covering mucosa in 11 children(79%). Small bowel series revealed thickened mucosal folds in 10 children(77%). Four(31%) had positive finding of 99mTc-antimony lymphoscintigraphy. 3) Responses to treatment in children with primary intestinal lymphangiectasia were graded as good if the symptoms resolved, and poor if there was no lasting resolution of symptoms and repeated albumin administration. Response to therapy was good in four and poor in six patients. The mean age at onset of symptoms was 8 years in good resonse group, and 2 years in poor response group (p<0.05).
CONCLUSION
The diagnosis of intestinal lymphangiectasia is confirmed by duodenoscopy and endoscopic small bowel biopsy in a child with diarrhea, edema, hypoalbuminemia, and lymphocytopenia. As compared with other diagnostic methods such as small bowel series and lymphoscintigraphy, duodenoscopy and endoscopic small bowel biopsy are very sensitive and should be performed early.