A case of primary intestinal lymphangiectasia

Abstract

A 26-year-old male patient who had an 8 years history of recurrent peripheral edema with diarrhea and hypoproteinemia was evaluated. Endoscopic jejunal and ileal biopsy revealed markedly dilated mucosal lymph vessels with no evidence of inflammation. 99mTc-labeled human serum albumin (HSA) scintigraphy showed significant activity accumulating in the gastrointestinal tract to represent 99mTc-HSA leakage into the bowel lumen. A diagnosis of protein losing enteropathy and intestinal lymphangiectasia could be made. After treatment with a high protein and fat restricted diet, his symptoms subsided and the serum protein level was normalized.

Fig. 1.

A barium meal showing irregular luminal narrowing in the jejunum and ileum (arrow).

Fig. 2.

Small intestinal enteroscopy performing under fluoroscopic guidance (A) and endoscopic findings showing multiple whitish granular spots scattered throughout the mucosa of the jejunum (B) and terminal ileum (C).

Fig. 3.

Microscopic section reveals markedly dilated submucosal lymphatics without inflammation (arrow). H & E stain (×1000)

Fig. 4.

^99mTc-HSA Scintigraphy: The radioactivity is noted in the heart, great vessels, liver and spleen at 30 minutes (A), in the right lower quadrant and transverse colon at 6 hours (B) and in the transverse and sigmoid colon at 24 hours (C) after intravenous injection of ^99mTc-HSA.