Published online Jun 21, 2015. doi: 10.3748/wjg.v21.i23.7320
Peer-review started: January 10, 2015
First decision: February 10, 2015
Revised: February 27, 2015
Accepted: March 31, 2015
Article in press: March 31, 2015
Published online: June 21, 2015
Processing time: 161 Days and 13.7 Hours
We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.
Core tip: This is a case of a 42-year-old man who presented with a grand mal seizure, malabsorption-derived electrolytes and a protein disorder caused by primary intestinal lymphangiectasia. After an extensive management and exclusion of secondary intestinal lymphangiectasia, a diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Although numerous therapeutic strategies are available, only octreotide therapy proved to be a potentially effective means of therapeutic resolution; however, a longer follow-up period is needed. More case reports and/or studies on similar cases are needed to determine the best possible treatment strategies for this rare disorder.