Oh TG, Chung JW, Kim HM, Han SJ, Lee JS, Park JY, Song SY. Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. World J Gastrointest Endosc 2011; 3(11): 235-240 [PMID: 22110841 DOI: 10.4253/wjge.v3.i11.235]
Corresponding Author of This Article
Si Young Song, MD, PhD, Division of Gastroenterology, Department of Internal Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, South Korea. sysong@yuhs.ac
Article-Type of This Article
Case Report
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World J Gastrointest Endosc. Nov 16, 2011; 3(11): 235-240 Published online Nov 16, 2011. doi: 10.4253/wjge.v3.i11.235
Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy
Tak Geun Oh, Joo Won Chung, Hee Man Kim, Seok-Joo Han, Jin Sung Lee, Jung Yeob Park, Si Young Song
Tak Geun Oh, Joo Won Chung, Hee Man Kim, Jung Yeob Park, Si Young Song, Division of Gastroenterology, Department of Internal Medicine, Yonsei Institute of Gastroenterology, Yonsei University College of Medicine, Seoul 120-752, South Korea
Seok-Joo Han, Department of Pediatric Surgery, Yonsei University College of Medicine, Seoul 120-752, South Korea
Jin Sung Lee, Department of Pediatrics, Yonsei University College of Medicine, Seoul 120-752, South Korea
Si Young Song, Division of Gastroenterology, Department of Internal Medicine, Brain Korea 21 Project for Medical Science and Severance Biomedical Science Institute, Yonsei University College of Medicine, Seoul 120-752, South Korea
Author contributions: Oh TG designed the case report as a main author; Chung JW and Kim HM contributed to provide background knowledge; Han SJ provided many informations about the patient; Lee JS provided genetic information and accessment; Park JY participated in endoscopic evaluation and accessment; Song SY supervised and checked up the manuscript finally as a corresponding author; all authors reviewed and approved the final manuscript.
Correspondence to: Si Young Song, MD, PhD, Division of Gastroenterology, Department of Internal Medicine, 50 Yonsei-ro, Seodaemun-gu, Seoul 120-752, South Korea. sysong@yuhs.ac
Telephone: +82-2-22281957 Fax: +82-2-22277900
Received: May 6, 2011 Revised: October 19, 2011 Accepted: October 28, 2011 Published online: November 16, 2011
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.
