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Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect

Aroor, Shrikiran and Mundkur, Suneel C and Kanaparthi, Shravan and Kumar, Sandeep (2017) Waldmann’s Disease (Primary Intestinal Lymphangiectasia) with Atrial Septal Defect. Journal of Clinical and Diagnostic Research, 11 (4). pp. 3-4. ISSN 0973-709X

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Abstract

Waldmann’s disease or Primary Intestinal Lymphangiectasia (PIL) is a rare disorder of gastrointestinal tract characterized by dilated lymphatics and widened villi causing leakage of lymph into intestinal lumen. Loss of lymph leads to hypoalbuminemia, hyogammaglobulinemia and lymphopenia. Secondary lymphangiectasia occurs secondary to an elevated lymphatic pressure as in lymphoma, systemic lupus erythematosus, constrictive pericarditis, cardiac surgeries (Fontan’s procedure), inflammatory bowel disease and malignancies. We, hereby present a five-year-old male child who presented with abdominal distension and poor weight gain. He had hypoalbuminemia, lymphocytopenia and hypogammaglobulinemia. Upper gastrointestinal endoscopy showed normal gastric mucosa and punctate white lesions in duodenal mucosa with biopsy confirming intestinal lymphangiectasia. Secondary causes of intestinal lymphangiectasia were ruled out. Echocardiography revealed atrial septal defect which is an uncommon association with Waldmann’s disease. He was started on low fat, high protein diet and medium chain triglyceride supplementation following which he improved symptomatically. High index of suspicion, early diagnosis and appropriate dietary treatment are necessary to alleviate symptoms as well as to achieve a sustainable growth and development in these children.

Item Type: Article
Uncontrolled Keywords: Anasarca; Protein loosing enteropathy; Recurrent diarrhoea
Subjects: Medicine > KMC Manipal > Paediatrics
Depositing User: KMC Library
Date Deposited: 15 Apr 2017 09:35
Last Modified: 15 Apr 2017 09:35
URI: http://eprints.manipal.edu/id/eprint/148707

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