Journal of Clinical and Diagnostic Research, ISSN - 0973 - 709X

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Case report
Year : 2017 | Month : April | Volume : 11 | Issue : 4 | Page : SD03 - SD04

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

Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar

1. Professor and Head, Department of Paediatrics, Kasturba Medical College, Manipal, Karnataka, India. 2. Additional Professor, Department of Paediatrics, Kasturba Medical College, Manipal, Karnataka, India. 3. Senior Resident, Department of Paediatrics, Kasturba Medical College, Manipal, Karnataka, India. 4. Assistant Professor, Department of Paediatrics, Kasturba Medical College, Manipal, Karnataka, India.

Correspondence Address :
Dr. Shravan Kanaparthi,
Flat 206, Mandavi Sapphire, Perampally Road, Manipal-576101, Karnataka, India.
E-mail: shravan.1354@gmail.com

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.

Keywords

Anasarca, Protein loosing enteropathy, Recurrent diarrhoea

How to cite this article :

Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar. WALDMANN’S DISEASE (PRIMARY INTESTINAL LYMPHANGIECTASIA) WITH ATRIAL SEPTAL DEFECT. Journal of Clinical and Diagnostic Research [serial online] 2017 April [cited: 2017 May 30 ]; 11:SD03-SD04. Available from
http://www.jcdr.net/back_issues.asp?issn=0973-709x&year=2017&month=April&volume=11&issue=4&page=SD03-SD04&id=9742

DOI and Others

DOI: 10.7860/JCDR/2017/24989.9742


Date of Submission: Oct 24, 2016
Date of Peer Review: Nov 15, 2016
Date of Acceptance: Dec 20, 2016
Date of Publishing: Apr 01, 2017

Financial OR OTHER COMPETING INTERESTS: None.

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