Waldmann’s disease (Primary intestinal lymphangiectasia) with atrial septal defect

Shrikiran Aroor, Suneel Mundkur, Shravan Kanaparthi, Sandeep Kumar

Research output: Contribution to journalArticle

2 Citations (Scopus)

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.

Original languageEnglish
Pages (from-to)SD03-SD04
JournalJournal of Clinical and Diagnostic Research
Volume11
Issue number4
DOIs
Publication statusPublished - 01-04-2017

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Intestinal Lymphangiectasis
Atrial Heart Septal Defects
Hypoalbuminemia
Lymphopenia
Lymph
Defects
Lymphangiectasis
Fontan Procedure
Constrictive Pericarditis
Echocardiography
Agammaglobulinemia
Gastrointestinal Endoscopy
Endoscopy
Biopsy
Conservation of Natural Resources
High Fat Diet
Nutrition
Gastric Mucosa
Growth and Development
Inflammatory Bowel Diseases

All Science Journal Classification (ASJC) codes

  • Medicine(all)
  • Clinical Biochemistry

Cite this

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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.",
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Waldmann’s disease (Primary intestinal lymphangiectasia) with atrial septal defect. / Aroor, Shrikiran; Mundkur, Suneel; Kanaparthi, Shravan; Kumar, Sandeep.

In: Journal of Clinical and Diagnostic Research, Vol. 11, No. 4, 01.04.2017, p. SD03-SD04.

Research output: Contribution to journalArticle

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