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World J Clin Cases. Oct 16, 2014; 2(10): 528-533
Published online Oct 16, 2014. doi: 10.12998/wjcc.v2.i10.528
Primary intestinal lymphangiectasia: Minireview
Sachin B Ingle, Chitra R Hinge (Ingle)
Sachin B Ingle, Department of Pathology, MIMSR Medical College, Latur, Maharashtra 4132512, India
Chitra R Hinge (Ingle), Department of Physiology, MIMSR Medical College, Latur, Maharashtra 4132512, India
Author contributions: Ingle SB and Hinge (Ingle) CR prepared the manuscript; Ingle SB critically revised the intellectual content and gave final approval of manuscript.
Correspondence to: Sachin B Ingle, Professor, Department of Pathology, MIMSR Medical College, Ambajogai road, Vishwanathpuram, Latur, Maharastra 4132512, India.
Telephone: +91-2382-227424 Fax: +91-2382-228939
Received: April 18, 2014
Revised: June 28, 2014
Accepted: July 25, 2014
Published online: October 16, 2014

Primary idiopathic intestinal lymphangiectasia is an unusual disease featured by the presence of dilated lymphatic channels which are located in the mucosa, submucosa or subserosa leading to protein loosing enteropathy.Most often affected were children and generally diagnosed before third year of life but may be rarely seen in adults too. Bilateral pitting oedema of lower limb is the main clinical manifestation mimicking the systemic disease and posing a real diagnostic dilemma to the clinicians to differentiate it from other common systemic diseases like Congestive cardiac failure, Nephrotic Syndrome, Protein Energy Malnutrition, etc. Diagnosis can be made on capsule endoscopy which can localise the lesion but unable to take biopsy samples. Thus, recently double-balloon enteroscopy and biopsy in combination can be used as an effective diagnostic tool to hit the correct diagnosis. Patients respond dramatically to diet constituting low long chain triglycerides and high protein content with supplements of medium chain triglyceride. So early diagnosis is important to prevent untoward complications related to disease or treatment for the sake of accurate pathological diagnosis.

Keywords: Primary idiopathic intestinal lymphangiectasia, Mucosa-submucosa, Protein loosing enteropathy, Double balloon endoscopy, Biopsy

Core tip: Waldmann’s disease is an unusual primary idiopathic intestinal lymphangiectasia, which results in protein loosing enteropathy. Recently, double balloon endoscopy and biopsy in combination is an effective diagnostic tool to hit the correct diagnosis to avoid untoward complications related to disease and treatment due to misdiagnosis. Thus, the clinician should keep in mind this rare condition as a differential diagnosis of oedema.