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Volume 09 Journal of Gastrointestinal & Digestive System
ISSN: 2161-069X Gastro Congress 2019
July 08-09, 2019
July 08-09, 2019 | Zurich, Switzerland
14th Euro-Global Gastroenterology Conference
Clinical profile, response to therapy and outcome of primary intestinal lymphangiectasia in children
Durga Prasad, Anshu Srivastava, Anil Tambe, Surender Kumar Yachha, Moinak Sen Sarma and Ujjal Poddar
Sanjay Gandhi Postgraduate Institute of Medical Sciences, India
Objective: Intestinal lymphangiectasia (IL, primary or secondary) is an important cause of protein-losing
enteropathy. We evaluated the clinico-laboratory profile, response to therapy, complications and outcome of children
with primary IL (PIL).
Methods: Consecutive children (≤18 years) diagnosed with PIL (clinical setting, typical small bowel histopathology
and exclusion of secondary causes) from 2007 to 2017 were evaluated.
Results: 28 children with PIL (16 boys, age at symptom onset-12 months and at diagnosis 8 years) were studied.
Pedal edema (93%), chronic diarrhea (78.6%) and recurrent anasarca (64%) were the common presentations.
Ascites, pleural and pericardial effusion was seen in 64% (n-18; chylous-5, non-chylous-13), 18% and 18% cases
respectively. Hypoproteinemia, hypoalbuminemia, hypocalcaemia and lymphopenia were present in 82%, 82%,
75% and 39% cases respectively. Duodenal biopsy established the diagnosis in 86% cases, while 14% required
distal small bowel biopsies. Dietary therapy was given in all and 6 cases required additional therapy (octreotide-6,
tranexamic acid-3 and total parenteral nutrition-1). Lymphedema (3/5 vs. 1/23), pleural effusion (4/5 vs. 1/23) and
need of additional therapy (4/5 vs. 2/23) was significantly more common in patients with chylous ascites (n=5) than
those without chylous ascites (n=23). 24 cases were in follow-up for 39 (6-120) months and showed improvement,
however 8 required readmission (symptom recurrence-6 [25%], complication- 2 [8.3%, Budd Chiari Syndrome-1
and abdominal B cell lymphoma-1]).
Conclusion: Presence of chylous ascites suggests severe disease in children with PIL. Majority of PIL children
respond to dietary therapy; only 20% need additional therapy. Symptom relapse and complications occur in 1/3rd of
cases and need long-term follow-up.
J Gastrointest Dig Syst 2019, Volume 09