Primary intestinal lymphangiectasia with a history of mesenteric lymphangioma
Main Article Content
Abstract
Primary intestinal lymphangiectasia (PIL) is a congenital and rare disorder of intestinal lymphangiectasia. The clinical manifestations of PIL are characterized by excessive loss of lymphatic fluid which includes proteins, fats, and lymphocytes into the intestinal lumen. We reported a case of a 14-year-old girl presented with persistent diarrhea and lower limbs edema. She had a history of surgery to remove mesenteric lymphangioma at 3 years of age and presented with persistent acute diarrhea and edema one year post surgery. Laboratory tests resuts showed hypoalbuminemia (19.9 g/L), hypovitaminosis D (25OH vitamin D levels 1.8 ng/mL) and hypocalcemia (iCa 0.81 mmol/L), lymphopenia (0.62 G/L), negative proteinuria. Abdominal MRI showed no abnormality. Endoscopy and biopsies of the duodenum, ileum showed intestinal lymphangiectasia. Lymphangiography DSA showed no lymphatic leakage but with dilated hepatic lymphatic. She was diagnosed with PIL and treated with a medium-chain triglyceride diet, subcutaneous octreotide and hepatic lymphatic sclerotherapy. Her edema resolved and her serum albumin remained stable.
Article Details
Keywords
Protein-losing enteropathy (PLE), primary intestinal lymphangiectasia (PIL), Waldmann’s disease
References
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