Video Capsule Endoscopy to Diagnose Primary Intestinal Lymphangiectasia in a 14-Month-Old Child

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To the Editor: The patient is a 14-month-old girl of Turkish origin with progressive, generalized edema, ascites, weight loss, and mild diarrhea since several weeks. Laboratory investigation revealed iron deficiency anemia (5.2 mmol/L), hypoalbuminemia (13 g/L), hypogammaglobulinemia (1.73 g/L), and increased fecal alpha 1-antitrypsin (11 mg/g; normal range <2.6 mg/g), indicative of protein-losing enteropathy. Fat-soluble vitamin levels were decreased (vitamin A: 0.7 μmol/L; vitamin E 17 μmol/L, vitamin D 25 OH 38 nmol/L); international normalized ratio was within normal range. There were no signs of underlying infections, celiac, liver, or cardiac disease. Esophagogastroduodenoscopy (proximal jejunum was reached) and ileocolonoscopy did not reveal macroscopic or histologic abnormalities, particularly no intestinal lymphangiectasia. Video capsule endoscopy (VCE) was performed, which showed a dense layer of whitish, swollen intestinal villi from the proximal jejunum to the distal ileum, characteristic for lymphangiectasia (Fig. 1). Furthermore, VCE revealed several erosions with active blood loss, possibly accounting for the anemia (Fig. 2). The diagnosis primary intestinal lymphangiectasia (PIL) was established and a low-fat diet with medium-chain triglyceride supplementation was initiated. This prevents overload of chyle in intestinal lymphatic vessels, which may provoke rupture and secondary lymph loss (1,2). Within weeks, albumin levels normalized and edema dissolved. After 6 months normal diet was reintroduced, without recurrence of clinical and biochemical abnormalities, indicating this had been a transient phenotype of PIL. Diagnosis of PIL can usually be established by esophagogastroduodenoscopy, with typical macroscopic and histological findings. Our case illustrates the value of VCE in children to establish the diagnosis PIL when findings by classical endoscopy are not conclusive. Furthermore, this case underlines the increasing notion that VCE can be performed safely below the age of 2 years, although a lower age limit to perform VCE is not yet defined (3,4).
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