Primary Intestinal Lymphangiectasia in an Infant with a History of Cowʼs Milk Protein Allergy—A Case Report

Abstract

Purpose: An unusual case of an infant who presented to our institution with a history of failure-to-thrive, cow's milk protein (CMP) allergy, and chronic diarrhea. Methods: Descriptive report. Results: 11-month-old African-American girl with failure-to-thrive and chronic diarrhea was transferred to our institution from an outside hospital. She had previously been clinically diagnosed with CMP allergy. On admission, she appeared extremely malnourished with a distended abdomen. Initial evaluation revealed severe hypoalbuminemia, hypogammaglobulinemia, hyper-IgE, and low levels of CD4 lymphocytes. Her stools were voluminous with increased alpha-1-antitrypsin. Upper and lower endoscopies were notable for diffuse, white lesions of her small bowel (Figure 1). Histology and electron microscopy (EM) showed dilated lymphatic ducts and large fat droplets, respectively (Figures 2 and 3). An unusual enteropathy included architectural distortion of her villi, described as “stubby” and atrophic, a thin brush-border membrane (Figure 4), and a mixed-cellular infiltrate of eosinophils, lymphocytes, and PMNs (Figure 5). Colonic histology was significant for eosinophilic colitis (Figure 6). Abdominal CT revealed bowel wall edema, but was otherwise unremarkable. Celiac serology and genetic markers were negative. Clinical status markedly improved on parenteral nutrition.Figure 1: Snow-white mucosal lesions in terminal ileum.Figure 3: EM showing large fat droplets within mucosa of small bowel.Conclusion: This is the first case report of primary intestinal lymphangiectasia in association with mixed-cellular enteropathy and clinical history of CMP allergy.