Protein-losing enteropathy in an infant with a lymphangiomyoma

A 3-month-old girl with intractable diarrhoea had protein-losing enteropathy secondary to a lymphangiomyoma. This is the first reported case of lymphangiomyoma in an infant. As enteral feeds were not tolerated, she was maintained on parenteral nutrition for 2 months. Pancreatic enzyme supplementation produced a rapid clinical improvement and normalization of serum albumin level. Weaning was subsequently tolerated and the vitamin, mineral, and trace element deficiencies improved. Subsequent recurrence was associated with normal pancreatic function and has proved refractory to treatment.