Primary intestinal lymphangiectasia with massive abdominal lymphatic malformation requiring surgical debulking

Primary intestinal lymphangiectasia is a rare disorder characterized by severe protein-losing enteropathy resulting in lymphopenia, hypoalbuminemia, and hypogammaglobulinemia. It may be associated with limb lymphedema, chylous ascites, and abdominal lymphatic malformations.We describe a 20 year-old female with progressive primary intestinal lymphangiectasia, severe bilateral lower extremity lymphedema and a massive abdominal and retroperitoneal lymphatic malformation who developed debilitating symptoms and protein/calorie malnutrition. Our treatment approach included multidisciplinary management and eventual surgical debulking resection.Although the management of primary intestinal lymphangiectasia is usually medical, in the rare case of primary intestinal lymphangiectasia with associated chylous ascites and extensive abdominal lymphatic malformation, surgical resection appears to be a safe and successful strategy to improve nutrition and quality of life. As in the present case, complex and severe vascular anomalies necessitate a combined treatment approach, which is available at multidisciplinary vascular anomalies centers.