Severe Form of Primary Intestinal Lymphangiectasia in an One-Year-Old Child

Abstract

Primary intestinal lymphangiectasia (Waldmann syndrome) is a congenital abnormality of the lymphatic system in children – a disease that occurs infrequently. There are about 80 published descriptions of this pathology in infants. The main clinical manifestation of intestinal lymphangiectasia is a syndrome of malabsorption: diarrhoea, nausea, vomiting, abdominal pain and peripheral oedema. In some cases, steatorrhoea of varying severity occurs. Long lasting of the disease, no effect on the therapy leads to such complications as chylous ascites and chylothorax. This paper presents a clinical case of an early debut primary intestinal lymphangiectasia in a 6-month-old child, complicated by development of concomitant immunodeficiency. The final diagnosis was set after the histological examination of biopsy material in jejunum. Substitution therapy with albumin had no positive effect. Because of the constant hypoproteinemia that had not been corrected with either albumin or ongoing corticosteroid therapy, it was decided to start the sandostatin therapy. With improvement in the patient’s condition, he was transferred from intensive care to the department of somatic pathology. Transfusions of albumin have been cancelled. The protein level increased up to 53 g/l, swelling disappeared, but general sponginess of the tissue remained. The patient was discharged from the hospital with recommendations to continue the combined therapy including sandostatin and compulsory monitoring of the concentration of immunoglobulins.