103 We report a patient with DiGeorge syndrome and protein losing enteropathy corrected by anterior aortopexy. At six months of age he presented with a generalized seizure due to hypocalcemia. He had a one month history of foul smelling, copious stools, periorbital and facial edema and abdominal distention. He was diagnosed with DiGeorge syndrome (hypoparathyroidism, T-cell immunodeficiency, ventricular septal defect, and a deletion at band 22q11.2). Hypomagnesemia, anemia, hypogammaglobulinemia and hypoalbuminemia were noted and a protein losing enteropathy (PLE) documented by an elevated fecal alpha 1-antitrypsin clearance of 136mg/dl. G. lamblia infection was identified and treated without benefit. Calcium and magnesium supplements, calcitriol, and elemental iron were started. Co-trimoxazole was given for P. carinii pneumonia prophylaxis and monthly infusions of intravenous immunoglobulins were administered. An endoscopy with biopsy confirmed the diagnosis of intestinal lymphangiectasia (IL) and visualized a large posterior indentation in the upper thoracic esophagus. No improvement was noted in the stools despite dietary manipulation. At ten months the child developed dysphagia with solids and cough. An UGI revealed a posterior indentation of the esophagus with marked narrowing of the trachea. An MRI revealed a right acsending aorta, transverse aorta progressing from right to left, posterior to the esophagus, and descending left thoracic aorta. The descending aorta gave rise to the left subclavian artery creating a vascular ring with a left sided ligamentum. An aortopexy and divisions of ligamentum arteriosum was performed. After the surgery, the patient tolerated solid food well and no longer had cough. His stools decreased in volume and frequency and his serum albumin and immunoglobulin levels normalized on a regular diet. At two years of age, he had normal parathyroid function, normal serum levels of immunoglobulins, and a repeat fecal alpha 1-antitrypsin was 12 mg/dl. Discussion: In this patient with DiGeorge syndrome, G. lamblia was thought to be the cause of malabsorbtion and PLE. However, persistence of PLE after treatment of G. lamblia enteritis, and rapid improvement after the surgery indicate, that IL was most likely caused by pressure of the anamalous vascular structures on the thoracic lymphatic drainage system. We report this case, because PLE and IL caused by an aberrant aortic arch and vascular ring have not been previously described in a patient with DiGeorge syndrome.