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OCTREOTIDE MANAGEMENT OF INTESTINAL LYMPHANGIECTASIA IN A TEENAGE HEART TRANSPLANT PATIENT
G Altit, H Patel, V MorinvilleMontreal Children's Hospital, Montreal, Quebec
AIMS: Intestinal lymphangiectasia, a disorder of obstructed lymphatic drainage, clinically presents as protein-losing enteropathy. Mild hypoproteinemia may require little intervention, but profound protein losses require therapy. Despite limited benefit, traditional care has centered around: 1) diuresis, 2) albumin infusion, 3) high protein/ high medium chain triglyceride diet, 4) intravenous immune globulin and 5) corticosteroids. Heparin and octreotide are other rarely-reported therapies. We hereby describe a teenage heart transplant recipient who developed severe hypoalbuminemia related to lymphangiectasia arising due to right heart dysfunction, successfully managed with subcutaneous octreotide.
METHODS: Retrospective chart review conducted subsequent to obtaining institutional approval.
RESULTS: A 15-year-old male, recipient of a heart transplant at one year of age, presented with a 9-month history of progressive hypoalbuminemia. Active medical issues included chronic renal failure, pacemaker dependence, and complex refractory right heart dysfunction. Ongoing medical therapies included an anti-rejection regimen including corticosteroid, as well as periodic intravenous immune globulin for chronically active parvovirus infection leading to aplastic anemia. At its nadir, blood albumin levels decreased to 14 g/L (normal range 31-48 g/L) and the patient developed a 5-kg weight gain with anasarca. Investigations revealed an elevated 24-hour alpha-1 antitrypsin stool clearance [1.8 g/L (normal range 0-0.72 g/L), when serum albumin levels were 18 g/L], confirming a protein-losing enteropathy. Abdominal ultrasound and computer tomography did not reveal other underlying pathologies to account for the excessive enteric protein losses. Upper endoscopy and small bowel biopsies confirmed the clinical impression of lymphangiectasia. The hypoalbuminemia was refractory to periodic 25% albumin infusions combined with attempts at dietary interventions with a high-protein, high medium-chain triglyceride specialized formula. The patient was hospitalized, intravenous albumin infusions were provided until normalization of blood albumin levels, and 50 micrograms twice a day subcutaneous octreotide was begun. Subsequent to the introduction of octreotide, despite marginal compliance with the specialized formula, the patient has maintained normal blood albumin levels to a current duration of 6 months without any further need for albumin infusions.
CONCLUSIONS: Lymphangiectasia is a difficult-to-manage condition. Octreotide use has only rarely been described. The mechanism of effect of octreotide may be via local action on vascular somatostatin receptors, leading to reduced lymph flow. Given the balance between harm and potential benefit, subcutaneous octreotide should be considered in the therapeutic options for pediatric lymphangiectasia.