Protein-losing enteropathy (PLE) remains a feared complication of Fontan palliation (FP), with few effective treatment options. While systemic corticosteroids aide some patients, side-effects may be prohibitive. A patient with FP and severe PLE refractory to standard therapies is presented. Use of oral budesonide (OB, Entocort® EC) resulted in significant improvement.

E.F. is a 9 y.o. black female, born with a hypoplastic left heart syndrome variant. She underwent Stage I palliation in infancy, Glenn anastamosis at age 6 months and extracardiac, unfenestrated FP at age 2 ½ years. Post-operative pleural effusions resolved after three weeks. Fifteen months after FP, E.F. presented with abdominal and facial swelling. Serum albumin levels (SAL) were <2 mg/dL; stool alpha-1-antitrypsin levels were increased. Cardiac catheterization revealed central venous pressures of 13 mmHg and good ventricular function. Narrowing of the LPA was successfully stented. Symptoms nonetheless persisted; the patient was subsequently lost to follow-up. She represented at 7 years of age with severe edema, refractory to heparin, diuretics, systemic steroids and diet. Albumin was required every 48 hours. A RAST panel revealed allergies to wheat, egg and dairy products. These were eliminated without improvement. OB was added and SAL increased >2.5 mg/dL for three weeks. OB was discontinued three months later, with return to her pre-treatment state. It was restarted after 9 months, with prompt improvement.

We hypothesize that food allergies may play a role in refractory PLE patients. Use of OB, with limited systemic absorption, may offer a therapeutic option for selected patients.