Bariatric surgery for treatment of morbid obesity in an adolescent with Marfan syndrome

Abstract

A 16 year old Caucasian female with genetically confirmed Marfan syndrome and extreme obesity (weight: 205 kg, height: 176 cm, BMI: 66 kg/m 2) was referred to Cincinnati Children's Hospital Comprehensive Weight Management Center for bariatric surgery. Her comorbidities attributed to obesity included: obstructive sleep apnea, hyperinsulinemia, hypertension, left ventricular hypertrophy, gastroesophageal reflux, urinary stress incontinence, and irregular menses. Her family history was concerning for sudden death from aortic dissections as early as the 2nd decade of life for other individuals with Marfan syndrome and concomitant severe obesity. Cardiac imaging at time of referral was limited secondary to poor acoustic windows for echocardiography and body habitus which restricted the use of traditional scanners. Initial challenges included estimating her surgical risk due to her connective tissue disease and the unknown status of her aorta. Additional cardiac Magnetic Resonance (MR) imaging demonstrated no aortic dilation. She underwent uneventful laparoscopic Roux-en-Y gastric bypass surgery and recovery. Six months later her weight had decreased to 162 kg (BMI: 52.0 kg/m 2, weight loss: 43 kg). Hyperinsulinemia improved (fasting insulin: 38.9 → 19.7 uU/ml), and left ventricular mass improved (55 → 39 g/m 2.7). This is the first case highlighting the safety and potential impact of surgical weight loss in the management of an adolescent with Marfan syndrome. Lifelong followup is recommended due to long term risks of Marfan syndrome, and independent nutritional risks of gastric bypass surgery.