Respiratory failure due to morbid obesity in a patient with Prader-Willi syndrome: an experience of long-term mechanical ventilation.

Prader-Willi syndrome (PWS) is characterized by obesity, mild mental retardation or learning disability, and behavior problems, especially in association with food and eating. A 19 year-old man, 150 cm, 140 kg (body mass index [BMI], 62.2 kg.m(-2)), whose condition had been diagnosed as PWS, received 41-day mechanical ventilation because of respiratory failure, chiefly due to morbid obesity. Because the patient frequently developed bronchoconstriction, metered-dose inhalers of a corticosteroid (beclomethasone dipropionate) and a beta2 agonist (salbutamol) were needed. To achieve adequate sedation, which was also crucial to control the bronchoconstriction, the concurrent use of midazolam, fentanyl, ketamine, and propofol was required. Pressure-control ventilation was useful to avoid high airway pressure due to low respiratory system compliance associated with the morbid obesity. Because it appeared that the basic problem leading to respiratory failure in this patient was morbid obesity, body weight reduction was considered to be mandatory. Thus, caloric intake was limited to 1000 kcal.day(-1), resulting in body weight reduction by 50 kg during the patient's stay in the intensive care unit (ICU). The patient was successfully extubated on ICU day 35.