Longterm ventilation for children with severe respiratory control disorders, myelomeningocele and Chiari II malformation

Case report. We report on three female patients with myelomeningocele, Chia ri ii malformation and severe respiratory control disorder despite neurosur gery. After life-threatening events (two patients) and negative weaning (on e patient) we decided a longterm home-care ventilation at the age of 5 mont hs, 3.1 respectively 10.1 years of age, Patients were ventilated by a press ure-controlled ventilator via nasal mask (one patient) respectively by a vo lume-controlled ventilator via tracheostoma (two patients). Over a period o f 2.7 respectively 5.2 years we could prevent two patients from life-threat ening event. During a follow-up of 7.3 years our third patient suffered fro m a prolonged convulsion,which led to a severe neurologic deterioration. Tw o of the three patients showed a positive neurologic development and social integration. Discussion. Longterm ventilation can prolong life expectancy a nd optimize neurological development and social integration for patients with myelomeni ngocele and severe respiratory control disorders despite neurosurgery. Acco rding to the complex neurologic situation,and the patient's and their famil y's stress the elective decision for a longterm ventilation has to be judgd ed critically and individually.