Early dentofacial orthopedic treatment of a patient with maxillary hypoplasia and congenital central hypoventilation syndrome

Congenital central hypoventilation syndrome (CCHS) is a respiratory disorder characterized by compromised central and peripheral chemoreflexes that lead to hypoventilation during sleep. All patients with CCHS require lifelong ventilatory support during sleep; however, nasal bilevel positive airway pressure (BiPAP) therapy can cause maxillary hypoplasia. In the present case, the patient had been diagnosed with CCHS and was initiated on BiPAP therapy at the age of two months. The patient presented to our orthodontic department with severe midface hypoplasia along with an anterior dental crossbite at the age of five years. An orthodontic appliance (facemask) was prescribed to assist forward growth of the nasomaxillary complex. The patient was instructed to wear the facemask together with the BiPAP appliance while sleeping and for as long as possible when awake. Orthopedic treatment for at least one year resulted in favorable forward growth of the maxilla and reduced facial concavity.