Combination of severe facial and cervical vascular malformation with obstructive sleep apnea syndrome: diagnostic and therapeutic approaches.

The combination of obstructive sleep apnea syndrome and vascular malformation within the head and neck region is a rare condition, and interestingly, only a few cases have recently been published. Propagation of the vascular mass to the larynx and pharynx can cause breathing and swallowing difficulties. Due to these sypmtoms, examination and initiation of appropriate therapy for such patients are indeed challenging. We reviewed the literature available and present our case of a 64 year old woman emphasizing the complaints of sleep apnea syndrome and vascular malformation of the face and neck region. Polygraphic examination detected severe obstructive sleep apnea syndrome. The MR examination of the neck revealed extensive vascular mass narrowing the pharyngo-laryngeal region, thereby causing temporal bone destruction on the right side with intracranial propagation. ENT examination demonstrated significant narrowing of the pharyngeal lumen and the laryngeal aditus caused by multiple hemangiomas. CPAP titration showed the minimalization of the apnea-hypopnea index on the effective pressure level. Regular CPAP usage resulted in diminishing a majority of the patient's complaints. Our examination clearly demonstrates, obstructive sleep apnea syndrome coupled with significantly obstructing vascular malformation in the head and neck region can be effectively treated safely with a CPAP device, if surgical therapy is not possible. We summarized our findings and the data available in the literature to set up recommendations for the appropriate examination and therapy (including mask fit, etc.) of vascular malformations and hemangiomas causing pharyngo-laryngeal obstruction.