脳障害と治療

概要

Respiratory and Cranial Complications during Anaesthesia in Pfeiffer Syndrome

Ela Erten, Nedim Çekmen, Ferruh Bilgin and Mehmet Emin Orhan

Pfeiffer syndrome (PS) is rarely encountered, even at major craniofacial centers. Published reports indicate high mortality rates (25-85%) for severely affected subtypes. PS is characterized by bilateral coronal craniosynostosis, midface hypoplasia, beaked nasal tip, broad and medially deviated thumbs and great toes. We present a case of a 12-monthsold male infant with PS type 2 who underwent a craniosynostosis and advancing a supraorbital bar operations during general anesthesia. For simple procedures, a safe anesthetic plan can be formulated if the anatomic factors affecting the airway are carefully considered. More extensive and prolonged patient diagnosed to have PS require more monitoring and include all the problems associated with difficult intubation, hazardous airway management, massive blood loss and fluid shift, shunt-dependent hydrocephalus, and long anesthetic times. This case is presented since anaesthesiologists should be aware of the high incidence of respiratory and cranial complications in PS.