Respiratory and Cranial Complications during Anaesthesia in Pfeiffer Syndrome

Pfeiffer syndrome (PS) is an autosomal dominant condition comprising bilateral coronal craniosynostosis, midface hypoplasia with a beaked nasal tip, and broad and medially deviated thumbs and great toes [1]. It is a clinically variable disorder and has been divided into three subtypes by Cohen in 1993 [2]. Originally, it was described in eight persons from three generations in a pedigree consistent with an autosomal dominant transmission. Since then, several reports have documented its high clinical and genetic heterogeneity [3]. The condition is usually detected in the newborn period or later, and very few prenatal ultrasound diagnoses have been reported. Type 1 is called classical PS, and its findings are moderate. Type 1 frequently shows normal neurological and mental development, and has better surveillance. Type 2 has an appearance of cloverleaf (trefoil) skull, and central nervous system is frequently involved. Type 3 differs with absence of cloverleaf skull from Type 2. Type 2 and 3 has a bad prognosis due to severe neurological complications and various visceral anomalies, and frequently death is seen at early ages [4-7]. These patients are candidates of difficult intubation because of existence of maxillary hypoplasia and associated narrow larynx and pharynx together with possibility of tracheal anomalies [8].