GENERAL ANESTHESIA IN A PATIENT WITH CLEIDOCRANIAL DYSPLASIA

Cleidocranial dysplasia (CD) is a rare disorder that involves developmental abnormalities of bony structures. CD is characterized as an autosomal dominant skeletal dysplasia with a variety of clinical manifestations; most commonly supernumerary teeth, brachycephalic skull, short stature, and hypoplastic or aplastic clavicles. In a systematic review of a study population comparing CD individuals with non-affected relatives as controls, scoliosis and upper respiratory complications (including recurrent sinus infections, collapsing nasal passages, sleep apnea, and wheezing) were noted to be significantly increased in CD individuals in comparison with controls. Other possible manifestations of CD that involve upper airway structures include maxillary hypoplasia, high vaulted palate, and palatal clefting. The variety of CD associated structural abnormalities can potentially pose challenges to anesthetic management; however, there is limited literature describing anesthetic implications of CD patients undergoing surgery. Potential difficulties for airway management and neuraxial placement are described in the anesthetic management of a patient with CD who underwent a variety of obstetrical procedures. We present our approach to airway assessment and anesthetic management of a patient with CD undergoing a general surgical procedure.