Anesthetic care of a child with Kabuki syndrome

Kabuki syndrome (KS) is a rare genetic disorder associated with unique facial features, developmental delay, and multiple end-organ abnormalities. Specific phenotypic findings include long palpebral fissures, eversion of the lateral third of the lower eyelid, arched eyebrows with sparseness of the lateral one-third, a short columella with a depressed nasal tip, and prominent ears. Patients with KS frequently have associated skeletal deformities, short stature, cleft lip and palate, dental anomalies, joint laxity, cardiovascular abnormalities, renal malformations, recurrent pneumonia, recurrent otitis media, and hearing loss. Given the potential for end-organ involvement and congenital malformations, surgical intervention may be required to address these concerns. We present a patient with Kabuki syndrome who required anesthetic care during two operative interventions: laryngoscopy and supraglottoplasty at 5 months of age and open reduction for hip dislocation at 9 months of age. Previous reports of anesthetic care for patients with Kabuki syndrome are reviewed with discussion of perioperative concerns and options for anesthesia.