Anesthetic care of a child with Kabuki syndrome

Abstract

Kabuki syndrome (KS) is a rare genetic disorder associ- ated with unique facial features, developmental delay, and multiple end-organ abnormalities. Specific pheno- typic 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, re- current 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 dur- ing two operative interventions: laryngoscopy and supra- glottoplasty at 5 months of age and open reduction for hip dislocation at 9 months of age. Previous reports of anes- thetic care for patients with Kabuki syndrome are re- viewed with discussion of perioperative concerns and op- tions for anesthesia.

Keywords

• kabuki syndrome
• pediatric anesthesiology
• periopera- tive care
• congenital heart disease
• hypotonia