Anesthetic implications of Pallister-Hall Syndrome in patients with a bifid epiglottis
Download full article

Kevin T. Riutort, Neil G. Feinglass, Sorin J. Brull

Department of Anesthesiology Mayo Clinic, Jacksonville, FL

Abstract

Purpose: We report a rare case of a patient with preoperative diagnosis of Pallister-Hall Syndrome (PHS) and a bifid epiglottis, and discuss briefly the anesthetic implications of this rare syndrome. Although PHS is very rare, the associated visceral and endocrine malformations place PHS patient at risk.

Clinical Features: PHS is an autosomal dominant congenital disorder with visceral and endocrine malformations. Craniofacial aberrations include bifid epiglottis and uvula, microglossia, micrognathia, hard palate malformation, cleft larynx, gingival cysts, and mandibular hypoplasia. Preoperative evaluation should focus on airway management using fiberoptic techniques. Intraoperatively, renal, endocrine, and neurological functional derangements must be anticipated. Post-operatively, oxygenation and ventilation monitoring is warranted because of airway anatomy malformations.

Conclusion: A high index of suspicion should be maintained in patients with congenital visceral and endocrine malformations.

Keywords: airway management; bifid epiglottis; congenital malformation; difficult airway; fiberoptic intubation; Pallister-Hall syndrom