Airway Management in a Patient with Infantile Systemic Hyalinosis

Fatemah Qasem, Ahmad Abotaiban

Abstract

Infantile Systemic Hyalinosis (ISH) is a rare inherited disease that is found among some families in Arab region where consguinity is not uncommon. Patients with ISH have distinctive clinical features that label them as difficult intubations. This includes limited mouth opening, restricted neck movement, protrusion of the mandible, and gingival hyperplasia. We describe the anesthetic management in 24 year old lady who presented for an elective procedure and the different ways to approach these patients.

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