---

Anaesthetic challenges faced in a child with Morquio syndrome- An orphan disease

Full Text PDF

---

Case Report

Author Details : Tasneem Fathima Ahmed*, S Navaneetha Krishnan, Lakshmi Ramakrishnan

Volume : 10, Issue : 1, Year : 2023

Article Page : 98-101

https://doi.org/10.18231/j.ijca.2023.017

Suggest article by email

Get Permission

Abstract

Introduction: Morquio syndrome is a progressive lysosomal storage disorder leading to accumulation of glycosaminoglycans in the soft tissue, cartilage, and bone causing skeletal dysplasia.
Case Report: We present a four-year-old child diagnosed with right side inguinal hernia posted for herniotomy. He had a large head with short neck and kyphoscoliosis. Pre-operative MRI whole spine shows a hypoplastic dens causing canal stenosis, indenting the cord at C1 level. General Anesthesia was planned. With a video laryngoscope on standy by, patient was induced after stabilizing the neck with a custom neck collar and the airway was secured with a Proseal Laryngeal Mask Airway.
Discussion: The airway management can be challenging because of accumulation of mucopolysaccharides in the soft tissue of upper airway and limited mouth opening due to involvement of temporo mandibular joints.
Conclusion: A thorough pre-operative evaluation of airway must be done and a well planned anaesthetic technique must be executed.
 

Keywords: Morquio syndrome, Kyphoscoliosis.

How to cite : Ahmed T F, Krishnan S N, Ramakrishnan L, Anaesthetic challenges faced in a child with Morquio syndrome- An orphan disease. Indian J Clin Anaesth 2023;10(1):98-101

This is an Open Access (OA) journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.