2023, Vol. 6, Issue 1, Part A

Morquio syndrome, also described as Mucopolysaccharidoses (MPS) type IV A in literature, are rare progressive and autosomal recessive lysosomal storage diseases characterised by deficiency of enzymes N- acetyl-galactosamine-6-sulphate sulphatase and beta-galactosidase that decreases the catabolism of the glycosaminoglycans (GAGs) that cause accumulation of glycosaminoglycans in soft tissue, bone and cartilage giving rise to severe skeletal dysplasias including critical pectus carinatum, kyphoscoliosis, odontoid hypoplasia resulting in notable atlanto-axial instability, cervical stenosis, other joint deformities and dwarfism. It also poses major airway challenges as there is limited mouth opening, deformation of the oropharyngeal and laryngeal tissues, hypertrophied tonsils adenoids resulting in marked sleep apnea and macroglossia. Keratan sulphate (predominant GAG) accumulates in the hyaline cartilage of the trachea giving rise to tracheal stenosis and tracheomalacia. Pulmonary, cardiac and other vital organs dysfunction also adds to the risk of anesthesia related complications. In this article, authors will discuss the case of a 9 years old female child with Morquio syndrome and the successful management of airway and general anaesthesia challenges they faced.