Anaesthetic challenges and perioperative management of a child with morquio syndrome posted for atlanto axial fusion and decompression: A case report

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 beta-galactosidase that decreases the catabolism glycosaminoglycans (GAGs) cause accumulation soft tissue, bone cartilage giving rise to severe skeletal dysplasias including critical pectus carinatum, kyphoscoliosis, odontoid hypoplasia resulting notable atlanto-axial instability, cervical stenosis, other joint deformities dwarfism. It poses major airway challenges there is limited mouth opening, deformation oropharyngeal laryngeal tissues, hypertrophied tonsils adenoids marked sleep apnea macroglossia. Keratan sulphate (predominant GAG) accumulates hyaline trachea tracheal stenosis tracheomalacia. Pulmonary, cardiac vital organs dysfunction adds risk anesthesia related complications. In this article, authors will discuss case a 9 years old female child with syndrome successful management general anaesthesia they faced.