Morquio’s syndrome, a mucopolysaccharidosis IV storage disorder with an incidence of 1 in 200,000 children.The enzyme deficiency in type A and B is N acetyl galactosamine 6sulfatase and B galactosidase respectively.

CLINICAL PROFILE: 4 year old male child with morquios syndrome presented with progressive weakness of all four limbs and right side UMN facial palsy with cervical spine instability postedfor surgical decompression and dorsal fusion of cervical spine .

PRE ANAESTHETIC EVALUATION: large tongue, short neck, deformed pinna , low set ears,barrel chest, knock knee and flat foot. Spine imaging- assimilation of posterior arch of C1 within foramen magnum causing significant cervical canal stenosis , flattened cervical vertebra, atlanto – axial instability.hypoplasia of dense of C2 with Significant cord compression at craniovertebral junction at C1-C2 level. cord compression at D10-11. X- ray pelvis showed bilateral femoral head flattening

DISCUSSION: Uniqueness of airway anatomy due to deposition of mucopolysacrides in soft tissues of oropharynx and spine deformities in morquios syndrome often results in failed mask ventilation and intubation along with potential spinal cord injury. Thorough assessment of airway, cardio respiratory and neurological evaluation is important during pre-operative assessment. Spontaneous respiration , Manual inline stabilisation with head in neutral position for endotracheal intubation is recommended if facilities for fiber optic intubation in paediatric age group is not available..protection of eyes, padding of pressure points and sufficient knowledge of paediatric physiology can lead to successful anaesthesia outcome.