Sun14November1246PM 23

Management of the airway may be difficult in which of the following syndromes and conditions?

True / False

Crouzon's syndrome Correct

Cystic hygroma Correct

Edwards' syndrome Correct

Hurler's syndrome Correct

Goldenhar's syndrome Correct

Explanation

All of the syndromes and conditions listed are associated with difficult airway management.

Crouzon's syndrome is an autosomal dominant craniofacial disorder. Features of this syndrome include hypoplastic maxillae and a high arched palate, which is occasionally cleft.

A cystic hygroma may occasionally appear high in the anterior triangle and have an associated intraoral lymphangioma. The airway compromise can be compounded by mediastinal extension of the hygroma.

Trisomy 18 or Edwards' syndrome is associated with apnoeic spells and micrognathia.

Goldenhar's syndrome (oculoauriculovertebral dysplasia) is a combination of micrognathia and an abnormal cervical spine, including odontoid elongation. The risk of cord injury is high and the airway management plan may require tracheostomy.

Hurler's syndrome is an autosomal recessive disorder characterised by an L-iduronidase deficiency. Deposition of acid mucopolysaccharide occurs in every organ system and they are prone to upper airway obstruction (large tongue, laryngeal and pulmonary lymphoid tissue infiltrates).

Answer Statistics

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Average score: 79.52%

Times answered: 291