Congenital Syndromes and Conditions

Abstract

Robin sequence is a clinical triad of micrognathia, glossoptosis and airway obstruction, often in association with cleft palate. The small jaw limits oropharyngeal space, making it difficult to compress the tongue sufficiently with the laryngoscope blade to gain a view of the larynx during direct laryngoscopy. The airway can be maintained with a supraglottic airway which bypasses airway obstruction at the level of the tongue. A hyperangulated videolaryngoscope facilitates intubation. Achondroplasia is the commonest cause of dwarfism. The craniocervical junction is affected and children with achondroplasia often need surgical decompression of the foramen magnum. Airway management is usually straightforward, limiting neck movement as much as possible. Children with Trisomy 21 often present for anesthesia for ear nose and throat surgery. Congenital heart disease, anxiety, atlantoaxial instability and obstructive sleep apnea are potential concerns for anesthesia.

Review Questions

• 1. Robin Sequence is associated with mandibular hypoplasia. Why does this cause airway obstruction and difficulty with intubation?

• 2. A 7 year old child with Trisomy 21 presents for tonsillectomy. He has no neck symptoms.

  • (a) Why is he at risk of neurological problems with anesthesia and surgery?

  • (b) Would you request cervical spine X-rays before anesthesia?

  • (c) What precautions would you take to minimize the risk of neurological sequelae?