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A 6-year-old child with Crouzon syndrome (CS) presents with bilateral inguinal hernia for repair. His parents report that a previous craniosynostosis repair, at the age of 9 months, and a LeFort III osteotomy, at the age of 4 years, were both associated with a difficult airway and that the anesthesia practitioners had told them to pass this information onto the next practitioner. The child is otherwise well and of normal intelligence.

47.2.1 What Is Crouzon Syndrome?

Crouzon and Apert syndromes are the most common of the craniosynostosis syndromes. In addition to craniosynostosis, these children also have fusion of the bony sutures in the cranial base and midface, and shallow eye sockets. This gives the appearance of a flat midface and eyes which protrude. Children with Apert syndrome (AS) also have syndactaly (webbing) of the hands and feet. The infant's shallow midface and/or small or partially obstructed nasal passages can cause airway compromise and airway management difficulties, as in this case. Evaluation by ENT specialists is important and a tracheotomy may be necessary to relieve chronic airway obstruction.1

CS occurs in approximately one in 25,000 births. It may be transmitted as an autosomal dominant genetic condition or appear as a fresh mutation (no affected parents). The appearance of an infant with CS can vary in severity from a mild presentation with subtle midface characteristics to severe forms with multiple fused cranial sutures and marked midface and eye problems. The incidence of AS is approximately one in 100,000 births and most cases are fresh mutations. The general features of a child with AS are similar to those in CS. However, there is not as much variability between cases and the degree of presentation is more severe.

47.2.2 Why Is the Team Approach So Important in Cases Like This?

Essential to the handling of this patient on an elective surgery basis is communication and preparation. The surgeon, on scheduling the case, should contact the anesthesia practitioner to advise them of the potential difficult airway management to prevent surprises and case cancellation. Should prior notification not occur, it is perfectly reasonable to delay this elective surgical procedure until information regarding the patient's past medical history is obtained.

The situation in an emergency is obviously much different with teamwork taking a responsive as opposed to a proactive posture, although many of the same specialists are usually involved in the care of these patients.

47.3.1 How Do You Assess the Airway of This Child? What If He Is Uncooperative?

As 2 years have passed since the last surgery, one can expect some growth changes in the airway (see Chapter 42). This patient ought to be approached as a new difficult airway. It is incumbent upon the anesthesia practitioner to take a good history, paying particular attention as to whether this child has difficulty in ...

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