Skip to Main Content

++

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 ...

Want remote access to your institution's subscription?

Sign in to your MyAccess profile while you are actively authenticated on this site via your institution (you will be able to verify this by looking at the top right corner of the screen - if you see your institution's name, you are authenticated). Once logged in to your MyAccess profile, you will be able to access your institution's subscription for 90 days from any location. You must be logged in while authenticated at least once every 90 days to maintain this remote access.

Ok

About MyAccess

If your institution subscribes to this resource, and you don't have a MyAccess profile, please contact your library's reference desk for information on how to gain access to this resource from off-campus.

Subscription Options

AccessAnesthesiology Full Site: One-Year Subscription

Connect to the full suite of AccessAnesthesiology content and resources including procedural videos, interactive self-assessment, real-life cases, 20+ textbooks, and more

$995 USD
Buy Now

Pay Per View: Timed Access to all of AccessAnesthesiology

24 Hour Subscription $34.95

Buy Now

48 Hour Subscription $54.95

Buy Now

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.