Apert syndrome

I avoid performing advancement of the frontal bone (FOA) too early by performing cranial expansion with springs. When FOA is delayed to age 2, it is much more stable. Sometimes foramen magnum decompression is performed. The fingers are separated in the first few years of life. Later in life surgery will be required to advance the midface and correct the bite. This surgery may become urgent if midfacial hypoplasia causes secondary airway obstruction.

Reference:Occipital expansion without osteotomies in Apert syndrome. C Davis,
M MacFarlane, A Wickremesekera J. Craniofacial Surg (submitted)

Crouzon syndrome

Cranial expansion is frequently required within the first two years of life because of raised intracranial pressure.

A frontal advancement is done and will give better coverage of the eyes. Midface advancement may be required for airway problems or aesthetics and the timing varies according to the presenting problem.

A Spectaclesplasty in the early teens gives a more normal periorbital appearance and limits teasing during adolescence. A maxillary osteotomy is preformed at the completion of skeletal growth to correct the bite.

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Pfieffer syndrome

This syndrome is associated with raised intracranial pressure and midfacial hypoplasia.

Surgery is required to expand the cranium and advance the midface for better airway, dental occlusion and globe coverage.

Cloverleaf skull

Cloverleaf skull is caused by premature fusion of multiple sutures. It may be a consequence of several different syndromes. The priorities of treatment are to relieve the raised intracranial pressure, prevent brainstem herniation, protect the eyes and ensure patency of the airway.