Timing of Operations for Syndromic Craniosynostosis in Children

Apert Syndrome

• Fronto-orbital advancement at 9–12 months: Once the sutures, fontanelles, and synchondroses fuse, the risk of intracranial hypertension is increased. At that point, usually around 9–12 months of age, early fronto-orbital advancement with cranial vault remodeling is indicated, as this should lower the risk of elevated ICP. Careful monitoring and regular follow-ups are indicated throughout, since intracranial hypertension has been found in some patients even after surgery. Should signs of elevated ICP develop, further decompression and remodeling is advised.
• Midface advancement mid-childhood: The age for midface advancement varies with each patient. The management of the midface deformity is best undertaken in mid-childhood, together with a reshaping of the anterior cranial vault. An early midface advancement risks endangering tooth buds and thereby permanent dentition, as discussed above. Early maxillary advancement does not lead to normalization of maxillary sutural growth.

Crouzon Syndrome

• Fronto-orbital advancement at 9–12 months: The initial management in Crouzon syndrome usually requires a fronto-orbital advancement at the age of 9–12 months, unless the child presents with severe exophthalmos or breathing problems, when an immediate correction is advised.
• Repeat fronto-orbital advancement if signs of elevated ICP: The child is followed at regular intervals, and if signs of elevated ICP occur, a repeat cranial fault decompression and reshaping is carried out as required. The location of the cranial vault restriction influences the region for which further decompression and reshaping is planned. A higher risk of dural tears and greater blood loss should be anticipated during repeat procedures.
• Midface advancement at 5–7 years: The definitive management of the midface deformity via a monobloc, facial bipartition or Le Fort III osteotomy is carried out between 5–7 years of age. An early maxillary advancement as advocated by Tessier was subsequently been found by him and others not lead to a normalization of maxillary growth (42, 43, 44). Consequently, further elective surgery and orthodontic treatment might be required.

Pfeiffer Syndrome

• Typically same as Crouzon: The staging of reconstruction is, as always, dependent on individual variations, but otherwise it is similar to that for Crouzon syndrome.

Saethre-Chotzen Syndrome

• Correction of craniosynostosis at 8–12 months: Craniosynostosis in the child with Saethre-Chotzen syndrome is usually corrected between the age of 8–12 months, and the timing and nature of surgery are again specifically tailored to the child.
• Coordinated treatment plan for associated anomalies: The correction of associated anomalies such as cleft palate or webbed fingers will be defined in a coordinated treatment plan.