Treatment Algorithm and Long-Term Outcomes of Crouzon Syndrome

Purpose:  Crouzon syndrome is characterized by bicoronal craniosynostosis, maxillary hypoplasia, and exorbitism.(1-3)  Treatment of Crouzon syndrome requires multiple surgeries throughout a child’s life and few studies have described long-term outcomes of such interventions.(4)  We present our surgical algorithm, long-term surgical outcomes, and psychosocial status of Crouzon patients.(5)

Methods: A retrospective review was performed on patients diagnosed with Crouzon syndrome at the UCLA Craniofacial Clinic (n=84) between 1975-2011.  Patients treated for 15 years or more following frontofacial advancement with complete radiographic and clinical evaluations were included in the study (n=41). A complete psychosocial review was performed with a face-to-face or over-the-phone interview with patients and their families. Highest level of education completed, relationship status, and work history were all queried.

Results: The average age of patients included in the review was 31.6 years with a mean follow-up for 21.4 years. Primary procedures performed were strip craniectomy (n=5) and fronto-orbital advancement (n=31) during infancy.  Five patients received monobloc procedures at an early age instead of fronto-orbital advancements during infancy. Between 6-8 years of age, patients received one of the following operations: monobloc osteotomy with facial bipartition (n=6, 14%), monobloc osteotomy alone (n=11, 27%), or LeFort III distraction (n=12, 49%).  In 94% of patients who received midface advancement, patients displayed symptoms of airway obstruction or excessive globe exposure.  At skeletal maturity, 93% (n=38) of patients received Le Fort I advancement for class III malocclusion.  Psychosocial evaluation demonstrated that 40 out of 41 patients reported a normal social life.  The majority of patients received regular education (n=37) and a minority required special education (n=5). 19 patients were married, 15 were in a relationship, and the remainder single.

Conclusions: Long-term outcomes studies are essential to evaluating the extensive treatment Crouzon patients endure during their lifetime.  Our current report reviews our surgical algorithm and the functional, morphologic, and psychological improvement of patients who have completed treatment.