Assessment of long-term quality of life in patients with syndromic craniosynostosis

Several studies have analyzed the long-term stability of cranioplasty and midface
distraction in patients with craniosynostosis; however, few studies have investigated
long-term quality of life (QOL) and complications in adults with syndromic craniosynostosis.
This study aimed to investigate the QOL (social, physical, and psychosocial) of patients
with adult syndromic craniosynostosis. Patients aged ≥20 years with syndromic craniosynostosis,
who were surgically treated at a single craniofacial institution, were included in
this study. We investigated everyday inconvenience (using the World Health Organization
Disability Assessment Schedule questionnaire), any ongoing treatment, marital status,
and number of children. Totally, 18 patients aged 22–48 years (mean: 31.4 ± 9.2 years)
answered the questionnaire (Crouzon syndrome, 9; Apert syndrome, 5; Pfeiffer syndrome,
4). Of these, only one Crouzon syndrome patient was married; she was also the only
one with a child. Apert syndrome patients were found to have difficulty in understanding,
communication, and self-care because of their mental retardation and hand and foot
handicaps; however, their participation in society was the most aggressive. In contrast,
Crouzon syndrome patients had especially poor participation in society. In all patients,
any ongoing hospital treatment was due to ophthalmological conditions. Crouzon syndrome
patients have extremely poor QOL; the absence of mental retardation and hand and foot
handicaps forces them to live in mainstream society, for which they are emotionally
ill-equipped. It is necessary to treat these patients without any residual deformity
to provide psychological support and to create an accepting society.