title: First Vault Expansion in Apert and Crouzon-Pfeiffer Syndromes: Front or Back?
abstract: BACKGROUND
  Children with Apert and Crouzon-Pfeiffer syndromes are at risk of intracranial hypertension. Until 2005, when the authors switched to occipital expansion, their institution's preferred treatment was fronto-orbital advancement. However, it was still unclear whether (1) occipitofrontal head circumference (i.e., intracranial volume) was greater after occipital expansion than after fronto-orbital advancement; (2) the incidences of tonsillar herniation and papilledema were lower; and (3) visual acuity was better during follow-up. In these patients, the authors therefore compared fronto-orbital advancement with occipital expansion as the first surgical procedure.
  
  
  METHODS
  Measurements included repeated occipitofrontal head circumference as a measure for intracranial volume; neuroimaging to evaluate tonsillar herniation; funduscopy to identify papilledema; and visual acuity testing.
  
  
  RESULTS
  The authors included 37 patients (Apert syndrome, n = 18; Crouzon-Pfeiffer syndrome, n = 19). Eighteen underwent fronto-orbital advancement and 19 underwent occipital expansion (age at surgery, 1.0 versus 1.5 years; p = 0.13). Follow-up time in both groups was 5.7 years. The increase in occipitofrontal head circumference (+1.09 SD) was greater after occipital expansion than after fronto-orbital advancement (+0.32 SD) (p = 0.03). After occipital expansion, fewer patients with Crouzon-Pfeiffer syndrome had tonsillar herniation (occipital, three of 11; fronto-orbital advancement, seven of eight; p = 0.02); for both syndromes together, fewer patients had papilledema (occipital, four of 19; fronto-orbital advancement, 11 of 18; p = 0.02). Visual acuity was similar after fronto-orbital advancement and occipital expansion (0.09 versus 0.13 logarithm of the minimum angle of resolution) (p = 0.28).
  
  
  CONCLUSIONS
  The authors' preference for occipital expansion as the initial craniofacial procedure in Apert and Crouzon-Pfeiffer syndromes is supported by the greater increase it produces in intracranial volume (as evidenced by the occipitofrontal head circumference), which reduces the incidences of tonsillar herniation and papilledema.
  
  
  METHODS
  Therapeutic, III.
authors.affiliation: Rotterdam, The Netherlands From the Dutch Craniofacial Center, the Pediatric Intensive Care Unit; and the Departments of Radiology, Ophthalmology, and Neurosurgery, Sophia Children's Hospital-Erasmus University Medical Center.
authors.forename: Bart
authors.initials: B
authors.lastname: Spruijt
authors.forename: Bianca F M
authors.initials: BFM
authors.lastname: Rijken
authors.forename: Bianca K
authors.initials: BK
authors.lastname: den Ottelander
authors.forename: Koen F M
authors.initials: KFM
authors.lastname: Joosten
authors.forename: Maarten H
authors.initials: MH
authors.lastname: Lequin
authors.forename: Sjoukje E
authors.initials: SE
authors.lastname: Loudon
authors.forename: Marie-Lise C
authors.initials: MC
authors.lastname: van Veelen
authors.forename: Irene M J
authors.initials: IMJ
authors.lastname: Mathijssen
mesh_terms: D000168:Acrocephalosyndactylia / Q000175:diagnosis / Q000601:surgery*; D002508:Cephalometry; D002675:Child, Preschool; D003394:Craniofacial Dysostosis / Q000175:diagnosis / Q000601:surgery*; D003399:Craniotomy / Q000379:methods*; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D019651:Plastic Surgery Procedures / Q000379:methods*; D012189:Retrospective Studies; D013577:Syndrome; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome