BACKGROUND: The facial features of children with FGFR3Pro250Arg mutation (Muenke 
syndrome) differ from those with the other eponymous craniosynostotic disorders. 
We documented midfacial growth and position of the forehead after fronto-orbital 
advancement (FOA) in patients with the FGFR3 mutation.
METHODS: We retrospectively reviewed all patients who had an FGFR3Pro250Arg 
mutation and craniosynostosis. Only patients who had FOA in infancy or early 
childhood were included. The clinical records were evaluated for type of sutural 
fusion; midfacial hypoplasia and other clinical data, including age at 
operation; type of procedures and fixation (wire vs resorbable plate); frequency 
of frontal readvancement, forehead augmentation, midfacial advancement; and 
complications. Preoperative and postoperative sagittal orbital-globe 
relationship was measured by direct anthropometry. Outcome of FOA was graded 
according to the Whittaker classification as category I, no revision; category 
II, minor revisions, that is, foreheadplasty; category III, alternative bony 
work; category IV; redo of initial procedure (ie, secondary FOA). Midfacial 
position was determined by clinical examination and lateral cephalometry.
RESULTS: A total of 21 study patients with Muenke syndrome (8 males and 13 
females) were analyzed. The types of craniosynostosis were bilateral coronal 
(n=15), of which 3 also had concurrent sagittal fusion, and unilateral coronal 
(n=5). Two patients had early endoscopic suturectomy, but later required FOA. 
Mean age at FOA was 22.9 months (range, 3-128 months). Secondary FOA was 
necessary in 40% of patients (n=8), and secondary foreheadplasty in 25% (n=5) of 
patients. No frontal revisions were needed in the remaining 35% of patients 
(n=7). Mean age at initial FOA was significantly younger in the group requiring 
repeat FOA or foreheadplasty compared with patients who did not require revision 
(P<0.05). Location of synostosis, type of fixation, and bone grafting did not 
significantly affect the need for revision. Only 30% (n=6) of patients developed 
midfacial retrusion.
CONCLUSIONS: The frequency of frontal revision in patients with Muenke syndrome 
who had FOA in infancy and early childhood is lower than previously reported. 
Age at forehead advancement inversely correlated with the incidence of relapse 
and need for secondary frontal procedures. Midfacial retrusion is relatively 
uncommon in FGFR3Pro250Arg patients.