Juvenile active ossifying fibroma is a rare lesion seldom seen by neur
osurgeons. It originates in the paranasal sinuses during childhood, gr
ows slowly, and encroaches on adjacent orbital and cranial compartment
s. In the past 3 years, four patients with this lesion were seen (thre
e men and one woman; mean age 28 years). The clinical presentations we
re different with each patient: sinusitis, meningitis, periorbital pai
n, and a unique case of a juvenile active ossifying fibroma presenting
with high-grade internal carotid artery stenosis and ischemic symptom
s. Three patients were treated by transfacial approaches: two with a t
ransfrontal-nasal approach and one with a transfrontal-nasoorbital app
roach. Two open resections resulted in gross-total excision and no rec
urrence as of the 2-year follow-up review. In the third patient, the t
umor-encased carotid artery was preserved at the expense of a complete
resection; that patient underwent superficial temporal artery-middle
cerebral artery bypass and remains without ischemic symptoms or tumor
recurrence at 2 years. The fourth patient underwent three subtotal end
oscopic resections and is also without symptomatic recurrence at 2 yea
rs. Three points must be made concerning these lesions. First, the cli
nical and radiographic characteristics of juvenile active ossifying fi
broma may not be easily recognized by neurosurgeons, which could lead
to misdiagnosis and mismanagement of these lesions. Second, this tumor
can encase the carotid artery and cause severe stenosis or occlusion.
Third, complete resection of the tumor is required to effect a cure,
and transfacial approaches, which give wide exposure of the sinuses, a
ppear to yield better, more radical resections than endoscopic procedu
res.