From July 1987 to January 1998, 14 patients, ages 1 to 19 years (mean
6 years), were seen with nasal dermoid sinus cysts, a congenital lesio
n with the potential for intracranial extension. Each patient was asse
ssed clinically for cyst location, symptoms, associated craniofacial d
eformity, and other congenital anomalies. In 5 (36 percent) of the 14
patients, ages 4 to 48 months (mean 25 months), computed tomographic s
cans indicated indirect signs of intracranial extension, which were co
nfirmed intraoperatively and histologically in all 5 patients. After n
eurosurgical consultation, a combined single-stage intracranial-extrac
ranial approach was used to excise the lesion. No perioperative compli
cations occurred. Clinical assessment (follow-up 20 to 40 months, mean
31 months), confirmed by postoperative CT scan 1 year after surgery,
indicated no evidence of recurrence, residual skeletal contour defects
, or deformity; forehead and nasal growth were qualitatively normal, a
nd scar appearance was satisfactory. Our experience indicates that int
racranial extension of nasal dermoid sinus cysts seen at a tertiary ca
re referral center are not rare, that computed tomography scan permits
accurate diagnosis, and that the single-stage intracranial-extracrani
al approach to resection is effective and results in minimal morbidity
.