Both nasal cerebral heterotopia and encephalocele in the same patient

OBJECTIVE AND PATIENT: Both nasal cerebral heterotopia and encephalocele are rare congenital benign masses of neurogenic origin caused by an embryonic developmental abnormality. It is generally accepted that nasal heterotopia is a sequelae to encephalocele. This report presents an unusual case of nasal cerebral heterotopia and encephalocele arising in the same patient. The patient had a firm, solid mass measuring 1.5 x 1.0 cm on the bridge of the nose covered with normal skin and another mass in the nasal cavity obstructing the right nasal cavity. Computed tomography (CT) demonstrated that the nasal bone separated these masses. CT also showed a bony defect at the skull base. Surgery consisted of dividing the encephalocele and closure of the skull base fistulae, along with nasal subcutaneous mass enucleation.

Intraoperative examination indicated the existence of a pit on the nasal bone where the pedicle of the nasal subcutaneous mass connected. Microscopic examination of the nasal cavity mass demonstrated meningoencephalocele, and examination of the nasal subcutaneous mass demonstrated nasal cerebral heterotopia, which was confirmed by immunohistochemical staining. After 10 months, complete removal of the subcutaneous nasal mass was recognized and there was no evidence of recurrence.

Findings in this case suggest that the nasal cerebral heterotopias will result from encephalocele.