[Congenital intranasal cephalocele: diagnosis and treatment]

Abstract

The term cephalocele indicates a rare congenital malformation in which intracranial contents are extended through a defect in the cranium and dura mater. Intranasal cephaloceles belong to the group of basal cephaloceles. They can easily be misdiagnosed as nasal polyps and this can be potentially fatal after erroneous polypectomy. Three cases of transethmoidal cephalocele are presented, each with intermittent cerebrospinal fluid (CSF) rhinorrhea. The presence of a positive 2-transferrin-band in the immunological tests performed on the nasal fluid proved particularly helpful in diagnosing CSF. Other clinical sings were nasal obstruction associated with a solid intranasal mass, recurrent sinusitis and extensive pneumocephalus associated with headache after forceful nose-blowing. In all cases CT-scan delineated the osseous defect in the anterior skull-base, although MRI proved superior in soft-tissue resolution and multiplanar scanning. In one case surgery was a frontal craniotomy combined with endonasal endoscopic ethmoidectomy while in the other two a transethmoid approach was used. The present report emphasizes the distinctive clinical features of congenital intranasal cephaloceles and indicates the diagnostic and surgical procedures.