Camurati-Engelmann disease combined with transethmoidal meningoencephalocele: illustrative case

Abstract

BACKGOUND Camurati-Engelmann disease (CED) is a rare disorder characterized by progressive cranial hyperostosis and diaphyseal sclerosis of the long bones. Chronic intracranial hypertension gradually occurs due to progressive cranial vault hyperostosis. OBSERVATIONS A 57-year-old man who had been diagnosed with CED at 9 years old suddenly developed cerebrospinal fluid rhinorrhea. A bone defect of the right cribriform plate and protrusion of brain tissue from the right cribriform plate into the right nasal cavity were identified. The patient underwent endoscopic resection of the meningoencephalocele combined with the bath-plug procedure. After surgery, cerebrospinal fluid rhinorrhea disappeared. LESSONS Chronic intracranial hypertension due to progressive cranial vault hyperostosis in CED may cause a bone defect and meningoencephalocele in the anterior skull base, resulting in cerebrospinal fluid rhinorrhea.