Abstract
BACKGROUNDCamurati-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.OBSERVATIONSA 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.LESSONSChronic 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.
Highlights
Camurati-Engelmann disease (CED) is a rare disorder characterized by progressive cranial hyperostosis and diaphyseal sclerosis of the long bones
We report a case of a patient with CED combined with transethmoidal meningoencephalocele
A 57-year-old man who had been diagnosed with CamuratiEngelmann disease (CED) at 9 years of age based on a marfanoid body, pain in the extremities, and fusiform-shaped cortical thickening of the diaphysis of the extremities suddenly developed cerebrospinal fluid (CSF) rhinorrhea
Summary
Camurati-Engelmann disease (CED) is a rare disorder characterized by progressive cranial hyperostosis and diaphyseal sclerosis of the long bones. OBSERVATIONS A 57-year-old man who had been diagnosed with CED at 9 years old suddenly developed cerebrospinal fluid rhinorrhea. 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. Camurati-Engelmann disease (CED) is a disorder characterized by progressive cranial hyperostosis and diaphyseal sclerosis of the long bones; only approximately 300 cases of CED have been reported.[1]. Chronic intracranial hypertension gradually occurs due to progressive cranial vault hyperostosis.[1]. We report a case of a patient with CED combined with transethmoidal meningoencephalocele
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