SUMMARY Suboccipital craniotomy and cervical laminectomy were performed in a patient with Camurati-Englemann's disease to relieve symptoms of medullary compression. In spite of surgical decompression, the patient expired on the fourth postoperative day from cerebellar herniation.Camurati-Englemann's disease, also known as progressive diaphyseal dysplasia, is an unusual disorder of bone metabolism.' The lesions are primarily characterised by symmetrical, fusiform hyperostosis and sclerosis of long bones and of the cranial base.2 Pain in the lower extremities, general muscle weakness, and a waddling gait are the most common neurological disturbances.3 Cranial nerve deficits can occur with involvement of the skull.4 Surgical treatment has included decompression of the optic or auditory nerves.24 Rarely, a patient will have signs and symptoms of increased intracranial pressure (ICP).5 Both the aetiology and surgical management of increased ICP in Camurati-Englemann's disease have yet to be clarified. by bony overgrowth. He had broad, thick extremities and digits. Neurological examination revealed diminished auditory acuity bilaterally and weakness of facial muscles, and nystagmus on lateral gaze. He had a wide based gait, mild dysmetria, hyperactive deep tendon reflexes in his legs, and Babinski's sign was present bilaterally. Fundi were normal. Methods and resultsLaboratory investigation revealed an alkaline phosphatase of 657 IU/100 ml (normal = 25-100 IU), a parathormone level of 59 pg/ml (normal = 230-630 pg/ml). Serum calcium, full blood count, urinalysis and electrocardiogram were normal. A chest radiograph was normal except for thickened ribs and clavicles. A metabolic bone survey showed a diffuse, symmetrical increase in cortical bone thickness with loss of intramedullary cavities. Plain radiograph skull, CT and cerebral angiography were performed but were difficult to interpret because of attenuation caused by thick, dense bone. Magnetic resonance imaging revealed diffuse cranial hyperostosis with no pneumatization of the paranasal or temporal sinuses (fig a). The cerebral hemispheres were normal. There was tonsillar and vermian herniation caudal to Cl.A decompressive suboccipital craniotomy and Cl-C2 laminectomy were performed to decompress the posterior fossa and cervico-medullary junction. The operation was lengthy and technically difficult because of the thickness and density of the bone. Gross inspection of an intraoperative, nondecalcified specimen revealed apparently normal bone. A thick dense dural band between the arch of C1 and the foramen magnum was identified and divided. The medulla and spinal cord pulsated normally. As the dura was opened, the patient's temperature rose sud- 1349Protected by copyright. on 11 May 2018 by guest.
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