Neurology India Medknow Publications on behalf of the Neurological Society of India ISSN: 0028-3886 EISSN: 1998-4022 Vol. 58, Num. 1, 2010, pp. 144-145

Neurology India, Vol. 58, No. 1, January-February, 2010, pp. 144-145

Letter To Editor

Gorham's disease of the calvarium

Ji Zhang, Jin Li, Liu Ling¹ , Yue Kang Zhang

Departments of Neurosurgery, ¹ Radiology, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, P. R. China.

Correspondence Address: Ji Zhang, Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, P. R, zhangjiweihui@yahoo.com.cn

Date of Acceptance: 29-Oct-2009

Code Number: ni10036

DOI: 10.4103/0028-3886.60412

Sir,

A 40-year-old man had an asymptomatic skull introcession over the right cranial vault that gradually enlarged over a period of two years. Physical examination revealed a large area of depression measuring 3 x 5 cm in size over the right parieto-occipital region. Neurological examination was normal. Plain X-ray film of the skull revealed destruction of the bone with sclerotic margins over the right parietal region [Figure - 1]. A computed tomography (CT) scan demonstrated the absence of diploe and outer table of right parietal bone; and also showed hyperdense area in the right parieto-occipital bone including hypodense cysts within it [Figure - 2]. Three-dimensional CT of cranium revealed a well-defined skull introcession with incomplete disappearance of the central bone matrix [Figure - 3]. A magnetic resonance imaging (MRI) study disclosed the intact brain tissue and the right parieto-occipital scalp depression with loss of subjacent calvarial marrow signal intensity [Figure - 4]. A Technetium-99 m-methylene diphosphate showed increased uptake at the margin of the right parieto-occipital bone. A biopsy revealed massive fibrous tissue, dilated vessels, sequestrums, and chronic inflammatory cells [Figure - 5]. The biopsy result was consistent with Gorhams′s disease (GD). The patient underwent surgery for resection of the inner table and peripheral portion of the skull introcession. Cranioplasty was also performed with artificial bone graft. Postoperative CT scan obtained three days after operation showed the bone defect completely repaired [Figure - 6]. The pathological examination indicated the same results as the initial biopsy. The patient recovered well and was discharged five days after the operation without any complication.

Gorham′s disease (GD) is characterized by spontaneous and progressive localized osteolysis associated with proliferation of vascular or lymphatic structures of bone resulting in bony destruction and resorption. ^[1],[2] GD of the calvarium is extremely rare and only eight cases have been reported in the English literature [Table - 1]. The clinical manifestations of GD are determined by the location of involvement. Our patient presented with incidental introcession over his calvarium and with progressive evolution. The diagnosis of GD is mainly based on clinical, radiological and histopathological findings. ^[3] Radiological findings and the biochemical results are usually non-specific, but helpful to exclude other diagnosis. Biopsy and histological examinations are quite important. The current treatment of GD primarily focuses on stopping osteoclastic activity. Surgery and/or radiation therapy have been used for treatment in most cases with variable outcomes. ^[4] Early surgical therapy, if possible, represents the optimal management modality of calvarium GD.

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