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| LETTER TO EDITOR |
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| Year : 2010 | Volume
: 58
| Issue : 1 | Page : 144-145 |
Gorham's disease of the calvarium
Ji Zhang1, Jin Li1, Liu Ling2, Yue Kang Zhang1
1 Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, P. R, China
2 Department of Radiology, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, P. R, China
| Date of Acceptance | 29-Oct-2009 |
| Date of Web Publication | 8-Mar-2010 |
Correspondence Address:
Yue Kang Zhang
Department of Neurosurgery, West China Hospital, Sichuan University, 37 Guo Xue Xiang, Wu Hou District, Chengdu - 610 041, P. R
China
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0028-3886.60412
How to cite this article:
Zhang J, Li J, Ling L, Zhang YK. Gorham's disease of the calvarium. Neurol India 2010;58:144-5 |
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.
| » References | |  |
| 1. | Choma ND, Biscotti CV, Bauer TW, Mehta AC, Licata AA. Gorham's syndrome: A case report and review of the literature. Am J Med 1987;83:1151-6.  |
| 2. | Gorham LW, Stout AP. Massive osteolysis (acute spontaneous absorption of bone, disappearing bone): Its relation to hemangiomatosis. J Bone Joint Surg 1955;37A:985-1004. |
| 3. | Möller G, Priemel M, Amling M, Werner M, Kuhlmey AS, Delling G. The Gorham-Stout syndrome (Gorham's massive osteolysis), A report of six cases with histopathological findings. J Bone Joint Surg Br 1999;81:501-6. |
| 4. | Mignogna MD, Fedele S, Lo Russo L, Ciccarelli R. Treatment of Gorham's disease with zoledronic acid. Oral Oncol 2005;41:747-50. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
[Table 1]
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