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Frontiers of Medicine

ISSN 2095-0217

ISSN 2095-0225(Online)

CN 11-5983/R

Postal Subscription Code 80-967

2018 Impact Factor: 1.847

Front Med Chin    2009, Vol. 3 Issue (2) : 211-215     DOI: 10.1007/s11684-009-0024-7
RESEARCH ARTICLE |
Fibrous dysplasia involving the calvarium in children?
Chunquan CAI1,3, Qian LI2, Qingjiang ZHANG3, Changhong SHEN1()
1. Department of Neurosurgery, General Hospital of Tianjin Medical University, Tianjin 300052, China; 2. Department of Medical Imaging, Tianjin Medical University, Tianjin 300070, China; 3. Department of Pediatric Neurosurgery, Tianjin Children’s Hospital, Tianjin 300074, China
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Abstract  

To gain a broader appreciation of the clinical presentation, operative treatment, and outcome of fibrous dysplasia involving the calvarium in children, we retrospectively reviewed a series of cases of fibrous dysplasia involving the calvarium (4 males and 2 females) with patients’ age ranging from 5 to 12 years old. The clinical manifestation, radiographic findings, surgical treatment, outcome and follow-up were evaluated on the basis of medical records. Fibrous dysplasia in the series was monostotic, involving frontal bone (2 cases), temporal bone (1 case), parietal bone (2 cases) and occipital bone (1 case). The patients most commonly presented with enlarging mass and cosmetic complaints. The treatment given, depending on clinical presentation, was simple biopsy with conservative follow-up (2 cases) to cranial resection (4 cases). All the cases were histopathologically confirmed as fibrous dysplasia. It was demonstrated thatfibrous dysplasia involving the calvarium is a typically benign but slowly progressive disorder of bone. Modern imaging modalities and histopathologic analysis have made diagnosis relatively straightforward. Surgery should be reserved for patients with functional impairment or cosmetic deformity. Because of the benign nature of the condition, the surgery itself should be contemplated with great caution in children.

Keywords fibrous dysplasia      calvarium      monostotic      child     
Corresponding Authors: SHEN Changhong,Email:soy@tijmu.edu.cn   
Issue Date: 05 June 2009
URL:  
http://academic.hep.com.cn/fmd/EN/10.1007/s11684-009-0024-7     OR     http://academic.hep.com.cn/fmd/EN/Y2009/V3/I2/211
Fig.1  Photograph of a patient with fibrous dysplasia and marked cosmetic deformity that progressed in right frontal bone (case 4)
no.age(yr)/sexsymptomslocationsurgical procedureoutcome and follow-up
15/maleenlarging massparietal bonebiopsystable, 27 months
29/maleenlarging mass and eye proptosisfrontal bone(orbital roof)facial degloving andcraniofacial resectionstable, 48 months
315/femaleenlarging massand headachetemporal bonecraniotomystable, 50 months
412/maleenlarging massfrontal boneplanted a titanium plate after frontal craniotomystable, 18 months
57.5/fameleenlarging massoccipital bonebiopsyexpanding, 24 months
611/maleenlarging massparietal bonecraniotomystable, 30 months
Tab.1  Summary of patients’ characteristics
Fig.2  Head CT images obtained in a patient with fibrous dysplasia (case 3). (a) Axial-CT showing the expansion of the temporal bone of fibrous dysplasia; (b) three-dimensional CT demonstrating the extension of lesion; (c) postoperative axial-CT showing the absence of the lesion and defect of the calvarium.
Fig.3  Head CT images obtained in a patient with fibrous dysplasia (case 4). (a) Axial-CT showing the expansion of the frontal bone of fibrous dysplasia; (b) axial-CT with bone-window showing an oval lucent region surrounded by a capsule of dense bone (black arrow); (c) three-dimensional CT demonstrating the extension of lesion.
Fig.4  Intraoperative photographs (case 4). (a) The fibrous dysplasia bone was resected, and neither the dura, nor the surrounding tissues were invaded; (b) a titanium armor plate was planted to repair skull after frontal craniotomy.
Fig.5  Histological examination showing typical features of fibrous dysplasia (HE staining, × 100 and inset × 400—right lower corner)
Fig.6  A specimen’s section showings a hemorrhagic and necrotic cystic lesion in the expanded bone (case 4, the same patient with Fig.3)
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