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Rare incidence of Gorham disease with limited involvement of the maxilla: case report
ARTICLE IN PRESS
YBJOM-4747; No. of Pages 2
Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery xxx (2015) xxx–xxx
Letter to the Editor
Rare incidence of Gorham disease with limited involvement of the maxilla: case report Gorham disease is a rare disease of the bony skeleton that is characterised by osteolysis. The cause is unknown. The axial skeleton and mandible are the most commonly affected, and in rare incidences, the maxilla. We know of only three cases in the maxilla alone. Limited knowledge about the origins and pathophysiology of the disease make diagnosis challenging. To increase awareness, we describe a rare case, which was limited to the maxilla. A 54-year-old woman presented for evaluation of hyperplastic tissue after an unexplained loss of alveolar bone in the right maxilla. The history of increased mobility and subsequent loss of teeth 1-8 was relevant. She had ulcerated tissue around teeth 3 and 4. Radiographic examination showed extensive bone loss, erosion, and thinning of the right maxilla, sparing the nasopalatine canal (Fig. 1). An excisional biopsy was recommended, and histopathological examination was consistent with focal gingival fibrous hyperplasia. There was no sign of active disease, and she was followed up to ensure healing and resolution of the lesion. The course of the disease is unpredictable and bony resorption may halt spontaneously.1,2 The diagnosis can be difficult and is done by exclusion, which is based on history, and radiographic, histological, and laboratory findings.3 Presentation may include intermittent facial swelling, mild to moderate pain, mobility and loss of teeth, increased risk of pathological fractures, and deformities of the face.3 Johnson and McClure2
described two histopathological phases: one active with osteolysis, pain, and soft-tissue swelling, and the second quiescent, with fibrosis, and no active bony resorption, swelling, or pain.1–3 The usual findings on radiographic examination include localised osteolysis and ill-defined destruction of cortical bone.3 We diagnosed Gorham disease in a quiescent phase with limited involvement of the maxilla. The history, and radiographic and histopathological findings were consistent with this.2,3 Gorham disease presents as a local, but aggressive resorption of affected bones. The diagnosis relies on clinical, radiographic, and histopathological examination. The involvement of the maxillofacial skeleton can have serious complications that affect eating, breathing, and appearance. Considering the severity of possible outcomes, it should always be included in the differential diagnosis of diseases with unexplained osteolysis, despite its rarity.
Conflict of Interest We have no conflicts of interest.
Ethics statement/confirmation of patient permission We followed the guidelines of our Institutional Review Board. No personal details, identifying information, or images are included in the paper.
Source of grants None.
References
Fig. 1. Panoramic computed tomograph that depicts the extensive bony destruction limited to the right maxillary quadrant. Teeth 1-8 are missing as a result of the resorption of alveolar bone.
1. Heffez L, Doku HC, Carter BL, et al. Perspectives on massive osteolysis. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1983;55:331–43. 2. Johnson PM, McClure JG. Observations on massive osteolysis: a review of the literature and report of a case. Radiology 1958;71:28–42.
http://dx.doi.org/10.1016/j.bjoms.2016.01.006 0266-4356/© 2016 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons.
Please cite this article in press as: Zakhary I, Khanafer A. Rare incidence of Gorham disease with limited involvement of the maxilla: case report. Br J Oral Maxillofac Surg (2015), http://dx.doi.org/10.1016/j.bjoms.2016.01.006
YBJOM-4747; No. of Pages 2
2
ARTICLE IN PRESS Letter to the Editor / British Journal of Oral and Maxillofacial Surgery xxx (2015) xxx–xxx
3. Perschbacher SE, Perschbacher KA, Pharoah MJ, et al. Gorham’s disease of the maxilla: a case report. Dentomaxillofac Radiol 2010;39:119–23.
Ibrahim Zakhary ∗ Abraham Khanafer Department of Oral & Maxillofacial Surgery, University of Detroit Mercy School of Dentistry 2700, Martin Luther King Jr. Blvd., Detroit, MI 48208-2576
∗ Corresponding
author at: University of Detroit Mercy School of Dentistry 2700 Martin Luther King Jr. Blvd., Detroit, MI 48208-2576, Department of Oral & Maxillofacial Surgery Tel.: +313-494-6678. E-mail address: [email protected] (I. Zakhary)
Please cite this article in press as: Zakhary I, Khanafer A. Rare incidence of Gorham disease with limited involvement of the maxilla: case report. Br J Oral Maxillofac Surg (2015), http://dx.doi.org/10.1016/j.bjoms.2016.01.006
YBJOM-4747; No. of Pages 2
Available online at www.sciencedirect.com
British Journal of Oral and Maxillofacial Surgery xxx (2015) xxx–xxx
Letter to the Editor
Rare incidence of Gorham disease with limited involvement of the maxilla: case report Gorham disease is a rare disease of the bony skeleton that is characterised by osteolysis. The cause is unknown. The axial skeleton and mandible are the most commonly affected, and in rare incidences, the maxilla. We know of only three cases in the maxilla alone. Limited knowledge about the origins and pathophysiology of the disease make diagnosis challenging. To increase awareness, we describe a rare case, which was limited to the maxilla. A 54-year-old woman presented for evaluation of hyperplastic tissue after an unexplained loss of alveolar bone in the right maxilla. The history of increased mobility and subsequent loss of teeth 1-8 was relevant. She had ulcerated tissue around teeth 3 and 4. Radiographic examination showed extensive bone loss, erosion, and thinning of the right maxilla, sparing the nasopalatine canal (Fig. 1). An excisional biopsy was recommended, and histopathological examination was consistent with focal gingival fibrous hyperplasia. There was no sign of active disease, and she was followed up to ensure healing and resolution of the lesion. The course of the disease is unpredictable and bony resorption may halt spontaneously.1,2 The diagnosis can be difficult and is done by exclusion, which is based on history, and radiographic, histological, and laboratory findings.3 Presentation may include intermittent facial swelling, mild to moderate pain, mobility and loss of teeth, increased risk of pathological fractures, and deformities of the face.3 Johnson and McClure2
described two histopathological phases: one active with osteolysis, pain, and soft-tissue swelling, and the second quiescent, with fibrosis, and no active bony resorption, swelling, or pain.1–3 The usual findings on radiographic examination include localised osteolysis and ill-defined destruction of cortical bone.3 We diagnosed Gorham disease in a quiescent phase with limited involvement of the maxilla. The history, and radiographic and histopathological findings were consistent with this.2,3 Gorham disease presents as a local, but aggressive resorption of affected bones. The diagnosis relies on clinical, radiographic, and histopathological examination. The involvement of the maxillofacial skeleton can have serious complications that affect eating, breathing, and appearance. Considering the severity of possible outcomes, it should always be included in the differential diagnosis of diseases with unexplained osteolysis, despite its rarity.
Conflict of Interest We have no conflicts of interest.
Ethics statement/confirmation of patient permission We followed the guidelines of our Institutional Review Board. No personal details, identifying information, or images are included in the paper.
Source of grants None.
References
Fig. 1. Panoramic computed tomograph that depicts the extensive bony destruction limited to the right maxillary quadrant. Teeth 1-8 are missing as a result of the resorption of alveolar bone.
1. Heffez L, Doku HC, Carter BL, et al. Perspectives on massive osteolysis. Report of a case and review of the literature. Oral Surg Oral Med Oral Pathol 1983;55:331–43. 2. Johnson PM, McClure JG. Observations on massive osteolysis: a review of the literature and report of a case. Radiology 1958;71:28–42.
http://dx.doi.org/10.1016/j.bjoms.2016.01.006 0266-4356/© 2016 Published by Elsevier Ltd. on behalf of The British Association of Oral and Maxillofacial Surgeons.
Please cite this article in press as: Zakhary I, Khanafer A. Rare incidence of Gorham disease with limited involvement of the maxilla: case report. Br J Oral Maxillofac Surg (2015), http://dx.doi.org/10.1016/j.bjoms.2016.01.006
YBJOM-4747; No. of Pages 2
2
ARTICLE IN PRESS Letter to the Editor / British Journal of Oral and Maxillofacial Surgery xxx (2015) xxx–xxx
3. Perschbacher SE, Perschbacher KA, Pharoah MJ, et al. Gorham’s disease of the maxilla: a case report. Dentomaxillofac Radiol 2010;39:119–23.
Ibrahim Zakhary ∗ Abraham Khanafer Department of Oral & Maxillofacial Surgery, University of Detroit Mercy School of Dentistry 2700, Martin Luther King Jr. Blvd., Detroit, MI 48208-2576
∗ Corresponding
author at: University of Detroit Mercy School of Dentistry 2700 Martin Luther King Jr. Blvd., Detroit, MI 48208-2576, Department of Oral & Maxillofacial Surgery Tel.: +313-494-6678. E-mail address: [email protected] (I. Zakhary)
Please cite this article in press as: Zakhary I, Khanafer A. Rare incidence of Gorham disease with limited involvement of the maxilla: case report. Br J Oral Maxillofac Surg (2015), http://dx.doi.org/10.1016/j.bjoms.2016.01.006