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Gorham's disease affecting the maxillofacial skeleton.

Y Anavi1, W R Sabes, S Mintz

  • 1Oral and Maxillofacial Surgery, Detroit Receiving Hospital, Michigan.

Head & Neck
|November 1, 1989
PubMed
Summary
This summary is machine-generated.

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Gorham's disease, a rare condition affecting head and neck bones, often presents with pain and fractures. Accurate diagnosis requires correlating histology with clinical signs, with surgical reconstruction showing promise.

Area of Science:

  • Maxillofacial Surgery
  • Oncology
  • Pathology

Background:

  • Gorham's disease (GD) is a rare, idiopathic condition characterized by the progressive loss of bone.
  • Maxillofacial involvement is documented in 21 cases, with this report detailing an additional case.

Observation:

  • GD predominantly affects the mandible, often with associated maxilla or other head/neck bone involvement.
  • Common symptoms include pain, tooth mobility, pathological fractures, and facial deformity, with a mean diagnostic delay of 6.4 years.
  • Initial biopsy misdiagnosis occurred in 45% of reported cases.

Findings:

  • This case represents the sole instance of successful treatment via excision and autogenous bone graft reconstruction.
  • The disease shows a slight male predominance.

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Implications:

  • Accurate diagnosis hinges on integrating histological findings with clinical presentation.
  • Surgical intervention, specifically excision and autogenous bone grafting, offers a potentially curative approach for maxillofacial Gorham's disease.
  • Further research into diagnostic criteria and treatment modalities for this rare condition is warranted.