Fibrous Dysplasia in Humerus Shaft - Fixation or Curettage?: A Case Report
Aman Rai1, J K Giriraj Harshvardhan1, Sundar Suryakumar1
1Department of Orthopedic Surgery, Sri Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India.
Insights
Fibrous dysplasia in long bones can cause pathological fractures. Intramedullary fixation effectively treats these fractures, relieving pain and restoring mobility without bone grafting.
Area of Science:
- Orthopedics
- Skeletal Dysplasias
- Pathology
Background:
- Fibrous dysplasia is a benign skeletal disorder affecting osteoblastic differentiation.
- It commonly impacts the lower extremity and craniofacial skeleton, presenting in the first three decades of life.
- Radiologically, it appears as an expansile, lytic intramedullary lesion.
Observation:
- A 24-year-old woman presented with a 3-year history of right arm pain, worsening over 3 months, with swelling.
- Radiological imaging revealed an expansile lytic lesion in the humerus diaphysis, indicative of pathological fractures.
- Histopathology confirmed fibrous dysplasia without malignancy.
Findings:
- Intramedullary fixation using an interlocking nail was performed for the pathological humerus fracture.
- The patient experienced significant pain relief and improved shoulder range of motion post-surgery.
- Radiological and functional outcomes indicated successful fracture healing and good functional recovery.
Implications:
- Intramedullary stabilization of long bone fibrous dysplasia with pathological fractures can restore function and alleviate pain.
- This approach may obviate the need for curettage or bone grafting in managing such cases.
- Early intervention and appropriate surgical management are crucial for favorable outcomes in fibrous dysplasia.
Introduction:
Fibrous dysplasia is a benign skeletal condition characterized by abnormal osteoblastic differentiation and maturation, typically affecting the lower extremity and craniofacial skeleton. It usually manifests in the first three decades of life, with symptoms starting in early childhood. Radiologically, it appears as an expansile, lytic, intramedullary, diaphyseal, or metaphyseal lesion. Treatment options include nonsurgical surveillance, pharmacotherapy, and surgery. Bisphosphonates can manage painless lesions, whereas surgery is necessary for pathological fractures. Alternative treatments include curettage, bone grafting, and internal fixation.
Case Report:
A 24-year-old woman experienced pain in her right arm for 3 years, which had worsened over the past 3 months and was accompanied by swelling for 2 months. The pain was insidious, gradually progressive, and diffuse over the diaphyseal region of the humerus. Examination revealed bony swelling and thickening, restricted shoulder movement and no distal neurovascular deficit.Radiologically, there was an expansile lytic lesion involving the middle and distal third of the diaphysis of the right humerus, suggesting pathological fractures. A magnetic resonance imaging scan revealed an expansile intramedullary lesion involving almost the entire shaft of the right humerus, sparing the proximal metaphyseal region. Histopathological examination revealed hemorrhagic giant cells with fibroblastic proliferation, with no signs of malignancy. Intramedullary fixation was performed with an intramedullary interlocking nail. She began gentle shoulder range-of-motion exercises and pendulum exercises. The patient experienced significant pain relief with good functional outcome and signs of fracture healing.
Conclusion:
Long bones with fibrous dysplasia that manifest as pathological fractures may be painful and have limited mobility. The fracture can heal, pain can be reduced, and the functional range of motion can be restored by stabilizing the lesion with an intramedullary implant without curettage or bone grafting.
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