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Repeat Intracranial Expansion After Skull Regrowth in Hyperostotic Disease: Technical Note
Timothy Wong1, Yehuda Herschman2, Nitesh V Patel2
1Rutgers, Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA.
World Neurosurgery
|February 1, 2017
Summary
Camurati-Engelmann disease (CED) is a progressive genetic disorder causing bone overgrowth. A novel surgical approach using multiple craniectomies and titanium mesh cranioplasties may offer long-term relief for recurrent symptoms.
Area of Science:
- Neurosurgery
- Genetics
- Orthopedics
Background:
- Camurati-Engelmann disease (CED) is a rare autosomal-dominant disorder characterized by progressive hyperostosis, leading to increased intracranial pressure and cranial nerve dysfunction.
- Surgical decompression offers symptomatic relief but recurrence due to bony regrowth is a concern.
Observation:
- A 46-year-old female CED patient presented with recurrent increased intracranial pressure two decades after initial surgical interventions.
- Skull imaging revealed significant cranial bone rethickening, drastically reducing intracranial volume.
Findings:
- A radical craniectomy involving four bone flaps and subsequent cranioplasties with titanium mesh was performed.
- This intervention aimed to preserve the skull's natural contour while addressing hyperostosis.
Implications:
- CED should be managed as a chronic condition due to its progressive nature.
- Multiple craniectomies with titanium mesh cranioplasties may provide more durable symptomatic control and reduce recurrence risk in CED patients.
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