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Published on: June 21, 2019
Vertex extradural hematoma in association with Paget's disease of the skull
V G Ramesh1, K Deiveegan, V Soundappan
1Institute of Neurology, Madras Medical College and Govt. General Hospital, Chennai 600-003, India. drvgramesh@hotmail.com
Abstract:
Paget's disease of skull usually causes neurological complications such as basilar invagination, cranial neuropathies, etc. Occurrence of extradural hematoma in association with Paget's disease of skull is rare. A 48 year old man presented with headache and right upper limb weakness, two days after a fall from scooter. CT Scan of brain showed a large extradural hematoma at the vertex with the cranial vault showing features of Paget's disease. At surgery, no skull fracture or injury to the superior sagittal sinus was evident. There was diffuse oozing from the inner table of the skull, which showed features of Paget's disease. The extradural hematoma was evacuated and the patient made good recovery. He deteriorated a few hours after surgery. Follow up CT Scan showed diffuse brain swelling with minimal recollection of hematoma. Re-exploration showed a small recollection which was evacuated and the part of the oozing diseased skull was excised. The patient recovered completely. This is the first reported case of vertex extradural hematoma in association with the Paget's disease of skull. The unusual features are the absence of skull fracture and injury to the superior sagittal sinus. The increased vascularity of the skull due to Paget's disease has caused the hematoma by diffuse oozing from the inner table.
Insights
This study reports a rare case of extradural hematoma in a patient with Paget's disease of the skull. The hematoma, located at the vertex, formed due to oozing from the diseased skull, not a fracture.
Area of Science:
- Neurology
- Neurosurgery
- Medical Imaging
Background:
- Paget's disease of the skull can lead to neurological issues like basilar invagination and cranial neuropathies.
- Extradural hematomas are uncommon complications of Paget's disease of the skull.
Observation:
- A 48-year-old man with Paget's disease of the skull presented with headache and limb weakness after a fall.
- CT scans revealed a vertex extradural hematoma without skull fracture or superior sagittal sinus injury.
- Intraoperative findings included diffuse oozing from the inner table of the skull.
Findings:
- The extradural hematoma was successfully evacuated, but the patient experienced deterioration due to brain swelling.
- A second surgery to excise diseased skull and evacuate a hematoma recurrence led to complete recovery.
- This case highlights vertex extradural hematoma in Paget's disease of the skull, likely caused by increased vascularity and oozing.
Implications:
- This case expands the understanding of potential neurological complications associated with Paget's disease of the skull.
- It underscores the importance of considering unusual etiologies for extradural hematomas, even in the absence of trauma.
- The findings suggest that increased skull vascularity in Paget's disease can precipitate bleeding and hematoma formation.
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