Calvarial hyperostosis
Calvarial Hyperostosis is a medical condition characterized by the abnormal thickening of the skull bones, specifically the calvaria, which is the upper part of the skull that encloses the brain. This condition can lead to various symptoms and complications due to the increased pressure on the brain and the encroachment on intracranial space. The exact cause of Calvarial Hyperostosis is not well understood, but it is believed to involve a combination of genetic and environmental factors.
Causes and Pathophysiology
The pathophysiology of Calvarial Hyperostosis involves the overproduction of bone tissue in the skull. This can be due to a variety of factors, including genetic mutations, hormonal imbalances, or as a secondary effect of other diseases. In some cases, it is associated with syndromes such as Craniodiaphyseal Dysplasia and Van Buchem Disease, which are characterized by generalized skeletal dysplasia.
Symptoms
Symptoms of Calvarial Hyperostosis may vary depending on the severity and extent of bone overgrowth. Common symptoms include:
- Headaches
- Visual disturbances
- Hearing loss
- Facial deformity
- Neurological deficits due to pressure on the brain
Diagnosis
Diagnosis of Calvarial Hyperostosis typically involves a combination of clinical evaluation and imaging studies. Magnetic Resonance Imaging (MRI) and Computed Tomography (CT) scans are particularly useful in assessing the extent of bone thickening and its effect on intracranial structures.
Treatment
Treatment options for Calvarial Hyperostosis are limited and mainly focus on managing symptoms and preventing complications. Surgical intervention may be considered in severe cases to relieve intracranial pressure or correct deformities. However, surgery carries risks and may not completely resolve the condition.
Prognosis
The prognosis for individuals with Calvarial Hyperostosis varies. In mild cases, the condition may be asymptomatic and not require treatment. In more severe cases, however, it can lead to significant morbidity due to neurological complications.
Epidemiology
The prevalence of Calvarial Hyperostosis is not well documented, as many cases may be asymptomatic or misdiagnosed. It can occur in individuals of any age but is typically diagnosed in childhood or early adulthood.
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Contributors: Prab R. Tumpati, MD