Bannayan-Zonana Syndrome is a rare hamartomatous condition through the occurrence of a lot of subcutaneous hemangiomas, lipomas and macrocephaly. Bannayan-Riley-Ruvalcaba Syndrome is another term for this kind of condition. In an autosomal current form of the illness is hereditary though wild instances have been reported.
Furthermore, this condition is very closely connected to Cowden’s syndrome with numerous hamartomas. People with this disorder often present with macrocephaly with developmental postponement and hypotonia, which are identified during the preliminary years of life. Hamartomatous develops such as vascular deformities, subcutaneous and intestinal polyposis are the common findings.
Since the recently discovered findings in the genes, there is a proof that Cowden’s syndrome and Bannayan-Zonana Syndrome share a common abnormality in the genes. However, prior to those findings, there was no increased risk of growth in family members. Also, visceral and intracranial connection may occur in rare conditions and can result to indicative automatic compression and bleeding mostly of the roots of spinal nerve and spinal cord and may require surgical resection.
In addition, Bannayan-Zonana Syndrome is classified as an unusual illness. This shows that this condition affects not more than 200,000 people in the population of the United States. The complications of this condition include rectal hemorrhage and intussusceptions.
Moreover, some people who are suffering from this condition may have hamartomatous lesions building intracerebral bleeding or cord compression, or they may unusually have other malignant tumors. Thus, it is essential that neurosurgeons are conscious of the entity. Keep in mind that the early diagnosis of this disorder is also vital for genetic counseling.
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