Since this syndrome encompasses multiple phenotypic characteristics and difficulty in treating/ supervision, it is crucial to have patients be set with a multidisciplinary team, that consists of nurses, radiologist, pathologist, audiologist, otolaryngologist, geneticist, ophthalmologist, neurologist, and lastly a neurosurgeon (1).
The ideal way to manage these patients through early detection, making sure patients/ populations at higher risk are screened, patients are monitored, and determining treatment from patients’ medical history; including whether or not the syndrome was caused sporadically or familial (1). It is extremely important to screen children for this syndrome, via eye and skin examination, since they have a higher risk of developing …show more content…
In attempting to treat patients via surgery after unsuccessful attempts with radiotherapy is a huge risk in causing patient death. Treatment also includes rehabilitation for those who have lost their hearing due to VS and can provide hearing aid or cochlear implants; however even with the implants many patients’ hearing are not improved due to damage of the cochlear (if so patients can instead have an auditory brainstem or midbrain implantation) (2, 6). If cochlear implants are successive then patients’ speech improves dramatically (6). Usually the implants are placed in after the removal of the initial case of VS the patient has encountered (6). Unfortunately, these devices are not that effective in protecting the hearing ability in patients and interferes with MRI scanning due to the implants themselves being magnets (6). Meningiomas are treated with more efficiency through surgical means, but not very effective through radiotherapy than it is for treating VS (6). Spinal tumors are also removed through surgical means (occurred in about 30% of patients); …show more content…
Genetic testing, in Europe (specifically in the UK), children aged ten and are at higher risk are often subjected to genetic testing and be monitored via MRI scanning to attempt to detect tumors as early as possible (1). Compared to the US, the genetic testing completely depends on individuals with family history and often the testing occurs a earlier before any signs would indicate such a diagnosis in order to make such preparation, if needed (1). The genetic tests utilized MLPA (multiple ligation dependent probe amplification); however, its 92% ability to detect mutations in tumors of patients, either non-mosaic or mosaic, is more effective than in sporadic patient’s whose samples were derived from blood (6). However, determining if a patient has mosaicism is much more difficult due to the fact genetic tests use blood samples that may not genetically match with what is found in the tumor (6). For children with a relative with the syndrome are often tested at the age of 10, routinely checked with MRI (bi-yearly to yearly when tumors form), visual examinations (as early as when they are born), and audiological tests (yearly) (2,
The ideal way to manage these patients through early detection, making sure patients/ populations at higher risk are screened, patients are monitored, and determining treatment from patients’ medical history; including whether or not the syndrome was caused sporadically or familial (1). It is extremely important to screen children for this syndrome, via eye and skin examination, since they have a higher risk of developing …show more content…
In attempting to treat patients via surgery after unsuccessful attempts with radiotherapy is a huge risk in causing patient death. Treatment also includes rehabilitation for those who have lost their hearing due to VS and can provide hearing aid or cochlear implants; however even with the implants many patients’ hearing are not improved due to damage of the cochlear (if so patients can instead have an auditory brainstem or midbrain implantation) (2, 6). If cochlear implants are successive then patients’ speech improves dramatically (6). Usually the implants are placed in after the removal of the initial case of VS the patient has encountered (6). Unfortunately, these devices are not that effective in protecting the hearing ability in patients and interferes with MRI scanning due to the implants themselves being magnets (6). Meningiomas are treated with more efficiency through surgical means, but not very effective through radiotherapy than it is for treating VS (6). Spinal tumors are also removed through surgical means (occurred in about 30% of patients); …show more content…
Genetic testing, in Europe (specifically in the UK), children aged ten and are at higher risk are often subjected to genetic testing and be monitored via MRI scanning to attempt to detect tumors as early as possible (1). Compared to the US, the genetic testing completely depends on individuals with family history and often the testing occurs a earlier before any signs would indicate such a diagnosis in order to make such preparation, if needed (1). The genetic tests utilized MLPA (multiple ligation dependent probe amplification); however, its 92% ability to detect mutations in tumors of patients, either non-mosaic or mosaic, is more effective than in sporadic patient’s whose samples were derived from blood (6). However, determining if a patient has mosaicism is much more difficult due to the fact genetic tests use blood samples that may not genetically match with what is found in the tumor (6). For children with a relative with the syndrome are often tested at the age of 10, routinely checked with MRI (bi-yearly to yearly when tumors form), visual examinations (as early as when they are born), and audiological tests (yearly) (2,