The treatment of neurocutaneous melanosis
A regular annual check of the moles by a dermatologist is important because they carry a high risk of degeneration. This means that the naevi can develop into melanomas (skin cancer). During the first weeks of life of an infant suffering from neurocutaneous melanocytosis, the large-area nevi can be abraded (dermabrasion).
However, due to the presence of numerous nevi, this is not completely possible. Therefore, abnormal nevi must be observed or, in case of doubt, removed. If additional neurological symptoms occur, such as internal hydrocephalus, neurosurgical surgery must be considered.
The affected patients must also be neurologically monitored throughout their lives using MRI and computed tomography (CT). If additional neurological symptoms occur, such as internal hydrocephalus, neurosurgical surgery must be considered. In addition, patients must be neurologically monitored throughout their lives using MRI and computed tomography (CT).
Prognosis
Patients with asymptomatic neurocutaneous melanocytosis have a normal life expectancy. In contrast, patients with a disease with neurological symptoms have an unfavorable prognosis.A large proportion of those affected die within the first three years after the symptoms have developed, as there is a high risk of a tumor of the meninges. In addition, further malformations of the brain often occur, with many patients developing hydrocephalus internus (“hydrocephalus”).
This is an enlargement of the fluid-filled cavities (ventricles) of the brain. The cerebrospinal fluid (cerebrospinal fluid) normally flows from the brain into the spinal cord. In neurocutaneous melanocytosis, moles form in the area of the outflow, which increase in size over time and thus impede the outflow of cerebrospinal fluid from the brain. As a result, the cerebral pressure increases and can cause severe damage to the brain.