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Basal Cell Nevus Syndrome: Symptoms and Treatment for Basal Cell Nevus Syndrome

The Basal cell nevus syndrome or nevoid basal cell carcinoma syndrome also known as the Gorlin's syndrome is a genetic disorder that involves a number of body organs. There are visual and internal defects that affect the skin, the central nervous system, the eyes, the endocrine glands, and the bones. The illness also causes an abnormal facial countenance and susceptibility towards malignant disorders of the skin. The rare condition takes place when the child inherits the autosomal gene which becomes the dominant trait in the individual. Hence the child who receives the unnatural gene from either parent is likely to contain the disorder. Those inherited with this disorder have remarkable physical features. They usually have eyes that are placed wide set, abnormally wide nose, and a leaden bulging eye brow.

Those who are screened with having this defective gene develop these unnatural facial features and are highly likely to develop basal cell carcinoma somewhere at the onset of puberty. Moreover the involvement of the nervous system in this disorder also causes great anguish to the patient. The individual can suffer from hydrocephalus, which is the accumulation of fluid in the brain thus causing a visual enlargement of the head, convulsions, some degree of retardation, ear problems resulting in deafness or tumors in the brain such as medulloblastoma. Where defects in the eye are concerned, the individual may have flaws in the iris or the lens and sometimes blindness can also occur. Imperfections in the bones comprise tumors or projection in the upper and lower jaw. These abnormalities may cause the jaw to form unnatural tooth growth and sudden fractures. Some patients are also affected with spinal or back bone defects that cause the back to curve inward, including scoliosis and kyphosis and also conditions involving rib abnormalities.

 

Basal Cell Nevus Syndrome: Basal Cell Carcinoma Prevention


In all the characterizing features of this disorder are not only ungainly to bear but also painful and may develop further complications owing to the present nature of the defects. This particular condition is usually treated by a number of specialists dealing with the various systems that have been affected. While an oncologist maybe sought to treat the tumor, an orthopedic doctor may be involved in the treatment of bony developmental deficiencies. If there is a family history of this condition it is necessary to seek advice from your medical practitioner while planning a child. Moreover if any child displays symptoms that are tend to suggest or imply the outset of this disorder, then medical help should be sought to clear all doubts. In many cases, an increasing number of couples and parents are opting for genetic counseling that will help reveal if the child may be affected, if there is a history of the condition.

 
   
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