Neurofibromatosis is a major cause of scoliosis. Neurofibromatosis is caused bygenetic defects that lead to abnormalities of neural crest cell and multi-system damage. Neurofibromatosis is divided into type I and type II based on clinical manifestations and gene mapping.
Cafe-au-lait spots and multiple neurofibromatosis of peripheral nerves are visible on surface of the skin after we exam the body of patient with Type I,which are more in non-exposed parts of the trunk. Also Lisch nodule is visible on eyes, that is upper eyelid fibroma or plexiform neurofibroma. The mass is palpable on orbit and moves when eyes goggle. Orange circular miliary nodules are visible on iris nodulessthrough lit lamp. It is hamartoma and specific performance of type I , can increase with age. Diagnostic criteria is more than 6 nodules with a diameter greater than 5mm (diameter is greater than 15mm after puberty ) with high diagnostic valuebefore puberty as well as the features of freckles on the body armpit.
Type I of neurofibromatosis easily lead to scoliosis. Patient will have early onset of the disease (usually before puberty) with rapid development of scoliosis and obviousdeformity. The curvature is stiff, and bone strength is decreased by itself etc.Orthopedic brace is difficult to deal with it.
Relative blog: The Application of Spinal Orthosis VI
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