Von Recklinghausens
INFORMATION ON VON RECKLINGHAUSEN'S DISEASE:-
Von Recklinghausen’s disease may be defined as a rare genetic disorder of the nervous system that mainly affect the development and growth of neural (nerve) cell tissues. Von Recklinghausen’s disorders cause tumors to grow on nerves and produce other abnormalities such as change in skin, changes and bone deformities.
CAUSES OF VON RECKLINGHAUSEN'S DISEASE:-
The only and the important reason for the development of Von Recklinghausen’s disease is mutation in gene of an individual.
SYMPTOMS OF VON RECKLINGHAUSEN'S DISEASE:-
1. Skin abnormalities.
2. Bone deformities.
3. Epilepsy
4. Progressive vision loss
5. Hearing disturbances
6. Balance disorders
7. Sensation disorders
8. Paresthesias
9. Spinal tumors
10. Brain tumors
DIAGNOSIS OF VON RECKLINGHAUSEN'S DISEASE:-
Clinically Von Recklinghausen’s disease can be identified by:-
1. CT scan.
2. MRI.
3. X- Ray.
4. DNA testing.
TREATMENT OF VON RECKLINGHAUSEN'S DISEASE:-
Treatment for Von Recklinghausen’s disease may involve the use of braces and or pulsating electromagnetic fields.
Bracing can prove to be both preventive and therapeutic. Children should be fitted with polypropylene knee-ankle-foot orthoses as soon as they start to stand or walk around furniture.
The source of pulsating electromagnetic fields may be external, such as the clamshell device over an ankle-foot orthoses, or an internal unit implanted in the soft tissue around the area of pseudarthrosis, usually in conjunction with an Autogenous bone graft. The effectiveness of this form of treatment remains highly controversial.
Autogenous bone is placed into the excised pseudarthrosis site by surgical method. An intramedullary rod is placed from the proximal tibia across the pseudarthrosis site, incorporating the graft and extending down through the ankle across the talus and into the calcaneus. Rod replacement is required as the patient grows, and continuous bracing is necessary.
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