Wockhardt Hospital on the Bannerghatta Road in Bangalore successfully performed a rare and complicated spinal deformity correction surgery to save a 14 year old boy from leading a complete disabled life.
The teenaged patient was suffering from Neurofibromatosis which is an autosomal dominant disorder that affects the bone, the nervous system, soft tissue, and the skin with a severe spinal deformity. There have been only a few cases reported the world over on surgical treatment for a rotational dislocation of the spine in neurofibromatosis.
Doctors at Wockhardt Hospitals led by Dr K N Krishna, senior consultant in the brain and spine service performed an immediate surgery to prevent further deterioration of his condition.
“The case required considerable study and preparation as there is so little documentation on the surgery at this stage of Neurofibromatosis. A high level of expertise is needed to perform this surgery” said Dr. Krishna.
“In India, unlike western countries, spinal deformity in Neurofibromatosis tends to go undiagnosed due to lack of awareness and also very few hospitals have the infrastructure and expertise to treat this condition. In most cases patients advance to a stage where the spinal deformity can become severe, spinal cord damage can become irreparable and correction very dangerous” said Dr Dilip Gopalakrishnan, consultant spine surgeon, Wockhardt Hospitals.
The complex surgery involved spinal decompression, stabilization of the deformity and fusion of the spine thus restoring the function of the spinal cord and protecting it from further damage. The patient is now able to stand on his feet unassisted with relatively no pain and will soon be discharged. He will be able to attend school in January.
According to international studies, type 1 neurofibromatosis occurs in approximately 1 of 2,500-3,300 live births, regardless of race, sex, or ethnic background. The carrier incidence at birth is 0.0004, and the gene frequency is 0.0002. The incidence of type 2 neurofibromatosis is 1 case per 50,000-120,000 population.
There are two major subtypes type 1 neurofibromatosis, which is the most common subtype and is referred to as peripheral neurofibromatosis, and type 2 neurofibromatosis, which is referred to as central neurofibromatosis.
The severity of both types of neurofibromatosis is varies considerably. In families where more than one person has NF, the symptoms and complications for each affected person can be different. During diagnosis, it is not possible to assess whether a case will be mild or lead to severe complications. Both types of neurofibromatosis are a dominant genetic disorders. An affected person has 50 per cent chance of passing it on with each pregnancy. Neurofibromatosis also can be the result of a spontaneous mutation in the genetic material of the sperm or egg at conception in families with no previous history of NF. About half of cases are inherited, and the other half are due to spontaneous genetic mutation.