13 year old girl presented to KDAH with history of Spinal deformity. She came with complaints of worsening for spinal deformity, rib hump and breast asymmetry. Scoliosis is a medical condition in which a person's spine has a sideways curve. The curve is usually "S"- or "C"-shaped. Scanogram X-ray of the Spine was done which showed severe spinal deformity with the main curve of thoracic spine measuring about 85 degrees. The proximal thoracic curve and the lumbar curve where also significant (structural) and measured about 36 degrees. MRI Scan of the whole spine was done and it showed a large cystic cavity in the spinal cord (syrinx) with herniation of the brain at the cranio spinal Junction. This is called as the Arnold Chiari Malformation. She also had hydrocephalus. Scoliosis associated with Arnold Chiari malformation is subclassified as "syndromic scoliosis". 13 year old female child with scolosis. She presented with worsening deformity and rib hump.
Scoliosis with Arnold Chiari malformation on MRI. Patient has a large syrinx extending from upper cervical spine to lower thoracic spine.
Normally, the cerebellum sits at the back of the skull, just above the spine. When a child has a Chiari malformation, the space for the cerebellum is sometimes too small or unusually shaped. This can squeeze part of the cerebellum and even part of the brain down through a funnel-like hole below the skull. This hole is called the foramen magnum. A Chiari malformation can put pressure on the cerebellum, brain stem, and spinal cord and can keep those body parts from working properly. It also can block the movement of cerebrospinal fluid (CSF), the liquid that surrounds the brain and spinal cord and protects them from injury. When cerebrospinal fluid doesn't flow properly, it can cause hydrocephalus i.e a buildup of cerebrospinal fluid in the brain and spinal cord). This buildup can block communication of the brain's messages to the body and cause headaches, pain, numbness, and weakness.
The problem list can be summarized as:
1. Worsening Spine deformity with main structural cure of 85degrees.
2. Arnold chiari malformation.
3. Large Syrinx from upper cervical to lower thoracic spine.
4. Hydrocephalus
The Surgery: The Surgery is these patients are associated with significant risk of getting paralysis. The Surgery was planned in stages by Dr Abhijit Pawar. At stage one the decompression of the cranio vertebral junction (foramen magnum) was planned to remove the outflow obstruction to the cerebrospinal fluid. Surgery was done under neuromonitoring successfully. The child was observed for serial follow up for six month and the MRI was repeated at the end of 7 month. The follow up MRI showed the syrinx had significantly decreased in size.
X-ray picture showing good correction of spinal deformity and restoration of alignment.
The final scoliosis correction was planned. The major thoracic deformity and other 2 curves were corrected under precise neuromitoring. MEP and SSEP were monitored by the neurophysician continuously to ensure the patient does not get paralysis. All pedicle screws were placed on the concave side of the deformity and alternate on the convex side. Computer guided navigation techniques were used for placement of pedicular screws which ensure safe placement and minimal exposure to radiation. Precautions were taken to minimize the blood loss. The surgery was complete in about 5 hrs and the patient was shifted to recovery room in normal neurological condition. The pain was controlled post operatively by the expert pain management team at Kokilaben. The next day in the morning the child was made to sit and stand bed side. Active physiotherapy and walking was started on day 2. On 4 th post operative day the child was discharged. The child started going to school 1 month post surgery. She is not active and gets involved in sports 4 month post surgery.
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