Frequently Asked Questions about Early Onset Scoliosis

What to Expect

Myths


What is early onset scoliosis?
Early-onset scoliosis is an abnormal side-to-side curvature of the spine that is diagnosed before age 10 years. There are several types of early onset scoliosis: congenital scoliosis, neuromuscular scoliosis, syndromic scoliosis, idiopathic scoliosis which can be either infantile or juvenile.
How will my child to be evaluated for early onset scoliosis?
The process of evaluating a child with early onset scoliosis consists of a detailed history and physical examination. X-rays of the spine and neck in multiple views will be necessary. Sometimes a computerized tomographic scan (CT) or a magnetic resonance imaging (MRI) will be done to get a three-dimensional view of the spine and to establish measurements of the chest wall deformity. Frequently an ultrasound of the kidneys and heart will be necessary since both organs are formed in the embryo at the same time as the vertebra. Consultations with other specialists such as geneticists, pediatrics and pediatric pneumologists will be required.
What are the possible complications of early onset scoliosis?
The spine curvature may increase (progress), as shown by x-rays taken over time. If the curve progresses, there is asymmetry of the chest wall and the function of the lungs can be affected.
What is thoracic insufficiency syndrome?
Thoracic insufficiency syndrome or TIS is defined as the inability of the thorax to support a normal respiration or lung growth. It can result from spinal deformity or from a primary chest wall problem. The common problems or deficiencies are: chest wall deformity, chest wall asymmetry and respiratory function.
What are the options for treatment of early onset scoliosis?
Treatment is individualized for each patient. The orthopaedic surgeon and his team of consultants are involved in the decision making process and will select the optimal treatment for your child. Treatment options are: observation, casting, bracing and surgery.
What type of surgery is done?
The purpose of surgery is to control the spinal deformity and allow for the chest wall and lungs to grow. The most common two surgeries are: Spine-based growing rods or rib-based growing rods (VEPTR). The surgical team will decide which system is best for your child; alternatives will be discussed with you.
How does the VEPTR work? (Vertebral Expandable Titanium Prosthetic Rib)
The VEPTR is an expandable titanium device that is attached to the ribs at the upper portion of the chest and to the lower vertebral bodies or the iliac crest (hip bone). As the VEPTR is expanded it controls or improves the spinal deformity and does not allow the deformity to progress. The goal of this operation is to make one (or both) sides of the chest more normal in shape. A larger chest can provide more room for the lungs to expand or grow.
How is the VEPTR surgery done?
The surgery is done under general anesthesia. Very frequently spinal cord function is monitored. The surgeon makes an incision in the upper portion of the child's back and attaches the VEPTR to the ribs. The lower portion of the VEPTR is inserted through incisions over the lower spine or the iliac crest. An initial expansion is done to correct some of the spinal deformity and to improve the space available for the lungs.
What are the risks of VEPTR surgery?
The risks associated with a VEPTR surgery include infection, bleeding, nerve and spinal cord injury, loosening of the device making it necessary for it to be reimplanted and other risks that depend on the nature of the chest and spinal deformities as well as the health of the child.
What happens in the immediate post-operative period?
Frequent change in position and sitting is important. The child is given medicines for pain and antibiotics for several days. They will be seen by a respiratory therapist for breathing exercises. The average time in-hospital is between 3 and 5 days depending on the child's principal diagnosis.
What happens after the initial VEPTR implantation?
After the initial surgery to place the vertical expandable prosthetic titanium rib (VEPTR), your child will be evaluated on a regular basis. Expansion surgeries will be scheduled about twice a year to expand the device depending on your child's growth.
What is an expansion surgery?
VEPTR expansion or lengthening is a short surgical procedure under general anesthesia involving an approximately 1-inch incision. Typically, children spend one night in the hospital.
What happens at the end of growth?
At the end of growth, or at 15-16 years of age there are several alternatives: observation or fusion of the vertebra with or without instrumentation. The decision depends on the child’s specific diagnoses and the results obtained with the VEPTR.

What to Expect

Myths