July 10, 2002 at 9:27 PM CDT - Updated July 28 at 4:05 PM
Infantile Scoliosis Infantile scoliosis is a form of scoliosis that is present at birth or becomes apparent within the first year of life. Instead of developing in a straight pattern, the spine forms an abnormal sideways curvature – usually in the pattern of an “S” or “C.” The condition is relatively rare and occurs more commonly in girls than boys by a ratio of 2.5 to 1.
In some cases of infantile scoliosis, the degree of curvature is so severe one side of the middle of the body is unable to grow. The chest area is compressed, interfering with the ability of the lung on the affected side to expand and grow. Children can develop a condition called thoracic insufficiency syndrome and have difficulty breathing. (Thoracic insufficiency can also be caused by other problems, such as when a child has fused ribs or missing ribs.) In severe cases, a child may require placement on a ventilator.
Stabilizing the spine with bracing is usually not an effective treatment for infantile scoliosis. If the degree of abnormal curvature is severe or compromising a child’s health, surgeons may insert a growing rod in the child’s back. The top and bottom of the rod is attached to the spine with hooks. Every few months, the rod is surgically expanded to accommodate the growth of the child.
There are some drawbacks to the traditional spinal rod. Sometimes the hooks become dislodged and need to be reattached. To place the rod in the proper place, doctors may have to remove portions of the bones in the spinal column. That can lead to a stiffness of the spine and reduce the ability to make future corrections.
Treating Thoracic Insufficiency Syndrome Some doctors are testing a new device for children with severe thoracic insufficiency syndrome, such as those with severe infantile scoliosis. The device is called the Vertical Expandable Prosthetic Titanium Rib (VEPTR, also known as the titanium rib). One end of the titanium rib is attached to an upper rib. The other end is attached to the lower spine (for scoliosis) or lower rib (for rib malformations). As the child grows, doctors make a small incision in the back, pull out a locking clip, lengthen the rod, and then put in a new clip.
Like a car jack, the device supports the spine and/or ribs and expands the chest cavity. That gives room for the lungs to grow and eases the child’s breathing. The device, which is currently in an FDA clinical trial, can be used in children as young as six months and as old as about 17. Early treatment is recommended to give children’s lungs a chance to grow normally and provide the fullest lung capacity in adulthood. While the titanium rib can provide some children with immediate improvement in symptoms, in many cases, it simply stabilizes their condition. For children with severe scoliosis, the rib can prevent the abnormal curvature from getting worse, buying time until doctors can provide permanent correction when the child is older.
The following sites are participating in the trial of the titanium rib:
Los Angeles Children’s Hospital Los Angeles, CA
Children’s Hospital Boston, MA
Shriners Hospital for Children Philadelphia, PA
Children’s Hospital of Pittsburgh Pittsburgh, PA
CHRISTUS Santa Rosa Children’s Hospital San Antonio, TX
Primary Children’s Medical Center Salt Lake City, UT
Children’s Hospital and Regional Medical Center Seattle, WA
For general information, visit the web page of the Titanium Rib Project at http://www.titaniumribproject.8m.com. For information or referral, contact the Shriners Hospital for Children in Philadelphia, PA at (800) 281-4050. The following sites are participating in the trial of the titanium rib: Los Angeles Children’s Hospital, Los Angeles, CA; Children’s Hospital, Boston, MA; Shriners Hospital for Children, Philadelphia, PA; Children’s Hospital of Pittsburgh, Pittsburgh, PA; CHRISTUS Santa Rosa Children’s Hospital, San Antonio, TX; Primary Children’s Medical Center, Salt Lake City, UT; Children’s Hospital and Regional Medical Center, Seattle, WA
For general information on scoliosis: National Institute of Arthritis and Musculoskeletal and Skin Diseases, NIH, 1 AMS Circle, Bethesda, MD 20892-3675, http://www.niams.nih.gov