Pediatric Syndromic Scoliosis

by HSS on the Move

The vast majority of scoliosis cases—a condition of the spine in which the spine curves to varying degrees—are  due  to unknown causes and can develop during the infantile, juvenile or adolescent age groups. Syndromic cases of scoliosis represent a unique group of these spine conditions. Diseases such as Marfan’s Syndrome, Ehlers-Danlos Syndrome, osteogenesis imperfecta, neurofibromatosis, Prader-Willi Syndrome, arthrogryposis, and Riley-Day Syndrome are some of the common syndromic causes of this condition. Dr. John Blanco, Pediatric Orthopedic Surgeon, discusses this disorder and common treatments:

• Syndromic scoliosis often results in curves in the spine early in life. As children with this condition grow, the curvature can progress and worsen. It’s important for a pediatric orthopedic surgeon to monitor the condition closely, because many of these curves may eventually need to be treated.

• Parents of children with syndromic scoliosis should also have their child evaluated by a geneticist and neurologist to determine which one of the disorders mentioned above, or others, could be the cause of the spine curvature. Sometimes these patients may have respiratory and cardiac conditions, related to the syndrome or secondary to severe spinal curvatures. Due to this risk, it is also important for these patients to be evaluated by a pediatric pulmonologist and cardiologist.

• A non-operative therapy for early scoliosis is to use a cast or a brace to attempt to prevent or delay the progression of the spine curvature.

• Because pediatric patients are growing rapidly, performing traditional spinal fusion surgery to stabilize the spine’s vertebrae and stop further growth should be avoided for this condition as long as possible, preferably after the age of 10.

There are other surgical options that do not require fusion. These include inserting growing rods—expandable devices that are attached to the top and base of the spine to guide and support straight growth.  Also, the VEPTR (Vertical Expandable Prosthetic Titanium Rib) technique uses another device to straighten the spine and separate the ribs, which may prevent respiratory problems. These treatments can help to control curve progression and allow for continued spinal growth and lung development in patients with significant curves at a young age if non-operative methods do not work.