Keeping an eye on the curve
Regular scoliosis exams are important

Daniel Hedequist, MD, fits 13-year-old Jillian Ricciardelli with a Boston brace.

Shopping for bathing suits, Erin Williams saw that her 6-year-old daughter Megan's back and shoulders were visibly uneven. A trip to the pediatrician and a standard Adam's forward bend test showed that Megan had scoliosis. Instead of resting flat, her back had a small hump on one side. X-rays showed a total of three spinal curves, one at 40 degrees and two in the high 30s. Because of the multiple curves and their degree of severity, Megan was referred to Children's Hospital Boston.

Healthy spines naturally curve toward the front and back, but scoliosis is an unnatural side-to-side curve of the spineóeither in a C or S shapeóthat measures at least 10 degrees on x-ray. Scoliosis, which can be classified as structural or nonstructural, affects 2 percent of women and 0.5 percent of men. Structural scoliosis appears in an abnormal spine with a fixed lateral curvature and can have a range of causes, including disease, congenital and post-traumatic. Nonstructural scoliosis, also called functional scoliosis, occurs in a normal spine and is caused by a secondary condition such as an inflammation, spasm or variation in leg length. It often goes away once the secondary condition is treated. Additional forms of scoliosis include neuromuscular, associated with muscular dystrophies or cerebral palsy; and syndromic scoliosis, associated with neurofibromatosis, Marfan's disease and other connective tissue disorders.

The large majority of scoliosis cases (over 80 percent), however, are idiopathic and according to the National Institute of Arthritis and Musculoskeletal and Skin Diseases, three to five out of every 1,000 children develop spinal curves that are considered large enough to require treatment. Idiopathic scoliosis can run in families and usually occurs in children after age 10. Girls are much more likely to have scoliosis and to require surgery to correct it. Other forms of idiopathic scoliosis can occur in children younger than 10 years of age, but are very rare.

Standard school screenings for scoliosis occur in kindergarten, fourth, seventh and 10th grades. Between the ages of 10 and 14, regular screening is especially important, as children often experience an accelerated growth rate. "Idiopathic scoliosis usually pops up around early adolescence," says Daniel Hedequist, MD, assistant in Orthopaedic Surgery at Children's. "As they develop, their spines are growing rapidly, and if they have a curve, it can also grow rapidly."

The Adam's forward bend test that Megan underwent is the standard method for identifying scoliosis. "During the test, we look for specific asymmetries of the body when it is in certain positions," says Dr. Hedequist. "Are the shoulders level? Is one shoulder higher than the other? Is there symmetry of scapula in the back? Is there symmetry of the waist? Does one side look straight where the other side has more curves? Do the levels of the pelvis look equal?"

"We also look for signs of problems with the spinal cord in the middle of the back," he adds. "Do they have a hairy patch, do they have skin dimpling or a discoloration over the spinal column itself, all of which might mean something is wrong inside the spinal cord?"

It is important for pediatricians and general practitioners to check for scoliosis at routine physical check-ups, as only about half the states in the United States require public schools to test for the disorder. In New England, Connecticut, Maine, Massachusetts, and Rhode Island require school testing, but New Hampshire and Vermont do not.

Identifying curves by shape and location

When an Adam's forward bend test reveals signs of scoliosis, patients should be referred to an orthopaedic specialist for diagnosis and possible treatment. X-rays are usually required and, to minimize a child's exposure to radiation, it is best that only the orthopaedic specialist orders x-rays, based on knowledge of what will provide the most appropriate and valuable information for treating and diagnosing spinal deformities. "A lot of patients referred to Children's don't end up having scoliosis, but are appropriate referrals as they have some signs of scoliosis," says Dr. Hedequist. "Among children with straight spines, 30 to 40 percent will have some signs of scoliosis that merit closer examination through a referral, whether it is asymmetry of the shoulder blades, inequality of the shoulders or inequality of the waist," he adds.

Once a diagnosis of scoliosis is confirmed, the next step is treating it. And fortunately for Megan, scoliosis treatment has come a long way. Like most children with curves between 30 and 50 degrees, she was fitted with a Boston back brace. Unlike the old-style Milwaukee braces, with metal rods connecting the shoulders to a neck ring, the Boston back brace is made of lightweight plastic and is barely visible under clothes. Many patients, who otherwise would have spent months in the hospital after surgery, can now benefit from wearing a close-fitting brace like the Boston back brace or the Wilmington brace, both of which are custom made to fit a child or teen. "The goal of the brace is to stop the curve from progressing," says Dr. Hedequist. "If a curve can be treated with a brace instead of surgery, that's the best option."

It's important for kids who wear braces because of scoliosis to be active and fit. "Regular activities help children maintain their self-confidence, increase the strength and flexibility in their back and of course, exercise is an important part of staying healthy," says Dr. Hedequist.

Unfortunately, in Megan's case, her curves continued to progress. Just two years after being fitted for the brace, she required corrective surgery. "Once a curve is greater than 50 degrees, a corrective procedure can be done to straighten the spine by permanently implanting metal rods parallel to the spinal column," says Dr. Hedequist. Megan spent just six days in the hospital, went back to school in three and a half weeks and began regular activities in less than three months. Today Megan is doing well, playing softball, lacrosse and riding her bike. Children's Hospital Boston is one of a select group of hospitals involved in a prospective national study to determine the surgical outcomes of patients with idiopathic scoliosis.

 

	For more information or to refer patients, contact Children's Spinal Program in the Department of Orthopaedic Surgery at (617) 355-6021.