Surgery for Achondroplasia in Children

There is no way to prevent or cure achondroplasia, a genetic condition that causes short stature. However, doctors at Hassenfeld Children’s Hospital at NYU Langone may recommend orthopedic surgery or neurosurgery when the condition leads to problems with the spine or other parts of the body.

Surgery may occur in childhood or in adulthood, depending on when symptoms arise.

Spinal Fusion

Spinal fusion surgery may be needed to correct progressive spinal deformities, such as severe kyphosis, which is a curvature in the middle of the back. Our orthopedic surgeons are highly experienced in performing this surgery in children with achondroplasia, many of whom have significant changes in the structure of the back as well as spinal cord compression.

During spinal fusion, the surgeon uses rods, screws, and small pieces of bone—called bone grafts—to fuse the vertebrae, which are tiny bones in the spine, that are causing the curvature. This can also prevent the curve from returning.

In children with achondroplasia, spinal fusion surgery is a major operation that is performed using general anesthesia and takes four to five hours to complete. The rods can be left in place permanently, and most people do not require a brace after surgery. The vertebrae usually fuse together completely within three to six months.

After surgery, your child may remain in the hospital for a few days, until he or she can walk and perform tasks such as dressing and climbing steps. Your child may continue to recuperate at home for three to four weeks, avoiding extreme bending, twisting, stooping, or lifting of heavy objects. He or she can typically return to regular activities within two to three months.

Complications, such as pain and incomplete healing of the vertebrae, are rare.

Decompression Surgery

In some people with achondroplasia, the vertebrae may compress the spinal cord or brainstem—the stem-like base of the brain that connects to the spinal cord—causing severe pain and muscle weakness. Our neurosurgeons, working alongside our orthopedic surgeons, may perform decompression surgery, which involves removing bone from the foramen magnum—the area that connects the spine and skull—or along the spinal column to relieve pressure on the spinal cord or brainstem.

Decompression is often performed at the same time as spinal fusion surgery. Your child may return to regular activities within a few weeks, although recovery may take longer if the procedure was performed at the same time as spinal fusion.

Although this procedure is usually done during childhood, it may also be performed when an adult with achondroplasia has spinal compression.

Guided Growth Plate Surgery

Guided growth plate surgery, also known as hemiepiphysiodesis, may be recommended to correct a curvature, or bowing, in the legs. In guided growth plate surgery, the surgeon makes a small incision at the growth plate and attaches a titanium plate to one side of the bone with titanium screws. This temporarily stops the longer-length portion of the leg from growing, so that the shorter side can continue to grow and catch up. This procedure is often used in children who are still growing and have time for the bowing to correct during growth.

Growth plate surgery is usually an outpatient procedure that can be completed in about one hour. Your child may need to use crutches for a few weeks after surgery to allow the bones to heal.

Osteotomy

An osteotomy is an operation sometimes used to reset severely misshapen bones in the lower legs in children, adolescents, and some adults with severe bowing. The surgeon cuts the tibia, a large bone on the inner portion of the lower leg, and the fibula, a thinner bone parallel to the tibia.

The surgeon realigns the bones with internal pins, plates, and screws or with an external fixator—a frame worn outside the body and attached to the bone with pins—that is attached during osteotomy surgery. Wearing the fixator corrects bone alignment and reverses abnormal rotation of the leg bone. Additional rods may be used to stabilize the frame, which your child may wear for 8 to 12 weeks.

Internal fixators, which are less cumbersome than external devices, may be used in children older than age 8 and adults who have less severe bowing. A second operation is performed to remove these devices.

Osteotomy and additional surgery to remove internal fixators are performed using general anesthesia. Most children stay in the hospital for two to three days after osteotomy.

Other Types of Surgery

Our orthopedic surgeons may recommend procedures that lengthen the legs and arms. Our surgeons can discuss different options with families who are interested in these treatments.