Congenital Femoral Deficiency
CONGENITAL FEMORAL DEFICIENCY:
“CFD” represents a spectrum of congenital defects around the femur bone, the hip joint above, the knee joint below, and the muscles that cross the hip joint. The main feature of this congenital disorder is that the femur bone is short. In most children, not only is the femur bone severely shortened, but the hip joint is affected to some degree. In some cases, a hip joint may never form at all.
CFD may be associated with other limb deficiency abnormalities, with the most common one being a fibula hemimelia. Except when associated with very rare genetic defects, children with CFD are often very healthy and have normal intelligence. They can essentially lead normal lives.
For general treatment of Congenital Femoral Deficiency, please click here.
TREATMENT OF HIP DISORDERS ASSOCIATED WITH CONGENITAL FEMORAL DEFICIENCY
For children with CFD, there is variable hip involvement. Some hip abnormalities are very mild and do not require any treatment, and others are so severe that they cannot be reconstructed. Hip reconstruction, therefore, is focused on those children between these two extremes.
Type of Hip Deformity
For those children born with femoral deficiency, most will have deformities where the hip joint is flexed up, it is positioned away from the body (abducted), and twisted outward (external rotation) compared to normal. In addition, the neck-shaft angle is often quite abnormal. The average person has a angle from the neck of the femur to the shaft of the femur of approximately 135 degrees. Those with femoral deficiencies can have angles well less than 90 degrees. As the ball (head) of the femur is not positioned properly in the socket, the socket can also develop abnormally. Look at the picture to the right. This is a young patient with mild femoral deficiency on the right limb (which is on the left side of the x-ray). The left side is normal. Note that the neck shaft angle (yellow lines) is much less on the right side compared to the left (red lines). The socket is also more steep on the abnormal right side compared to the normal left side.
Purpose of Reconstruction
For those children that are planning on going limb lengthening surgeries, a hip reconstruction may be recommended prior to the initial lengthening surgery. The purpose of the reconstruction is two-fold. First, the reconstruction is designed to lengthen and stretch muscles and to try and recreate a more normal bony anatomy. Second, and very important, if the hip is not reconstructed prior to lengthening surgery, the hip may be at higher risk for dislocating during the lengthening surgery.
How the Reconstruction is Performed
The hip reconstruction is commonly known as the "super hip" procedure. The surgery is an "a la carte" type surgery designed to "fix" the parts of the hip that needs to be fixed prior to lengthening. It may involve one or all of the following aspects:
- Soft Tissue Releases: there are several muscles that are lengthen and/or released during the procedure to allow more range of motion. these include the hip flexors, the hip abductors (gluteal muscles), and one of the hip external rotators (piriformis tendon). The goal is to allow more range of motion but to decrease the overall weakness of the muscles around the hip due to the surgery.
- Femoral (Thigh Bone) Osteotomy: most of the time, the top of the femur bone will need to be cut in order to restore a more normal neck-shaft angle. Expect that some type of metal hardware will be used to hold the bone in place. Prior to limb lengthening, this hardware will need to be surgically removed.
- Pelvis Bone Osteotomy: If the socket is found to be too steep, then the pelvis bone may need to be cut as well.
The series of pictures below demonstrate a young boy before and after his "super hip" which was performed by a COA surgeon at age 2.
Depending on the age of your child and the extent of surgery that is performed, your child may need to be placed in a hip spica cast. These casts can be challenging to manage in a toddler. Rest assured, the COA team is here to help you get through it. Schedule an appointment today to meet one of our experienced COA limb team doctors.