Congenital Limb Deficiencies Approach


 

CONGENITAL FEMORAL DEFICIENCY

Congenital Femoral Deficiency is a rare hip disorder that we, at the Paley Institute, specialize in treating. The exact management depends on the type of CFD.  The approach is not just a, “wait and see” one. We are able to predict the limb length difference at maturity and then create a life plan for the child which includes a preparatory surgery by age three and then limb lengthening planned and performed starting at approximately age 5.


At the Paley Institute, we treat a great number of CFD patients. There are essentially four types with the first three in increasing severity and the fourth involving the knee. Treatment often includes an extensive SUPER hip operation that treats the deformity of the hip and then lengthening of the thigh and leg, at a later date. This surgery should only be performed by surgeons who are familiar with CFD and frequently operate on these types of cases. The procedure is quite complicated and if it is not done with appropriate support from physical therapists familiar with the disease, there is a risk of significant failure.  With appropriate treatment, the lower extremity can often be made quite functional with no prosthesis or amputation needed. The leg often has involvement as well with another congenital deficiency called fibular hemimelia which is discussed in another section.


The first step in treatment of CFD requires placement of a special orthotic once the child becomes of walking age, at about a year old. Physical therapy for stretching is not necessary as the deformity is a structural one and cannot be stretched or strengthened.


The first surgery in all Type 1, 2 and 3 CFD cases is to prepare the hip and femur for lengthening at a later date. The surgery entails realigning the sometimes severe deformity of the hip and placing a plate on the femur. In some cases, the hip socket is deepened at that time.  The knee is straightened and stabilized with ligaments, if needed. The child heals over a six to eight week period and one to three years later we incrementally lengthen the femur. This is the most complicated of all lengthening procedures because the knee and hip need to be protected with physical therapy, bracing and often external fixation. Depending on the limb length discrepancy prediction, we determine the number of lengthening procedures needed to equalize limb length. The first lengthening is usually never more than five centimeters (two inches). With lengthening surgeries spaced approximately three years apart, the procedure can be done with an external fixator or an internal lengthening rod, depending on the size of the child and the size of the femur bone.


The caveat to that is that in some Type 2 and Type 3 cases, the child may be treated with a rotationplasty. This is an uncommon procedure where the lower extremity is rotated one hundred and eighty degrees allowing the ankle to act as a knee. Although it sounds strange, a rotationplasty often makes the patient quite functional with just one procedure as opposed to multiple lengthenings.


Overall, following the right course of treatment, patients with CFD live functional, fulfilling and happy lives.


FIBULAR HEMIMELIA

Fibular hemimelia is one of the most common conditions that we treat at the Paley Institute.  Between 18 and 36 months of age, we perform the initial surgery which entails placing the foot in the corrected position under the tibia. This operation has been termed a SUPERankle and is adjusted based on the deformity present and the type.


A first lengthening of the tibia often follows soon after the initial SUPERankle surgery. The need for knee surgery and/or femoral (thigh) lengthening all depends on the severity and presence of the knee ligament absence and/or femoral shortening. The number of necessary lengthenings is often predicted at the first visit.  


Treatment requires a keen familiarity with FH as well as its different types and associated problems of the femur and knee. The SUPERankle, which includes releases, osteotomies of the heel and often the foot, shortening of the tibia, and freeing of nerves and blood vessels, may be quite complicated and difficult. Associated problems such as a knock knee deformity may be treated with a small procedure called guided growth. Complex limb lengthening with protection of the knee is often needed. Ligament reconstruction of the knee is commonly needed as well.


Results have been outstanding leading to quite good function of the extremity.


TIBIAL HEMIMELIA

TH is specifically treated depending on the type and severity.  Fixing the foot deformity as well as aligning and lengthening the tibia is often required. Utilizing the fibula as a tibia is often the first line of treatment.  At times, the knee needs to be created from the patella (knee cap). Each patient must be individualized in order to create a sensible life plan for function.


Treatment is based on severity and involves correction of the foot deformity, utilizing the fibula as a tibia, ankle fusion, and reconstruction of the knee.


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Lucky Fin Project

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David S. Feldman, MD
901 45th Street, Kimmel Building
West Palm Beach, FL 33407
Phone: 561-762-3005
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