Fibular Hemimelia

A little info about fibular hemimelia and Caleb's treatment:

Fibular Hemimelia occurs 1 out of every 40,000 live births which amounts to 100 babies born with FH in the United States per year.

There are many degrees of the condition. Some children have either partial absence of the fibula while others have complete absence.

Classic presentations of FH usually show a bowed tibia and dimpling on the skin on the tibia.

The affected limb is shorter than the other. The limb length discrepancy varies from minimal to significant.

The foot is abnormal in children with FH. Many children are born missing toes and the foot is not positioned properly under the leg so it usually 'bends' outward giving it a crooked appearance.

Many children with FH may also have problems with their hip, femur, and/or knee.

*In Caleb's case, his fibula is completely missing but he has all five of his toes. Caleb's limb length discrepancy at this moment is 7.2 cm. His projected LLD at fully grown is 10.2 cm. His knee has a minor deformity stemming at the base of the femur which has resulted in knock-knee. Knock-knee is a condition where the knee turns inward..

Treatment:

The main concern with FH is foot stability. To address this concern, Caleb will have a very complex superankle procedure in which soft tissues connecting to the ankle will be released, in addition to tibial, ankle, and heel bone reconstruction to align the foot correctly under the leg. The foot will be kept in position with wires attached to an external fixator. This surgery will occur simultaneously with the lengthening surgery. The Taylor spatial frame will be used for the foot correction and the lengthening. This external fixator will have to be adjusted (or turned) everyday.

Caleb will be in the hospital 3-4 days. To help minimize pain, Caleb will have an epidural placed in his back for the first 2 days. The anticipate that the hardest day for Caleb will be when the epidural is removed. During this time, Caleb will have to learn to manage pain with oral pain medication and I.V. meds if applicable.  The length of our hospital stay is dependent on pain management.

The second part of the surgery will be bone lengthening. In order to lengthen the tibia, the tibia bone has to be broken so that there is a small space in between the bone for new bone to form. Everyday the pins are adjusted, lengthening the bone, with our lengthening goal being between 5-7 cm. Lengthening begins after we leave the hospital and I will be taught to properly turn the pins and clean the pin sites. Caleb will be turning pins at a rate of .75mm per day. This phase of the lengthening takes between 2-3 months. During this time our primary focus will be physical therapy, pain management, and infection. Caleb will have to go to PT (physical therapy) everyday at St. Mary's in West Palm Beach. While bone can grow at a rate of 1 mm per day, muscles and ligaments take longer to stretch. PT sessions are extremely important to help compensate for this.

The last part of the surgery is to address Caleb's knock-knee. A plate and screws will be attached to the inner part of Caleb's knee to help it from turning inwards. This plate will stay in Caleb's leg for a year and will have to be removed later on.

After the stretching phase is complete, the next phase is consolidation. during this phase the fixator still remains but the turning of the pins stops to allow for bone consolidation. This phase is equal to the time it takes during the pin turning.

The last phase is casting and it lasts about a month. This allows for the bone to harden. I'll be looking forward to this day, as the fixator will come off!!