Patients who suffer from Jones type I tibial hemimelia will see the quadriceps deficient or absent. The anchor and the knee are unstable with flexion contractures or dislocation. Now, the patella is also dysplastic or absent. The ankle is in equinovarus with the foot supinated. In almost every case, the ankle and the knee remain unstable.
Many reconstructive procedures have been performed in an attempt to help the patients lead a better quality of life. However, the recommended treatment has always been amputation followed by long-term prosthetic replacement. A new technique in the market known as femoral fibula calcaneal arthrodesis is also available. It is described as a cost-effective technique allowing patients to walk on their feet with sensation. This indicates that the process can be an excellent alternative for patients who prefer limb salvage to amputation.
Classifications
A systems have been proposed for different types of tibial hemimelia. Here, we have followed the Jones classification system, which shows range from most deficient to least deficient. Here, Type 1 is divided into two groups: Type 1A, which is characterized by the total absence of visible tibia, and Type 1B, which is characterized by the presence of unified proximal tibial epiphysis. Keep in mind that an ossified proximal part of the tibia with distal tibial aplasia characterizes Type 2. Conversely, type 3 is characterized by an ossified distal part of the tibia with proximal tibial aplasia. Finally comes type 4, characterized by a short tibia with distal tibiofibular diastasis.
Surgical techniques used
The procedure aims to correct the deformity and gain adequate length by surgical treatment for tibial hemimelia. Three stages of reconstruction are considered essential.
- Loosening Stage
During this procedure, the patient is under general anesthesia, and here, with the use of a tourniquet, incisions are made over both ends of the fibula. The previous soft tissue contracts, which are present below the femoral condyles and above the talar dome, are released. In case of severe contractors at the knee, the doctor will release the origin of the gastrocnemius, and the Achilles tendon is then anatomized. In children who have thickened fibula that is markedly curved, a double oblique diaphyseal osteotomy is performed. Here, 2 Steinmann pins are passed, one of which is approximately passed to the lower end of the femur and the other distally through the talus.
- Lengthening Stage
A ring fixator is applied to achieve gradual lengthening. This fixator is applied from foot to distal femur. Once the desired length of leg has been achieved, the fibula automatically becomes centralized. This provides length and shape to the leg. The patient must be closely observed for any neurovascular complications during the lengthening.
- Stabilisation stage
Finally, after the lengthening of the leg is done, the fibular ends are stabilized to the femoral condyles proximately and to the body of the talus distally using Kirschner wires. Now, a foot-to-groin cast is applied here. The foot deformity is corrected using posteromedial release. Neurovascular variations must be kept in mind when attempting to reconstruct the foot. The feet are corrected to the plantigrade position if there is a bilateral involvement. However, in the case of unilateral involvement, 5 degrees to 10 degrees of equinovalgus helps to compensate for the residual shortening of the limb. Finally, weight-bearing is permitted in a snug-fitting cast.
Final discussion
Tibial hemimelia is quite challenging congenital anomaly and is associated with a range of other congenital defects and duplications. Now, the condition is marked by shortening and bowing of the leg. To date, reconstructive options have been limited, and the most accepted treatment has been amputation, which is then followed by suitable prosthetic replacement. It is always suggested that treatment be started as early as possible to achieve early and maximum alignment between the knee and the ankle. Numerous reconstructive methods have been described to avoid amputation. However, the final result will depend entirely on the condition of the patient and the best available alternative.
Many studies have shown that femoral fibulo calcaneus arthrodesis is an effective option for treating Type 1 tibial hemimelia and patients who wish to salvage the extremity and avoid long-term prosthetic fittings. It is a cost-effective one-time procedure resulting in a stable extremity and allows patients to walk on their feet with sensation.
Conclusion
Trusting the right professional for support is always essential regardless of the patient’s condition. You can always rely on Trishla Ortho experts for quality treatment. They have certified and experienced professionals who understand the importance of the process and will be able to provide the best care possible. No matter the severity, they will devise a treatment option that works well for the patient and guarantees he can live a better quality of life.
