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Orthotic Intervention Options For Limb Salvage Treatment Of Fibular Hemimelia In A Single Case


Justina Shipley CO, MEd, FAAOP

Background

The patient presents with left fibular hemimelia with the absence of fifth metatarsal and phalanx, the navicular and talus are present, with some tibial bowing. Patient is currently 18 yo has opted for limb salvage, has severe valgus at the ankle, severe hallux valgus, is in fixed plantar flexion of about 20 and the tibia has fallen off of the talus causing rotation at the ankle in the medial plane. The patient currently bears weight on the medial malleolus, has constant issues with skin break down and has a 3.4 cm leg length discrepancy. Patient breaks most devices within a short time frame because he is very active and plays sports. The problem is trying to find a device that would be durable, minimize skin problems and breakdown but maximize function of foot and ankle in their current state. [Image ]

Introduction

Fibular hemimelia is a clinical spectrum disorder that includes a congenital deficiency of the fibula. As a spectrum disorder it primarily manifests as partial and complete absence of the fibula but may also include other anomalies. Most of these anomalies include bone and joint issues associated with the lower extremities, but can occasionally include the hands. The severe shortening of the involved lower extremity and the equinovalgus deformity of the ankle are usually the cause of the major functional deficits associated with fibular hemimelia (Catagni et al., 2010).

According to the Dalmonte classification system, there are 3 types of severity for the spectrum. Type I classification is relatively mild and includes only slight deformation that consists of shortening of the tibia and fibula, but no other angular deformities that would be considered significant. Severe shortening of the tibia and fibula is classified as a type II and includes the absence of the lateral malleolus. This leads to ankle instability including equinovalgus, valgus deformity at the knee and the possibility of missing one or two rays on the foot. The most severe presentations classified as a type III involve the complete absence of the fibula. The associated tibia can also include shortening and deformation with the possibility of femoral involvement as well. The equinovalgus foot will also be present in type III fibular hemimelia (Stanitski & Stanitski, 2003).

Treatment options include leg lengthening and amputation. Management of severe cases of fibular hemimelia remains controversial; most treatment plans are not only based on the severity of the deformity but also on the preference of the patient and their family. Each treatment plan is individualized and may include more than one procedure (Catagni, et al. 2010). Symes or Boyd amputations are routinely recommended and have become an accepted form of treatment for the disorder (Birch et al., 1999). Many families refuse amputation and medical staff must respect their decision. The complications associated with both amputation and repeated lengthening must be considered and explained to the family as they are making a decision concerning a treatment plan (Catagni, et al. 2010).

Patient & Methods

In the presentation of this challenging case overview, the patient's family refused amputation as an option and moved forward with limb salvage procedures. This scenario includes an orthotic intervention solution for the single case study: At a young age, the patient was prescribed a thermoplastic AFO, which would slide off so it was replaced with a double upright AFO and a shoe lift. Throughout the pediatric treatment of the deformity the patient has had serial casting several times to try and control the Achilles tendon tightness, as well as botox injections. The patient had a tibial corticotomy with Ilizarov fixator that bridged the foot. The lengthening procedure resulted in 7.5 cm of distraction and tibial bowing was corrected. Follow-up complications of pin site infection and a knee flexion contracture occurred. The patient was also prescribed a clam shell AFO and a dyna-splint for knee flexion contracture after the pin removal from the foot. After frame removal, the patient was placed in a rocker bottom shoe and a valgus prevention AFO. Heel cord tightness was still an issue and valgus deformity of the foot continued to worsen. Surgery was performed and a screw was placed in the medial malleolus to help with the valgus deformity, this procedure was performed with a left opening wedge osteotomy with iliac crest bone graft, peroneus brevis, peroneus longus and Achilles Z-lengthening followed by casting. A thermoplastic AFO with valgus prevention was continued along with a shoe lift as a follow up to the procedure. Continued issues with valgus deformity at the ankle and hallux valgus, with plantar flexion and leg length discrepancy continued to make orthotic intervention more difficult. An AFO of graphite laminate with accommodation for plantar flexion and valgus with extra padding is currently being worn. The shoe lift was used at the beginning of this prescription, but has been discontinued by the patient. [Image ]

Results

The patient is now over 18 and has opted to continue with the limb salvage option at this time. Continued complications will manifest due to a severe equinovalgus deformity and increased plantar flexion contracture at the ankle. The patient is still wearing the carbon graphite custom ankle foot orthosis with accommodation for some plantar flexion and for severe ankle valgus. [Image ]

Bibliography

  1. Birch, J.G., Stewart, J., Walsh, M.B., Small, J.M., Morton, A., Koch, K.D., Smith, C., Cummings, D., Buchanan, R. (1999). Syme amputation for the treatment of fibular deficiency. Journal of Bone and Joint Surgery, 81(11) 1511-1518.
  2. Catagni, M.A., Radwan, M., Lovisetti, L., Guerreschi, F., Elmoghazy, N.A. (2011). Limb lengthening and deformity correction by the ilizarov technique in type III fibular hemimelia. Clinical Orthopaedics and Related Research, 469 (4), 1175-1180.
  3. Stanitski, D.F., Stanitski, C.L. (2003). Fibluar hemimelia: a new classification system. Journal of Pediatric Orthopedics 23, 30-34.

FDA Disclosure

The FDA has cleared all pharmaceuticals and/or medical devices for the use described in this presentation.

Financial Disclosure

Justina S. Shipley, CO, MEd, FAAOP: 3C (Comfort Products; Fillauer Companies); 9 (Louisiana Association of Orthotists and Prosthetists; Orthotics and Prosthetics Activities Foundation)

All aspects of this device were financed by the Shriners Hospital for Children in Shreveport Louisiana

Justina Shipley is affiliated with Shriners Hospitals for Children, Shreveport, Louisiana USA