Also known as congenital absence of the fibula, congenital fibular deficiency, paraxial fibular hemimelia and aplasia/hypoplasia of the fibula, fibular hemimelia . Fibular hemimelia. Disease definition. Fibular hemimelia is a congenital longitudinal limb deficiency characterized by complete or partial absence of the fibula. consists of shortening or entire absence of the fibula; previously known as fibular hemimelia; the most common congenital long bone deficiency.
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Surgical reconstruction for fibular hemimelia
The main difference between the groups was that families of hemimeia treated by amputation had lower economic and educational levels and were more ethnically diverse that those of the limb reconstruction group. Most patients with type 1 FH do not require any foot surgery; rather, treatment consists of lengthening the tibia and fibula with no foot fixation.
The leg is lengthened. While tibial fibulxr alone requires daily physical therapy, combined femur and tibial lengthening mandates strict lengthening-specific physical therapy [ 33 ]. Therefore, the decision to undergo the procedure is a personal one and not one that should be dictated by the surgeon. For specific medical advice, diagnoses, and treatment, consult your doctor.
This limb length discrepancy follows a Shapiro 1a curve, meaning its growth inhibition remains constant [ 5 ]. Use these two wires to guide the proximal osteotomy plane of cut Figs. Developmental patterns in lower-extremity length discrepancies.
In most of these cases the distal tibia is also maloriented into procurvatum and valgus. Genetic counseling Most cases are sporadic. But they do know that nothing a mom does during pregnancy causes the problem. Retrieved from ” https: The exception to this is when FH is associated with deficiency in more than one limb; for example, bilateral FH is often an autosomal dominant condition.
The third element of the foot deformity is the absence of some of the toes, including the foot metatarsal bones the long bones that lead down to the toes. If the lengthening option is not available at the treating center, patients should be offered a second opinion at a referral center that has expertise in lengthening reconstruction surgery.
The ankle joint, which is often a ball and socket type, cannot compensate for a valgus knee since it usually has valgus instability dynamic valgus. The tibial plafond is maloriented into valgus procurvatum b. Knee joint deformities The knee joint frequently has a valgus deformity. The ankle joint function with FH may range from: These three wires are parallel to the sole of the fibulag.
Nevertheless, if an amputation could be avoided and the foot and ankle and leg reconstructed to nearly normal function comparable to that afforded by a below-the-knee prosthetic, most parents and most individuals will choose to have the fkbular.
Many patients with FH are missing one or more toes. Hemimelia can also be intercalary in which case the foot remains unaffected. In light of these results all patients should be given the option of surgical reconstruction versus amputation.
In many developing nations, amputation may be culturally unacceptable and good prosthetics unobtainable.
Most feet have a ball and socket ankle joint with a fibula that is relatively short compared to the tibia at the level of the ankle joint. The hemimeli recurs through the proximal tibia. Paley Growth OS1 only ; App name 2: American Academy of Orthopaedic Surgeons; These authors compared their group with an amputation group and concluded that amputation was preferable to lengthening.
A lateral longitudinal incision is made along the posterior aspect of the tibia. Return to the distal incision and identify the flexor hallucis longus wiggle the big toe.
Fibular Hemimelia | Paley Orthopedic & Spine Institute
Fibular hemimelia Hemimelia in an amphibian affecting both calf and foot on one side Classification and external resources Specialty medical genetics ICD – 10 Q If a patient is complaining of a lot of pain, especially during the day while at rest, the cause of the pain should be sought. Cleidocranial dysostosis Sprengel’s deformity Wallis—Zieff—Goldblatt syndrome. In all cases, the desired lengthening was achieved and all patients were able to wear normal hemimellia without orthoses.
In this surgery, the orthopedic team places a lengthening device on the shorter leg. Springer, Berlin, Heidelberg, pp — The amount of overlap represents the amount of shortening required to acutely correct the foot.
A similar valgus tendency hemimeoia observed with progressive valgus deformity in children with FH after amputation [ 28 ]. This does not prove, however, that the best treatment for severe cases of FH is amputation with hemimmelia fitting. In the grade 3 FH group, hemikelia patients required six stages of reconstruction a stage referred to as a lengthening or a deformity correctionfour required five stages, six required four stages, three required three stages, four required two stages and one required one stage.
J Pediatr Orthop B. Open in a separate window. The tarsal tunnel is decompressed. The foot is fully realigned and stable. Serial lengthenings and epiphysiodesis, performed at well-spaced intervals during childhood, will equalize the leg length discrepancy.
The talar neck may be very short and have little concave offset. Parents of males treated by amputation perceived a lower school-related QoL for their child, a finding possibly explainable by socio-economic and ethnic differences between fibilar groups. The few series in which good results were obtained, even in severe cases of FH, reported that the final result was a stable plantigrade foot [ 43 fobular 46 ].
The reconstructive life plan would consist of:. Under tourniquet control make a medial longitudinal incision at the postero-medial border of fibulqr distal tibia extending past the ankle joint Fig. That combined with modern prosthetics leads to unrestricted excellent function. J Bone Joint Surg Am. The total leg length discrepancy at skeletal maturity and the separate bone segment femur, tibia, foot height discrepancy at maturity can be calculated using the multiplier method for limb length discrepancy prediction [ 18 ].
Identify and incise the lateral capsule of the fibylar ankle joint.