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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Children between the ages of 4.
Therefore an osteotomy or chondrotomy of the coalition must be performed prior to displacing the subtalar coalition osteotomy. For this surgery to work, kids must still be growing. If one examines the few series in the literature that report good functional results after limb lengthening, the predominant difference is that in the final result not only was the leg length discrepancy addressed successfully but the foot deformities were also addressed successfully.
They may order these tests to learn more:.
Once the calcaneus is medialized, varusized and distalized, it should be pinned with two retrograde wires through the foot. Both osteotomies are completed creating a trapezoidal segment of bone. The less dysplastic the talus is to begin with, the greater the potential range of ankle motion; the more dysplastic the talar anatomy, the greater the limitation of ankle hemielia. Paley type 2 FH.
The choice is therefore that of the parents as to which procedure they prefer for their child. If femoral lengthening is considered, it is factored into the surgical life plan discussed previously.
The reconstructive life plan would consist of: If there is one conjoint tendon present, and it appears to be a brevis tendon attached to lateral calcaneus or to lateral side of foot but not extending to first metatarsal fibulae, then lengthen this tendon in a Z-fashion.
At the ankle there is a dysplasia of the distal tibia and of the talus, which ranges from mild valgus of the distal tibia to severe dysplasia with flat malformed, maloriented joint surfaces. If the subtalar coalition connects the talus and calcaneus in a normal position such that the heel is in line with the ankle bone, then it does not contribute to additional deformity of the foot.
Children with deficient or absent cruciate ligaments often do very well and can engage actively in various sports that other children their age can participate in. Classification of FH Fig. Now displace the subtalar osteotomy by levering the calcaneus to move distal and medial using a small Hohmann elevator. Fibular hemimelia Hemimelia in an amphibian affecting both calf and foot on one side Classification and external resources Specialty medical genetics ICD – 10 Q Orthop Today 31 4: Both groups of patients were satisfied with their results, were equally and functionally active and had no pain.
In such cases, it is not unusual to perform the SUPERankle procedure with application of the external fixator for lengthening tibia and femur. All patients should be given the option of lengthening reconstruction surgery versus amputation. American Academy of Orthopaedic Surgeons; This effectively lengthens the tendon without weakening it Fig. If there are two tendons, mobilize them and only lengthen the brevis and never the longus. Acknowledgements The author would like to thank Pamela Boullier Ross who illustrated all of the figures in this manuscript.
Surgical reconstruction for fibular hemimelia
Amstutz HC Natural history and treatment of congenital absence of the fibula. Unlike femoral lengthening for congenital femoral deficiency, knee reconstruction or stabilization of the knee are not required in order to proceed with tibial lengthening.
Simultaneous femur and tibia lengthening with external fixation is used when the femur and tibia shortening is of significant magnitude. A lateral longitudinal incision is hemijelia along the posterior aspect of the tibia.
Toe and metatarsal surgery Many patients with FH are missing one or more toes. The ligament deficiency and subluxation are often not symptomatic at a young age, but these become a bigger problem when the child becomes taller and hemimeli.
Using standardized evaluation tools, both groups showed comparable documented hemimelai adjustment, QoL and physical function. Knee Deformity In patients with fibular hemimelia, the knee joint frequently has a valgus deformity knocked knee.
Insert one frontal plane 12 and one sagittal plane 34 guide wire at each level of planned osteotomy.
Surgical reconstruction for fibular hemimelia
In normal anatomy, the fibula contributes to the stability of the ankle. Bohne WH, Root L. Antenatal diagnosis Prenatal diagnosis of fibular hemimelia has been reported. Separate the muscles from fibulwr side of it and then cut the septum all the way down to where it passes over the deep peroneal nerve Fig.
The etiology of FH remains unknown, and in most cases it is usually not an inheritable condition, with the vast majority of children born with this condition having no family history of other birth defects.