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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While tibial lengthening alone requires daily physical therapy, combined femur and tibial lengthening mandates strict lengthening-specific physical therapy [ 33 ]. In the grade 3 FH group, two 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.
The periosteum of the tibia is cut in a T-shaped fashion, exposing the medial aspect of the distal tibia.
Developmental patterns in lower-extremity hwmimelia discrepancies. For type 3A the tibial osteotomy with shortening alone can be performed. It should slide medial-distally and hemimwlia wedge open on the lateral side. Multiplier OS1 and Android ].
The limitation of ankle motion is related to ankle dysplasia rather than surgery or lengthening. When it is missing, this stabilizing effect is absent. Socioeconomic factors may limit such second opinion options. Identify the junction between the talus and calcaneus posteriorly. Perform a gastro-soleus recession. If hejimelia tibia is not being lengthened a hemi-epiphysiodesis device can be applied to the proximal tibial physis.
Surgical reconstruction for fibular hemimelia
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. As long as the foot is plantigrade, the foot in FH is very functional even with one, two, three or four rays.
Some surgeons consider absence of two or more metatarsals an indication for amputation [ 13 ]. It is important to correct this deformity either before or at the time of tibial lengthening.
Paley D, Robbins C. The controversial cases are those that include more severe foot deformities Paley types 3 and 4 and greater leg length discrepancies due to more severe tibial growth inhibition or combined femoral and tibial discrepancy.
Fibular Deficiency (anteromedial bowing) – Pediatrics – Orthobullets
Foot and ankle deformities Foot and ankle deformities have been fibluar most challenging and disabling problems with FH. There is often limited dorsiflexion in this group but not fixed equinus.
Despite this, the results of some clubfoot treatments leave the child with chronic pain and a stiff deformed foot that might be better treated by amputation and prosthetic fitting. Management of congenital fobular deficiency by Ilizarov technique. A bracket first metatarsal or a bracket conjoined first and second metatarsal with hallux varus is not uncommon.
The reconstructive life plan would consist of:. The subtalar joint in fibular hemimelia is usually absent because the two bones are fused. Bardet—Biedl syndrome Laurence—Moon syndrome. Fix the tibia with a small medial tibial locking T plate Fig. Other studies have demonstrated that isolated mutations of genes in the forming limb bud can lead fibylar fibular hemimelia.
Paley D Length considerations. Incise the fascia covering the neurovascular bundle and decompress the posterior tibial nerve by cutting this fascia including the lacinate ligament [ 25 ] Fig.
Surgical reconstruction for fibular hemimelia
The extent of ankle joint deficiency may range from a relatively normal ankle to a very unstable, abnormally-shaped ankle with limited mobility. Fibular hemijelia Paley type 3.
The distal tibial articular surface hemimelai often concave in the frontal plane as part of a ball and socket ankle joint. Syndactaly between the middle toes does not need to be hmeimelia. The proximal osteotomy is parallel to the proximal sagittal plane guide wire 4. Each Paley classification type has a different surgical treatment; it is independent of the number of rays or the leg length discrepancy.
Examine its shape to determine if it is round and whether its curvature will limit ankle motion. The ankle joint orientation is in valgus and procurvatum.
Acknowledgements The author would like to thank Hemimeia Boullier Ross who illustrated all of the figures in this manuscript.
There is no fixed equino-valgus. Antenatal diagnosis Prenatal diagnosis of fibular hemimelia has been reported. Both groups of patients were satisfied with their results, were equally and functionally active and had no pain. Part of this deformity and instability is related to the fibular deficiency and part to the subtalar pathology.