Case of incomplete fibular hemimelia with tarsal coalition, pes planus, ball and socket ankle (original) (raw)
Related papers
Residual malformations and leg length discrepancy after treatment of fibular hemimelia
Journal of Orthopaedic Surgery and Research, 2011
Background: Fibular hemimelia has been reported as the most common congenital longitudinal deficiency of the long bones. Previous studies have focused on the best treatment options for this congenital condition. There is very little to our knowledge in the literature focused on residual persisting malformations and leg length discrepancy after treatment.
Postaxial hypoplasia of the lower extremity (fibular hemimelia) presenting in a young adult male
Radiology Case Reports
Postaxial hypoplasia of the lower extremity, formerly termed as fibular hemimelia, is characterized by lower limb length discrepancy and a broad spectrum of anomalies involving the ipsilateral limb. It is a rare skeletal abnormality with an incidence of 5.7-20 cases per 1 million births. Herein, we present a young man with postaxial hypoplasia of the lower extremity who admitted to the hospital for a reason other than musculoskeletal complaints. While his limb length discrepancy was rather mild, the accompanying tarsal coalition was of an extensive form involving talus, calcaneus, navicular, and cuboid. Such extensive fusions of the hindfoot are very rare, and they are commonly associated with congenital syndromes such as postaxial hypoplasia of the lower extremity and Alpert syndrome. Therefore, further investigation for accompanying abnormalities is needed in cases with extensive fusions of the hindfoot.
Knee
Background: Fibular hemimelia is the most frequently occurring congenital anomaly of long bones. These patients, among other deficiencies, have a poor development of the anterior cruciate ligament (ACL). Unless it causes clinically assessed instability of the knee, nonsurgical treatment is given. When surgical treatment is required, correction of angular limb deformity must be realized prior to ACL reconstruction. Methods: We present the case of a 16-year old patient with congenital fibular hemimelia. Physical examination showed genu valgum, anteromedial rotatory instability and recurvatum of the right knee. We decided to perform surgical correction of the angular deformities and ACL reconstruction in the same surgical time. Results: Twelve months after surgery, the patient had no evidence of clinical instability, with a range of motion from −5°-110°of the right knee. No claudication or gait instability was found. The KT-1000 arthrometer showed a difference of 2 mm between both knees. Conclusion: The ACL reconstruction and corrective osteotomies of angular deformities performed in a single surgical procedure had a good clinical result in a 12 month follow up-period, restoring stability of the knee and allowing a normal gait cycle.
MANAGEMENT OF FIBULAR HEMIMELIA
2000
We reviewed retrospectively 22 patients (23 limb segments) with fibular hemimelia treated by amputation or limb lengthening to evaluate these methods of treatment. There were 12 boys and 10 girls, all with associated anomalies in the lower limbs. Twelve patients (13 limb segments) had early amputation and prosthetic fitting and ten had tibial lengthening using the Ilizarov technique.
European Journal of Medical Genetics, 2019
Considering the paucity of reports on large series of patients with tibial hemimelia, we assessed the clinical spectrum of this rare congenital disorder in patients seen at a single Indian center over 10 years. Methods: Retrospective review of medical records of patients seen at single center in 10 years. Results: Thirty-five cases of TH, mostly Jones types Ia (18) and II (10), were diagnosed in 24 patients (13 had unilateral TH). Associated foot deformities included equinovarus (22), varus foot (10), absence of the medial row of toes (5) and polydactyly (3). Upper limbs anomalies included split-hand deformity (five patients) and radial club hand (two patients). Nine limbs of seven patients were surgically reconstructed. Modified orthosis was provided to seven patients, custom designed prosthesis fitment in six and amputation with prosthesis fitment in one. Patients presenting at adolescence or later were habituated to their deformity for indoor ambulation; families declined amputation.
Congenital Lower Limb Deformities
2018
Congenital deformities of the lower limb are difficult pathologies for treatment. In this chapter fibular hemimelia, tibial hemimelia, proximal focal femoral deficiency (PFFD), congenital pseudoarthrosis of the tibia are going to be explained. Fibular hemimelia should be named postaxial hypoplasia of lower extremity because it is accompanied by other deformities of lower extremity such as proximal femoral deficiency, coxa vara, congenital short femur, instabilities of knee, tarsal coalition, and ball-and-socket ankle. The purpose is to correct rotational and angular deformity, to correct ankle instability and foot deformity, to eliminate difference of length of the extremity, and to obtain an extremity that can bear weight. Tibial hemimelia or congenital absent tibia is a rare anomaly. Although the conventional treatment is amputation and prosthetic fitting in most centers, centralization of the fibula between the femoral condyles and correction of deformities to attain a functional...
Cureus, 2022
Congenital limb deformities, with a birth frequency of 0.55 per 1,000, are extremely rare prenatal defects that can present with either partial or complete lack of a limb or a specific portion of a limb. Amelia is a sporadic anomaly that is defined by the complete absence of a limb's skeletal elements, whereas hypomelia is defined by the incomplete development of a limb's skeletal elements. We present the case of a neonate with gross facial deformities in the form of the absence of both external ears and a saddle-shaped nose. The absence of the right lower limb bud was seen. The left lower limb was underdeveloped, noted only up to the thigh region with the hypoplastic distal part of the leg and absent foot. Genitals and the anus were absent. To the best of our knowledge, this case is exceptional in that congenital limb abnormalities are present at birth along with accompanying genital underdevelopment.
Ankle Reconstruction in Fibular Hemimelia: New Approach
HSS Journal ®, 2016
Background: Fibular hemimelia is a congenital disorder that is characterized by the absence of the fibula that could be either partial or complete. Successful management aims to restore normal weight bearing and normal limb length. The introduction of the Ilizarov method of limb lengthening has provided an attractive alternative to amputation. During lengthening, the tight posterolateral soft-tissue structures, the thick fibrous fibular band, and the shortened Achilles tendon become tighter and transfer a valgus force to the talus and calcaneus, further aggravating the deformity. Questions/Purposes: We have developed a strategy to address this in patients with Paley type III fibular hemimelia via ankle reconstruction that provides posterolateral stability and buttressing of the ankle and hind foot by reconstructing the lateral buttress. This is achieved through excision of the fibrous fibular anlage, centralization of the ankle, restoring talocalcaneal coronal alignment, and reconstruction of the lateral malleolus by transplanting the cartilaginous remnant of the lateral malleolus or by crafting a bone block autograft taken from the iliac crest or tibia. Methods: A prospective non-randomized clinical trial included ten ankles in eight patients with fibular hemimelia Paley type III (two patients had bilateral deformity). The patients' ages ranged from 7 to 36 months. Results: After a follow-up ranging from 48 to 96 months, a stable plantigrade foot was achieved in nine ankles; one ankle had residual equinus, five ankles had residual valgus heel, and eight ankles had complete range of motion of the ankle, whereas one patient lost 5°of dorsiflexion. One ankle had equinus deformity. Conclusions: To achieve satisfactory results, a stable plantigrade foot and ankle is necessary in patients with fibular hemimelia before attempting to equalize limb length discrepancy. It is important to reconstruct the ankle through an extra-articular soft tissue release, anlage resection, osteotomies, and restoring the abnormal talocalcaneal relationship before any attempt to equalize LLD.