Congenital Lower Limb Deformities (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.
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.
Case of incomplete fibular hemimelia with tarsal coalition, pes planus, ball and socket ankle
Journal of Surgery and Medicine, 2019
Fibular hemimelia (FH) is a congenital disease with a clinical spectrum ranging from mild fibular hypoplasia to fibular aplasia. There is no proven genetic factor. Some anomalies can accompany FH such as tarsal coalition, ulnar hemimelia, amelia, syndactyly, several extremity anomalies, renal anomalies and cardiac anomalies. Our case is about unilateral and incomplete type of right-side FH in a 14 years old female patient. Tibia was curved (bowing) and short. Disparity of measure with left lower extremity was monitored. Tarsal coalition in osseous form, tibial curve anomaly and small bone part placed in fibula distal region compatible with FH, were visualized. There was curved joint form in the same ankle with hemimelia compatible with ball and socket ankle deformity. Calcaneal inclination angle was 120°. The findings were compatible with pes planus.
Deformity Reconstruction Surgery for Tibial Hemimelia
Children, 2021
Tibial hemimelia is a rare congenital deficiency with a wide spectrum of pathology and deformity. This paper aims to give a comprehensive review of tibial hemimelia, with a concise summary of the history, pathology, and clinical findings of tibial hemimelia, while providing treatment recommendations and a review of the current literature. Classifications and surgical treatments are discussed, including amputation, limb reconstruction, and lengthening. Type-specific treatments are also discussed, including staged distraction correction of joint contractures of knee and ankle, Weber patelloplasty, fibular centralization, knee and ankle arthrodesis, implantable articulated distractors, and the role of femoral shortening. Amputation is a simpler and easier solution for many patients; however, reconstruction options continue to evolve, improve, and provide better functional outcomes in many cases. Factors favoring surgical reconstruction include the presence of a knee joint/proximal tibi...
Management Of Fibular Hemimelia: Amputation Or Limb Lengthening
The Journal of Bone and Joint Surgery, 1997
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.
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.
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.
Acta orthopaedica et traumatologica turcica, 2006
Congenital tibial deficiency is a rare anomaly causing shortening and varus deformity of the lower extremity. Recent limb lengthening and foot repositioning techniques enable functional results with preservation of the foot. We present a five-year-old boy with bilateral congenital tibial deficiency of type 2 according to the Jones classification, who was treated with tibia-fibular fusion without Syme amputation. His ambulation depended on crawling. Radiographic examination showed a normally developed fibula with thickening in the distal portion, and only proximal tibia with absence of the distal part. He also had bilateral stiff equinovarus deformity. In a series of operations, he underwent bilateral tibia-fibular fusion and fibular osteotomy, subtalar arthrodesis and metatarsal osteotomy in the right foot, and subtalar arthrodesis in the left foot. At the end of a six-year treatment and follow-up, walking was achieved despite some degree of limping.
Amputation Versus Staged Reconstruction for Severe Fibular Hemimelia
JBJS Open Access
Background: Fibular hemimelia, a congenital disorder characterized by the partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency, is the most common deficiency of long bones. The purpose of the present study of children with congenital fibular hemimelia was to examine the functional and psychosocial outcomes at a minimum of 2 years after treatment either with amputation and a prosthesis or with reconstruction and lengthening. Methods: Twenty children who were managed with primary amputation were compared with 22 children who were managed with staged limb reconstruction. The average age of the patients at the time of evaluation was 9 years (range, 5 to 15 years). Patients and parents completed psychosocial, quality-of-life, and satisfaction surveys. Patients underwent instrumented gait analysis and a timed 25 or 50-yard dash. The number and nature of surgical procedures were recorded from a retrospective chart review. Results: Families of children managed with amputation had lower economic and educational levels and were more ethnically diverse compared with the families of children managed with limb reconstruction. Scores on psychosocial and quality-of-life surveys were comparable with those from healthy patient populations. Parents of males treated with amputation perceived a lower school-related quality of life for their child; socioeconomic and ethnic differences between groups might account for this finding. Statistically but not clinically significant differences were measured during instrumented gait analysis at a self-selected walking speed and during a timed 25 or 50-yard dash. The majority of patients and parents reported satisfaction with the treatment method selected and would select the same treatment method again. Conclusions: At this interim stage of growth, there were no significant functional or psychological differences between groups. Both groups were satisfied with the outcome in mid-childhood, irrespective of the selection of amputation or limb reconstruction. Level of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence. F ibular hemimelia is characterized by partial or complete absence of the fibula, tibial growth inhibition, and foot and ankle deformity and deficiency. The primary problems associated with fibular hemimelia include leg-length discrepancy and equinovalgus deformity of the foot. The goal of treatment is restoration of the limb through (1) the production of a plantigrade, painless foot and equalization of limb length or (2) foot ablation and prosthetic fitting. Studies have documented the success of amputation as treatment for severe deformity 1-8. Advances in limb-lengthening surgery, including more versatile external fixators 9-12 and sophisticated foot and ankle reconstructions 13-15 , have enabled staged Disclosure: The authors indicated that no external funding was received for any aspect of this work. On the Disclosure of Potential Conflicts of Interest forms, which are provided with the online version of the article, one or more of the authors checked "yes" to indicate that the author had a relevant financial relationship in the biomedical arena outside the submitted work and "yes" to indicate that the author had other relationships or activities that could be perceived to influence, or have the potential to influence, what was written in this work (http://links.lww.com/JBJSOA/A91).
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.