Postaxial hypoplasia of the lower extremity (fibular hemimelia) presenting in a young adult male (original) (raw)
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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.
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...
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.
Rare case of tibial hemimelia, preaxial polydactyly, and club foot
World journal of clinical cases, 2016
A seven-month old female presented with left tibial hemimelia (or congenital tibial aplasia; Weber type VIIb, Jones et al type 1a), seven-toed preaxial polydactyly, and severe club foot (congenital talipes equinovarus). Definitive amputation surgery disarticulated the lower limb at the knee. This case report describes the anatomical findings of a systematic post-amputation examination of the lower limb's superficial dissection, X-rays, and computed tomography (CT) scans. From the X-rays and CT scans, we found curved and overlapping preaxial supernumerary toes, hypoplastic first metatarsal, lack of middle and distal phalanges in one supernumerary toe, three tarsal bones, hypoplastic middle phalanx and no distal phalanx for fourth toe, and no middle or distal phalanges for fifth toe. The fibula articulated with the anteromedial calcaneus and the tibia was completely absent. We identified numerous muscles and nerves in the superficial dissection that are described in the results se...
Annals of the New York Academy of Sciences, 2017
Postaxial limb hypoplasia (PALH) is a group of nonhereditary diseases with congenital lower limb deficiency affecting the fibular ray, including fibular hemimelia, proximal femoral focal deficiency, and tarsal coalition. The etiology and the developmental biology of the anomaly are still not fully understood. Here, we review the previous classification systems, present the clinical features, and discuss the developmental biology of PALH.
A seven-month old female presented with left tibial hemimelia (or congenital tibial aplasia; Weber type VIIb, Jones et al type 1a), seven-toed preaxial polydactyly, and severe club foot (congenital talipes equinovarus). Definitive amputation surgery disarticulated the lower limb at the knee. This case report describes the anatomical findings of a systematic post-amputation examination of the lower limb’s superficial dissection, X-rays, and computed tomography (CT) scans. From the X-rays and CT scans, we found curved and overlapping preaxial supernumerary toes, hypoplastic first metatarsal, lack of middle and distal phalanges in one supernumerary toe, three tarsal bones, hypoplastic middle phalanx and no distal phalanx for fourth toe, and no middle or distal phalanges for fifth toe. The fibula articulated with the anteromedial calcaneus and the tibia was completely absent. We identified numerous muscles and nerves in the superficial dissection that are described in the results section of the case report. Due to the rarity of this combination of anatomical findings, descriptions of such cases are very infrequent in the literature. Key words: Club foot; Congenital talipes equinovarus; Preaxial polydactyly; Tibial hemimelia; Congenital tibial aplasia
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.
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).
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.
A Systematic Review of Ankle Reconstruction with Limb Lengthening in Fibular Hemimelia
The Egyptian Journal of Hospital Medicine, 2019
Background: fibular hemimelia (FH) is a congenital deficiency where part or all of the fibular bone is hypoplastic, dysplastic or aplastic associated with hypoplasia and dysplasia of the tibia and hypoplasia, dysplasia and aplasia of parts of the foot. Objective: this systematic review was aimed to review the different method of ankle reconstruction with limb lengthening in patients with Fibular Hemimelia to restore normal weight-bearing and normal limb length so that the patient can walk with a normal gait as possible. Material and Methods: online search was done using the Medline database on PUBMED, Google Scholar and SCINCEDIRECT from 2001 to 2018; all the English language published studies were identified with the search keywords of, ankle reconstruction with limb lengthening in fibular hemimelia, fibular hemimelia and treatment of fibular hemimelia. Literature search database on PUBMED, Google Scholar and SCINCEDIRECT showed 193 studies. Results: Our search revealed 6 studies a...