fibular hemimelia

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...

PURPOSE: Comparison of two hexapod frame systems in paediatric tibial deformity correction; the Taylor Spatial Frame (TSF) and Orthex Hexapod System. METHODS: Paediatric patients with congenital and acquired tibial deformities treated with either TSF (between 2014 and 2016) or Orthex (between 2017 and 2019) frames were included in a retrospective comparative study. Outcome measures were healing index, pin infection rate, regenerate quality and density, software residual rate, deformity correction accuracy, strut exchanges and quality of life (QoL)...

BACKGROUND: Hydroxyapatite (HA) coated flexible intramedullary nailing (FIN) stimulates osteogenic activity. The role of HA-coated intramedullary nails remains unclear in normal bone lengthening. The goal of this study was to quantify the influence of FIN on the External Fixation Index (EFI) in patients with congenital lower limb discrepancy. METHODS: Patients with femoral deficiency and fibular hemimelia underwent lengthening by the combined technique of external fixation with titanium (Ti) FIN or HA coated FIN and returned for follow-up at least 12 months after frame removal...

The congenitally shortened limb (CSL) with fibular deficiency or absence has historically been graded by plain radiography, while associated cartilaginous and arterial soft tissue anomalies have been comparatively neglected. Consistent pathological evidence of remnant cartilaginous bodies in place of the fibula presupposes earlier existence of a preformed cartilaginous template of the fibula. In complete fibular radiographic absences, often associated with midline metatarsal deficiencies, the two usual nutrient arteries to the fibula fail to form, as they normally would have, around the (16-18 mm stage) sixth embryonic week...

The purpose of this survey in Belgium and the Netherlands was to assess treatment variation in glenohumeral osteoarthritis between experienced and less experienced orthopedic surgeons, and to investigate perioperative treatment after shoulder arthroplasty in a large group of orthopedic surgeons. Orthopedic surgeons specialized in shoulder surgery were invited to complete a survey between November 2013 and February 2015. Seventy-one percent of the approached surgeons com-pleted the survey. Less experienced surgeons (< 6 years) and surgeons from the Netherlands find patient characteristics (e...

PURPOSE: Longitudinal fibular deficiency (LFD) is the most common congenital long bone deficiency. This study aimed to objectively assess the physical performance of children and adolescents with LFD compared with unaffected peers, and to examine trends over age for subgroups of the LFD population. METHODS: Differences between children with LFD and unaffected peers were examined with hand-held dynamometry for lower-limb muscle strength, Six-Minute Walk Test, Timed up and down stairs test, Star Excursion Balance Test, and Standing long jump...

Meromelia refers to the partial absence of at least 1 limb and is also referred to as "terminal transverse hemimelia." It can occur in either isolation or with other congenital malformations. There are very few publications in the literature that report meromelia cases accompanied by other congenital anomalies. Proximal focal femoral deficiency is another rare congenital skeletal abnormality and is characterized by the underdevelopment of the proximal part of the femur and shortening of the entire lower extremity...

CASE: A 20-year-old man with a history of right lower extremity fibular hemimelia previously treated with PRECICE femoral nail lengthening presented with a broken magnetic nail and a displaced fracture through an ununited distraction osteogenesis site. Using a combination of techniques, we removed the broken implant while maintaining the achieved limb length and preserving the native biology without bone grafting. CONCLUSION: The unique challenges associated with the removal of a broken PRECICE femoral nail are described, with a technique for implant removal that preserves the achieved length, the innate biology of the distraction osteogenesis site, and promoting union without bone grafting...

Proximal focal femoral deficiency (PFFD) is a rare congenital skeletal abnormality characterised by the partial absence of the proximal femoral segment with shortening of the entire lower extremity. It typically presents as a shortened thigh, which is flexed, externally rotated and abducted. Some other skeletal anomalies, especially fibular hemimelia, usually accompany the disorder. The diagnosis of PFFD is made in early childhood and based mainly on conventional radiography. However, the radiographic appearance of the disease evolves as the child grows...

Fibular hemimelia (FH) is a congenital deficiency in which a part or all of the fibular bone is hypoplastic or aplastic and associated with hypoplastic tibia and foot anomalies. The main differential diagnoses include proximal focal femoral dysplasia, Femur-Fibula-Ulna syndrome, and Femoral Hypoplasia-Unusual Facies syndrome. Proximal focal femoral dysplasia, which has a short, angulated femur with normal mineralization may be associated with FH. We report a case of unilateral FH with focal femoral deficiency detected at 18 weeks of gestation during a routine ultrasonographic anatomic screening...

BACKGROUND: Fibular hemimelia is the most common deficiency involving the long bones. Paley classification is based on the ankle joint morphology, identifies the basic pathology, and helps in planning the surgical management. Reconstruction surgery encompasses foot deformity correction and limb length equalization. The SUPERankle procedure is a combination of bone and soft tissue procedures that stabilizes the foot and addresses all deformities. METHODS: We retrospectively reviewed 29 consecutive patients (29 limb segments), surgically treated between December 2000 and December 2014...

BACKGROUND: Limb lengthening for congenital femoral deficiency (CFD) with or without fibular hemimelia can be performed with both external and internal devices. The purpose of this study is to compare clinical outcomes of femoral lengthening utilizing monolateral external fixation versus a magnetically motorized intramedullary nail in patients with CFD with or without fibular hemimelia. METHODS: This retrospective review included 62 patients with femoral lengthening, 32 patients had monolateral external fixation (group A), 30 patients had internal lengthening nail (group B)...

Meromelia is a rare skeletal abnormality characterized by the partial absence of at least one limb. Several mechanisms have been postulated to explain the etiopathogenesis of the disorder. Most of the cases of meromelia are reported to be sporadic. It can occur either in isolation or with other congenital malformations. VACTERL association, gastroschisis, atrial septal defect, proximal femoral focal deficiency, and fibular hemimelia are the congenital abnormalities reported to be in association with meromelia...

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...

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...

Longitudinal fibular deficiency (LFD), or fibular hemimelia, is congenital partial or complete absence of the fibula. We aimed to compare the lower limb function of children and young people with LFD to that of unaffected peers. A cross-sectional study of Australian children and young people with LFD, and of unaffected peers, was undertaken. Twenty-three (12 males) children and young people with LFD (74% of those eligible) and 213 unaffected peers, all aged 7⁻21 years were subject to the Knee Osteoarthritis Outcome Score (KOOS/KOOS-Child) and the Cumberland Ankle Instability Tool (CAIT/CAIT-Youth)...

Purpose: When using tension band plates for angular deformity correction, the literature is unclear regarding the most effective screw insertion angle to use. This study evaluates the correlation between initial screw angle and the average rate of correction during hemiepiphysiodesis using tension band plates. Methods: This retrospective study includes 35 patients (47 physes) with genu valgum deformity (17 idiopathic and 18 fibular hemimelia) who underwent insertion of Eight-Plates between 2010 and 2015...

Fibular hemimelia is a rare congenital malformation that is commonly associated with other lower limb abnormalities. This is a unique case of a bicruciate ligament, anterior cru ciate ligament/posterior cruciate ligament (ACL/PCL) deficiency in a 6-year-old female with fibular hemimelia in which we describe an ACL reconstruction using autograft-allograft hybrid technique. This case focuses on the technical aspects of an ACL reconstruction using a physeal-sparing technique with a hybrid ACL graft in a pediatric patient with fibular hemimelia...

RATIONALE: Tibial hemimelia is known as a rare congenital lower limb deficiency. It has been classified into different types based on Jones classification, and the traditional treatment of tibial hemimelia is amputation. Here we present a variant and unclassified case of tibial hemimelia, which was caused by osteomyelitis. And the lower limb with tibial hemimelia was salvaged by asymmetric limb lengthening. PATIENT CONCERNS: 19-year-old girl had the shortened and curved left lower extremity with walking abnormalities...

BACKGROUND: Fibular hemimelia is the most common congenital long-bone deficiency. It is usually unilateral and results in a limb-length discrepancy. The literature generally subscribes to the concept of constant inhibition, a process by which limb-length ratios between the shorter and longer extremity remain constant throughout growth, but scientific data supporting this concept are sparse. Additionally, recent literature suggests that these children have abnormal skeletal maturation...