Fibular Hemimelia Research Articles

The need for state-of-the-art orthopedic surgical technology in low- to middle income countries : The case of distraction epiphyseolysis for limb lengthening in children with fibular hemimelia in Uzbekistan.

Amongst low- to middle income countries (LMIC), outdated and poorly documented orthopedic interventions may pose patients at risk for complications, serious adverse events (SAE) and unsatisfactory results, contradicting the World Health Organization's (WHO) Safe Surgery mandates. Use cases of conditions managed inadequately in LMIC environments are necessary to increase awareness of stakeholders and overcome deficiencies in collaboration with high-income countries (HIC). Methodological and clinical experts from the BG Kliniken and the Charité, Berlin, Germany, were invited to investigate established processes for musculoskeletal disorders and injuries in Tashkent, Uzbekistan. Congenital lower leg discrepancy due to fibular hemimelia was recognized as an illustrative example to prove the effectiveness of local treatment guidelines, still relying on distraction epiphyseolysis (chondrodiatasis) via an Ilizarov frame. Routine data from hospital records, images and outpatient follow-up examinations were compared to estimates from a review of studies of limb-lengthening by other techniques. Data from 16 of 49 immature patients (10 males, 6 females, mean age at surgery, 4.2 ± 2.3 years) undergoing chondrodiatasis between 2017 and 2021 showed a mean limb elongation of 2.4 (95% confidence interval [CI], 1.9 to 2.9) cm after a median follow-up of 29 (range, 24 to 36) months. While no complications or SAE were noted, findings are compatible with a risk of 21% under an upper 97.5% confidence limit for null events. No reproducible classification of hemimelia, surgical or general complications, assessment of functional outcomes or health-related quality of life were available. The pooled average length gain from 21 studies on different other interventions with osteotomy enrolling 458 limbs was 5.1 (95% CI, 5.0 to 5.3) cm. Limb lengthening for hereditary fibular hemimelia in Uzbekistan revealed rather obsolete surgical algorithms, inadequate documentation, and unreliable outcome assessment. Resource limitations notwithstanding, knowledge transfer, implementation of current procedures and hardware, and international collaboration is vital to improve quality of care in this scenario and for the benefit of LMIC in general.

Fibular hemimelia: reconstruction of difficult cases with tibial lengthening and ankle arthrodesis.

Management of fibular hemimelia includes either prosthetic care with or without a suitable amputation or tibial lengthening. Many studies have documented the success of both procedures. Most parents of these children refuse an amputation or have no access to good prosthetic care. The author presents a limb-salvage procedure with tibial lengthening and ankle stabilization. Twelve children of fibular hemimelia with 14 extremities had been subjected to limb lengthening after lateral leg release. To correct the valgus procurvatum, double oblique diaphyseal osteotomy (DODO) of the tibia was performed in 11 extremities. The age of the patients ranged from two to 15years with the median of fiveyears. All were male. The proposed procedure included three stages of loosening, lengthening, and stabilization with ankle arthrodesis at a later stage. All patients returned for follow-up for the first fouryears and had been walking on their sensate feet. With DODO followed by fixator/traction could straighten and lengthen the tibia simultaneously and correct the valgus procurvatum. Ankle stabilization provided stability and a plantigrade foot. A follow-up of six to30years with a median of tenyears has been reported. A new procedure of loosening, lengthening, and stabilization of the leg with ankle arthrodesis has been proposed. A follow-up of 30years with a median of tenyears of the said procedure has been reported. The procedure provides a long-lasting plantigrade and painless foot that has sensation and proprioception. An amputation at any level has not been recommended.

GUIDED GROWTH SURGERY FOR TREATMENT OF PATELLAR INSTABILITY IN GENU VALGUM

IntroductionGenu valgum is a common presentation in paediatric patients with congenital limb deformities. The aim of this study is to assess the outcome of guided growth surgery in paediatric patients referred via our physiotherapy pathway with isolated genu valgum and associated patellar instability.Materials & MethodsPatients were identified from our prospective patellar instability database. Inclusion criteria was acquired or congenital genu valgum associated with patellar instability in skeletally immature patients. The mechanical lateral-distal femoral angle was assessed on long leg alignment radiographs (mLDFA <85 degrees). Surgical treatment was the placement of a guided growth plate (PediPlate, OrthoPediatrics, USA) on the medial distal femoral physis (hemi-epiphysiodesis). KOOS-child scores were collected pre-operatively and post-operatively (minimum at 6 months).ResultsEleven patients (seven female) with mean age of 12(range 5–15) were identified. Five patients had congenital talipes equinovarus(CTEV), one fibular hemimelia, one di-George syndrome, one septic growth arrest and three had idiopathic genu valgum. Pre- and post-operative KOOS-child scores showed overall improvement: 58(range 36–68) to 88(65–99) and knee symptoms subscores: 64(43–71) to 96(68–100) p<0.01, t-test.Mean follow-up was 10 months (range 3–23). No subsequent dislocations/subluxations occurred during follow-up.ConclusionsGuided growth surgery is an effective way of treating symptomatic patellar instability in skeletally immature patients with genu valgum in the absence of other structural pathology. It was most common in our cohort in patients with unilateral CTEV. We would recommend to screen syndromic and congenital limb deformity patients for patellar instability symptoms in the presence of genu valgum.

Limb lengthening and deformity correction in patients with severe fibular hemimelia: experience of the children's university hospital in Belgrade

Background Fibular hemimelia (FH) is a congenital disease manifested by valgus deformity and instability of the knee joint, shortening and deformity of the tibia, hypoplasia and deformities of the foot and ankle.The aim of this paper was to determine the efficacy of the strategy of separate reconstruction of the foot along with correction of tibia deformity, and then tibial lengthening in patients with FH of types 3 and 4 according to Paley.Methods This retrospective study is based on an analysis of the treatment of 12 children with severe FH. The patients, aged no older than 24 months, were treated for foot reconstruction along with correction of tibial deformity followed by a separate stage of tibial lengthening. Tibial lengthening was performed in the age 4.6 ± 1.2 years. The long-term result of treatment was evaluated at least 1 year after the end of tibial lengthening. Evaluation criteria after tibia lengthening were external osteosynthesis index, amount of lengthening, assessment of outcomes according to Lascombes. Walking ability was assessed using Gillette questionnaire.Results The approach we used gave excellent and good results in 83 % of cases after the first reconstructive stage. Complications and recurrences of deformities encountered during the first stage were eliminated during subsequent planned limb lengthening. The average magnitude of lengthening was 6.4 ± 2.4 cm (37.2 ± 12.4 % of the initial segment length). The index of external osteosynthesis was 22.9 ± 12.2 days/cm. Monofocal distraction osteosynthesis was used in 9 cases and bifocal osteosynthesis in three cases. The results of lengthening were classified by Lascombes as IA in 7 cases, IB in four cases, 2B in one case.Discussion In severe FH, the question of reconstruction or early amputation remains open. There are two opinions on the staging of reconstructive orthopedic surgery and tibial lengthening in young children with severe FH.Conclusion The strategy of reconstruction of the foot and ankle joint at an early age (16-24 months) in children with severe FH followed by lengthening of the lower leg (at the age of 4-6 years) proved to be effective and can be used when it is chosen by the patient's parents. In 3C type cases, the use of external fixation to correct the deformity and simultaneously lengthen the tibia at the first stage is an alternative reasonable strategy option.

Characteristics of terminal hemimelia: What is the difference between terminal hemimelia and classic fibular hemimelia?

Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia. In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared. Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years. Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening. level IV.

Bending osteotomy through the distal tibial physis for stable reduction of the ankle joint in fibular hemimelia. Update at skeletal maturity.

Fibular hemimelia is a complex longitudinal malformation of the lower extremity with partial or complete deficiency of the fibula resulting in dorso-fibular dislocation of the hindfoot. Typically associated are talocalcaneal coalition, absence of rays of the foot, diaphyseal tibial deformity of valgus-procurvatum type and longitudinal growth deficiency. We have addressed the deformity of the distal tibial epiphysis surgically by a metaphyseal osteotomy to bend through the physis inspired by the Pemberton's acetabular osteotomy in 7 to 21-month-old children. Short-term results of a maximum of 42 months of follow-up have been published. Meanwhile, the first 4 patients thus treated have reached skeletal maturity, and the long-term results are presented. Three patients with unilateral and one patient with bilateral fibular hemimelia were operated on as described before at ages 7, 9, 15, and 18 months. Subsequently, several other procedures have been performed on all patients mainly consisting of lengthening of the tibia combined with axial corrections as well as additional foot alignment if needed. The osteotomy leads to stable axial retainment of the hindfoot in all patients without premature closure of the physis. Most of them need adaptation of footwear. None of the patients at present would favor to have been treated by amputation. The technique has shown good clinical results with preserved growth of the physis of the distal tibia and full axial weight bearing at long-term. In selected cases, this technique should be considered as a valuable alternative to other reconstructions.

MENISCAL ANOMALIES IN CONGENITAL CRUCIATE LIGAMENT DEFICIENCY: EXPERIENCE IN A TERTIARY CHILDREN'S KNEE UNIT

ObjectivesCongenital cruciate ligament deficiency is a rare condition that may occur in isolation or in association with longitudinal limb deficiencies such as fibular hemimelia or proximal femoral focal deficiency. Often anomalies of the menisci and their attachments can be very abnormal and impact on surgical management by standard techniques. Arthroscopic surgical knee reconstruction is undertaken to improve symptomatic instability and/or to stabilise and protect the knee for future planned limb lengthening surgery.The aim of this study is to evaluate the arthroscopic findings of patients undergoing surgery for congenital cruciate ligament deficiency, and specifically to determine the frequency and types of meniscal anatomical variations seen in these cases.MethodsPatients undergoing surgery for congenital cruciate ligament deficiency were identified from a prospectively collated database. Diagnosis was confirmed through review of the clinical notes and imaging. Operative notes and 4K saved arthroscopic images and video recordings for these cases were reviewed.ResultsOver a six-year period (July 2017 – September 2023), 42 patients underwent surgery for congenital ligament deficiency and tibiofemoral instability (45 surgical episodes). Median age of patients at time of surgery was 10 years (range 4 – 17 years). The most frequent diagnosis was congenital longitudinal limb deficiency syndromes in 27 cases, with the most frequent being fibular hemimelia. Isolated congenital ligament deficiency without any other associated extra-articular manifestations occurred in 11 cases. Absence of meniscal root attachments or hypertrophy of meniscofemoral ligaments acting as ‘pseudo-cruciates’ were seen in over 25% of patients. In isolated ACL deficiency these were injured causing onset of instability symptoms and pain following trauma. Often these abnormal structures required addressing to allow surgical reconstruction.ConclusionsOur findings demonstrate that there are often meniscal variations seen in association with congenital absence or hypoplasia of the cruciate ligaments. In these patients hypertrophied meniscofemoral ligaments may act as cruciate-like structures and play a role in providing a degree of sagittal plane stability to the knee. However, when the knee becomes unstable to the point that cruciate ligament reconstruction is indicated, these meniscal variants may often require stabilisation using complex meniscal root repair techniques or variations to standard cruciate ligament reconstruction techniques to accommodate the variant anatomy.

Poster 321: MENISCAL RAMP LESIONS IN ADOLESCENT PATIENTS UNDERGOING PRIMARY ACL RECONSTRUCTION

Objectives: Meniscal ramp lesions describe a specific injury to the meniscocapsular junction at the posterior horn of the medial meniscus. The lesions are strongly associated with anterior cruciate ligament (ACL) injuries and may affect the biomechanical stability of the knee if left untreated. Despite the increasing rates of ACL reconstructions performed in adolescents each year, the prevalence and risk factors for ramp lesions in this patient population is poorly understood. The purpose of this study was to determine the prevalence and risk factors for ramp lesions in children and adolescent patients undergoing primary ACL reconstruction. Methods: All patients ≤19 who underwent primary ACL reconstruction at a single institutional between 2019 and 2021 were included. All surgeries were performed by three fellowship trained orthopedic surgeons. Patients were considered to have a ramp lesion if one was identified arthroscopically at the time of ACL reconstruction. Basic demographic data, preoperative imaging, and other arthroscopic findings at the time of ACL reconstruction were reviewed. Results: 247 consecutive patients who underwent ACL reconstruction were reviewed. Patients were excluded if they had had prior ACL injury, prior knee surgery, fibular hemimelia, or incomplete documentation. A total of 230 patients met inclusion criteria. Average age at time of injury was 15.7 years (range 4.0-18.2 years). A ramp lesion was identified in 12% of patients. Posteromedial tibial bone bruise on preoperative MRI and medial femoral chondromalacia at time of ACL reconstruction were associated with the presence of a ramp lesion, Figure 1A (OR = 2.6, 95% CI [1.15, 5.93]). A striation pattern of medial femoral chondromalacia was highly specific for the presence of a ramp lesion, Figure 1B (OR = 424.3, 95% CI [50.99, 3531.3]). Sensitivity and specificity for detecting a ramp lesion was 68% and 76% with preoperative MRI and 68% and 99.5% with medial condyle striations, respectively. Conclusions: The prevalence of a ramp lesion in our cohort was 12%, mirroring historical reports in adult patients. Posteromedial bone bruising and medial femoral chondromalacia, especially in a striation pattern, should increase suspicion for the presence of a ramp lesion in patients undergoing ACL reconstruction.

Left femoral hypoplasia associated with congenital fibular agenesis – A rare case report

Background and objectives. Unilateral or bilateral femoral hypoplasia represents a rare clinical entity and it can be isolated or associated with otherlimb malformations such as: congenital absence of the fibula (the most common association), the absence of lateral foot rays or clubfoot, or other organ defects such asspinal dysraphism, or congenital cardiac malformations. Only a few cases have been detected by antenatal ultrasonography so far. Materials and methods. We present the case of a newborn admitted at birth in the Neonatology Department of County Emergency Clinical Hospital of Constanta, diagnosed prenatally with left femur hypoplasia, confirmed postnatally. Results. The subject is a full-term female neonate, born at 38 weeks gestational age, with a birth weight of 2600 g, Apgar Score of 9. The mother is 39-year-old, Gravida-I, Para-I, with no medical history of consanguinity, genetic or congenital abnormalities, exposure to toxins, teratogen drugs, or viral infections. The 14 weeks and 16 weeks ultrasound revealed a shortened left femur, below the 5th percentile, with no other facial morphology, cardiac, neurologic, gastrointestinal, or genitourinary system abnormalities mentioned. A cesarean section was performed at 38 gestational weeks (premature rupture of membranes, breech presentation). The diagnosis was confirmed postpartum by X-ray. Conclusions. The presented case emphasizes the importance of prenatal care and early detection of skeletal abnormalities for parental education and genetic counseling, offering an informed choice whether to continue with the pregnancy and planning for postnatal care with a multidisciplinary team: obstetrics and gynecology, neonatology, and pediatric orthopedic surgery.

Classics. Fibular hemimelia or deficiency (defectus fibulae)

The author presents an original technique of reconstruction of the ankle joint (including reconstruction of the lateral malleolus) in the congenital absence of the fibula.

Outcomes of Limb Lengthening with Computer-assisted Hexapod External Fixators in the Treatment of Fibular Hemimelia.

Fibular hemimelia (FH) represents the most common deficiency of the long bones and is associated with multiple deformities. Reconstructive treatment with external fixators in FH restores normal lower extremity alignment and length with plantigrade feet for a balanced and effective gait. The aim of this study is to evaluate the outcomes of lower limb lengthening and simultaneous tri-plane deformity correction with a computer-assisted hexagonal external fixator in children with FH. A retrospective review was performed for FH cases treated with a computer-assisted hexagonal external fixator in a tertiary referral center. Leg length discrepancy (LLD), interphyseal angles, tibiocalcaneal distances, healing index (HI), and callus shapes were analyzed for radiologic evaluation, and the Pediatric Quality of Life Inventory (PedsQL) was used for functional assessment. Limbs with HI <50 days/cm, PedsQL >75, and without regenerate fractures were considered successful lengthenings. Twenty-four limbs of 23 patients were included. The limbs were lengthened for a mean of 7.24cm (range, 4.7 to 15.6). The initial LLD of 5.6cm (range, 0.5 to 19cm) increased to 1.7cm (range, 0.1 to 6cm), and the mean interphyseal angle was 12.7 degree (range, 1.5 to 54.2 degree), tibiocalcaneal distance was 0.85cm (range, 0.1 to 1.7) at final follow-up. The most common regenerated bone morphology was cylindrical, as seen in 11 limbs (45.8%). The average PedsQL score was 83.5 (range, 69.5 to 96.7). Sixteen limbs (66.7%) had successful lengthening at their first, and 4 limbs (80%) had successful lengthening at their second surgeries. Seven limbs had complications requiring surgical intervention (29.1%), with 3 (12.5%) regenerate fractures after external fixators removal. Limb reconstruction with computer-assisted hexapod fixators is a successful and reliable option for the treatment of LLD in FH, and patients demonstrate good functional outcomes. Surgeons should be aware of potential complications and should utilize prophylactic measures when necessary. Level III, retrospective comparative study.

Hydrotherapy for Patients With External Fixation: Effect on Infectious Events

Surgery to achieve long bone lengthening and deformity correction in skeletally immature patients is traditionally performed through external fixators. The incidence of infection during treatment is high. Hydrotherapy is highly beneficial during external fixation management, but the infection rates may impact its utilization. The objective of this paper is to document the incidence and duration of infection in patients who participated in a full hydrotherapy program when performed under medical supervision and in accordance with strict follow-up protocols. In this retrospective study, we documented the key characteristics of patients who participated in hydrotherapy after lower limb external fixation surgery including age, sex, diagnosis and surgery site, the incidence and duration of pin site infections, and the percentage of cases, which were able to comply with a full hydrotherapy protocol. The hydrotherapy pool met all daily water quality metrics. Thirty-four children (19 males and 15 females) had a mean±SD age of 10.9±4.6 years. Of the cases, 80% were unilateral and 20% were bilateral. Diagnoses included congenital hereditary (eg, achondroplasia), congenital nonhereditary conditions (eg, fibular hemimelia), and acquired conditions (eg, traumatic). The location of osteotomy site was quite evenly spread between the femur and the tibia/fibula. Three out of the 34 patients were unable to receive hydrotherapy due to continuous secretions; 2 patients did not miss any sessions. The mean number of treatments was 3 per week. During the entire period, a total of 1200 treatments were performed for these patients with only 32 cancellations (about 3%) due to secretions or other signs of infection. Spearman correlation coefficients showed significantly high correlations between the duration of external fixation and the duration of hydrotherapy. There are no significant correlations between infections and other factors (sex, age segment, whether the problem is unilateral or /bilateral, diagnosis, and duration of treatment). This is the first report, to our knowledge, that documents the low prevalence of infectious events during hydrotherapy treatment and demonstrates its feasibility. Level III; retrospective, comparative study.

Specific deletion of Axin1 leads to activation of β-catenin/BMP signaling resulting in fibular hemimelia phenotype in mice.

Axin1 is a key regulator of canonical Wnt signaling pathway. Roles of Axin1 in skeletal development and in disease occurrence have not been fully defined. Here, we report that Axin1 is essential for lower limb development. Specific deletion of Axin1 in limb mesenchymal cells leads to fibular hemimelia (FH)-like phenotype, associated with tarsal coalition. Further studies demonstrate that FH disease is associated with additional defects in Axin1 knockout (KO) mice, including decreased osteoclast formation and defects in angiogenesis. We then provide in vivo evidence showing that Axin1 controls limb development through both canonical β-catenin and BMP signaling pathways. We demonstrate that inhibition of β-catenin or BMP signaling could significantly reverse the FH phenotype in mice. Together, our findings reveal that integration of β-catenin and BMP signaling by Axin1 is required for lower limb development. Defect in Axin1 signaling could lead to the development of FH disease.

Amputate or not Amputate? A Fibular Hemimelia Case Report

Fibular hemimelia is the most frequent congenital defect of the long bones. It has a wide spectrum of clinical and radiological presentations ranging from minor hypoplasia to complete absence of the fibula. Treatment options range between orthotic support, limb reconstruction, and amputation. In the current article, we present a complex clinical case of a young patient with fibular hemimelia. The patient presented a dysmetria of the lower limbs of 21 cm, also an equinovalgus foot position, and several malformations of the foot and toes. She walked with adapted orthosis and crutches, which was proved difficult for her, and esthetically undesirable. Due to the impairment on her quality of life, she was referred initially to bone-lengthening surgery. However, this surgery did not obtain satisfactory results, in both esthetic and functional levels. Hence, the patient was referred to amputation, with a better outcome after. In this clinical case, and reviewing the current literature, we aim to reflect on the implications of the different surgical options in the treatment of fibular hemimelia, and the role of physical medicine and rehabilitation in the functional recovery of these patients. We invite readers to reflect on some questions: Is amputation the best treatment in cases of serious fibular hemimelia? Should a bone-lengthening procedure be attempted before amputation? Should the amputation be delayed? To amputate or not to amputate?

Lower Extremity Malformations.

Lower Extremity Malformations.

Fibular Hemimelia: A Case Series of Nine Patients Treated with Syme Amputation

Fibular Hemimelia: A Case Series of Nine Patients Treated with Syme Amputation

Leg lengthening and deformity correction in fibular hemimelia using ring external fixator

Background: The treatment of fibular hemimelia aims to achieve a functional limb nearly equal in length to the normal limb at maturity, with a plantigrade, stable and flexible foot. The aim of this work was to evaluate the functional results of reconstructive lengthening and deformity correction in fibular hemimelia using the ring external fixator. Methods: This observational study was carried out on 41 patients with fibular hemimelia. The cases either prospectively gathered (sixteen cases, 39 %) to start their management plan or retrospectively collected (twenty-five cases, 61 %) and followed up. Foot reconstruction surgery was done in 22 cases while lengthening surgery applied all cases. All patients were subjected to: history taking, clinical examination (local (lower limb), foot and knee), general examination and imaging. Results: regarding overall results, at the end of follow-up with an average period of 2 years, 4 patients had excellent results (9.76%), 21 patients (51.2%) had good results, 12 patients (29.28%) had fair results and 4 patients (9.76%) had poor result. Regarding subjective functional assessment, out of 41 patients, 20 of them (48.8%) were from 75 to 100% subjectively satisfied, 11 patients (26.8%) from 50 to 74% and 10 patients (24.4%) from 0 to 49% satisfied.

Congenital Anterior Cruciate Ligament Deficiency and Meniscal Tear with Fibular Hemimelia Deformities Managed By Reconstructive Procedures in Two Stages

Congenital cruciate deficiency is associated with many musculoskeletal and congenital abnormalities. Long-standing instability eventually predisposes them to meniscal tears or cartilage damage. We report a case of anterior cruciate ligament (ACL) aplasia with fibular hemimelia and a bucket handle meniscal tear successfully managed with staged procedures. The meniscal tear was repaired, and ACL reconstruction was done in the first stage and was previously published. Valgus deformity of the tibial shaft along with splaying of toes was managed at the second stage (5 months from the first stage), and follow-up result at 3 years from the first surgery is presented. Meniscal pathologies should be suspected in patients with congenital malformations, and correction of the deformity can prevent further episodes of meniscal injuries.

Fibular hemimelia and risk of patellar dislocations: A case series.

The likelihood of patellar instability and consequently, risk of patellar dislocations is higher in those with anatomical abnormalities. Fibular hemimelia is a congenital disorder resulting in partial or full absence of the fibula, often with absence of the lateral and cruciate ligaments, although this patient group rarely undergoes ligament reconstruction. There is potential for adverse outcomes, in the longer term, including, possible increased risk of patellar dislocation and pain in the knee and hip. We aim to investigate the potential risk of spontaneous, unprovoked patellar dislocation among patients with fibular hemimelia, through a review of medical records and radiological investigations. All patients with a diagnosis of fibular hemimelia were included (n = 25), regardless of ultimate approach to management. Tibiofemoral angle measurement and Caton-Deschamps indices were calculated where suitable radiology was available, to better establish extent of potential patellar instability. All the patients with normal Caton-Deschamps indices had only partial fibular absence, although this does not detract from absence or hypoplasia of the anterior cruciate ligament, as a risk factor for patellar dislocation by predisposing to anterior tibial translation. Notably, of the three patients with increased Caton-Deschamps indices, two had complete fibular absence and underwent definitive amputation surgery at age 18 months and 3 years, respectively. Ultimately, this was a young patient group and on-going follow-up might yield better understanding of knee stability. Maintaining a well-aligned lower limb throughout growth might be protective even in the presence of anatomical abnormalities. This article mainly aims to raise awareness among prosthetic and orthotic professionals regarding the increased risk of patella dislocations.

Management of Anterior Cruciate Ligament Aplasia for the Arthroscopy Surgeons: A Systematic Review.

Anterior Cruciate Ligament Aplasia (ACLA) is a rare condition which can sometimes remain asymptomatic into adult life. Although the quoted incidence is 1.7 per 100,000 live births, it is higher due to these silent cases and can be encountered by the arthroscopy surgeon doing reconstructive procedure for the ACL. The aim of this study is to get information on clinical presentation, imaging, arthroscopy findings and management of the knee in patients with ACL aplasia through a systematic review relevant to the arthroscopy surgeon in making a decision when they encounter such a case. We performed a systematic review of the literature to get information on this condition that may be relevant to the arthroscopy surgeon from PubMed, Ovid full text, Embase and Ovid Medline databases. After exclusions and inclusions, we found 31 articles relevant to the search which included 101 knees. Most of the publications were case reports due to the rarity of this condition. ACLA is commonly associated with Fibular Hemimelia, Congenital Dislocation of the Knee and Proximal Focal Femoral Deficiency (PFFD). Clinical findings, imaging appearances, other associated findings with ACLA have been listed and management options listed and discussed. Patients with ACLA are often asymptomatic and arthroscopic reconstruction of the ACL appears to be a viable option in selected symptomatic cases only. The arthroscopic surgeon should be aware of the clinical picture and intraarticular findings of ACLA to recognize the condition and make a decision on the optimal management. Further studies are required to report on long-term outcomes of the condition in terms of secondary osteoarthritis development as well as the procedures performed. We recommend forming a global registry to study and further understand the aspects of this condition. 4. The online version contains supplementary material available at 10.1007/s43465-022-00651-2.