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1.
Orthopadie (Heidelb) ; 52(9): 710-718, 2023 Sep.
Artículo en Alemán | MEDLINE | ID: mdl-37522930

RESUMEN

The history and development of circular fixation is closely aligned with the history of limb lengthening. Gavriil A. Ilizarov not only developed his circular fixator, but he also was the first to research and publish on distraction osteogenesis. Progress and dissemination of the method was initially based on individual pioneers; a standardized nomenclature and annual courses led to a wider dissemination. The introduction of the Taylor spatial frame in 1997 was a game changer. Although various groups had already developed Hexapod-fixators, it was the TSF that hit the market and achieved market presence. After the patent for TSF expired, many companies developed hexapod fixators with various modifications. A new version of the TSF, the Smart TSF, which included an intuitive planning software, was introduced in Baltimore in 2021. The introduction of intramedullary lengthening nails at the beginning of the 2000s and the wide dissemination starting approximately 2013 rendered external fixation obsolete for certain indications. However, ring fixators are still the method of choice for complex reconstruction of deformity in traumatology, as well as in pediatric orthopedics in the presence of open growth plates.


Asunto(s)
Fijadores Externos , Osteogénesis por Distracción , Masculino , Niño , Humanos , Fijación de Fractura , Clavos Ortopédicos , Programas Informáticos
2.
Acta Orthop ; 93: 696-702, 2022 09 02.
Artículo en Inglés | MEDLINE | ID: mdl-36069481

RESUMEN

BACKGROUND AND PURPOSE: We report results and complications of gradual bone lengthening for post-traumatic lower-limb length discrepancy (LLD) with Precice intramedullary lengthening nails in 34 adults. PATIENTS AND METHODS: Inclusion criteria were lower-limb lengthening using the Precice nail, posttraumatic etiology, age older than 18 years, and minimum follow-up of 12 months after implantation. 34 patients met the inclusion criteria. Radiological and clinical outcome data were collected. RESULTS: Precice lengthening was performed in the femur in 28 patients (21 antegrade, 7 retrograde) and in the tibia in 6. Mean patient age at time of surgery was 32 years (18-72). Mean preoperative LLD was 31 mm (20-71). Acute correction of axial or rotational malalignment was performed in 11 segments. At final follow-up (mean 2 years [1.1-3.6]), 33 of 34 nails had been removed. All lengthening sites were healed, and all patients mobilized with full weight-bearing. 14 adverse events occurred in 11 patients and were categorized as problems (n = 5), obstacles (n = 3), and complications (n = 6). Unplanned surgery was necessary in 7 patients, 3 of whom did not complete treatment with Precice. INTERPRETATION: Correction of posttraumatic LLD with or without axial malalignment using Precice intramedullary lengthening nails is associated with a low number of complications and good functional outcome. However, one-fifth of patients in this series needed further unplanned surgery for revision of obstacles or complications.

3.
Front Endocrinol (Lausanne) ; 13: 866170, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-35399930

RESUMEN

Background: Gait deviations, lower limb pain and joint stiffness represent key symptoms in patients with X-linked hypophosphatemia (XLH, OMIM 307800), a rare disorder of mineral homeostasis. While the pathomechanism for rickets is well understood, the direct role of PHEX (Phosphate-regulating neutral endopeptidase) deficiency in non-rachitic features including complex deformities, skull and dental affections remains unclear. FGF23-inhibiting antibody treatment can normalize serum phosphate levels and to improve rickets in XLH patients. However, linear growth remains impaired and effects on lower limb deformity and gait are insufficiently studied. Aims: To characterize and evaluate the course of lower limb deformity in a case series of pediatric XLH patients receiving Burosumab therapy. Methods: Comparative assessment of planar radiographs, gait analysis, biochemical and clinical features of pediatric patients before and ≥12 months after initiation of FGF23-inhibiting was performed prospectively. Lower limb maltorsion was quantified by torsional MRI and gait analysis. Standardized deformity analysis of lower limb anteroposterior radiographs was conducted. Results: Seven patients (age 9.0 +/-3.6 years) were eligible for this study. All patients received conventional treatment before onset of antibody treatment. Maltorsion of the femur was observed in 8/14 legs using torsional MRI (mean antetorsion 8.79°). Maltorsion of the tibia was observed in 9/14 legs (mean external torsion 2.8°). Gait analysis confirmed MRI findings with femoral external malrotation prior to and one year after onset of Burosumab therapy. Internal foot progression (intoeing gait) remained pathological in all cases (mean 2.2°). Knee rotation was pathologically internal 10/14 legs. Mean mechanical axis deviation (MAD) of 16.1mm prior to Burosumab changed in average by 3.9mm. Three children underwent guided growth procedures within the observation period. Mild postprocedural rebound of frontal axis deviation was observed under Burosumab treatment in one patient. Conclusions: This is the first study to investigate lower limb deformity parameters quantitatively in children with XLH receiving Burosumab. One year of Burosumab therapy was associated with persistent maltorsion and frontal axis deviation (varus/valgus) despite improved rickets in this small, prospective uncontrolled study.


Asunto(s)
Raquitismo Hipofosfatémico Familiar , Extremidad Inferior , Niño , Raquitismo Hipofosfatémico Familiar/complicaciones , Raquitismo Hipofosfatémico Familiar/tratamiento farmacológico , Factores de Crecimiento de Fibroblastos , Humanos , Extremidad Inferior/patología , Fosfatos , Estudios Prospectivos
4.
J Pediatr Orthop ; 42(2): e192-e200, 2022 Feb 01.
Artículo en Inglés | MEDLINE | ID: mdl-34799542

RESUMEN

BACKGROUND: The Precice intramedullary bone lengthening nail has been used in our department since 2013. We sought to determine the efficacy and safety of intramedullary limb lengthening with Precice nails in children and adolescents. METHODS: We retrospectively investigated patients 18 years and younger who underwent lower-limb lengthening using the Precice nail. Radiologic and clinical outcome data were obtained from a prospective database. The minimum postimplantation follow-up was 12 months. Between March 2013 and March 2020, 161 patients underwent limb lengthening with a Precice nail; 76 patients met the inclusion criteria. RESULTS: We used 84 nails in 76 patients (68 femurs and 16 tibias). Femoral nails were inserted using an antegrade approach in 57 patients and a retrograde approach in 11. The mean age at surgery was 16 years (range, 9 to 18 y). The mean lengthening was 33 mm (range, 14 to 80 mm) with additional acute axial or rotational malalignment correction in 16 segments. At the last follow-up (mean=2.1, years; range, 1 to 5 y), all regenerates had healed and all patients were mobile with full weight-bearing. Complications that necessitated surgical revision occurred in 6 patients (8%), and the desired lengthening was not achieved in 2 patients. Postlengthening malalignment occurred in 4 patients (5 tibial nails). The weight-bearing index, defined as days from surgery to full weight-bearing/cm of lengthening, was a mean of 45 days (range, 7 to 127 d/cm). CONCLUSIONS: The Precice nail facilitated reliable and safe bone lengthening and was associated with a low complication rate. Correction of additional malalignment was possible by applying intraoperative acute correction or guided growth. LEVEL OF EVIDENCE: Level IV-therapeutic study investigating the results of treatment.


Asunto(s)
Alargamiento Óseo , Fijación Intramedular de Fracturas , Adolescente , Clavos Ortopédicos , Niño , Fémur/diagnóstico por imagen , Fémur/cirugía , Fijación Intramedular de Fracturas/efectos adversos , Humanos , Diferencia de Longitud de las Piernas/diagnóstico por imagen , Diferencia de Longitud de las Piernas/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
5.
Foot Ankle Clin ; 26(4): 619-637, 2021 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-34752231

RESUMEN

The Ponseti method for treatment of congenital clubfoot is well established and has been introduced in most pediatric orthopedic centers worldwide. However, reported rates of recurrence are largely variable and open joint surgery is still performed frequently, even in the age group younger than 6 years of age. Preventing recurrence and residual deformity can be achieved by strict adherence to the Ponseti method, ensuring and enforcing brace compliance, frequent follow-up, and early treatment of recurrence. This review discusses reasons for clubfoot recurrence, prevention of clubfoot recurrence, and the treatment of recurrent congenital clubfoot within the realm of the Ponseti method.


Asunto(s)
Pie Equinovaro , Tirantes , Moldes Quirúrgicos , Niño , Pie Equinovaro/cirugía , Estudios de Seguimiento , Humanos , Manipulación Ortopédica , Recurrencia , Resultado del Tratamiento
6.
Front Endocrinol (Lausanne) ; 12: 754084, 2021.
Artículo en Inglés | MEDLINE | ID: mdl-34646241

RESUMEN

Background: X-linked hypophosphatemia (XLH) is a rare genetic disorder characterized by lower limb deformity, gait and joint problems, and pain. Hence, quality of life is substantially impaired. This study aimed to assess lower limb deformity, specific radiographic changes, and gait deviations among adolescents and adults with XLH. Design: Data on laboratory examination and gait analysis results were analyzed retrospectively. Deformities, osteoarthritis, pseudofractures, and enthesopathies on lower limb radiographs were investigated. Gait analysis findings were compared between the XLH group and the control group comprising healthy adults. Patients and Controls: Radiographic outcomes were assessed retrospectively in 43 patients with XLH (28 female, 15 male). Gait analysis data was available in 29 patients with confirmed XLH and compared to a healthy reference cohort (n=76). Results: Patients with XLH had a lower gait quality compared to healthy controls (Gait deviation index GDI 65.9% +/- 16.2). About 48.3% of the study population presented with a greater lateral trunk lean, commonly referred to as waddling gait. A higher BMI and mechanical axis deviation of the lower limbs were associated with lower gait scores and greater lateral trunk lean. Patients with radiologic signs of enthesopathies had a lower GDI. Conclusions: This study showed for the first time that lower limb deformity, BMI, and typical features of XLH such as enthesopathies negatively affected gait quality among adolescents and adults with XLH.


Asunto(s)
Raquitismo Hipofosfatémico Familiar/fisiopatología , Marcha , Extremidad Inferior/fisiopatología , Adolescente , Adulto , Anciano , Raquitismo Hipofosfatémico Familiar/diagnóstico por imagen , Raquitismo Hipofosfatémico Familiar/patología , Raquitismo Hipofosfatémico Familiar/cirugía , Femenino , Humanos , Extremidad Inferior/diagnóstico por imagen , Extremidad Inferior/patología , Masculino , Persona de Mediana Edad , Radiografía , Estudios Retrospectivos , Adulto Joven
7.
J Pediatr Orthop ; 40(8): 418-424, 2020 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-32205682

RESUMEN

BACKGROUND: We aimed to evaluate the effect of tibialis anterior tendon transfer (TATT) on foot motion in children with clubfoot recurrence after initial Ponseti treatment. METHODS: Children with dynamic clubfoot recurrence after initial Ponseti treatment who underwent TATT between 2014 and 2017 were considered for inclusion. Exclusion criteria were neurological disease, split transfer of the tendon, additional bone or joint invasive surgery, and initial treatment abroad. Of 94 children (143 TATT), 36 met the inclusion criteria. Seventeen (47%) of the 36 children with 25 clubfeet and a mean age at the time of surgery of 6.8 years participated in the study. Gait analysis, including the Oxford foot model, was conducted preoperatively and postoperatively. Furthermore, kinematic and kinetic data were compared with those of age-matched healthy children (n=18). RESULTS: Forefoot supination in relation to the hindfoot and tibia was reduced during swing and at initial contact after TATT compared with preoperative values. Forefoot supination in relation to the tibia at initial contact decreased from 12.4 to 5.2 degrees after TATT (control group, 6.0 degrees). The heel showed less dynamic varus and adduction movement after TATT compared with preoperatively. Maximum ankle power was reduced preoperatively and postoperatively compared with controls. Maximum ankle dorsiflexion slightly increased after TATT. CONCLUSIONS: Gait analysis showed normalization of the main components of dynamic clubfoot recurrence after TATT. This joint-sparing surgery efficiently corrects recurrent dynamic deformity. LEVEL OF EVIDENCE: Level II-therapeutic.


Asunto(s)
Pie Equinovaro , Ortesis del Pié , Prevención Secundaria , Supinación/fisiología , Transferencia Tendinosa , Fenómenos Biomecánicos , Niño , Preescolar , Pie Equinovaro/diagnóstico , Pie Equinovaro/cirugía , Femenino , Pie/fisiopatología , Análisis de la Marcha , Humanos , Masculino , Evaluación de Resultado en la Atención de Salud/métodos , Prevención Secundaria/instrumentación , Prevención Secundaria/métodos , Transferencia Tendinosa/efectos adversos , Transferencia Tendinosa/métodos
8.
Unfallchirurg ; 122(1): 33-43, 2019 Jan.
Artículo en Alemán | MEDLINE | ID: mdl-30607483

RESUMEN

BACKGROUND: Fractures in childhood can result in malalignment and/or leg shortening due to insufficient reduction, malunion or injury to the growth plate. OBJECTIVE: Different types of posttraumatic deformities around the knee as well as the diagnostic approach and correction are discussed. MATERIAL AND METHODS: While deformities without shortening can often be corrected during surgery with internal fixation, limb shortening with or without malalignment has to be treated with external fixation or internal lengthening nails. RESULTS: Modern six-axis external fixators enable simultaneous lengthening and axial correction in all planes in patients from childhood onwards. A correction using lengthening nails is possible at the end of growth or if the physis has already been closed by trauma. CONCLUSION: Precise analysis of the deformity and leg length discrepancy is necessary to select the best point in time and the right surgical procedure and hardware for correction.


Asunto(s)
Diferencia de Longitud de las Piernas , Osteotomía , Niño , Fijadores Externos , Fijación de Fractura , Humanos , Articulación de la Rodilla
9.
J Pediatr Orthop ; 38(7): 365-369, 2018 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-27574955

RESUMEN

BACKGROUND: Despite the popularity of tension band plating (TBP) current literature lacks clinical data concerning recurrence ("rebound") of frontal plane malalignment. This study investigated the rebound phenomenon after TBP in idiopathic genu varum/valgum deformities. We analyzed factors that may contribute to the development of rebound after removal of TBP. METHODS: Patients who had correction of idiopathic valgus or varus deformities by TBP at the distal femoral and/or proximal tibial growth plate were selected from a prospective consecutive database. Only patients who had plates removed for at least 1 year and had a long standing radiograph of the lower limbs before plate removal were included. Patients who had presumably not yet reached skeletal maturity (age under 14 y for girls and under 16 y for boys) were excluded. The change of the mechanical axis from plate removal to follow-up after skeletal maturity was evaluated and a statistical analysis was performed. RESULTS: Twenty-nine patients (64 extremities) were eligible. The mean follow-up was 39.1 months (range, 12.3 to 67.3 mo). The mean mechanical axis deviation (MAD) was +0.8 mm (range, -26 to +22 mm) after plate removal and -2.4 mm (range, -29 to +27 mm) at follow-up, accounting for a significant change of MAD (P=0.046). We observed a mean, relative recurrence of frontal plate malalignment into valgus direction of -3.2 mm (range, -48 to +23 mm). Twenty extremities (31%) showed <3 mm of MAD change; 27 extremities (42%) showed >3 mm of MAD change into valgus, and 17 extremities (27%) >3 mm of MAD change into varus direction. Patients with <3 mm MAD change had only 0.8 years, and those >3 mm a mean of 2.0 years of remaining growth until skeletal maturity. Each additional m/kg of body mass index increased the risk of valgus recurrence by 12.1%. The 3 genua vara patients revealed to have an even higher rate of malalignment recurrence (4 of 6 limbs) at final follow-up. CONCLUSIONS: Given our strict criteria, there is a high rate of radiologic recurrence of frontal plane malalignment after TBP. Children who are more than 1 year before skeletal maturity at TBP removal, and those with increased body mass index are at higher risk for rebound growth. LEVEL OF EVIDENCE: Level IV-consecutive therapeutic case series.


Asunto(s)
Placas Óseas , Fijación Interna de Fracturas/métodos , Genu Varum/cirugía , Placa de Crecimiento/cirugía , Adolescente , Factores de Edad , Placas Óseas/efectos adversos , Niño , Femenino , Fémur/diagnóstico por imagen , Fémur/cirugía , Fijación Interna de Fracturas/efectos adversos , Genu Varum/diagnóstico por imagen , Placa de Crecimiento/diagnóstico por imagen , Humanos , Masculino , Estudios Prospectivos , Radiografía , Recurrencia , Estudios Retrospectivos , Tibia/diagnóstico por imagen , Tibia/cirugía , Resultado del Tratamiento
10.
J Child Orthop ; 10(6): 521-528, 2016 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-27826907

RESUMEN

PURPOSE: Instability of the knee is a common finding in patients with congenital limb deficiency. The instability can be attributed to soft tissue abnormalities, frontal, sagittal or rotational deformity of the lower limb and bony dysplasia of the patella or of the femoral condyles. In most of the cases, these pathomorphologic changes stay asymptomatic in daily activity. However, instability can appear during deformity correction and bone-lengthening procedures, leading to flexion contracture or subluxation of the knee. METHODS: A review of pediatric orthopaedic literature on different factors of knee instability, state-of-the-art treatment options in congenital limb deficiency and in cases of lengthening-related knee subluxation is presented and the authors' preferred treatment methods are described. RESULTS: Leg lengthening and deformity correction in patients with congenital limb deficiencies can be achieved with various techniques, such as guided growth, monolateral or circular external fixation and intramedullary lengthening nails. Radiographic assessment and clinical examination of the knee stability are obligatory to estimate the grade of instability prior to surgical procedures. Preparatory surgery, as well as preventive measures such as bracing, bridging of the knee and intensive physical therapy, can help to avoid subluxation during lengthening in unstable knees. CONCLUSIONS: Adequate surgical techniques, preventive measures and early detection of signs of subluxation can lead to good functional results in patients with congenital limb deficiency.

11.
Foot Ankle Clin ; 20(4): 563-86, 2015 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-26589079

RESUMEN

Understanding the pathoanatomy of severe recurrent clubfoot and its implication on treatment options is important for the successful treatment. A comprehensive clinical evaluation of the different components helps in selecting procedures. Individual needs and social and psychological factors influencing treatment and the impact of treatment on the child have to be considered. With increasing dissemination and improved understanding of the Ponseti method, a further decrease in the frequency of severe recurrent clubfoot can be hoped for and expected.


Asunto(s)
Pie Equinovaro/terapia , Pie Equinovaro/cirugía , Humanos , Recurrencia , Grabación en Video
13.
J Bone Joint Surg Am ; 96(19): 1593-9, 2014 Oct 01.
Artículo en Inglés | MEDLINE | ID: mdl-25274784

RESUMEN

BACKGROUND: The aim of the study was to evaluate how clubfeet treated with the Ponseti method compare with control feet in gait analysis and whether additional information can be provided by the Oxford foot model. METHODS: All patients with a minimum age of three years in our prospective database of clubfeet treated with the Ponseti method were considered for inclusion. Exclusion criteria were an associated syndrome or neurological disease, positional (slight) clubfoot, and presentation at an age of more than three months. Of the 125 patients with 199 clubfeet who satisfied the criteria, thirty-six (29%) agreed to participate in the study. Four of these were excluded because of insufficient gait analysis data, leaving thirty-two patients with fifty clubfeet for evaluation. Clinical examination and three-dimensional gait analysis including the Oxford foot model were performed, and a disease-specific questionnaire was administered. Kinetic and kinematic results were compared with those of an age-matched control group (n = 15). RESULTS: The mean score on the disease-specific questionnaire was 83.5. Gait analysis showed significantly decreased range of motion, plantar flexion, and power of the ankle compared with controls. The mean external foot progression angle of 5.7° in the Ponseti group was slightly less than that in the controls. Slight intoeing occurred in 24%, and 12% did not achieve a neutral position during swing phase. Slight compensation was observed, including external rotation of the hip in 28%. The Oxford foot model revealed differences in foot motion between the groups. CONCLUSIONS: Clubfoot treatment with the Ponseti method yielded good clinical results with high functional scores. Three-dimensional gait analysis demonstrated distinctive but slight deviations. Intoeing was less frequent and less severe compared with groups in the literature. We recommend the use of three-dimensional gait analysis, including a foot model, as an objective tool for evaluation of the results of clubfoot treatment. LEVEL OF EVIDENCE: Therapeutic Level II. See Instructions for Authors for a complete description of levels of evidence.


Asunto(s)
Pie Equinovaro/fisiopatología , Pie Equinovaro/terapia , Marcha/fisiología , Niño , Preescolar , Bases de Datos Factuales , Femenino , Humanos , Masculino , Estudios Prospectivos , Encuestas y Cuestionarios
14.
Prenat Diagn ; 34(10): 940-5, 2014 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-24764248

RESUMEN

OBJECTIVES: Routine ultrasonography can detect congenital femoral deficiency (CFD) and fibular hemimelia (FH), but prenatal detection rate and its relation to deformity severity have never been reported. Whether mothers prefer prenatal diagnosis is also unknown. We aimed to determine whether mothers prefer prenatal diagnosis, to report detection rates for CFD and/or FH, and to correlate detection rates to severity of limb shortening. METHODS: Surveys were mailed to 171 mothers who gave birth to children with CFD/FH between 2000 and 2008. Bilateral femoral and tibial lengths were measured on postnatal radiographs. We calculated corresponding femoral/tibial lengths at gestational weeks 20 and 30. RESULTS: Sixty-five surveys were returned, and 56 radiographs were reviewed. Most mothers (63%) preferred prenatal diagnosis as it enables prenatal counseling. Congenital limb shortening was detected in 24 cases (37%) and was not detected in 41 cases (63%). Detection rate was 52% (12 of 23) in CFD cases, 23% (three of 13) in FH cases, and 30% (six of 20) in combined cases. CFD cases with severe shortening had a higher detection rate. CONCLUSIONS: Ultrasonographers should measure both femoral and tibial lengths. Unilateral shortening should result in pediatric orthopedic consultation to estimate limb-length discrepancy at maturity and discuss treatment.


Asunto(s)
Ectromelia/diagnóstico por imagen , Fémur/diagnóstico por imagen , Tibia/diagnóstico por imagen , Ultrasonografía Prenatal , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Embarazo , Radiografía , Estudios Retrospectivos
15.
J Child Orthop ; 8(1): 3-10, 2014 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-24488847

RESUMEN

BACKGROUND: Diagnosis and early treatment of developmental dysplasia of the hip (DDH) continue to be issues of discussion. In 1992, a nationwide general ultrasound screening program using Graf technique was introduced to detect DDH in Austria. We investigated the effects of this program on the rates of operative and conservative interventions and the influence of the program on the number of hospital admissions for the treatment of DDH. METHODS: All cases of DDH documented in Austrian hospitals from 1992 to 2008 were included in this retrospective study. The database of the Austrian Ministry of Health was used to extract documented diagnoses and treatments. RESULTS: Since the introduction of the screening program, the number of patients who require pelvic surgery to treat DDH has decreased by 46 % and the number of open reductions is as low as 0.16 per 1,000 live births. Hospital admissions for the treatment of DDH decreased from 9.5 to 3.6 per 1,000 live births. All noted results gained statistical significance. CONCLUSION: Compared with routine clinically based screening programs, our results confirm low numbers of open reductions and pelvic surgeries. We, therefore, advocate a standardized nationwide general ultrasound screening program to reduce the rates of operative interventions and hospital admissions associated with the treatment of DDH. LEVEL OF EVIDENCE: Level III, diagnostic.

16.
Artículo en Inglés | MEDLINE | ID: mdl-24023524

RESUMEN

PURPOSE: Cleidocranial dysplasia is an autosomal dominant disorder characterized by defective ossification of the intramembraneous ossification (primarily the clavicles, cranium, and pelvis), and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. Spine deformities were of progressive nature and considered to be the major orthopedic abnormalities encountered in our practice in patients with cleidocranial dysplasia. We aimed to further delineate the underlying spine pathology and its etiological understanding. Extraspinal deformities were dealt with respectively. MATERIAL AND METHODS: In this paper, we describe 7 patients who were consistent with the phenotypic and the genotypic characterization of cleidocranial dysplasia. Reformatted computed tomography (CT) scans have been applied in several instances to further understand the underlying pathology of progressive spine tilting. Radiographs were sufficient to illustrate other skeletal malformations. RESULTS: Anatomical survey demonstrates that a broad spectrum of frequently unrecognized orthopedic aberrations were encountered. We believe that torticollis has evolved in connection with the persistence of synchondrosis of the skull base and the upper cervical spine and these are strongly correlated to the well-known pathology of posterior occipital synchondrosis. Similarly, scoliosis and kyphoscoliosis resulted from the pathologic aberration of the cartilaginous stage of disrupted embryological development. All our results are discussed for the first time. Coxa vara, patellar dysplasia, and genu valgum were observed as extraspinal deformities. CONCLUSION: This paper includes for the first time the anatomical analysis of the malformation complex of the craniocervical and the entire spine in patients with cleidocranial dysplasia. Reformatted CT scan was the modality of choice. We were able to illustrate that the persistence of skull base and the cervical spine synchondrosis were correlated with the pathological mechanism of the posterior occipital synchondrosis. Therefore, injuries to the craniocervical region in these patients might lead to a wide range of dreadful complications, ranging from complete atlanto-occipital or atlanto-axial dislocation to nondisplaced occipital condyle avulsion fractures with the possibility of morbid and or mortal outcome. On the other hand, the persistence of a cartilaginous spine was the reason behind the progressive spine tilting. This pathological form can be considered as a notoriously unpredictable malformation complex. The value of presenting these patients is to demonsterate that the genotype is not a precise index to assess the severity and the natural history of the phenotype.

17.
Int Orthop ; 37(9): 1827-31, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23900385

RESUMEN

PURPOSE: The Ponseti method has become the gold standard for clubfoot treatment. Although promising short-term results have been published, only a few studies report results at the end of the bracing period. We aimed to evaluate the functional midterm results, rate of recurrence and need for subsequent surgery. METHODS: Patients from our prospective database of clubfeet treated with the Ponseti method with a minimum age of three years were identified. Exclusion criteria were syndrome or neurogenic association, address in a foreign country, presentation after six weeks of age, more than three casts applied elsewhere and correction with less than three casts. A total of 125 patients met the inclusion criteria. The Pediatric Outcomes Data Collection Instrument (PODCI), the disease-specific instrument (DSI) questionnaire and an invitation for a clinical examination were sent out. For patients not presenting for evaluation, data from the last follow-up were extracted. RESULTS: Seventy questionnaires (56 %) of patients with a mean age of 5.7 years (3.3-8.9 years) were returned. The DSI score (n = 65) was 85.3 (± 13.01 SD) and the PODCI score (n = 59) was 95.5 (± 6.3 SD). A total of 113 of 125 patients (90.4 %) with 182 clubfeet were examined in the study or seen in follow-up. During a mean follow-up of 5.2 years (range 3-8.5 years) a repeat tenotomy was performed in 4 % of cases, a percutaneous Achilles tendon lengthening in 3 %, a tibialis anterior tendon transfer in 13 % and open joint surgery in 5 %. The mean dorsiflexion with knee extended was 15.9° (range 0-32°; SD ± 5.5) with 16 feet (9 %) presenting less than 10°. CONCLUSIONS: The functional scores indicate that the Ponseti method results in mostly pain-free feet not limiting age-appropriate activity. In this consecutive case series open joint surgery could be avoided in 95 % of cases with a good functional and anatomic outcome.


Asunto(s)
Pie Equinovaro/cirugía , Tendón Calcáneo/cirugía , Moldes Quirúrgicos , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Masculino , Manipulación Ortopédica , Osteotomía , Recuperación de la Función , Tenotomía
18.
Int Orthop ; 37(9): 1747-53, 2013 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-23928728

RESUMEN

The Ponseti method has become the gold standard of care for the treatment of congenital club foot. Despite numerous articles in MEDLINE reporting results from around the globe there are still crucial details of the Ponseti method which seem to be less commonly known or considered. The Ponseti method is not only a detailed method of manipulation and casting but also of preventing and treating relapse. Recommendations on how to correct complex club foot have resulted in an almost 100 % initial correction rate. The foot abduction brace is crucial for preventing relapse and is still a challenge for families and sometimes doctors alike. Experience and knowledge on how to support the parents, how to set and apply the brace in the best possible way and how to solve problems that can be encountered during the bracing period are essential to ensure compliance. Regular follow-up visits are necessary to be able to detect early signs of recurrence and prevent full relapse by enforcing abduction bracing, recasting or performing tibialis anterior tendon transfer. Recent midterm outcome studies have shown that by following the Ponseti treatment regime in all aspects it is possible to prevent open joint surgery in almost all cases. The body of literature of the last decade has evaluated many steps and aspects of the Ponseti method and gives valuable answers to questions encountered in daily practice. This review of the current literature and recommendations on the different aspects of the Ponseti method aims to promote understanding of the treatment regime and its' details.


Asunto(s)
Pie Equinovaro/cirugía , Tendón Calcáneo/cirugía , Tirantes , Moldes Quirúrgicos , Humanos , Manipulación Ortopédica , Rango del Movimiento Articular , Tenotomía
19.
J Bone Joint Surg Am ; 94(4): 349-55, 2012 Feb 15.
Artículo en Inglés | MEDLINE | ID: mdl-22336974

RESUMEN

BACKGROUND: Anterior tibial tendon transfer is a common procedure for treatment of clubfoot recurrence. Fixation of the tendon usually includes passing the tendon through the lateral cuneiform. Drilling the bone and passing sutures through the plantar aspect of the foot may cause neurovascular damage. METHODS: Anterior tibial tendon transfer was performed through the lateral cuneiform in twelve cadaveric limbs. Drill holes were made perpendicular to the lateral cuneiform surface (group A), made perpendicular to the weight-bearing surface (group B), inclined 15° in the frontal and sagittal planes (group C), or aimed at the middle of the plantar aspect of the foot (group D). Two unmodified Keith needles and two blunted Keith needles were each passed ten times per foot. A dissection was performed. The average distance from the drill hole to the nerve structures and the number of punctures of nerve structures were reported. RESULTS: In group A, the drill hole was 1.7 mm from a medial plantar nerve branch and 5 mm from the nerve bifurcation. In group B, the hole was 0.3 mm from a branch of the lateral plantar nerve and 25.3 mm from the lateral plantar nerve bifurcation. The drill hole in group C was 1.7 mm from the lateral plantar nerve bifurcation. In group D, the drill direction resulted in an inclination of 22° in the frontal plane and 4° in the sagittal plane. The drill exited 7.7 mm from a medial plantar nerve branch and 4.3 mm from a lateral plantar nerve branch. The medial and lateral plantar nerve bifurcations were at a distance of 13 mm and 14.7 mm, respectively, from the drill hole in group D. Unmodified Keith needles punctured nerve structures twelve times in group A, twenty times in group B, six times in group C, and once in group D. Use of blunted Keith needles resulted in no nerve punctures. CONCLUSIONS: When anchoring the transferred anterior tibial tendon in the lateral cuneiform for the treatment of clubfoot recurrence, the drill should be aimed at the middle of the plantar surface of the foot to minimize the risk of nerve damage. Passing the sutures with a blunt needle might prevent damage to nerves or vessels when anterior tibial tendon transfer to the lateral cuneiform is performed for the treatment of clubfoot recurrence.


Asunto(s)
Pie/inervación , Complicaciones Intraoperatorias/prevención & control , Traumatismos de los Nervios Periféricos/prevención & control , Transferencia Tendinosa/métodos , Cadáver , Pie Equinovaro/cirugía , Femenino , Humanos , Masculino , Riesgo
20.
J Pediatr Orthop B ; 21(1): 52-8, 2012 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-22134652

RESUMEN

The treatment of neglected or relapsed clubfoot is still a challenge. Extensive open surgeries may lead to postoperative scarring and various complications.Gradual distraction using circular fixators for treatment of these cases was described by many researchers in the last decades. Different techniques were used with and without open surgeries. Recently the Taylor Spatial Frame was described for clubfoot correction using the principles of the Ponseti technique. Results of treatment using different techniques are described in this review. External fixation with soft tissue distraction even without open surgery is an effective treatment for neglected or relapsed clubfoot.


Asunto(s)
Pie Equinovaro/terapia , Fijadores Externos , Técnica de Ilizarov/instrumentación , Artrodesis , Artrogriposis/complicaciones , Artrogriposis/terapia , Pie Equinovaro/etiología , Humanos , Manipulación Ortopédica , Osteotomía , Recurrencia
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