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1.
Coluna/Columna ; 19(3): 189-193, July-Sept. 2020. tab, graf
Article de Anglais | LILACS | ID: biblio-1133571

RÉSUMÉ

ABSTRACT Objective To compare the use of a dynamic surgical guide (PediGuard®) and pilot hole preparation, with the use of a probe and the aid of fluoroscopy in osteoporotic or osteopenic patients undergoing pedicular fixation of the thoracic or lumbar spine. Methods One hundred and eight patients were randomized. A pilot hole was prepared with the dynamic surgical guide (PediGuard®), or with a probe with the aid of fluoroscopy. A total of 657 vertebral pedicles (120 thoracic and 180 lumbar) were included in the study. The parameters used for the comparison were: accuracy of the pedicular screw, number of fluoroscopic shots, and change in intraoperative trajectory of the perforation after detecting pedicle wall rupture. Results In the group with use of the dynamic surgical guide, malpositioning of the pedicle screws was observed in 8 (2.6%) patients and intraoperative change of perforation trajectory in 12 (4%) patients, and there were 52 fluoroscopic shots. In the group without use of the dynamic surgical guide (PediGuard®), misplacement of the pedicle screws was observed in 33 (11%) patients and intraoperative change of perforation trajectory in 47 (13.2%) patients, and there were 136 fluoroscopic shots. Conclusion The use of the dynamic surgical guide (PediGuard®) in patients with osteoporosis or osteopenia enabled more accurate placement of pedicular screws, with less change in the intraoperative course of the perforation and less intraoperative radiation. Level of Evidence II; Randomized clinical trial of lesser quality.


RESUMO Objetivo Comparar o uso de um guia cirúrgico dinâmico (PediGuard®) e o preparo de orifício piloto com uma sonda e o auxílio de fluoroscopia em pacientes com osteopenia ou osteoporose submetidos à fixação pedicular da coluna torácica ou lombar. Métodos Cento e oito pacientes foram randomizados. Um orifício piloto foi preparado com o guia cirúrgico dinâmico (PediGuard®) ou com uma sonda com auxílio de fluoroscopia. Foram incluídos no estudo 657 pedículos vertebrais (120 torácicos e 180 lombares). Os parâmetros usados para a comparação foram: acurácia da colocação do parafuso pedicular, número de disparos fluoroscópicos e mudança da trajetória intraoperatória da perfuração depois da detecção de ruptura da parede do pedículo. Resultados No grupo de pacientes em que se usou o guia cirúrgico dinâmico, observou-se mau posicionamento dos parafusos pediculares em oito (2,6%) pacientes e alteração da trajetória intraoperatória da perfuração em 12 (4%) pacientes, com 52 disparos fluoroscópicos. No grupo de pacientes em que o guia cirúrgico dinâmico (PediGuard®) não foi usado o mau posicionamento dos parafusos pediculares foi observado em 33 (11%) pacientes, a mudança intraoperatória da trajetória da perfuração foi vista em 47 (13,2%) pacientes, com 136 disparos fluoroscópicos. Conclusão O uso do guia cirúrgico dinâmico (PediGuard®) em pacientes com osteoporose ou osteopenia permitiu a colocação de parafusos pediculares com maior acurácia, com menor alteração da trajetória intraoperatória da perfuração e menor dose de radiação intraoperatória. Nível de Evidência II; Estudo clínico randomizado de menor qualidade.


RESUMEN Objetivo Comparar el uso de una guía quirúrgica dinámica (PediGuard®) y la preparación del orificio piloto con una sonda y la ayuda de fluoroscopia en pacientes con osteopenia u osteoporosis sometidos a fijación pedicular de la columna torácica o lumbar. Métodos Ciento ocho pacientes fueron asignados aleatoriamente. Se preparó un orificio piloto preparado con la guía quirúrgica dinámica (PediGuard®) o con una sonda con ayuda de fluoroscopia. Se incluyeron en el estudio 657 pedículos vertebrales (120 torácicos y 180 lumbares). Los parámetros utilizados para la comparación fueron: precisión de la colocación del tornillo pedicular, número de disparos del dispositivo de fluoroscopia y cambio en la trayectoria intraoperatoria de la perforación después de la detección de ruptura de la pared del pedículo. Resultados En el grupo de pacientes en el que se utilizó la guía quirúrgica dinámica, se observó mal posicionamiento de los tornillos pediculares en 8 (2,6%) pacientes y cambios de la trayectoria intraoperatoria de la perforación en 12 (4%) pacientes, con 52 disparos del aparato de fluoroscopia. En el grupo de pacientes en los que no se utilizó la guía quirúrgica dinámica (PediGuard®), se observó un mal posicionamiento de los tornillos pediculares en 33 (11%) pacientes, el cambio intraoperatorio de la trayectoria de perforación se observó en 47 (13,2%) pacientes, con 136 disparos fluoroscópicos. Conclusión El uso de la guía quirúrgica dinámica (PediGuard®) en pacientes con osteoporosis u osteopenia permitió la colocación de tornillos pediculares con mayor precisión, menos cambios en la trayectoria intraoperatoria de la perforación y dosis más baja de radiación intraoperatoria. Nivel de Evidencia II; Ensayo clínico aleatorizado de menor calidad.


Sujet(s)
Humains , Soupapes d'Orifice , Maladies osseuses métaboliques , Vis orthopédiques , Radioscopie
2.
Eur J Orthop Surg Traumatol ; 30(4): 621-627, 2020 May.
Article de Anglais | MEDLINE | ID: mdl-31863270

RÉSUMÉ

PURPOSE: The purpose of this study is to evaluate whether patients with high-tone neuromuscular early-onset scoliosis have different surgical outcome and complication rate, when compared to patients with low-tone neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. METHODS: This is a retrospective cohort study of 67 neuromuscular early-onset scoliosis patients, collected from a multicenter database, treated with a rib-to-pelvis rib-based dual growing system. All patients were divided into two groups: high tone and low tone. Pre-, intra- and postoperative data were compared between both groups. Complications were reported by a standardized system. RESULTS: Twenty-six high-tone and 41 low-tone patients were found homogeneous regarding gender, age at surgery, weight, height, estimated blood loss and surgery time. High-tone group (19/26 = 73.1%) experiences more postoperative complications than low-tone group (22/41 = 53.7%). Most common complications were infection, device migration, death and hardware failure. Permanent abandonment of rib-based growing technique and device removal was required in 21% of high-tone patients (P < 0.001). None of the low-tone patients required abandonment. CONCLUSION: High-tone patients had more complications than those with low tone in management of neuromuscular early-onset scoliosis treated with a rib-to-pelvis rib-based dual growing system. A different surgical approach may be required to treat the high-tone neuromuscular early-onset scoliosis.


Sujet(s)
Procédures orthopédiques , Complications postopératoires , Prothèses et implants , Côtes , Scoliose , Rachis , Âge de début , Développement osseux , Enfant , Femelle , Études de suivi , Humains , Incidence , Mâle , Hypertonie musculaire/complications , Hypertonie musculaire/diagnostic , Hypotonie musculaire/complications , Hypotonie musculaire/diagnostic , Procédures orthopédiques/effets indésirables , Procédures orthopédiques/instrumentation , Procédures orthopédiques/méthodes , Complications postopératoires/diagnostic , Complications postopératoires/épidémiologie , Complications postopératoires/étiologie , Porto Rico/épidémiologie , Études rétrospectives , Côtes/imagerie diagnostique , Côtes/chirurgie , Facteurs de risque , Scoliose/épidémiologie , Scoliose/physiopathologie , Scoliose/chirurgie , Rachis/imagerie diagnostique , Rachis/chirurgie , Résultat thérapeutique
3.
Surg Technol Int ; 26: 371-5, 2015 May.
Article de Anglais | MEDLINE | ID: mdl-26055034

RÉSUMÉ

INTRODUCTION: Surgical correction of spinal deformity in myelomeningocele is associated with high rates of pseudarthrosis and implant failure. The anterior fusion is traditionally a wide exposure from the thorax to the sacrum. We report minimally invasive lateral interbody fusion (MILIF) to address the issue of fusion between vertebrae with marginal posterior elements while minimizing the morbidity of an open approach. MATERIALS AND METHODS: We performed a single-center, retrospective review of patients with myelomeningocele and severe scoliosis who underwent posterior spinal fusion (PSF) and staged MILIF for anterior fusion of the thoracolumbar/lumbar spine. We identified four patients with high risk of curve progression who met the following inclusion criteria: diagnosis of myelomeningocele, severe scoliosis (Cobb angle>70°), PSF using greater than 80% pedicle screws, age greater than 10 years at time of surgery, and a minimum follow-up of two years. Radiographic, clinical, and complication data were reviewed. RESULTS: All four patients achieved fusion (100%). The average age at index surgery was 12.8 years (range, 11-16) and follow-up was 3.2 years (range, 2-4.9). The average preoperative coronal Cobb angle measured 111° (range, 74-140°). The average postoperative Cobb angle at follow-up measured 37° (range, 23-42°). The MILIF procedure was performed an average of six months after the index procedure. After anterior fusion, all patients spent one day in the pediatric ICU and an average of 5.5 days in the hospital (range, 4-7). One patient (25%) developed a postoperative wound infection after PSF which required irrigation and debridement in the operating room. CONCLUSION: MILIF as an adjunct to posterior spinal fusion for severe scoliosis associated with myelomeningocele may provide acceptable fusion rates, curve correction, maintenance of correction at mid-term follow-up, and be associated with less morbidity than the traditional anterior approach.


Sujet(s)
Myéloméningocèle/chirurgie , Scoliose/chirurgie , Arthrodèse vertébrale/méthodes , Adolescent , Enfant , Femelle , Humains , Mâle , Myéloméningocèle/imagerie diagnostique , Radiographie , Études rétrospectives , Scoliose/imagerie diagnostique
4.
Coluna/Columna ; 13(3): 210-213, Jul-Sep/2014. tab
Article de Anglais | LILACS | ID: lil-727083

RÉSUMÉ

OBJECTIVE: To determine the effectiveness of a pedicle probe to anticipate an impending breach and allow redirection during placement of a pilot pedicle hole. METHODS: Purposely four cortical wall sites were drilled: medial and lateral pedicle wall, and lateral and anterior wall of the vertebral body. The surgeon stopped probing when the sound changed, suggesting abutment against the cortical wall ("anticipation" of impending breach). A fluoroscopy image was then obtained. The surgeon then advanced the PediGuard through the cortex until the sound changed, indicating a breach. In the second part of the study three probes were used: 1) DSG (PediGuard) with curved tip with electronics ON; 2) DSG with electronics OFF; 3) standard Lenke probe. After the images were taken, the operating surgeon (blinded to x-rays) was instructed to redirect and continue drilling into the vertebral body. RESULTS: The surgeon accurately anticipated 60 of 75 (80%) of the breaches, 17 of 19 (89%) in the medial pedicle wall. In the second part of the study the DSG with electronics ON was superior to the DSG with electronics OFF as well as the standard Lenke probe (100% vs. 90% vs. 79%, p = 0.0191). CONCLUSION: Successful redirection by passing the pedicle probes into the vertebral body without a breach after anticipation of an impending pedicle wall breach occurred in 100% of the drillings when done with the DSG with the electronics ON vs only 84% when there was no electronic feedback. .


OBJETIVO: Avaliar a efetividade da sonda pedicular para prever a rotura iminente e permitir o redirecionamento durante o posicionamento de orifício piloto no pedículo. MÉTODOS: Intencionalmente, foram feitos quatro orifícios na parede cortical: parede medial e lateral do pedículo e parede lateral e anterior do corpo vertebral. O cirurgião parava a sondagem à mudança do som, que sugeria a proximidade da parede cortical ("previsão" de rotura iminente). A imagem por fluoroscopia era obtida. A seguir, o cirurgião avançava a sonda PediGuard através do osso cortical até a alteração do som, que indicava a rotura. Na segunda parte do estudo foram utilizadas três sondas: GCD (PediGuard) com ponta curva ligada, PediGuard curva desligada e sonda Lenke padrão. Depois que as radiografias eram feitas, o cirurgião (sem ver as imagens) era instruído a redirecionar e a continuar perfurando o corpo vertebral. RESULTADOS: O cirurgião previu com precisão 60 das 75 (80%) roturas, 17 de 19 (89%) na parede medial do pedículo. Na segunda parte do estudo, o guia cirúrgico dinâmico ligado foi superior à desligado, assim como à sonda Lenke padrão (100% vs. 90% vs. 79%, p = 0,0191). CONCLUSÃO: O redirecionamento bem-sucedido da sonda pedicular no interior do corpo vertebral, sem rotura devido à previsão de rotura iminente da parede do pedículo ocorreu em 100% das perfurações com a utilização do o guia cirúrgico dinâmico com o dispositivo ligado, em comparação com 84% das perfurações com o dispositivo desligado. .


OBJETIVO: Evaluar la efectividad de la sonda pedicular para prever la rotura inminente y permitir el redireccionamiento durante el posicionamiento de orificio piloto en el pedículo. MÉTODOS: Intencionalmente, fueron hechos cuatro orificios en la pared cortical: pared medial y lateral del pedículo y pared lateral y anterior del cuerpo vertebral. El cirujano paraba el sondeo al cambiar el sonido, que sugería la proximidad de la pared cortical ("previsión" de rotura inminente). Era obtenida imagen por fluoroscopia. A seguir, el cirujano avanzaba la sonda PediGuard a través del hueso cortical hasta la alteración del sonido, que indicaba la rotura. En la segunda parte del estudio fueron utilizadas tres sondas: ECMT (PediGuard) con punta curva encendida, PediGuard curva apagada y sonda Lenke estándar. Después que las radiografías eran realizadas, el cirujano (sin ver las imágenes) era instruido a redireccionar y a continuar perforando el cuerpo vertebral. RESULTADOS: El cirujano previno con precisión 60 de las 75 (80%) roturas, 17 de 19 (89%) en la pared medial del pedículo. En la segunda parte del estudio, la sonda ECMT encendida fue superior a la apagada, así como a la sonda Lenke estándar (100% vs. 90% vs. 79%, p = 0,0191). CONCLUSIÓN: El redireccionamiento exitoso de la sonda pedicular en el interior del cuerpo vertebral, sin rotura debido a la previsión de rotura inminente de la pared del pedículo ocurrió en 100% de las perforaciones con el uso de la sonda ECMT con el dispositivo encendido, en comparación con 84% de las perforaciones con el dispositivo apagado. .


Sujet(s)
Procédures de chirurgie opératoire/méthodes , Arthrodèse vertébrale/instrumentation , Radioscopie , Vis pédiculaires
5.
Spine (Phila Pa 1976) ; 38(25): E1583-8, 2013 Dec 01.
Article de Anglais | MEDLINE | ID: mdl-23963018

RÉSUMÉ

STUDY DESIGN: Dual-center, retrospective study. OBJECTIVE: To evaluate whether vertebral body stapling (VBS) influences curve progression between 30° and 39° in children younger than 10 years with idiopathic scoliosis. SUMMARY OF BACKGROUND DATA: Patients younger than 10 years with idiopathic scoliosis of more than 30° have a 100% risk of progression to spine fusion regardless of nonoperative treatment. VBS may represent an alternative fusionless treatment option for this group of high-risk patients. METHODS: Patients younger than 10 years with idiopathic thoracic or lumbar scoliosis of 30° to 39° who were treated with VBS with a minimum of 24 months of follow-up were studied. Outcome variables were curve progression and magnitude, surgical complications, and a need for reoperation. Preoperative and postoperative curve magnitudes were compared using a paired Student t test. Postoperative curve magnitudes were compared with one another using a paired Student t test. A P value of less than 0.05 was defined as statistically significant. RESULTS: Twelve patients were studied (female: n = 12; average age: 7.8 yr [range: 6.3-9.7 yr]). Thirteen curves were treated with VBS (thoracic: n = 9; lumbar: n = 4). The average follow-up was 3.4 years (range: 2.2-5.4 yr). The average preoperative curve magnitude was 33.4° (range: 30°-39°). The immediate postoperative curve magnitude (19.0°; range: 0°-29°) and curve magnitude at the most recent follow-up (23.0°; range: 10°-34°) were significantly less than the preoperative magnitude of 33.4°. Both thoracic curves (100%) and lumbar curves (100%) were treated successfully. Curve magnitudes did not change significantly postoperatively between the first erect radiographs and the most recent follow-up. Two patients had pneumothorax, and 1 patient had symptomatic pleural effusion. No patient required definitive fusion for curve progression. CONCLUSION: VBS is effective in controlling curve progression in the high-risk group of children younger than 10 years with idiopathic scoliosis between 30° and 39° in whom bracing may be ineffective.


Sujet(s)
Vertèbres lombales/anatomopathologie , Vertèbres lombales/chirurgie , Scoliose/chirurgie , Vertèbres thoraciques/anatomopathologie , Vertèbres thoraciques/chirurgie , Enfant , Femelle , Études de suivi , Humains , Mâle , Réintervention , Études rétrospectives , Arthrodèse vertébrale/méthodes , Résultat thérapeutique
6.
Coluna/Columna ; 12(2): 138-141, 2013. ilus, tab
Article de Anglais | LILACS | ID: lil-680730

RÉSUMÉ

OBJECTIVE: The purpose of this study is to report the results using PediGuard (electrical conductivity device) to reduce radiation exposure while drilling the pilot hole for pedicle screw placement. METHOD: Eighteen patients diagnosed with a degenerative lumbar spine, that required a posterior spinal fusion. Average age of the patients were 55 ± 12 years. Patients received postoperative CT scans of all screws. Scans were reviewed by an independent reviewer grading 'in' < 2 mm of breach , or 'out' > 2 mm of breach. In a randomized fashion, the surgeon created pilot holes with either his standard technique or by using the PediGuard. Fluoroscopy was used for each drilling as necessary. Once the pilot hole was created, the surgeon inserted titanium screws into the pedicle pilot holes. A total of 78 screws (39 standard probe and 39 PediGuard ) were analyzed. RESULTS: 78 screws (39 standard probe and 39 PediGuard were analyzed. No significant difference in breach rate > 2mm by either method (p=1.000), with one screw out in each group. Fluoroscopy shots averaged 5.2 (range, 0 to 15), average decrease of 2.3 (30%) per screw in the PediGuard group vs. 7.5 (range, 2 to 17) in the standard group (p< .001). CONCLUSION: This trial to assess pedicle probe location within the pedicle and vertebral body showed the number of fluoroscopy shots were reduced by 30%, compared to a standard probe while maintaining a 97.5% screw placement accuracy.


OBJETIVO: Relatar os resultados do uso do dispositivo PediGuard para reduzir a exposição à radiação durante a perfuração do orifício piloto para a colocação do parafuso pedicular. MÉTODOS: Dezoito pacientes com diagnóstico de doença degenerative da coluna lombar e submetidos à artrodese posterior (todos pelo investigador principal [CDC]) foram incluídos no estudo. A idade média dos pacientes era de 55 ± 12 anos. No pós-operatório foi realizada tomografia computadorizada em todos os pacientes para avaliação do posicionamento dos parafusos. Os exames foram revisados por um avaliador independente. Os parafusos foram considerados no interior do pedículo quando o rompimento da cortical era menor que 2mm, e localizados for a quando o rompimento era maior que 2mm. O orificio piloto foi re alizado foi realizado de modo randomizado por meio de sonda ou utilizando o PediGuard, tendo sido utilizada a fluoroscopia quando necessario. Parafusos pediculares de titânio foram utilizados de acordo com a técnica padrão. Foram utilizados 78 parafusos de titânio de acordo com a técnica padrão, 39 parafusos com orificio piloto realizado com sonda e 39 parafusos com orificio piloto realizado com o PediGuard. RESULTADOS: Não houve diferença significativa na taxa de rompimento do pedículo > 2 milímetros por qualquer um dos dois métodos (p = 1,000), tendo sido observado apenas 1 parafuso fora do pedículo vertebral em ambos os grupos. A media da utilização da fluoroscopia foi 5,2 disparos (variando de 0 a 15) por parafuso no grupo PediGuard vs 7,5 (variando de 2 a 17) no grupo em que a sonda foi utilizada (p <0,0001). Foi observada redução média de 2,3 disparos (30%) por parafuso com a utilização do PediGuard. Foram realizados 202 disparos na fluoroscopia no grupo do PediGuard e 293 no grupo padrão. CONCLUSÕES: Neste estudo prospectivo e randomizado foi observado que a utilização do PediGuard para o prepare do orifício piloto reduz os disparos da fluoroscopia em 30% em comparação com a utilização de sonda, mantendo a precisão da colocação do parafuso pedicular em 97,5% das perfurações.


OBJETIVO: La finalidad de este estudio es relatar los resultados al usar PediGuard (dispositivo de diferenciación de conductividad eléctrica) para reducir la exposición a la radiación al perforar el orificio piloto para la colocación de tornillos pediculares. MÉTODO: Dieciocho pacientes con diagnóstico de degeneración de la columna lumbar, que precisaban artrodesis espinal posterior. El promedio de edad de los pacientes fue 55 ± 12 años. Los pacientes fueron sometidos a TC de todos los tornillos después de la cirugía. Las TC fueron analizadas por un revisor independiente y graduadas como 'dentro' < 2 mm de vacío o 'fuera' > 2 mm de vacío. De modo aleatorio, el cirujano creó orificios piloto con su técnica estándar o usando PediGuard. La fluoroscopía fue usada para cada perforación, de acuerdo a la necesidad. Una vez creado el orificio piloto, el cirujano insertó tornillos de titanio en los orificios pediculares pilotos. Fue analizado un total de 78 tornillos (39 con sonda estándar y 39 con PediGuard). RESULTADOS: Fueron analizados setenta y ocho tornillos (39 con sonda estándar y 39 con PediGuard). No fue encontrada diferencia significativa en la tasa de vacíos > 2 mm en ninguno de los métodos (p = 1,000), con un tornillo fuera en cada grupo. Cada inyección de fluoroscopía fue en promedio 5,2 (franja 0 a 15), la reducción media fue 2,3 (30%) por tornillo en el grupo PediGuard en comparación con 7,5 (franja 2 a 17) en el grupo estándar (p < 0,001). CONCLUSIÓN: Este estudio evalúa la ubicación de la sonda pedicular en el interior del pedículo y el cuerpo vertebral mostró que el número de inyecciones de fluoroscopía fue reducido en 30% en comparación con la sonda estándar, al mismo tiempo en que mantuvo la precisión de 97,5% en la colocación del tornillo.


Sujet(s)
Humains , Rachis/chirurgie , Arthrodèse , Radioscopie , Exposition aux rayonnements , Vis pédiculaires
7.
J Pediatr Orthop ; 30(6): 521-6, 2010 Sep.
Article de Anglais | MEDLINE | ID: mdl-20733413

RÉSUMÉ

BACKGROUND: Spondylocostal dysplasia (SCD) constitutes a heterogeneous patient group with multiple vertebral formations and segmentation defects of the entire spine, with asymmetric rib malformations. Respiratory failure has been reported in spondylocostal dysplasia secondary to thoracic insufficiency syndrome. The vertical expandable prosthetic titanium rib (VEPTR) reconstructs the chest wall to address the thoracic insufficiency seen in this patient population. The purpose of this study is to evaluate spinal deformity correction and respiratory function outcomes in a spondylocostal dysplasia population treated with VEPTR. METHODS: A cohort of 20 patients with spondylocostal dysplasia and 2-year follow-up were evaluated from a multicenter IDE study of 214 patients who had surgery with the VEPTR device. Data collected included gender, nonskeletal malformations, age at surgery, number of procedures, estimated blood loss, length of stay, and surgical time. Clinical and radiographic parameters were collected, and respiratory function was assessed. RESULTS: In 14 of 20 patients (70%), spinal deformity was controlled evidenced by a decrease of the initial Cobb coronal angle at last follow-up. Fourteen patients (70%) maintained their oxygen level throughout treatment. At preoperative and last evaluation, assisted ventilation rating (AVR) scores showed that 5 patients improved their level of ventilation and 14 patients maintained their AVR level at room air. One patient decreased his level from supplemental oxygen to night ventilation. Mean thoracic spinal length (growth) by year was 0.82 cm. No mortality occurred in this group of patients. CONCLUSIONS: VEPTR implantation in SCD allows continued thoracic spine growth while controlling progressive spine deformity. The improved AVR ratings after surgery suggest a beneficial effect on the natural history of TIS in this population. Mortality and complication rate seem acceptable in this high-risk population of SCD patients. LEVEL OF EVIDENCE: Therapeutic study, Level IV, (case series, no comparison group).


Sujet(s)
Prothèses et implants , Insuffisance respiratoire/chirurgie , Côtes/chirurgie , Malformations multiples/chirurgie , Enfant , Enfant d'âge préscolaire , Femelle , Études de suivi , Cardiopathies congénitales/complications , Cardiopathies congénitales/chirurgie , Hernie diaphragmatique/complications , Hernie diaphragmatique/chirurgie , Humains , Nourrisson , Mâle , Oxygène/métabolisme , Conception de prothèse , Tests de la fonction respiratoire , Insuffisance respiratoire/étiologie , Études rétrospectives , Côtes/malformations , Syndrome , Titane , Résultat thérapeutique
8.
J Pediatr Orthop ; 30(3): 282-8, 2010.
Article de Anglais | MEDLINE | ID: mdl-20357596

RÉSUMÉ

BACKGROUND: A syndrome of children with short stature, bilateral hip dislocations, radial head dislocations, carpal coalitions, scoliosis, and cavus feet in Puerto Rican children, was reported by Steel et al in 1993. The syndrome was described as a unique entity with dismal results after conventional treatment of dislocated hips. The purpose of this study is to reevaluate this patient population with a longer follow-up and delineate the clinical and radiologic features, treatment outcomes, and the genetic characteristics. METHODS: This is a retrospective cohort study of 32 patients in whom we evaluated the clinical, imaging data, and genetic characteristics. We compare the findings and quality of life in patients with this syndrome who have had attempts at reduction of the hips versus those who did not have the treatment. RESULTS: Congenital hip dislocations were present in 100% of the patients. There was no attempt at reduction in 39% (25/64) of the hips. In the remaining 61% (39/64), the hips were treated with a variety of modalities fraught with complications. Of those treated, 85% (33/39) remain dislocated, the rest of the hips continue subluxated with acetabular dysplasia and pain. The group of hips that were not treated reported fewer complaints and limitation in daily activities compared with the hips that had attempts at reduction. CONCLUSIONS: Steel syndrome is a distinct clinical entity characterized by short stature, bilateral hip and radial head dislocation, carpal coalition, scoliosis, cavus feet, and characteristic facial features with dismal results for attempts at reduction of the hips. LEVEL OF EVIDENCE: Prognostic Study Level II.


Sujet(s)
Malformations multiples/physiopathologie , Luxation congénitale de la hanche/physiopathologie , Scoliose/physiopathologie , Malformations multiples/imagerie diagnostique , Adolescent , Adulte , Taille , Os du carpe/malformations , Os du carpe/imagerie diagnostique , Enfant , Enfant d'âge préscolaire , Études de cohortes , Femelle , Études de suivi , Luxation congénitale de la hanche/imagerie diagnostique , Luxation congénitale de la hanche/thérapie , Humains , Luxations/congénital , Luxations/imagerie diagnostique , Mâle , Adulte d'âge moyen , Porto Rico , Qualité de vie , Radiographie , Radius/imagerie diagnostique , Radius/physiopathologie , Études rétrospectives , Scoliose/imagerie diagnostique , Syndrome , Facteurs temps , Jeune adulte
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