Prophylactic Intramedullary Rodding After Femoral Lengthening in Patients With Achondroplasia and Hypochondroplasia.

BACKGROUND: Femoral fracture after femoral lengthening in patients with achondroplasia and hypochondroplasia is a frequent complication, occurring in up to 30%. The purpose of this study is to demonstrate the effectiveness of prophylactic intramedullary rodding in preventing this complication. METHODS: Multicenter retrospective study involving 86 femoral lengthening procedures in 43 patients with achondroplasia or hypochondroplasia. Forty-two femora (21 patients) were prophylactically managed with intramedullary Rush rodding after external fixation removal (11 females and 10 males, mean age 14.6 years) compared with 44 femora (22 patients) without prophylactic intramedullary rodding (13 females and 9 males, mean age 15.2 years). The mean amount of lengthening in the rodding group was 13.3 cm (52.6%) with an External Fixation Index of 25.8 days/cm; in patients without rodding was 14.3 cm (61.5%) and 24.5 days/cm, respectively. RESULTS: Seven cases (15.9%) without rodding developed fractures. Four of them required surgical correction due to displacement or shortening. Only 1 patient (2.4%) had fracture of the femur after prophylactic rodding, and surgery was not required. The incidence of femur fracture was significantly lower in the prophylactic rodding group compared with the nonrodding group (2.4% vs. 15.9%, respectively; P =0.034). There were no cases of infection or avascular necrosis. CONCLUSIONS: Prophylactic intramedullary rodding is a safe and effective method for preventing femoral fractures after femoral lengthening in patients with achondroplasia or hypochondroplasia. LEVEL OF EVIDENCE: Level III-a retrospective comparative study.

Errors en ortopèdia pediàtrica / No disponible

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[Optimization of radiological scoliosis assessment]. / Optimización del estudio radiológico de la escoliosis.

Most scoliosis are idiopathic (80%) and occur more frequently in adolescent girls. Plain radiography is the imaging method of choice, both for the initial study and follow-up studies but has the disadvantage of using ionizing radiation. The breasts are exposed to x-ray along these repeated examinations. The authors present a range of recommendations in order to optimize radiographic exam technique for both conventional and digital x-ray settings to prevent unnecessary patients' radiation exposure and to reduce the risk of breast cancer in patients with scoliosis. With analogue systems, leaded breast protectors should always be used, and with any radiographic equipment, analog or digital radiography, the examination should be performed in postero-anterior projection and optimized low-dose techniques. The ALARA (as low as reasonable achievable) rule should always be followed to achieve diagnostic quality images with the lowest feasible dose.

Optimización del estudio radiológico de la escoliosis / Optimization of radiological scoliosis assessment

La mayoría de las escoliosis son idiopáticas (80%) y ocurren más frecuentemente en adolescentes y en el sexo femenino. La radiografía simple es el método de imagen de elección, tanto en el estudio inicial como en el seguimiento evolutivo, pero tiene el inconveniente de utilizar radiaciones ionizantes. Las mamas quedan en el campo de exposición durante estos estudios repetidos. Los autores presentan una serie de recomendaciones para optimizar la técnica de las exploraciones y los equipos radiográficos, tanto analógicos como digitales, para evitar dosis de irradiación innecesarias a los pacientes y reducir el riesgo de cáncer de mama en los pacientes con escoliosis. Cuando se utilizan equipos analógicos siempre debe utilizarse protector mamario plomado y, en todos los equipos, sean analógicos o digitales, hay que realizar la radiografía en proyección posteroanterior y con técnicas optimizadas de baja dosis. Hay que seguir siempre el principio ALARA (as low as reasonable achievable) para obtener imágenes diagnósticas de calidad con la dosis lo más baja posible (AU)

Most scoliosis are idiopathic (80%) and occur more frequently in adolescent girls. Plain radiography is the imaging method of choice, both for the initial study and follow-up studies but has the disadvantage of using ionizing radiation. The breasts are exposed to x-ray along these repeated examinations. The authors present a range of recommendations in order to optimize radiographic exam technique for both conventional and digital x-ray settings to prevent unnecessary patients' radiation exposure and to reduce the risk of breast cancer in patients with scoliosis. With analogue systems, leaded breast protectors should always be used, and with any radiographic equipment, analog or digital radiography, the examination should be performed in postero-anterior projection and optimized low-dose techniques. The ALARA (as low as reasonable achievable) rule should always be followed to achieve diagnostic quality images with the lowest feasible dose (AU)

Tratamiento prenatal del mielomeningocele / Prenatal treatment of myelomeningocele

El mielomeningocele es la malformación congénita, dentro de los defectos del tubo neural, más grave compatible con la vida. El diagnóstico prenatal suele realizarse en la ecografía morfológica aunque recientemente se han descrito marcadores precoces de primer trimestre. En 2011 se publicó el estudio Management of Mielomeningocele Study (MOMS), estudio aleatorizado comparando los fetos operados prenatalmente con los operados postnatalmente. Los resultados mostraron la reducción de la necesidad de derivaciones ventrículo-peritoneales y una mejoría de la función motora con la intervención prenatal sin reportar una importante morbilidad materna. Desde hace años, en el Hospital Universitari Vall d’Hebron se está trabajando en experimentación animal, inicialmente mediante la creación de un modelo animal de mielomeningocele y posteriormente de diferentes técnicas de reparación. Esta investigación traslacional ha sido aplicada a la práctica clínica. Desde el año 2010 se ofrece un programa multidisciplinar de cirugía prenatal del mielomeningocele(AU)

Myelomeningocele is the most severe congenital malformation among neural tube defects that are compatible with life. Although prenatal diagnosis is usually performed with the 20-22nd week scan, early first trimester markers have been recently described. Management of Myelomeningocele Study (MOMS), a randomized study that compares the prenatally operated fetuses with those that were operated on post-natally, was published in 2011.The results showed a reduction in the need for peritoneal shunts and improved motor function with the prenatal intervention without reporting any significant maternal morbidity. The Hospital Universitari Vall d’Hebron has been working on animal experimentation for many years. Initially, they created an animal model of myelomeningocele, and later on developed several repair techniques. This translational research has been applied to clinical practice. Since 2010, we have offered a multidisciplinary program of prenatal myelomeningocele surgery(AU)

Treatment of congenital pseudarthrosis of the tibia with vascularized fibular periosteal transplant.

The vascularized fibular periosteal flap has been recently described and showed solid angio and osteogenic features. We report the use of a free vascularized fibular periosteal transplant in the treatment of a El-Rosasy-Paley Type III congenital pseudarthrosis of the tibia in a 7-year-old boy, with a prior unsuccessful surgery at the age of three. The contralateral fibular periosteum was used to replace two-thirds of the hamartomatous tibial periosteum. We did not proceed to debriding the focus of the pseudarthrosis nor addressed the tibial recurvatum or revised the previous tibial rod. Consolidation was achieved radiologically at 3 months, allowing for the tibial rod to be removed. One year postoperatively, the patient ambulated without the use of a protective orthesis and resumed his sports practice. This novel pathogenesis-based technique showed promising results and a prompt healing of such a difficult orthopedic condition.

Nena de 13 anys amb lesions òssies lítiques focals al peu / No disponible

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