ABSTRACT
La pseudoartrosis congénita de la clavícula es una malformación rara y benigna, caracterizada por la ausencia del tercio medio de la clavícula. Suele ser unilateral y mayoritaria en el lado derecho. La etiología es desconocida y se postulan diversas teorías etiopatogénicas (vascular, embriológica y genética).Puede detectarse en el período neonatal o, más frecuentemente, durante la infancia. En ocasiones, puede ser sintomática. Puede requerir tratamiento mediante reconstrucción quirúrgica por injerto óseo.Se presentan 2 casos, uno de diagnóstico neonatal y otro de 3 años de edad realizados con 24 h de diferencia. Se destaca la consideración de este diagnóstico como diferencial de fractura obstétrica o postraumática, displasia cleidocraneal y neurofibromatosis de tipo 1.
The congenital pseudoarthrosis of the clavicle is a rare and benign malformation, characterized by the absence of the middle third of the clavicle. It is usually unilateral and the majority on the right side. The etiology is unknown, postulating diverse etiopathogenic theories (vascular, embryological and genetic).It can be detected in the neonatal period or, more frequently, during childhood. Occasionally it can be symptomatic. It may require treatment by surgical reconstruction by bone graft. Two cases are presented, one of neonatal diagnosis and another one of 3 years of age performed with 24 hours of difference. We emphasize on its consideration as a differential diagnosis of obstetric or post-traumatic fracture, cleidocranial dysplasia and neurofibromatosis type I.
Subject(s)
Humans , Female , Infant, Newborn , Child, Preschool , Pseudarthrosis/congenital , Clavicle/abnormalities , Pseudarthrosis/diagnostic imaging , Congenital Abnormalities , Diagnosis, DifferentialABSTRACT
Objetivo: Reportar la capacidad de la proteína morfogenética de lograr la consolidación ósea en pacientes con seudoartrosis congénita de tibia. Pacientes y métodos: Se realizó en tres pacientes con diagnóstico de seudoartrosis congénita de tibia, se hizo seguimiento durante seis meses a cada uno observando la consolidación y capacidad funcional de la extremidad. Resultados: Se logró consolidación en todos los casos presentándose ésta a los tres meses y medio en promedio. Conclusiones: La proteína morfogenética tiene utilidad como coadyuvante para conseguir la regeneración y consolidación ósea en pacientes con seudoartrosis congénita de tibia.
Objective: To report the ability of bone morphogenetic protein to achieve bone healing in patients with congenital pseudoarthrosis of the tibia. Patients and methods: Three patients with a diagnosis of congenital pseudoarthrosis of the tibia were followed-up for six months. Bone healing and functional capacity of the limb were observed. Results: Bone healing occurred in all cases at a mean of three and half months of treatment. Conclusions: Morphogenetic protein is useful as an adjuvant to achieve bone regeneration and healing in patients with congenital pseudoarthrosis of the tibia.
Subject(s)
Child , Child, Preschool , Humans , Male , Bone Morphogenetic Proteins/administration & dosage , Bone Regeneration/drug effects , Pseudarthrosis/congenital , Follow-Up Studies , Pseudarthrosis/pathology , Pseudarthrosis/therapy , Treatment Outcome , Tibia/pathologyABSTRACT
Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging problems in pediatric orthopaedics. The treatment goals are osteosynthesis, stabilization of the ankle mortise by fibular stabilization, and lower limb-length equalization. Each of these goals is difficult to accomplish but regardless of the surgical options, the basic biological considerations are the same: pseudarthrosis resection, biological bone bridging of the defect by stable fixation, and the correction of any angular deformity. The Ilizarov method is certainly valuable for the treatment of CPT because it can address not only pseudarthrosis but also all complex deformities associated with this condition. Leg-length discrepancy can be managed by proximal tibial lengthening using distraction osteogenesis combined with or without contralateral epiphysiodesis. However, treatment of CPT is fraught with complications due to the complex nature of the disease, and failure is common. Residual challenges, such as refracture, growth disturbance, and poor foot and ankle function with stiffness, are frequent and perplexing. Refracture is the most common and serious complication after primary healing and might result in the re-establishment of pseudarthrosis. Therefore, an effective, safe and practical treatment method that minimizes the residual challenges after healing and accomplishes the multiple goals of treatment is needed. This review describes a multi-targeted approach for tackling these challenges, which utilizes the Ilizarov technique in atrophic-type CPT.
Subject(s)
Humans , Ilizarov Technique/adverse effects , Pseudarthrosis/congenital , Tibia/surgeryABSTRACT
Os autores apresentam o caso de um paciente do sexo masculino, com pseudartrose congênita e encurtamento importante da tíbia esquerda, após inümeros procedimentos cirúrgicos convencionais sem êxito, e seu resultado pelo método de Ilizarov.
Subject(s)
Humans , Male , Adult , Tibial Fractures/surgery , Tibial Fractures/congenital , Pseudarthrosis/congenital , Pseudarthrosis/surgery , Ilizarov Technique/methodsABSTRACT
Presentamos una serie de 5 pacientes con diagnóstico de SCT tipo II de Boyd, intervenidos entre los 18 meses y los 7 años 2 meses (m: 4a 2m) 4 pacientes con un total de 9 cirugías previas y uno solo sin antecedentes quirúrgicos. El acortamiento previo varió entre 3 y 7 cm (m: 4.98 cm). A todos los pacientes se les transfirió un peroné vascularizado con una longitud entre 8.7 y 14 cm (m: 11.14 cm), utilizando fijación externa en 3 y clavo centromedular en 2 casos. En todos los casos se obtuvo consolidación ósea: la unión proximal entre las 6 y 24 semanas y la distal entre las 6 y 16 semanas. En cuatro pacientes se retiró la osteosíntesis y se inició el apoyo gradual a partir de las 16 semanas. Se midió el índice de hipertrofia del peroné transplantado según de Boer y Wood, encontrando hipertrofia mayor del 20 por ciento a partir de la cuarta semana e hipertrofia endóstica entre la octava y décima semanas. Las complicaciones fueron: neuropraxia bilateral del sural externo que revirtió rápidamente (un caso), contractura del flexor propio del dedo grueso manejada conservadoramente (un caso), infección del tracto de los clavos en un fijador que requirió de su retiro precozmente (un caso) y consolidación en valgo de tobillo que requirió de osteotomía correctora (un caso). El acortamiento residual al máximo seguimiento fue de entre 0 a 2.5 cm (m: 1.12 cm). La transferencia microvascular de peroné es el método más efectivo en el menejo actual de SCT tipo II de Boyd o multioperadas. Su morbilidad es menor que la reportada para otros métodos convencionales más reconocidos
Subject(s)
Infant , Child, Preschool , Child , Humans , Male , Female , Orthopedics , Pseudarthrosis/congenital , Surgical Procedures, Operative , Tibia/abnormalities , Fibula/transplantationABSTRACT
Os autores apresentam o estudo prospectivo de 25 pacientes portadores de psudoartrose congênita da tíbia, tratados pelo método de Ilizarov. A classificaçao adotada foi a de Ilizarov (1972), modificada por Catagni (1986), preconizando para cada tipo de pseudartrose um tipo de montagem específica. Ressaltam a maior incidência de lesao no terço distal da tíbia (84 por cento). A idade média dos pacientes era de 8,1 anos. A grande maioria apresentava pseudartrose atrófica dos tipos A1 e B2 (80 por cento), cujo tempo médio de consolidaçao foi de 14,5 meses. As pseudartroses hipertróficas de tipos A2, A2D e A3 apresentaram consolidaçao em média de 8,8 meses. O tempo médio geral de consolidaçao foi de 12,4 meses. Encontrou-se como doença associada a neurofibromatose, com incidência de 76 por cento. Relatam como caso raro, sem qualquer citaçao na literatura, associaçao com hemimelia paraxial fibular total. Registram como resultado 92 por cento de consolidaçao, correçao da discrepância de suas medidas em mais de 50 por cento e correçao das deformidades em 70 por cento. Discutem as complicaçoes do método e concluem pela validade deste tipo de tratamento.
Subject(s)
Humans , Male , Female , Child, Preschool , Child , Ilizarov Technique , Pseudarthrosis/congenital , Pseudarthrosis/surgery , Tibia/surgery , Follow-Up Studies , Prospective Studies , Treatment OutcomeABSTRACT
Os autores apresentam sua experiência no tratamento de cinco pacientes portadores de pseudartrose congênita dos ossos do antebraço. Relatam os casos descritos na literatura e ressaltam a raridade desta doença. Descrevem a técnica cirúrgica de reconstruçäo utilizando o transplante de fíbula vascularizada e mostram os bons resultados obtidos.
Subject(s)
Humans , Male , Female , Child , Adolescent , Forearm/surgery , Fibula/transplantation , Pseudarthrosis/surgery , Forearm , Pseudarthrosis/congenitalABSTRACT
Five children with congenital pseudarthrosis of the tibia were treated by resection of the lesion and reconstruction of the extremity with a free vascularized fibular graft (FVFG). Two of five cases had had multiple surgical procedures before FVFG. These two cases were complicated with the non-union of the proximal grafthost junction. Of these, one case achieved bone union after further resection of the non-union and adding allogenic bone graft and another case denied further operation and was lost to follow-up 6 months postoperatively. Three cases, two of which underwent FVFG as a primary treatment and another one after failure from the Sofield procedure respectively, were initially treated successfully and solid union of the grafthost junctions was within four months postoperatively. Problems including: anterior bowing, refracture, valgus deformity of the ankle and leg length discrepancy occurred in all four followed cases.
Subject(s)
Adolescent , Child , Child, Preschool , Female , Fibula/blood supply , Humans , Male , Pseudarthrosis/congenital , Tibia/abnormalitiesABSTRACT
É apresentada uma revisäo do tema Pseudoartrose Congênita da Clavícula, abordando os aspectos clínicos, radiológicos e anatomopatológicos. Apresenta-se o caso de um menino de sete anos, com esta patologia na clavícula direita