Enfermedad de Caffey: reporte de un caso clínico / Caffey's disease: report of a clinical case / Doença de Caffey: relato de um caso clínico

Introducción: La enfermedad de Caffey es una patología ósea inflamatoria, rara, autolimitada, casi exclusiva de lactantes. Objetivos: Jerarquizar el abordaje diagnóstico de una patología poco frecuente. Caso clínico: 4 meses 22 días, varón, consulta por irritabilidad y edema de miembro inferior izquierdo de 4 días de evolución. Sin traumatismos ni fiebre. Examen físico: edema indurado en tercio medio e inferior de pierna izquierda, no rubor ni calor local. Dolor a la palpación de cara anterior y lateral de tibia y peroné. Limitación funcional, no resaltos óseos. Radiografía: engrosamiento del periostio en tibia y peroné a nivel diafisario. Hemograma: Glóbulos blancos 15.380 KU/L, Hemoglobina 10,8 g/dL, Plaquetas 816.400 10/ul, proteína C reactiva 13,90 mg/dl. Con planteo de probable infección osteoarticular se inicia clindamicina ( gentamicina e ingresa a cuidados moderados. Dada la persistencia de edema e irritabilidad, al quinto día se solicita resonancia magnética: hallazgos sugerentes de un probable proceso inflamatorio- infeccioso de partes blandas con compromiso óseo. Completa 14 días de clindamicina y 7 días de gentamicina intravenosa, hemocultivo negativo. Persiste con edema, irritabilidad y dolor. A los 21 días, se revalora la presentación clínica-imagenológica, se plantea enfermedad de Caffey. Se inicia anti-inflamatorio con buena evolución. Conclusiones: La enfermedad de Caffey es una colagenopatía rara, que afecta lactantes. El diagnóstico es clínico - radiológico (irritabilidad, tumefacción de partes blandas y alteraciones radiológicas). El pronóstico a largo plazo suele ser favorable. Es importante considerar el diagnóstico en lactantes que se presentan con esta sintomatología para evitar retrasos diagnósticos e instauración de tratamientos innecesarios.

Introduction: Caffey's disease is a rare disease that is reported almost exclusively in infants. Objective: Describe the case of a rare pathology, prioritizing the diagnostic approach. Clinical case: 4 month -old, healthy male. Consultation due to irritability and edema of the left lower limb for 4 days. No trauma or fever. Physical examination: indurated edema in the left leg, no redness or local heat. Pain on palpation of the anterior and lateral aspect of the tibia and fibula. Functional limitation, no bony protusions. Leg x-ray: thickening of the periosteum in the tibia and fibula at the diaphyseal level. Hemogram: White Blood Cells 15,380 KU/L Hemoglobin: 10.8 g/dL. Platelets: 816,400 10/ul, C-reactive protein: 13.90 mg/dl. He was admitted with a suggestion of probable osteoarticular infection. Clindamycin ( gentamicin is started. Given the persistence of edema and irritability despite treatment, on the fifth day an MRI was requested: findings suggestive of a probable inflammatory-infectious process of soft tissues with bone involvement. Completed 14 days of clindamycin and 7 days of intravenous gentamicin, blood culture negative. It persists with edema, irritability and pain. After 21 days, the clinical-imaging presentation was reassessed and Caffey's disease was considered. Anti-inflammatory begins with good evolution. Conclusions: Caffey's disease is a rare collagenopathy, that affects infants. The diagnosis is clinical - radiological (irritability, soft tissue swelling and radiological alterations). The long-term prognosis is usually favorable. It is important to consider the diagnosis in infants who present with these symptoms to avoid diagnostic delays and initiation of unnecessary treatments.

Introdução: A doença de Caffey é uma patologia óssea inflamatória rara, autolimitada, quase exclusiva de lactentes. Objetivos: Priorizar a abordagem diagnóstica de uma patologia rara. Caso clínico: 4 meses 22 dias, sexo masculino, consulta por irritabilidade e edema do membro inferior esquerdo de 4 dias de evolução. Sem trauma ou febre. Exame físico: edema endurecido em terço médio e inferior da perna esquerda, sem vermelhidão ou calor local. Dor à palpação das faces anterior e lateral da tíbia e fíbula. Limitação funcional, sem saliências ósseas. Radiografia: espessamento do periósteo na tíbia e fíbula ao nível diafisário. Hemograma: Glóbulos brancos 15.380 KU/L, Hemoglobina 10,8 g/dL, Plaquetas 816.400 10/ul, Proteína C reativa 13,90 mg/dl. Com sugestão de provável infecção osteoarticular, foi iniciada clindamicina + gentamicina e internado em cuidados moderados. Dada a persistência do edema e da irritabilidade, no quinto dia foi solicitada ressonância magnética: achados sugestivos de provável processo inflamatório-infeccioso de partes moles com envolvimento ósseo. Completou 14 dias de clindamicina e 7 dias de gentamicina intravenosa, hemocultura negativa. Persiste com edema, irritabilidade e dor. Após 21 dias, o quadro clínico-imagem foi reavaliado e considerada doença de Caffey. O antiinflamatório começa com uma boa evolução. Conclusões: A doença de Caffey é uma colagenopatia rara que afeta lactentes. O diagnóstico é clínico-radiológico (irritabilidade, edema de partes moles e alterações radiológicas). O prognóstico a longo prazo é geralmente favorável. É importante considerar o diagnóstico em lactentes que apresentam esses sintomas para evitar atrasos no diagnóstico e início de tratamentos desnecessários.

Long Backbone Fracture Caused by Blunt Force: 99 Cases Analysis / 法医学杂志

OBJECTIVE@#To investigate the main point of long backbone fracture caused by blunt force in forensic clinical identification and to provide a reference for the inspection and appraisal practices of such injury.@*METHODS@#Ninety-nine cases of adult long backbone fractures were collected from January 2006 to December 2013 in Gutian County of Fujian Province. According to the terms of fracture location, mode of injury, type, the data were summarized.@*RESULTS@#In the 99 cases, there were 36 cases caused by hitting, kicking, and falling and 63 cases caused by vehicle collision. The majority of the former was ulna, and those of the latter were tibia and fibula. The types of fracture were transverse one, short oblique one, long oblique one, and spiral one.@*CONCLUSION@#Different types of long backbone fracture, not only causing stress load of fractures as well as structural differences related to each segment.

Ossificação do pedículo vascular de um retalho livre de fíbula: relato de caso / Ossification of vascular pedicle in fibula free flap: a case report

Introdução: Várias são as vantagens da utilização de retalhos fibulares para as reconstruções de defeitos craniomaxilofaciais, incluindo a baixa morbidade da área doadora, boa qualidade óssea possibilitando a realização de implantes osteointegrados quando indicados, além da possibilidade de inclusão de uma ilha de pele quando indicado. Durante a dissecção do retalho, próximo à região do pedículo vascular, normalmente inclui-se um cuff muscular e uma faixa de periósteo. O potencial osteogênico do periósteo transplantado tem sido objeto de estudo. Relato de caso: paciente de 15 anos, submetido à reconstrução microcirúrgica com um retalho fibular para um defeito mandibular pós-ressecção de um sarcoma ósseo. Evoluiu com aumento de volume, de consistência óssea na região cervical próximo à cervicotomia realizada para anastomose vascular. Exames de imagem mostravam características ósseas da massa. Foi então submetido à nova cervicotomia e exploração da massa, sendo observada uma formação de tecido ósseo no local da anastomose vascular. Exame anatomopatológico da peça mostrava formação de tecido ósseo adjacente ao retalho periostal. Discussão: Durante a dissecção do retalho fibular, a osteotomia é realizada a alguns centímetros da articulação do joelho, isto a fim de facilitar a dissecção do pedículo vascular na região do oco poplíteo. O pedículo vascular fica então envolto por uma cuff muscular e por uma tira de periósteo. Este mantém sua capacidade osteogênica, que pode ser ativada de acordo com o estímulo do local. A ossificação do periósteo do pedículo vascular de retalhos livres de fíbula permanece um evento raro, porém relatado por centros diferentes.

Introduction: The use of fibula flaps for the reconstruction of craniomaxillofacial defects has many advantages, including the low morbidity of the donor area, good bone quality for use of osseointegrated implants, and the possibility to include a skin island, when indicated. During the dissection of the flap, a muscle "cuff" and a periosteal strip are usually included near the region of the vascular pedicle. The osteogenic potential of the transplanted periosteum has been the object of studies. Case report: A 15-year-old male patient underwent microsurgical reconstruction using a fibula flap for a mandibular defect caused by the resection of a bone sarcoma. He developed increased volume and bone consistency in the cervical region next to the area where a cervicotomy was performed for vascular anastomosis. Imaging examinations showed the characteristics of the bone mass. He then underwent a new cervicotomy and mass exploratory surgery because bone tissue formation was observed at the site of vascular anastomosis. Anatomopathological examination of the specimen showed bone tissue formation next to the periosteal flap. Discussion: During fibula flap dissection, osteotomy is performed a few centimeters from the knee joint to facilitate the dissection of the vascular pedicle in the region of the popliteal fossa. Then, the vascular pedicle is surrounded by a muscle cuff and periosteal strip. This maintains its osteogenic capacity, which can be activated according to the stimulus of the area. Although periosteal ossification of the vascular pedicle in fibula free flaps is a rare event, it has been reported in different centers.

Perone distal estudio anatomico con placa de osteosintesis / Anatomical study of fibula plate location osteosynthesis

La fractura de tobillo es una patologíatraumática muy frecuente, que cuando debe ser intervenidaquirúrgicamente, lo habitual es el uso de placade osteosíntesis, la misma puede ser colocada en unade sus dos caras, externa o posterior. La externa se encuentradividida por una cresta en una superficie triangularanterior subcutánea y una pósteroexterna, diferentesautores recomiendan la colocación en una u otracara. El objetivo de este trabajo es evaluar cuál de lascaras del tercio distal del peroné, es la más adecuadaanatómicamente para la colocación de la placa deosteosíntesis.MATERIALES Y MÉTODOS: Se evaluó el tercio distal de25 perones, 15 correspondieron al lado izquierdo y 10 allado derecho las muestras fueron obtenidas de la Cátedrade Anatomía Normal. Facultad de Ciencias Médicas(UNC. Córdoba), se realizaron mediciones tanto de lacara ánteroexterna como de la pósteroexterna. Para lamedición se utilizó un calibre digital.RESULTADOS: Los resultados obtenidos indicaron unadistancia promedio de la cara ánteroexterna de 75 mmdesde el maléolo hasta el vértice de la cresta (rango 65mm a 92 mm) y una distancia promedio de 65 mm en lacara pósteroexterna desde la fosita de los peroneos hastala unión de ambas (rango 60 mm a 72.3 mm).CONCLUSIÓN: El estudio anatómico del peroné distalnos permite afirmar que ninguna de sus caras terminansiendo ideales para la colocación de una placa, estaosteosíntesis de 7 orificios puede utilizarse en ambascaras ánteroexterna y pósteroexterna, cuando se tratade una fractura transindesmal, pero no es anatómicamenteviable colocar en la cara ánteroexterna cuandohablamos de fracturas suprasindesmales. Debe colocarseen la cara pósteroexterna aunque en esta partetambién interfiera la unión de ambas caras...

The fibula is a long thin bone thatarticulates with the tibia at both ends. A study wasconducted over 25 perones anatomical and analyzed thedistal third. Our goal is to evaluate which of the faces ofthe distal fibula is the most appropriate anatomically tothe placement of the osteosynthesis plate.MATERIALS AND METHODS: Was evaluated peronesdistal third of 25, 15 corresponded to the left and rightside 10 samples were obtained from plants of NormalAnatomy Chair Faculty of Medical Sciences (UNCCordoba), were measured both as the anterolateral theposterolateral was used for measuring a digital caliper.RESULTS: The results showed an average distance of75 mm anterolateral from the malleolus to the apex of theridge (range 65 to 92 mm) and an average distance of 65mm in the posterolateral from the pit of the peroneal tothe junction of two (range 60 to 72.3 mm).CONCLUSION: We conclude that none of its faces endup being suitable for placement of a plate, theosteosynthesis plate of holes 7 may be used on both sidesanterolateral and posterolateral when dealing with fractureanatomically syndesmotic but not feasible to placeon the face when speaking of fractures anterolateralsuprasindesmales. Should be placed in the posterolateralbut also interfere in this part of the union of both sides...

Ankle Deformity Secondary to Acquired Fibular Segmental Defect in Children

BACKGROUND: The authors report the long-term effect of acquired pseudoarthrosis of the fibula on ankle development in children during skeletal growth, and the results of a long-term follow-up of Langenskiold's supramalleolar synostosis to correct an ankle deformity induced by an acquired fibular segmental defect in children. METHODS: Since 1980, 19 children with acquired pseudoarthrosis of the fibula were treated and followed up for an average of 11 years. Pseudoarthrosis was the result of a fibulectomy for tumor surgery, osteomyelitis of the fibula and traumatic segmental loss of the fibula in 10, 6, and 3 cases, respectively. Initially, a Langenskiold's operation (in 4 cases) and fusion of the lateral malleolus to the distal tibial epiphysis (in 1 case) were performed, whereas only skeletal growth was monitored in the other 14 cases. After a mean follow-up of 11 years, the valgus deformity and external tibial torsion of the ankle joint associated with proximal migration of the lateral malleolus needed to be treated with a supramallolar osteotomy in 12 cases (63%). These ankle deformities were evaluated using the serial radiographs and limb length scintigraphs. RESULTS: In all cases, early closure of the lateral part of the distal tibial physis, upward migration of the lateral malleolus, unstable valgus deformity and external tibial torsion of the ankle joint developed during a mean follow-up of 11 years (range, 5 to 21 years). The mean valgus deformity and external tibial torsion of the ankle at the final follow-up were 15.2degrees (range, 5degrees to 35degrees) and 10degrees (range, 5degrees to 12degrees), respectively. In 12 cases (12/19, 63%), a supramalleolar corrective osteotomy was performed but three children had a recurrence requiring an additional supramalleolar corrective osteotomy 2-4 times. CONCLUSIONS: A valgus deformity and external tibial torsion are inevitable after acquired pseudoarthrosis of the fibula in children. Both Langenskiold supramalleolar synostosis to prevent these ankle deformities and supramalleolar corrective osteotomy to correct them in children are effective initially. However, both procedures cannot maintain the permanent ankle stability during skeletal maturity. Therefore any type of prophylactic surgery should be carried out before epiphyseal closure of the distal tibia occurs, but the possibility of a recurrence of the ankle deformities and the need for final corrective surgery after skeletal maturity should be considered.

Congenital osteofibrous dysplasia of the tibia, associated with pseudoarthrosis of the ipsilateral fibula

We describe an otherwise normal male neonate who presented shortly after birth with rare congenital osteofibrous dysplasia of the right tibia associated with pseudoarthrosis of the ipsilateral fibula. The lesion was curetted, and the defect was packed with a fibular bone graft from the other leg. Histopathological examination was typical for osteofibrous dysplasia. The ipsilateral fibular pseudoarthrosis was observed with no active intervention. Seven years follow-up showed good functional recovery without recurrence of the lesion. The case is a new presentation of congenital osteofibrous dysplasia, and is presented to draw attention to this rare condition that must be considered in the differential diagnosis of congenital lesions of the tibia

Carcinoma of uterine cervix with isolated metastasis to fibula and its unusual behavior: report of a case and review of literature.

Bone metastasis from carcinoma cervix is uncommon, especially in the distal appendicular skeleton. A 36 year old lady presented with carcinoma of uterine cervix, FIGO, stage IIb. She was treated with radical radiotherapy. Nine months later, she developed an isolated lytic lesion in right fibula, which turned out to be a metastatic lesion. The patient is doing well, 3 years after the surgical excision of metastasis. This is one of the few documented cases of metastasis to fibula, arising from carcinoma of uterine cervix and probably the first with isolated metastasis of this site. Unlike the dismal outcome commonly seen in patients with bone secondaries, she continues to be disease free and alive at 39 months of follow up, after the development of skeletal metastasis.

Bilateral Transverse (Bowdler) Fibular Spurs with Hypophosphatasia in an Adolescent Girl

Hypophosphatasia is a clinically heterogeneous inheritable disorder characterized by defective bone mineralization and the deficiency of serum and tissue liver/bone/kidney alkaline phosphatase activities. Due to the mineralization defect of the bones, various skeletal findings can be radiologically observed in hypophosphatasia. Bowing and Bowdler spurs of long bones are the characteristic findings. The Bowdler spurs reported on in the previous pertinent literature were observed in the perinatal aged patients and these lesions have rarely involved adolescents. We herein report on a 14-year-old girl with fibular Bowdler spurs.

Carcinoma epidermoide asociado a osteomielitis crónica del peroné. Presentación de un caso y revisión de la literatura / Epidermiomoid carcinoma associated to chronic osteomyelitis of the fibula. Reporte of a case and review of the literature

El carcinoma epidermoide es una neoplasia maligna que se origina del epitelio; en algunas ocasiones se presenta asociada a la osteomielitis crónica: El sitio de mayor presencia es la tibia. Es de difícil diagnóstico dado que se asocia a úlceras generalmente infectadas, con problemas de cicatrización. El diagnóstico histopatológico también es complejo por la gran metaplasia celular que se produce en las úlceras. Un dato que apoya el diagnóstico es la dificultad para la cicatrización a pesar del tratamiento. El diagnóstico se consigue con biopsias de diferentes sitios de la úlcera y el tratamiento generalmente tiene que ser radical. Se reporta un caso de carcinoma epidermoide

Melorreostosis. Presentación de un caso y revisión de la literatura / Melorheostosis. Case report

La melorreostosis es una enfermedad poco común, se describió desde 1933, su etiología es poco precisa aun cuando se cree que es de origen genético con defectos en la capa mesodérmica. Se describe el caso de una niña diagnosticada y tratada en el Hospital Shriners. En esta patología se observa, a nivel óseo, el engrosamiento de la cortical y según su extensión, en los huesos de todo un miembro o en una porción de un solo hueso producirá una deformación total o parcial del miembro

Adamantinoma: relato de um caso / Adamantinoma: a case report

O adamantinoma, também chamado ameloblastoma, é neoplasia rara dos ossos longos (pouco mais de 100 casos descritos na literatura) e com origem näo estabelecida. Por possuir aspectos que lembram o ameloblastoma da mandíbula, é geralmente correlacionado com aquele tumor, inclusive no próprio nome. Com a finalidade de dar conhecimento à literatura, os autores apresentam um caso considerado clássico de adamantinoma e fazem breve revisäo da clínica, radiologia e patologia deste tumor

Tumor maligno primario de hueso y embarazo: comunicación de un caso y revisión de la literatura / Primary malignant tumor of bone and pregnancy: report of a case and review of the literature

Se presenta I gesta de 18 años con diagnóstico de embarazo de 33 semanas y tromboflebitis de pierna izquierda cuya enfermedad comenzó a los 2 meses de embarazo con edema; radiología realizada reveló tumor maligno de peroné izquierdo; la paciente fue referida a otra institución donde tuvo parto vaginal de recién nacida que le sobrevive luego de la muerte de la madre ocurrida 14 meses depués del diagnóstico. La literatura revisada expresa que el estudio radiológico debe ser realizado ante dolor y/o edema persistente, independientemente de la edad del embarazo, ya que hay poca evidencia de efecto teratogénico en embrión humano para dosis menores de 15 rads, que pueden usarse también radionúcleidos (99 technetium) con fines diagnósticos; una vez obtenido radiodiagnóstico el siguiente paso es el estudio histológico mediante biopsia con aguja fina o biopsia abierta; que el embarazo no tiene efecto deletéreo en el curso de los sarcomas ni viceversa, por lo tanto no hay indicación de interrupción del embarazo salvo en los casos en que se requiera quimioterapia en primer trimestre; que no se ha demostrado la transmisión genética ni tampoco han sido descritas metástasis fetales y/o placentarias