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1.
Rev.chil.ortop.traumatol. ; 63(2): 128-133, ago.2022. ilus
Artigo em Espanhol | LILACS | ID: biblio-1436775

RESUMO

INTRODUCCIÓN El granuloma eosinofílico (GE) es una patología infrecuente, sobre todo en adultos, que puede afectar la columna cervical. A pesar de la vasta literatura, esta enfermedad afecta principalmente a la población infantil, y no hay un consenso sobre el manejo en adultos. Con el objetivo de aportar conocimiento respecto a esta patología poco frecuente, se presenta un caso clínico de GE cervical en un paciente de 16 años, a quien se trató de manera conservadora, con buenos resultados y retorno completo a sus actividades. CASO CLÍNICO Un hombre de 16 años, seleccionado de rugby, consultó por dolor cervical axial persistente y nocturno de 6 semanas de evolución, sin trauma evidente. Al examen, destacó dolor a la compresión axial sin compromiso neurológico asociado. Los exámenes de tomografía computarizada (TC) y resonancia magnética (RM) revelaron lesión lítica en el cuerpo de C3 de características agresivas, de presentación monostótica en tomografía por emisión de positrones-tomografía computada (TEP-TC) compatible con tumor primario vertebral. Se decidió realizar biopsia percutánea bajo TC, para definir el diagnóstico y manejo adecuado, la cual fue compatible con células de Langerhans. Al no presentar clínica ni imagenología de inestabilidad ósea evidente o compromiso neurológico, se manejó con tratamiento conservador, inmovilización cervical, analgesia oral, y seguimiento estrecho. A los cuatro meses de evolución, se presentó con una TC con cambios reparativos del cuerpo vertebral y sin dolor, y logró retomar sus actividad habituales. CONCLUSIONES El diagnóstico de GE es infrecuente a esta edad, y se debe plantear entre diagnósticos diferenciales de lesiones líticas agresivas primarias vertebrales. Es necesario el uso de imágenes, y la biopsia vertebral es fundamental para confirmar el diagnóstico. Su manejo va a depender de la sintomatología, del compromiso de estructuras vecinas, y de la estabilidad de la vértebra afectada. El manejo conservador con seguimiento clínico e imagenológico es una opción viable.


INTRODUCTION Eosinophilic granuloma (EG) is a rare, tumor-like lesion, infrequently affecting the cervical spine, particularly in adults. Although vastly described in literature, this pathology mainly affects children, and there is still no consensus on its treatment in older patients. With the goal of contributing to increase the knowledge regarding this infrequent pathology, we present a case of a C3 eosinophilic granuloma in a 16-year-old patient, who was treated conservatively, with good results, including complete return to his previous activities. CLINICAL CASE a 16-year-old male, elite rugby player, presented with a history of persistent neck pain, mainly at night, with no previous trauma. Upon physical examination, he reported neck pain with axial compression of the head, without neurological impairment. Both computed tomography (CT) and magnetic resonance imaging (MRI) scan revealed an aggressive lytic lesion in the C3 vertebral body, a with monostotic presentation on positron emission tomography-computed tomography (PET-CT) compatible with a primary spine tumor. A CT-guided percutaneous biopsy was obtained to establish the diagnosis and provide the proper management. The results were compatible with Langerhans cells. As he presented no symptoms or imaging findings of evident bone instability, as well as no neurological impairment, the patient was treated conservatively, with a cervical brace, oral pain medication and close followup. A CT obtained after four months of treatment showed reparative changes of the C3 vertebral body; at this point, the patient reported no neck pain, so he was able to return to his previous activities. CONCLUSIONS Although an EG is rare at this age, it should be considered in the differential diagnosis of primary vertebral aggressive lytic lesions. Imaging and a vertebral biopsy are paramount to confirm the diagnosis. The treatment modality depends on the symptoms, the involvement of adjacent structures, and the stability of the affected vertebra. Conservative management including clinical and imaging followup is a viable option.


Assuntos
Humanos , Masculino , Adolescente , Doenças da Coluna Vertebral/diagnóstico por imagem , Granuloma Eosinófilo/diagnóstico por imagem , Doenças da Coluna Vertebral/terapia , Imageamento por Ressonância Magnética/métodos , Tomografia Computadorizada por Raios X/métodos , Granuloma Eosinófilo/terapia
2.
Journal of Practical Radiology ; (12): 20-23, 2015.
Artigo em Chinês | WPRIM | ID: wpr-473552

RESUMO

Objective To investigate diagnostic magnetic resonance imaging features of orbital Langerhans cell histocytosis (LCH)and improve its diagnostic accuracy.Methods The symptoms and image data of fourteen histophathology verified orbital LCH cases are reviewed and analyzed.Results Nine patients had swollen eyelids,accompanying with symptoms of inflammation, esphthalmos and orbital masses.One case had cough symptom and another had diabetes insipidus.Of these fourteen cases,seven occurred in right orbital,six occurred in left orbital and one involved bilateral orbital.As to the location of LCH,six cases located in super-lateral wall of the orbit,five cases located in lateral wall of the orbital,and three cases located in roof of orbital.On MRI, thirteen cases lesions show hypo or iso signal intensity on T1 WI,and eleven cases lesions show heterogeneous hyper or iso signal in-tensity on T2 WI.The lesions of eosinophilic granuloma has clear border,which differentiate it from other types.After contrast en-hancement,MR imaging showed marked inhomogeneous enhancement.Conclusion MRI is the primary modality in diagnosing of or-bital LCH,clearly and accurately manifesting the extent of orbital LCH.It will be helpful to diagnose LCH timely if combining with clinical data.MR could provide reliable information for making surgical operation and treatment plan.

3.
Journal of the Korean Society of Magnetic Resonance in Medicine ; : 262-265, 2012.
Artigo em Inglês | WPRIM | ID: wpr-189234

RESUMO

Langerhans cell histiocytosis (LCH) can affect many different organs. However, LCH with pancreatic involvement is very rare with a few reports about imaging findings. We present a case of multisystemic LCH with pancreatic involvement in a five-week-old infant. Pancreas lesion showed hypoechoic on ultrasonography, low density with poor enhancement on CT, and restricted diffusion on diffusion-weighted imaging. Although LCH with pancreatic involvement is rare, LCH should be considered in the differential diagnosis of pancreatic mass in children.


Assuntos
Criança , Humanos , Lactente , Diagnóstico Diferencial , Difusão , Histiocitose de Células de Langerhans , Pâncreas
4.
Journal of Chongqing Medical University ; (12)2003.
Artigo em Chinês | WPRIM | ID: wpr-571666

RESUMO

Objective:To review the X-ray manifestations of the skeletal lesions in children with Langerhans cell histocytosis(LCH),and to evaluate the X-ray examination in the diagnosis of LCH.Methods:The skeletal plain films of 35 children with LCH were analysed retrospectively.AⅡ cases were confirmed by pathology.Results:Among 35 cases,skull destructions were found in 24(68.6%),vertebral destruction in 8(22.9%),long bone destruction in 12(34.3%),flat bone and irregular bone destruction in 4(11.4%).Eosinophilic granulomas mainly involved skull,vertefra and long bones,while Letterer-Siwes disease,Hand-Schuller-Christian disease and intermediary type mainly involved skull bones.Conclusion:Multilocation,diversity and changeability of the skeletal lesions are the x-ray features of LCH in children.X-ray examination and follow-up play an important role in the diagnosis of LCH.

5.
Korean Journal of Pediatric Hematology-Oncology ; : 236-243, 2003.
Artigo em Coreano | WPRIM | ID: wpr-190114

RESUMO

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder characterized by the proliferation of activated Langerhans cells. Although current therapies are very effective at inducing remission, multiple recurrences and long-term sequelae are common for young patients. For this reason, more effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2-chlorodeoxyadenosine (2-CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or refractory LCH. METHODS: Four children with recurrent or refractory LCH received 2-CdA (5~7 mg/m2/day for 5 days, given as a 24-hr continuous infusion and repeated every 21~28 days for 5~7 courses). RESULTS: All four patients had multiorgan involvement, and were heavily pretreated. Of the two children with recurrent diseases, one had complete response and the other showed no active disease except for the remaining diabetes insipidus. Two infants who showed poor early response to previous combination chemotherapy also responded poorly: partial response in one, and progressive disease resulting in death in the other. Toxicity consisted mainly of myelosuppression, but significant infections did not occur. The peripheral neuropathy was not seen. CONCLUSION: 2-CdA, tolerable in children without significant side effects, might be effective for the treatment of recurrent LCH in children. However, the efficacy in infants with multi-system, refractory diseases needs further study. The feasibility of 2-CdA treatment as the first-line therapy for high-risk diseases, and the possibility of combination with other agents needs to be addressed in the future.


Assuntos
Criança , Humanos , Lactente , Cladribina , Diabetes Insípido , Quimioterapia Combinada , Histiócitos , Histiocitose de Células de Langerhans , Células de Langerhans , Linfócitos , Doenças do Sistema Nervoso Periférico , Recidiva
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