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Comparative Study of Neurosurgical Complications of Thalassemia and Sickle Cell Disease

Open Journal of Modern Neurosurgery 2024年第3期14卷 190-202页

作者： Léon Boukassa Didace Massamba Miabaou Hugues Brieux Ekouélé Mbaki Sinclair Brice Kinata Bambino Olivier Brice Ngackosso Rel Boukaka Kala Gédéon Colin Thouassa Ruth Ibara Wamé Department of Neurosurgery Brazzaville University Hospital Center Brazzaville Republic of the Congo Department of General Surgery Brazzaville University Hospital Center Brazzaville Republic of the Congo

Introduction: Sickle cell disease and thalassemia are the most frequent hemoglobinopathies. During their evolution, they present certain complications, among which are two neurosurgical emergencies, namely spontaneous cranial epidural hematoma and non traumatic radiculo-medullary compression, with some particularities for each. Method: In order to highlight these particularities, we compared the characteristics of these two complications, from a number of publications reported between 2000 and 2021. Results: Sickle cell disease was complicated by spontaneous cranial epidural hematoma. Forty-two cases were reported, the mean age was 14.7 years (2 - 21 years) and the sex ratio was 6.4. The clinical presentation combined, in a non-traumatic context, signs of intracranial hypertension with those of neurological focalization. Neuroimaging showed epidural-type collection, often frontal and parietal in location. The incriminating mechanisms were ischemia, hemorrhage and extra medullary hematopoiesis. The treatment was surgical. Non traumatic radiculo-medullary was the complication of thalassemia. Of the 77 cases reported, the mean age was 27.5 years (9 - 66 years) and the sex ratio was 4.1. The lesions were epidural with a clear thoracic predominance and resulted from extra marrow hematopoiesis. Treatment included: hypertransfusion, radiotherapy, hydroxyurea and surgery. Vital and functional prognosis were globally satisfactory when the management was rapid. Conclusion: Cranial and spinal epidural lesions, respective complications of sickle cell disease and thalassemia, result from similar mechanisms. Their prognosis depends on the rapidity of management. .

关键词： Hemoglobinopathy Sickle Cell Disease Thalassemia Spontaneous Epidural Hematoma extra marrow hematopoiesis Slow marrow Compression

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