Del-Rio Camacho G, Orozco A L, Pérez-Higueras A, Camino López M, Al-Assir I, Ruiz-Moreno M
Department of Paediatrics, Fundación Jiménez Diaz, Madrid, Spain.
Pediatr Radiol. 2001 Feb;31(2):125-8. doi: 10.1007/s002470000313.
A girl with Down's syndrome, moyamoya disease and sagittal sinus thrombosis is described. She was diagnosed after acute neurological deterioration by MRI and angiography. Recombinant tissue plasminogen activator (r-TPA) was injected locally to recanalise the thrombus. The patient's condition significantly improved and she was discharged. After 2 years of follow-up the child remains asymptomatic. Moyamoya syndrome and cerebral venous thrombosis should not be overlooked as a cause of acute neurological deterioration in a child with Down's syndrome. MRA appears to be a safe and accurate alternative to traditional angiography for the diagnosis of moyamoya disease. Local fibrinolysis with r-TPA is the treatment of choice for cerebral venous thrombosis due to its safety and efficacy.
本文描述了一名患有唐氏综合征、烟雾病和矢状窦血栓形成的女孩。她在急性神经功能恶化后通过MRI和血管造影确诊。局部注射重组组织型纤溶酶原激活剂（r-TPA）以使血栓再通。患者病情显著改善并出院。经过2年的随访，该患儿仍无症状。烟雾病综合征和脑静脉血栓形成不应被忽视，它们可能是唐氏综合征患儿急性神经功能恶化的原因。MRA似乎是诊断烟雾病的一种安全且准确的传统血管造影替代方法。由于其安全性和有效性，r-TPA局部纤溶是脑静脉血栓形成的首选治疗方法。
