Morais Barbara Albuquerque, Cardeal Daniel Dante, Ribeiro E Ribeiro Renan, Frassetto Fernando Pereira, Andrade Fernanda Goncalves, Matushita Hamilton, Teixeira Manoel Jacobsen
Hospital das Clínicas, School of Medicine, University of São Paulo, Street Eneas de Carvalho, 155, Pinheiros, São Paulo, Brazil.
Childs Nerv Syst. 2017 Aug;33(8):1399-1403. doi: 10.1007/s00381-017-3415-0. Epub 2017 Apr 25.
Intramedullary hemangioblastomas are rare benign vascular tumors, infrequent in pediatric patients. Clinical symptoms vary according to the age of presentation, tumor size, location, and concomitant syringomyelia. This is the second reported case of hemangioblastoma presenting with acute hydrocephalus.
A 3-month-old infant with acute hydrocephalus was asymptomatic after a ventriculoperitoneal shunt was placed. She returned 3 months later with irritability, acute paraplegia, and respiratory distress. Magnetic resonance imaging (MRI) showed an intramedullary T8-T9 tumor with syringomyelia. She underwent surgical resection with good results during the 6-month follow-up.
Intramedullary tumors may present as hydrocephalus and other nonspecific symptoms, with invariably delayed diagnosis in children, but must be considered in suspicious cases.
髓内血管母细胞瘤是罕见的良性血管肿瘤，在儿科患者中不常见。临床症状因发病年龄、肿瘤大小、位置以及是否合并脊髓空洞症而异。这是第二例报告的以急性脑积水为表现的血管母细胞瘤病例。
一名3个月大患急性脑积水的婴儿在进行脑室腹腔分流术后无症状。3个月后她再次就诊，出现易激惹、急性截瘫和呼吸窘迫。磁共振成像（MRI）显示T8 - T9节段髓内肿瘤并伴有脊髓空洞症。她接受了手术切除，6个月随访期间效果良好。
髓内肿瘤可能表现为脑积水和其他非特异性症状，儿童中诊断往往延迟，但在可疑病例中必须予以考虑。
