Pediatric Neurology Section, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.
Pediatric Neurology Section, Hospital Infantil Universitario Niño Jesús, Madrid, Spain.
Pediatr Neurol. 2017 Dec;77:48-53. doi: 10.1016/j.pediatrneurol.2017.08.013. Epub 2017 Sep 1.
We investigated the clinical characteristics of a pediatric population with hemato-oncological disease and intracranial hypertension, analyze the therapeutic response and outcome, and compare its characteristics with respect to a control group with idiopathic intracranial hypertension.
We retrospectively analyzed patients with hemato-oncological disease and secondary intracranial hypertension in our center during the past five years. We compared these individuals with a historical cohort with idiopathic intracranial hypertension from our institution (control group).
We identified eight patients, all with leukemia, and 21 controls. Mean age at diagnosis was 10.6 years, and 62% of individuals were female. Most of them were under treatment with drugs (62% corticosteroids, 75% active chemotherapy). Mean opening pressure of cerebrospinal fluid was 35 cm HO. All had headache, but only 28% complained of visual symptoms. Only 12.5% exhibited papilledema at the time of diagnosis (versus 71% in controls). All of them were treated with acetazolamide, with average therapy duration of nine months, and all had a favorable outcome (versus 57% of controls who needed second-line treatment). None of them showed long-term visual complications (versus 20% of controls).
Patients with hemato-oncological disease and secondary intracranial hypertension may not develop typical symptomatology. Thus, diagnosis and recognition of this entity among this cohort may be difficult. Associated factors are diverse and do not show an obvious causal relationship. A high index of suspicion must be maintained for diagnosis, because a favorable outcome is expected with prompt treatment. Acetazolamide is effective as a first-line therapy and caused few side effects.
我们研究了伴有血液肿瘤疾病和颅内高压的儿科患者的临床特征，分析了治疗反应和结果，并将其与特发性颅内高压的对照组进行了比较。
我们回顾性分析了过去五年内在我院就诊的伴有血液肿瘤疾病和继发性颅内高压的患者。将这些患者与我院的特发性颅内高压患者（对照组）进行比较。
我们共发现 8 例患者，均患有白血病，21 例对照组患者。诊断时的平均年龄为 10.6 岁，62%的患者为女性。他们大多接受药物治疗（62%的患者接受皮质类固醇治疗，75%的患者接受活性化疗）。脑脊液的平均开放压力为 35cmH2O。所有患者均有头痛，但只有 28%的患者有视觉症状。只有 12.5%的患者在诊断时出现视乳头水肿（对照组为 71%）。所有患者均接受乙酰唑胺治疗，平均治疗时间为 9 个月，所有患者均有良好的预后（对照组中需要二线治疗的患者为 57%）。他们均无长期视觉并发症（对照组为 20%）。
血液肿瘤疾病和继发性颅内高压患者可能不会出现典型的症状。因此，在该队列中诊断和识别该疾病可能较为困难。相关因素多种多样，且无明显的因果关系。必须保持高度怀疑，以便及时诊断和治疗，从而获得良好的预后。乙酰唑胺作为一线治疗有效，且副作用较少。
