Cochrane D D, Adderley R, White C P, Norman M, Steinbok P
Department of Pediatric Neurosurgery, British Columbia's Children's Hospital, Vancouver, Canada.
Pediatr Neurosurg. 1990;16(4-5):232-9. doi: 10.1159/000120533.
Apnea is a known complication of the Chiari II malformation presenting in infancy. Obstructive apnea secondary to bilateral abductor palsy or laryngomalacia and centrally mediated expiratory apnea with cyanosis can occur. Observations of 9 patients suggest that these forms of apnea may represent stages in a continuum of brain stem dysfunction due to the combined effects of the hindbrain malformation and its compression, hydrocephalus and progressive arachnoiditis. Obstructive apnea in some patients may be reversed by optimal control of hydrocephalus with or without cervical decompression. These patients may also develop episodes of cyanotic expiratory apnea of central origin (PEAC). This form of apnea does not respond to surgical or medical treatment and may show progressive worsening over time. Five of 6 patients with this form of apneic spell died suddenly, 2 of these died despite full recuscitative efforts. It is recommended that reports of treatment address results for both forms of apnea.
呼吸暂停是Chiari II型畸形在婴儿期出现时已知的并发症。可发生双侧外展肌麻痹或喉软化继发的阻塞性呼吸暂停以及伴有发绀的中枢性介导的呼气性呼吸暂停。对9例患者的观察表明,这些呼吸暂停形式可能代表由于后脑畸形及其压迫、脑积水和进行性蛛网膜炎的综合作用导致的脑干功能障碍连续体中的阶段。部分患者的阻塞性呼吸暂停可通过对脑积水进行最佳控制(无论是否进行颈椎减压)而得到缓解。这些患者还可能出现中枢性起源的青紫性呼气性呼吸暂停(PEAC)发作。这种呼吸暂停形式对手术或药物治疗均无反应,且可能随时间逐渐恶化。6例出现这种呼吸暂停发作形式的患者中有5例突然死亡,其中2例尽管全力复苏仍死亡。建议治疗报告阐述这两种呼吸暂停形式的治疗结果。