Bhangoo Ranjeev, Sgouros Spyros, Walsh A Richard, Clarke Jane R
Department of Neurosurgery, Birmingham Children's Hospital, Steelhouse Lane, Birmingham, B4 6NH, UK.
Childs Nerv Syst. 2006 Feb;22(2):113-6. doi: 10.1007/s00381-005-1252-z. Epub 2005 Sep 24.
In the absence of syringobulbia, hindbrain hernia does not commonly cause permanent respiratory complications. We present two patients who developed permanent central nocturnal hypoventilation following acute deterioration of hindbrain-hernia-related syringomyelia despite successful surgery.
Two children (one boy and one girl, aged 7 and 13 years, respectively) presented with acute neurological deterioration. The boy presented with a 6-week history of progressive tiredness and weakness and a short history of respiratory depression. The girl presented with a 2-year history of left hemiparesis and a short history of left hemiplegia and respiratory depression. On magnetic resonance scan, both had hindbrain hernia, hydrocephalus and cervical syringomyelia. Following cranio-vertebral decompression and, later, a ventriculoperitoneal shunt, the syringomyelia remained well controlled in both patients. In contrast, their respiratory depression improved minimally. Repeated multichannel respiratory monitoring revealed a persistently slow sleeping respiratory rate, with nocturnal hypercapnia and hypoxemia, managed successfully by night-time non-invasive ventilation. Neither the boy nor the girl, after 7 and 4 years, respectively, has developed any complications of nocturnal hypoventilation. When awake, both breathe normally.
Both children appear to have suffered a permanent impairment of respiratory control, resulting in nocturnal hypoventilation. This is usually seen with brain stem lesions and is unexpected when syringomyelia does not extend above C2, in the absence of syringobulbia.
Central nocturnal hypoventilation is postulated to be due to permanent damage of central brain stem pathways controlling the respiratory muscles, due to local pressure at the cranio-cervical junction associated with acute deterioration of the hindbrain herniation.
Sudden deterioration of hindbrain hernia and cervical syringomyelia can rarely impair the central mechanisms of respiratory control, with long-term implications on quality of life.
在没有延髓空洞症的情况下，后脑疝通常不会导致永久性呼吸并发症。我们报告了两名患者，尽管手术成功，但在后脑疝相关脊髓空洞症急性恶化后出现了永久性中枢性夜间通气不足。
两名儿童（一名男孩和一名女孩，分别为7岁和13岁）出现急性神经功能恶化。男孩有6周进行性疲劳和虚弱病史，近期有呼吸抑制史。女孩有2年左侧偏瘫病史，近期有左侧偏瘫和呼吸抑制史。磁共振扫描显示，两人均有后脑疝、脑积水和颈段脊髓空洞症。在进行颅颈减压，随后进行脑室腹腔分流术后，两名患者的脊髓空洞症均得到良好控制。相比之下，他们的呼吸抑制改善甚微。重复的多通道呼吸监测显示睡眠呼吸频率持续缓慢，伴有夜间高碳酸血症和低氧血症，通过夜间无创通气成功处理。分别在7年和4年后，男孩和女孩均未出现夜间通气不足的任何并发症。清醒时，两人呼吸均正常。
两名儿童似乎都遭受了呼吸控制的永久性损害，导致夜间通气不足。这通常见于脑干病变，在没有延髓空洞症且脊髓空洞症未延伸至C2以上时是出乎意料的。
中枢性夜间通气不足被推测是由于控制呼吸肌的脑干中枢通路受到永久性损伤，这是由于颅颈交界处与后脑疝急性恶化相关的局部压力所致。
后脑疝和颈段脊髓空洞症的突然恶化很少会损害呼吸控制的中枢机制，对生活质量有长期影响。
