Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia.
Polysomnography as an indicator for cervicomedullary decompression to treat foramen magnum stenosis in achondroplasia.
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多导睡眠图作为颈髓减压治疗软骨发育不全枕骨大孔狭窄的指标。
DOI：
10.1007/s00381-018-3880-0
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发表时间：
2018
期刊：
影响因子：
--
通讯作者：
Fujii Y.
中科院分区：
文献类型：
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作者：
Sano M;Takahashi N;Nagasaki K;Oishi M;Yoshimura J;Fujii Y.
目的软骨发育不全所致枕骨大孔狭窄导致颈髓受压的治疗仍存在争议，特别是对于无症状或症状轻微的患者。我们检查了多导睡眠图（PSG）作为颈髓减压治疗指标的有效性。方法我们回顾性分析了2008年至2015年治疗的9例软骨发育不全病例（平均年龄1岁9个月）。所有患者均接受PSG、磁共振成像（MRI）和耳鼻喉纤维镜检查。我们分析了人口统计数据、临床表现、呼吸障碍的程度和类型、枕骨大孔狭窄的严重程度和伴随的颈髓压迫、治疗（保守或手术）和临床结果。结果 9 名患者中有 8 名在白天没有出现严重症状。然而，MRI 显示 4 例重度、4 例中度和 1 例轻度颈髓受压，PSG 显示 4 例重度睡眠呼吸暂停，5 例中度睡眠呼吸暂停。所有睡眠呼吸暂停病例均为阻塞性或阻塞性为主。纤维镜检查未发现上气道狭窄 6 例，轻度狭窄 3 例。 4 例 PSG 出现严重睡眠相关呼吸障碍且颈髓重度或中度受压的患者接受颈髓减压术治疗。其中3名患者术后很快就出现睡眠呼吸改善，1名患者因气管插管时受压导致双侧声带麻痹而需要临时气管切开术。结论多导睡眠图可以作为颈髓减压手术的有用指标，特别是对于看似无症状的软骨发育不全伴枕骨大孔严重狭窄的病例。
ObjectiveManagement of cervicomedullary compression due to foramen magnum stenosis in achondroplasia remains controversial, especially for patients with no symptoms or mild symptoms. We examined the effectiveness of polysomnography (PSG) as an indicator for cervicomedullary decompression treatment.MethodsWe retrospectively reviewed nine achondroplasia cases (mean age 1 year and 9 months) treated from 2008 to 2015. All patients were examined by PSG, magnetic resonance imaging (MRI), and otolaryngeal fibroscopy. We analyzed demographic data, clinical presentation, degree and type of respiratory impairment, severity of foramen magnum stenosis and concomitant cervicomedullary compression, treatment (conservative or surgical), and clinical outcome.ResultsEight of nine patients presented with no severe symptoms in the daytime. However, MRI revealed four severe, four moderate, and one mild case of cervicomedullary compression, and PSG demonstrated severe sleep apnea in four cases and moderate sleep apnea in five cases. All sleep apnea cases were obstructive or obstructive-dominant. Fibroscopy revealed no upper airway stenosis in six cases and mild stenosis in three cases. Four patients who had severe sleep-related respiratory disturbance on PSG and severe or moderate cervicomedullary compression were treated by cervicomedullary decompression. Three of these patients demonstrated improved sleep respiration soon after surgery, while one required temporary tracheostomy due to bilateral vocal cord paralysis caused by compression during intratracheal intubation.ConclusionPolysomnography can be a useful indicator for cervicomedullary decompression surgery, especially in cases of seemingly asymptomatic achondroplasia with severe foramen magnum stenosis.