Department of Anesthesiology, Tohoku University School of Medicine, 1-1 Seiryoumachi, Aoba-ku, Sendai, Miyagi, Japan.
J Anesth. 2012 Dec;26(6):922-4. doi: 10.1007/s00540-012-1451-1. Epub 2012 Jul 12.
We encountered a 2-year-old female infant with congenital central hypoventilation syndrome (CCHS) who underwent an abdominal operation for strangulated ileus. Prior to the surgery, at home, the infant had been receiving non-invasive positive-pressure ventilation (NPPV) support only during sleep. However, after postoperative extubation, the blood oxygen saturation (SpO(2)) decreased to approximately 90 % with NPPV during sleep alone, necessitating the use of biphasic cuirass ventilation (BCV) along with NPPV for 2 days. The infant was weaned from the BCV on hospital day 9, and was discharged from the intensive care unit (ICU) on hospital day 13. Although it has been said that CCHS is not under the control of the respiratory center, there are no reports of the true CO(2) response curves in these patients. Therefore, during respiratory management in the ICU post-surgery, we examined (with the consent of the mother) the relationship of the end-tidal carbon dioxide (ETCO(2)) to the tidal volume and respiratory rate, for a period of 6 min in the absence of sedation, using a respiratory profile monitor. Electrocardiographic and SpO(2) monitoring was also conducted at the same time, to ensure the patient's safety. In this patient, while the ETCO(2) increased, the tidal volume and respiratory rate remained unchanged. No relationship was found between the tidal volume and the respiratory rate. Various modalities have been used for the treatment of CCHS (tracheotomy, NPPV, and diaphragmatic pacing). Treatment of these patients in the ICU should be tailored to the needs of individual patients and their families.
我们遇到了一名患有先天性中枢性低通气综合征（CCHS）的 2 岁女性婴儿，她因绞窄性肠梗阻接受了腹部手术。在手术前，在家中，婴儿仅在睡眠时接受无创正压通气（NPPV）支持。然而，手术后拔管后，婴儿在睡眠时单独使用 NPPV 时血氧饱和度（SpO2）降至约 90%，需要使用双相胸甲通气（BCV）和 NPPV 治疗 2 天。婴儿在住院第 9 天从 BCV 脱机，在住院第 13 天从重症监护病房（ICU）出院。虽然有人说 CCHS 不受呼吸中枢控制，但这些患者的真正 CO2 反应曲线尚无报道。因此，在手术后 ICU 的呼吸管理中，我们（在母亲同意的情况下）使用呼吸监测仪在没有镇静的情况下检查了 6 分钟内呼气末二氧化碳（ETCO2）与潮气量和呼吸率的关系。同时还进行了心电图和 SpO2 监测，以确保患者的安全。在该患者中，随着 ETCO2 的增加，潮气量和呼吸率保持不变。未发现潮气量与呼吸率之间存在关系。已经使用了各种方法来治疗 CCHS（气管切开术、NPPV 和膈肌起搏）。在 ICU 中治疗这些患者应根据患者及其家属的需求进行调整。
