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寰枢椎融合术患儿行全身麻醉的挑战:患儿患有弓型手畸形综合征和扩张型心肌病。

Challenges of anaesthetising a child with Bow Hunter's syndrome and dilated cardiomyopathy for occipitocervical fusion.

机构信息

Department of Anaesthesia, Christian Medical College, Vellore, Tamilnadu, India.

Department of Neuroanaesthesia, Christian Medical College, Vellore, Tamil Nadu, India

出版信息

BMJ Case Rep. 2023 May 2;16(5):e253834. doi: 10.1136/bcr-2022-253834.

Abstract

Paediatric Bow Hunter's syndrome (BHS), or rotational vertebral artery syndrome, is a rare cause of posterior circulation insufficiency in children. It results from mechanical obstruction of the vertebral artery by the transverse process of cervical vertebrae resulting in vertebrobasilar insufficiency during the neck rotation to the sides. Paediatric dilated cardiomyopathy (DCM) is a rare myocardial disease that presents with ventricular dilatation and cardiac dysfunction. This case report describes the successful anaesthetic management of an boy with BHS due to atlantoaxial dislocation and DCM. The child was anaesthetised by keeping the following anaesthetic goals in mind such as maintenance of the heart rate, rhythm, preload, afterload and contractility close to the baseline for both DCM and BHS. Haemodynamic management with optimal fluids, inotrope and a vasopressor and titrating its volume and doses using multimodal haemodynamic monitoring while keeping both cardio and neuroprotective strategies, and the multimodal analgesia techniques helped the child for faster recovery.

摘要

儿童弓型Hunter 综合征(BHS)或旋转椎动脉综合征是儿童后循环缺血的罕见原因。它是由于颈椎横突对椎动脉的机械性阻塞,导致在颈部向两侧旋转时出现椎基底动脉供血不足。儿童扩张型心肌病(DCM)是一种罕见的心肌疾病,表现为心室扩张和心功能障碍。本病例报告描述了一名患有寰枢椎半脱位和 DCM 的男孩成功的麻醉管理。该患儿在麻醉时牢记以下麻醉目标,如维持心率、节律、前负荷、后负荷和收缩力接近 DCM 和 BHS 的基线。通过使用多模式血流动力学监测来优化液体、正性肌力药和血管加压药的使用,并滴定其容量和剂量,同时保持心脏和神经保护策略,以及多模式镇痛技术,有助于患儿更快恢复。

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本文引用的文献

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Bow Hunter's Syndrome: A rare cause of vertebrobasilar insufficiency.弓状猎人综合征:椎基底动脉供血不足的罕见病因。
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An unusual case of pediatric bow hunter's stroke.一例罕见的儿童弓猎者卒中病例。
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