Aggressive bronchoscopic management of plastic bronchitis
Aggressive bronchoscopic management of plastic bronchitis
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DOI：
10.1016/j.ijporl.2010.02.005
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发表时间：
2010-07-01
影响因子：
1.5
通讯作者：
Choi, Sukgi
中科院分区：
文献类型：
--
作者：
Preciado, Diego;Verghese, Susan;Choi, Sukgi
可塑性支气管炎或“可塑性支气管炎”是一种罕见的疾病，其特征是形成厚的、坚韧的、树枝状的粘液性气管支气管管型，导致危及生命的气道阻塞和肺衰竭。我们回顾了三名儿童谁经历了单心室生理学Fontan程序后，复发性可塑性支气管炎。三级转诊儿童医院3例可塑性支气管炎患者的病例系列。所有患者都需要反复进行支气管镜检查，其中一个需要在一周内进行四次单独的支气管镜检查，以去除刚性管型。两名儿童因严重呼吸衰竭需要体外膜肺氧合（ECMO）。所有人都得到了持续的密集医疗支助。所有患儿的肺功能均恢复正常，但2个月后复发，其中1例随后因肺功能衰竭而死亡。可塑性支气管炎是一种不寻常的疾病，原因不明，发生在多个临床环境中，但特别是在那些接受Fontan手术的儿童中。这种令人痛苦的情况的管理是困难的，早期诊断和积极的措施，以消除刚性石膏结合重症医学护理是必要的。Fontan手术儿童的内在心肺生理学，包括心律失常、缺氧和肺动脉高压的风险，使这种情况更加复杂。(C)2010爱思唯尔爱尔兰有限公司版权所有。
Plastic bronchitis or "Bronchitis Plastica" is a rare disease characterized by the formation of thick, tenacious, arborizing mucofibrinous tracheobronchial casts that result in life-threatening airway obstruction and pulmonary failure. We review three children who developed recurrent plastic bronchitis after undergoing a Fontan procedure for single ventricle physiology. Case series of three patients with plastic bronchitis at a tertiary referral children's hospital. All patients required repeated bronchoscopies, one requiring four separate ones over a week's period, for removal of the rigid casts. Extra-corporal membrane oxygenation (ECMO) was needed in two children because of severe respiratory failure. All were also managed with adjunctive intensive medical support. Pulmonary function returned to normal in all children, but recurred 2 months later in one who subsequently expired due to pulmonary failure. Plastic bronchitis is an unusual condition of unknown cause that occurs in multiple clinical settings, but especially in those children who have undergone a Fontan operation. Management of this distressing situation is difficult and early diagnosis and aggressive measures to remove rigid casts combined with intensive medical care are necessary. The intrinsic cardiopulmonary physiology of children with Fontan procedures, including the risk of arrhythmias, hypo-oxygenation, and pulmonary hypertension make this condition even more complex. (C) 2010 Elsevier Ireland Ltd. All rights reserved.