摘要
目的总结窒息性胸廓发育不良(Jeune综合征)的外科治疗经验。方法回顾性分析2018年8月至2020年4月我院收治的15例窒息性胸廓发育不良患者的临床资料,其中男7例、女8例,年龄1~25(8.87±6.71)岁。采用特制钢板纠正病变肋骨与肋软骨生长方向,同时对两侧胸壁凹陷与前胸壁前凸畸形进行矫形,增加胸腔容积,矫正胸廓畸形。结果所有患者术后廓外观发生改变,形状接近正常,缺氧症状得到改善。手术时间(147.73±59.78)min,术中出血量(105.67±91.90)mL,ICU监护时间(14.20±13.54)d,住院时间(26.00±17.87)d。15例患者中,11例术后直接拔除气管插管,4例术后气管切开并行辅助呼吸,辅助呼吸时间分别为19 d、13 d、22 d、12 d。术后胸围明显增大,血氧饱和度明显改善。5例术后出现心功能不全,3例经强心处理得到改善,2例因心功能衰竭分别于术后第2 d和第31 d死亡。术后10例出现腹胀,其中5例较为顽固,经综合治疗消除。术后所有患者均接受随访,CT复查胸廓外观明显改善,肺部无压迫征象。1例13岁患者术后3个月出现呼吸不适,自行吸氧后症状缓解。1例25岁患者出院后半个月出现心功能不全,强心治疗后症状消失。4例患者分别于术后13个月、13个月、15个月、17个月取出钢板,术后胸廓外观保持良好,影像学检查显示骨性结构位置正常,肺野清晰,无慢性炎症征象。结论本方法是一种安全简单的手术方法,不仅可消除胸壁畸形,而且能明显增加胸腔容积,但远期效果尚需进一步评估。
Objective To summarize the experience of surgical treatment of asphyxiating thoracic dysplasia(Jeune syndrome).Methods A total of 15 patients with asphyxiating thoracic dysplasia from August 2018 to April 2020 in our hospital were retrospectively included.There were 7 males and 8 females,aged 1-25(8.87±6.71)years.Special steel bars were used to correct the growth direction of the rib and costal cartilage.Meanwhile,the concave and convex deformities of the chest wall on both sides were corrected to increase the chest volume and correct the thoracic deformity.Results The contour appearance of the chest wall of all patients changed after the operation.The shape was close to normal,and the symptoms of hypoxia were improved.The operation time was 147.73±59.78 min,intraoperative bleeding volume was 105.67±91.90 mL,ICU stay time was 14.20±13.54 d and hospital stay time was 26.00±17.87 d.Eleven patients were directly extubated after the operation,4 patients underwent tracheotomy and received assisted respiration,and the assisted respiration time was 19,13,22 and 12 days,respectively.The postoperative chest circumference was significantly increased,and the blood oxygen saturation was significantly improved.There were 5 patients with cardiac insufficiency,and 3 of them were improved by cardiotonic therapy,2 of them died of heart failure on the 2 nd and 31 st day after the operation,respectively.Abdominal distention occurred in 10 patients after operation,and 5 of them were obstinate and eliminated by comprehensive treatment.All patients were followed up.The appearance of thorax was improved obviously and there was no sign of compression in lungs.One 13-year-old patient developed respiratory discomfort 3 months after the operation,and the symptoms were relieved after self-administration of oxygen.A 25-year-old patient developed cardiac insufficiency half a month after the discharge,and the symptoms disappeared after cardiotonic treatment.Four patients took out the steel bars in 13,13,15 and 17 months after the operatio
作者
王文林
龙伟光
陈春梅
王文杰
刘洋
蔡斌
罗娟
陈凯
WANG Wenlin;LONG Weiguang;CHEN Chunmei;WANG Wenjie;LIU Yang;CAI Bin;LUO Juan;CHEN Kai(Institute of Chest Wall Surgery,The Second People's Hospital of Guangdong Province,Guangzhou,510317,P.R.China)
出处
《中国胸心血管外科临床杂志》
CSCD
北大核心
2021年第8期984-989,共6页
Chinese Journal of Clinical Thoracic and Cardiovascular Surgery
关键词
窒息性胸廓发育不良
手术
治疗
Asphyxiating thoracic dysplasia
operation
treatment
