摘要
目的评估马凡综合征(Marfan syndrome,MFS)患者的气胸发生率及其临床特点,并分析其相关危险因素。方法回顾性收集中国科学技术大学附属第一医院(安徽省立医院)2017年3月1日—2022年12月31日MFS住院患者的病例资料,分析其气胸特点及相关危险因素。结果共纳入53例患者(其中男性29例,55%),中位年龄为32岁(范围13~65岁),所有患者均无气胸家族病史。4例(8%)曾发生1次自发性气胸,均在同侧肺尖部发现肺大疱,其中3例(75%)为单发肺大疱伴有胸廓畸形。在49例既往未发生气胸患者中,9例(18%)有肺大疱,其中6例(67%)位于肺尖部,余3例为中、下肺叶(33%)。此外,6例(12%)伴有胸廓畸形。MFS伴有肺大疱患者的气胸发生率显著高于无肺大疱患者(31%比0%,P=0.002),伴有胸廓畸形患者的气胸发生率显著性高于胸廓正常患者(33%比2%,P=0.013)。4例气胸患者中2例行肺大疱切除术,1例行胸腔闭式引流术,另1例行胸腔穿刺抽气,随访期间均未见气胸复发。既往文献复习纳入国内14篇病例报道中16例MFS患者并发自发性气胸(其中男11例,69%),中位年龄17岁(范围13~28岁)。仅有3例(19%)有气胸家族史,14例(88%)均合并胸廓畸形。5例(31%)气胸患者发现肺大疱,其中4例行胸部CT发现肺大疱位于同侧上叶。7例(44%)患者气胸曾经复发。结论MFS并发自发性气胸的发病率显著增高,多数患者没有气胸家族病史,其发病与危险因素肺大疱(尤其是肺尖部)和胸廓畸形相关,发生气胸后应及时手术干预。
Objective To evaluate the incidence and characteristics of spontaneous pneumothorax in patients with Marfan syndrome(MFS),and to analyze its related factors.Methods The medical record of patients with MFS who were hospitalized in the First Affiliated Hospital of University of Science and Technology of China from March 1,2017 to December 31,2022 were retrospectively collected,to analyze the incidence of pneumothorax and related risk factors.Results A total of 53 patients(including 29 males,55%)were included,with a median age of 32 years(ranging from 13 to 65 years).All patients denied family history of pneumothorax.Four cases(8%)had one episode of spontaneous pneumothorax,with pulmonary bullae involved the ipsilateral apex.Among them,3 cases(75%)had single pulmonary bulla and thoracic deformity.Of the 49 patients without previous pneumothorax history,9(18%)had pulmonary bullae,of which 6(67%)were located at the apex of the lung,and the remaining 3(33%)were in the middle and lower lobes of the lung;In addition,6 cases(12%)were coexisted with thoracic deformity.The incidence of pneumothorax in patients with pulmonary bullae in MFS was significantly higher than those without pulmonary bullae(31%vs.0%,P=0.002),and the incidence of pneumothorax in patients with thoracic deformities was significantly higher than those with normal thorax(33%vs.2%,P=0.013).Of the 4 patients with pneumothorax,2 underwent bullectomy,1 underwent thoracic drainage,and the other underwent thoracic puncture and aspiration procedures,respectively.No recurrence of pneumothorax was observed during the follow-up period.Sixteen cases of MFS with spontaneous pneumothorax(11 males,69%)were reported in Chinese literature,with a median age of 17 years(ranging from 13 to 28 years).Among them,3 cases(19%)had a family history of pneumothorax,and 14 cases(88%)had thoracic deformities.Bullae was found in all 5 patients with pneumothorax,4 of them with chest CT images involved the ipsilateral upper lobe.Forty-four percents of 16 patients had pneumothorax recurrence.Conclusions The incidence of MFS complicated with spontaneous pneumothorax is significantly higher.However,most of them have no family history of pneumothorax.The incidence of pneumothorax with MFS is associated with pulmonary bullae(especially the apex of lung)and thoracic deformity.Timely surgical intervention should be carried out when pneumothorax happened.
作者
汪禹烁
刘婷
纪子梅
许实成
李建安
胡晓文
WANG Yushuo;LIU Ting;JI Zimei;XU Shicheng;LI Jian'an;HU Xiaowen(Department of Pulmonary and Critical Care Medicine,the First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei,Anhui 230001,P.R.China;Wannan Medical College,Wuhu,Anhui 241002,P.R.China;Department of Medical Imaging,the First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei,Anhui 230001,P.R.China;Department of Cardiovascular Surgery,the First Affiliated Hospital of USTC,Division of Life Sciences and Medicine,University of Science and Technology of China,Hefei,Anhui 230001,P.R.China)
出处
《中国呼吸与危重监护杂志》
CAS
CSCD
北大核心
2023年第4期256-261,共6页
Chinese Journal of Respiratory and Critical Care Medicine
基金
安徽省重点专科建设项目(2021szdzk05)。
关键词
马凡综合征
气胸
肺大疱
胸廓畸形
Marfan syndrome
pneumothorax
bullae
thoracic deformity
