·临床诊疗提示·

肺隔离症患者53例临床诊疗分析

苏娜1,姜鲁宁2*,蒋胜华2,宋敏1

  基金项目:山东省医药卫生科技发展计划项目(2016WS0176)

1.272100山东省济宁市,济宁医学院

2.272100山东省济宁市,济宁医学院附属医院呼吸内科

*通信作者:姜鲁宁,教授;E-mail:18454727155@163.com

  【摘要】 背景 肺隔离症是一种罕见的先天性肺发育异常疾病,临床误诊率高。目的 总结肺隔离症的临床特点、影像学表现、诊断以及治疗体会,以降低误诊率。方法 收集2008-01-01至2016-10-01济宁医学院附属医院收治并确诊的53例肺隔离症患者的临床资料,对其临床特点、影像学表现、诊断、治疗以及预后进行分析。结果 53例患者中,临床表现为咳嗽37例、咯血23例、咳痰20例、发热12例、胸闷8例、胸痛6例、无明显症状者9例。病变位于左下肺34例、右下肺17例、左上肺2例。影像学表现为实性肿块影19例,囊状影16例,不规则斑片状影12例,空洞4例,肺不张1例,胸腔积液1例。隔离肺的供应血管34例来源于胸降主动脉,17例来源于腹主动脉,1例来源于胸主动脉和膈肌动脉,1例来源于腹主动脉和左膈上动脉。53例患者确诊30例,23例出现误诊,误诊率为43.4%;48例行胸腔镜下肺叶切除术,术后继发肺部感染1例,2例咯血患者行异常动脉介入栓塞治疗,余3例行对症治疗。所有手术患者未再复发。结论 肺隔离症临床表现无特异性,误诊率高,增强CT和CT血管造影(CTA)是临床常用的诊断方法,确诊后应积极手术,胸腔镜下肺叶切除术是临床最有效的治疗方法。

  【关键词】 支气管肺隔离症;体征和症状;诊断;治疗

  【中图分类号】R 562.2 【文献标识码】A DOI:10.3969/j.issn.1007-9572.2017.00.181

  苏娜,姜鲁宁,蒋胜华,等.肺隔离症患者53例临床诊疗分析[J].中国全科医学,2017,20(36):4567-4570.www.chinagp.net]

  SU N,JIANG L N,JIANG S H,et al.Pulmonary sequestration:a review of the diagnosis and treatment of 53 cases[J].Chinese General Practice,2017,20(36):4567-4570.

 

Pulmonary Sequestration:a Review of the Diagnosis and Treatment of 53 Cases

 SU Na1,JIANG Lu-ning2*,JIANG Sheng-hua2,SONG min1

1.Jining Medical University,Jining 272100,China

2.Department of Respiratory Medicine,Affiliated Hospital of Jining Medical University,Jining 272100,China

*Corresponding author:JIANG Lu-ning,Professor;E-mail:18454727155@163.com

 

  Abstract】 Background Pulmonary sequestration is a rare congenital pulmonary malformation with high rate of misdiagnosis.

Objective To summarize the clinical features,imaging findings,diagnosis and treatment of pulmonary sequestration,in order to decrease the misdiagnosis rate of this disease.

Methods We collected the clinical data of 53 pulmonary sequestration patients who were diagnosed and treated in Affiliated Hospital of Jining Medical University from January 1,2008 to October 1,2016 and analyzed their clinical features,imaging findings,diagnosis,treatment and prognosis.

Results The clinical manifestations of these patients were cough(37 cases),hemoptysis(23 cases),expectoration(20 cases),fever(12 cases),chest tightness(8 cases),chest pain(6 cases),non-obvious symptoms(9 cases).The lesions were located in the left lower lung in 34 cases,in the right lower lung in 17 cases,and in the left upper lung in 2 cases.Imaging showed solid mass in 19 cases,cystic shadow in 16 cases,irregular patchy shadow in 12 cases,cavity in 4 cases,atelectasis in 1 case,pleural effusion in 1 case.The anomalous arteries supplying blood to bronchopulmonary sequestration were found from descending thoracic aorta in 34 cases,from abdominal aorta in 17 cases,from thoracic aorta and diaphragm artery in 1 case and from abdominal aorta and left superior phrenic artery in 1 case.Thirty cases were diagnosed confirmedly but 23 cases were misdiagnosed,the misdiagnosis rate was 43.4%.Three cases received symptomatic treatment,the other 48 cases received thoracoscopic lobectomy,of whom 1 had postoperative lung infection,and 2 received abnormal arterial embolization due to hemoptysis.No recurrence was found in all surgical patients.

Conclusion Pulmonary sequestration has high misdiagnose rate due to its atypical clinical manifestations.Enhanced CT and CTA are frequently used to help the diagnosis.When the patient is diagnosed confirmedly,surgery should be performed as early as possible.Thoracoscopic lobectomy is the most effective surgical treatment for this disease.

  Key words】 Bronchopulmonary sequestration;Signs and symptoms;Diagnosis;Therapy

 

  

肺隔离症是一种少见的先天性肺发育异常疾病,是胚胎发育缺陷导致的血管异常,表现为异常的肺组织与正常的支气管、肺血管分离,肺组织由体循环供血[1],其在先天性肺畸形中占0.15%~6.40%[2]。肺隔离症的临床表现为咳嗽、咳痰、咯血、胸痛等,易被误诊为肺炎、支气管扩张等疾病,误诊率较高,病情常迁延不愈。本文通过分析53例肺隔离症患者的临床资料,总结肺隔离症的临床特点、诊断方法以及治疗体会,以降低误诊率。

资料与方法

1.1 一般资料 收集2008-01-01至2016-10-01济宁医学院附属医院收治并确诊的53例肺隔离症患者的临床资料,其中男34例,女19例;年龄27~75岁,中位年龄47.9岁。

1.2 方法 CT检查或病理检查发现肺部病灶来自体循环供血为诊断标准。收集并总结53例患者的临床特点、影像学表现、诊断方法、治疗以及预后。

结果

2.1 临床特点 临床症状:咳嗽37例、咯血23例、咳痰20例、发热12例、胸闷8例、胸痛6例、无明显症状因体检发现9例。2例并发真菌感染。病变部位:左下肺34例、右下肺17例、左上肺2例。病程2 d~7年。

2.2 影像学表现以及血供来源 胸部CT示:实性肿块影19例,密度均匀,边缘光滑;囊状影16例,囊腔单个或多个,其中1例伴有液平;不规则斑片状影12例,可见大小不等的低密度区域;空洞4例,其中2例伴有液平(见图1A);肺不张1例(见图1B、C);胸腔积液1例。隔离肺的血供来源:34例来源于胸降主动脉,17例来源于腹主动脉,1例来源于胸主动脉和膈肌动脉,1例来源于腹主动脉和左膈上动脉。

2.3 术前诊断及误诊 所有患者行常规CT平扫检查,其中4例患者发现异常体循环血供(见图1D、E),确诊为肺隔离症;26例不排除肺隔离症,与家属商议后行进一步检查,20例经增强CT确诊(见图1F),5例经CT血管造影(CTA)确诊,1例孕妇经MRI确诊;23例患者CT平扫未提及肺隔离症且未进行进一步检查,误诊为支气管扩张并感染8例、肺囊肿6例、肺占位4例、肺炎3例、肺结核1例、肺囊肿1例,最终经手术确诊。

2.4 治疗及预后 53例患者中,48例行电视胸腔镜辅助手术(VATS)切除病变肺叶(见图1G、H),术中均发现异常供血动脉;其中1例增强CT发现异常血管1支,术中发现供血动脉2支;术后出现肺部感染1例。5例行保守治疗,其中2例咯血患者行异常动脉介入栓塞治疗,1例胸腔积液患者行胸腔闭式引流及抗感染治疗,病情好转后出院,2例肺炎患者经抗感染治疗后出院。对所有患者进行随访,随访时间1个月~8年,手术患者均未复发,异常血管栓塞患者未再出血,3例保守治疗者出现复发,其中2例再住院两次,1例再住院3次。

3 讨论

肺隔离症是由先天性肺发育畸形所致,胚胎期前肠和毛细血管与背主动脉相通,因发育障碍导致血管无法闭锁[3],该血管牵引部分肺组织与肺主体分离,独立发育,称为隔离肺,这就是目前受到普遍认可的牵引学说[4]。隔离肺由主动脉分支供血,其含氧量低于肺动脉,因此并无正常的肺功能。隔离肺可与正常支气管相通,也可不通,相通者容易出现反复感染。解剖学以隔离肺和正常肺组织是否被同一个胸膜包裹为标准[5],分为叶内型肺隔离症和叶外型肺隔离症。临床上叶内型肺隔离症较叶外型肺隔离症常见,叶内型隔离肺与正常支气管相通,出现症状早,病灶易出现反复感染,患者多以咳嗽、咳痰、发热等感染症状就诊,临床易诊断;叶外型肺隔离症与正常支气管不相通,常无明显症状,临床不易发现。本研究手术证实51例为叶内型肺隔离症,仅有2例为叶外型肺隔离症。

肺隔离症好发于青年男性,好发年龄为10~40岁,病变主要位于左下肺[6]。LEE等[7]报道540例肺隔离症患者中只有2%位于上叶,而本研究中仅有2例位于左上叶。本研究患者中男34例,女19例;年龄27~75岁;临床表现多样,无明显症状因体检发现9例。多数患者以肺部感染症状就诊,CT平扫仅起提示作用,尚不能确诊,临床易误诊。本研究中23例误诊,误诊率为43.4%,分析其主要的误诊原因如下:(1)临床表现缺乏特异性,肺隔离症患者常表现为反复感染,不易与肺炎、肺脓肿、支气管扩张等疾病鉴别。(2)本病没有受到临床医生的足够重视,呼吸内科和胸外科医生对本病了解较多,其他科的医生可能很少听说过,因此,诊断疾病时易忽略此病。(3)CT检查表现多样,有的呈囊性改变,可以有液平,易误诊为肺脓肿、肺囊肿等;有的呈团状影,易误诊为肿瘤;有的表现为空洞,易误诊为结核;个别病例表现为蜂窝状阴影,易误诊为支气管扩张症。此外,相当一部分患者症状反复加重、迁延不愈,发展为肺实变、机化、炎性假瘤等疾病[8],掩盖了原疾病的特点,误诊率高,因此当患者肺下叶反复出现感染症状时,临床工作者应该考虑此病,降低误诊率。

 

肺隔离症临床表现无特异性,影像学检查是术前诊断的关键,发现隔离肺组织的异常供血动脉是诊断标准[9]。选择性血管数字减影(DSA)一直被认为是诊断肺隔离症的金标准[10],但由于其为有创检查,费用高,仅能显影血管,不能看到肺内病变,限制了其在临床的应用。增强CT和CTA可以显示异常供血动脉的数目、来源和走形,同时显示肺部病灶;增强CT还可显示病灶是否强化,可与肺部肿瘤进行鉴别,目前已取代CTA成为诊断肺隔离症的首选检查方法[11];胸部MRI不需要造影剂,对检查者无辐射,但其检查时间长,价格稍高,肺内气体会产生伪影,检查效果劣于增强CT,但MRI是妊娠患者的理想检查方法。本研究经平扫CT确诊4例,经增强CT确诊20例,经CTA确诊5例经,MRI确诊1例,余23例未行进一步检查而误诊。综上,对于反复迁延不愈的肺部感染患者,应考虑到肺隔离症的可能,进一步行增强CT或CTA以确诊。

肺隔离症患者多以肺部感染症状就诊,抗感染治疗有效,

 

本文创新点:

 

收集了较大样本的病例资料,从临床特点、影像学表现、确诊方法、误诊原因、治疗手段以及预后等方面进行了全面的统计和详细的分析,加深了临床工作者对该病的认识。

 

可暂时减轻症状,但感染易反复发作,迁延不愈,随着年龄的增长,肺隔离症患者的病情会逐渐加重,容易出现并发症,如气胸[12]、 胸腔粘连[13]、癌变[14]、支气管扩张、真菌感染等,晚期供血动脉血液分流加压可导致心力衰竭,异常血管供血来源于体循环,压力高,易发生粥样硬化[15],出现并发症不仅使治疗困难增加,也明显降低了治疗效果。本研究2例并发真菌感染,保守治疗效果不佳,因此,肺隔离症一经确诊,应进行积极的手术治疗。肺隔离症的手术治疗主要包括肺叶切除(叶内型肺隔离症)和单纯肿块切除(叶外型肺隔离症)。VATS切除肺叶为临床治疗肺隔离症常用的手术方式,与传统开胸手术相比,具有创伤小、恢复快、住院时间短等优势[16]。KARAKAS等[17]研究发现VATS和开胸术的手术时间和病死率无明显差异,VATS后并发症发生率(31%)较开胸术(49%)低,对于肾衰竭、肺不张、严重贫血等危重患者,VATS更具有优势。手术的关键是处理异常血管,由于反复感染、渗出,病灶易与周围组织粘连,分离血管时难度增加,异常血管常退化变性使管壁弹性降低[18-19],盲目钳夹容易损伤血管,因血液来源于体循环,压力高,血管损伤后容易出现大出血,因此处理血管时一定要小心谨慎。异常血管主要来自于胸主动脉、腹主动脉,其次为肋间动脉、锁骨下动脉等[20],本研究34例来源于胸降主动脉,17例来源于腹主动脉,1例来源于胸主动脉和膈肌动脉,1例来源于腹主动脉和左膈上动脉。血液主要回流至肺静脉,其次为奇静脉、半奇静脉、上腔静脉[21]。供血动脉最多可达5条[22],本研究中有2例供血动脉为2条,余为1条,且1例术中所见与影像学表现不一致,增强CT见1条异常血管,术中发现2条,因此即使术中找到异常血管的数量与影像学表现一致,也要继续仔细寻找,以防漏掉异常血管。对于以咯血为主要症状而无法手术的患者,可行异常供血血管介入栓塞治疗,通过栓塞供血动脉而使病灶缺血、萎缩和机化,此种方法目前临床应用不多,远期疗效有待进一步观察。本研究48例患者行VATS肺叶切除术后并发肺部感染1例,术后所有患者未再复发,2例咯血患者行介入栓塞治疗,此后未再出血,余3例经对症治疗后好转出院。

肺隔离症临床表现无特异性,影像学检查发现异常供血动脉为术前诊断的关键,对于有反复发作的肺部慢性感染病史或疑诊为肺脓肿、支气管扩张等治疗后效果不佳者,应及时完善胸部增强CT或CTA来明确诊断,一旦确诊,应积极进行手术治疗。

作者贡献:苏娜进行研究课题设计、临床资料收集和分析,撰写论文并对文章负责;蒋胜华、宋敏进行课题评估、相关课题资料收集;姜鲁宁负责质量控制与审校。

本文无利益冲突。

 

本研究不足:

1)对患者治疗后的随访时间长短不一,不能更好地评估手术的长期效果;(2)本文未能提供手术患者异常血供的病理图片,无法使临床工作者更直观地认识此病。

 

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(收稿日期:2017-06-14;修回日期:2017-11-10)

(本文编辑:崔莎)