肺隔离症的诊断与治疗
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摘要
目的:肺隔离症是一种较少见的先天性肺发育异常,目前临床上误诊率仍较高。本文对肺隔离症的发生与分型、临床诊断、治疗以及预后进行概括性的综述,探讨肺隔离症的诊疗方法,以期减少误诊率,提高外科治疗水平。
     方法:回顾性分析我院2003年8月~2009年8月手术治疗12例肺隔离症患者的临床资料。
     结果:2003年8月~2009年8月手术治疗肺隔离症12例,占我院同期行肺叶切除术治疗的所有肺良恶性病变的0.85%(12/1411)。本组病例男性4例,女性8例,平均年龄40岁。其中5例见异常血管进入病灶诊断为肺隔离症,其余误诊为肺囊肿合并感染、支气管扩张症、肺脓肿或肺癌。左侧8例,右侧4例,其中呈蜂窝状囊性改变的有6例,密度不均匀软组织密度块4例,密度均匀软组织密度块2例。全部为叶内型,均行病变肺叶切除术,有1例出现术中大出血,其余患者手术顺利,无术后并发症,无围手术期死亡。
     结论:肺隔离症是一种少见的先天性肺发育异常,术前误诊率较高,一些影像学特征和临床表现可提示肺隔离症的诊断,一旦确诊,应采取手术治疗。提高术前诊断率及肺下叶切除术中想到可能存在肺隔离症,是预防术中大出血的关键。手术治疗肺隔离症效果满意。
Objective:Pulmonary sequestration is a rare congenital malformation of lung and its misdiagnosis rate is extremely high. It is of great significance to make a positive diagnosis before operation.
     Methods:14 patients of pulmonary sequestration from August 2003 to August 2009 were retrospectively studied. Their clinical data were analyzed.
     Result:Between August 2003 and August 2009,12 patients with pulmonary sequestration were operated.5 patients were diagnosed preoperatively which showed an abnormal feeding artery. Others were misdiagnosed as cyst of lung,bronchiectasis, lung cancer or pulmonary abscess. It was diagnosed as intralobar in all cases at surgery.8 patients had lesions in left lower lung and 4 patient in right lower lung.1 patient got massive hemorrhage in surgical operation Success of pulmonary lobectomy had been achieved in all cases without any death during the operation or postoperative complication.
     Conclusions:Pulmonary sequestration is a rare congenital malformation of lung and its misdiagnosis rate is extremely high. Some radiological features and clinical manifestations are sufficiently suggestive of diagnosis of pulmonary sequestration. Once the diagnosis is conformed, operation should be performed. The key to prevent massive hemorrhage in treatment is to improve preoperative diagnosis rate and pay attention to pulmonary sequestration when resect the lower lung. Surgical treatment can obtain excellent results.
引文
1. Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lung:a report of seven cases. J Patho,1946,58(3):457-467.
    2. Halkic N, Cuenoud PF, Corthesy ME, et al. Pulmonary sequestration:a review of 26 cases. Eur J Cardiothorac Surg,1998,14(2):127-133.
    3. Ferretti GR, Jouvan FB, Coulomb M. MDCT demonstration of intralobar pulmonary sequestration of the right upper lobe in an adult. AJR Am J Roentgenol,2005,185(6):1663-1664.
    4. Sersar SI, Diasty M, Ibrahim HR,et al. Lower lobe segment and pulmonary sequestration. J Thorac Cardiovase Surg,2004,127(3):898-899.
    5. Shibli M, Connery C, Shapiro JM. Intralobar and extralobar bronchopulmonary sequestration complicated by nocardia asteroides infection. Southern Med J,2003,96(1):78-90.
    6. AlbrenchtsenD. Pulmonary sequestration:A case of an intermediate link between extra-and intralobar sequestration and its haemodynamics. Scand J Thorac Cardiovasc Surg.1974,8(1):64-66.
    7.黄孝迈.现代胸外科学.北京:人民军医出版社,1991:80.
    8. Nicolette LA, Kosloske AM, Bartow SA, Murphy S. Intralobar pulmonary sequestration:a clinical and pathological spectrum. J Pediatr Surg. 1993,28(6):802-805.
    9. 钱可宝,向旭东,段林灿,等.肺隔离症的诊断和外科治疗.临床误诊误治,2009,(04):28:29.
    10.何建行,杨运有.730例胸腔镜手术临床应用研究.广东医学,1998,19(11):826-828.
    11. Juettner FM, Pinter HH, Friehs GB. Bronchial carcinoid arising in intralobar bronchopulmonary sequestration with vascular supply from the left gastric artery. J Thorac Cardiovasc Surg.1985,90(1):25-28.
    12. Peter BK, Didier S, Sven H, et al. Thoracoscopic treatment of pulmonary sequestation. European Journal of CardioThoracic Surgery,2006,29(5): 815-818.
    13. Savic B, Birtel FJ, Tholen W, et al. Lung sequestration:report of seven cases and review of 540 published cases. Thorax,1979,34(1):96-101.
    14. Zylak CJ, Eyler WR, Spizarny DL, et al. Developmental lung anomalies in the adult:radiologic pathologic correlation. RadioGraphics,2002,22(3):25-43.
    15. Thomas HB, Thomas BF. Congenital lesions of the lung and emphysema (pulmonary sequestration) in:David CS, Frank CS. Gibbon's surgery of the chest. Third Edition. Philadelphia:W. B. Saunders company,1976,6(1):262-291.
    16. Carter R. Pulmonary sequestration. Ann Thorac Surg,1969,7(1):68-88
    17. Smith RA. A theory of the origin of intralabar sequestration of lung.Thorax,1956, 11(3):102-110.
    18. Epping H, Shorenstein W. Krankheitender Lungen [inGerman].Ergeb Allg Pathol, 1902,8(1):267-285.
    19.张熙曾,吴德泰,白人华.肺隔离症(附2例报告).天津医药肿瘤学附刊,1982,9(1):49-52.
    20.胡为伟,赵松.肺隔离症的外科诊疗.中国现代医学杂志,2004,4(12):135-136.
    21.陈灏珠.实用内科学.下册.12版.北京:人民卫生出版社,2006:1785.
    22. Cockayne EA,Gladstone RJ. Acaseof accessory lungs associated with hernia through acongenital defect of the diaphragm. J Anat 1947,52(1):64-96.
    23. Bolck F.Zur Frageder Entstechungvon Nebenlungen [inGerman]. Virchows Arch 1950,319(1):20-43.
    24. Cole FH,Alley FH,Jones RS. Aberrant systemic arteries to the lower lung. Surg Gynecol Obstet 1951,9(3):589-596.
    25. Boyden EA.Bronchogenic cysts and the theory of intralobar sequestrations. Jthorac Cardiovasc Surg 1958; 35(5):604-616.
    26. Gebauer PW, Mason CB. Intralobar pulmonary sequestration associated with anomalous pulmonary vessels:anonentity. DisChest 1959,30(12):282-288.
    27. Kyllonen KE. Intralobar pulmonary sequestration and a theory as to it saetiology. Acta ChirScand 1964,12(7):307-310.
    28. Blesovsky A. Pulmonary sequestration:a report of an unusual case and review of the literature. Thorax 1967,22(1):351-357.
    29. Gerle RD, Jaretzki AM, Ashley CA, Berne AS. Congenital bronchopulmonary foregut malformation:pulmonary sequestration communicating with the gastrointestinal tract. Nengl J Med 1968,278(5):1413-1419.
    30. Moscarella AA,Wyllie RH. Congenital communication between the esophagus and isolate dectopic pulmonary tissue. J thorac Caridovasc Surg 1968; 5(5):672-676.
    31. Stocker JT, Malczak HT. A study of pulmonary ligament arteries:relationship to intralobar sequestration. Chest 1984.8(6):611-615.
    32.李树本,何建行,杨运有,等.叶内型肺隔离症的诊断与外科治疗.中华心血管外科杂志,2006,22(4):271-272
    33. DeParedes CG, Pierce WS, Johnson DG. Pulmonary sequestration in infants and children:a 20-year experience and review of the literature. J Paediatr Surg 1970, 5(1):136-147.
    34. Sabiston S,著.石应康,译.胸外科学6版.北京:人民卫生出版社,2000:581-583.
    35. Curtis MR, Mooney DP, Vaccaro TJ et al. Prenatal ultrasound characterization of the suprarenal mass:distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med 1997,16(6):75-83.
    36. White J, Chan Y F, Neuberger S, Wilson T. Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration:report of three cases and literature review. Prenat Diagn 1994,14(1):653-658.
    37. Chan YF, Oldfield R, Vogel S, Ferguson S. Pulmonary sequestration presenting as a prenatally detected suprarenal lesion in a neonate. J Pediatr Surg 2000,35(1): 1367-1369.
    38. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986,3(1):106-121.
    39. Savic B, Birtel FJ, Tholen W et al. Lung sequestration:report seven cases and review of 540 published cases. Thorax 1979,34(4):96-101.
    40. Dressler S.Massive pleural effusion and hypoplasia of the lung accompanying extrapulmonary sequestration. Hum Pathol 1981; 12(1):862-864.
    41. Conran RM, Stocker ST. Extralobar sequestration with frequently associated congenital cystic adenoid malformation, type 2:Report of 50 cases. Pediatr Dev Pathol 1999,2(5):454-463.
    42. McMullin N, Doi O, Kent M. The spectrum of bronchopulomonary foregut malformations:a case of combined bronchogenic cyst and sequestration. Pediatr Surg Int 1987,2(4):304-306.
    43. Heithoff KB, Shashikant MS, Williams HJ et al. Bronchopulmonary foregut malformations:a unifying etiological concept.AJR Am J Roentgenol 1976; 126: 46-55.
    44. Buntain WL, Woolley MM, Mahom GH et al. Pulmonary sequestration in children:a twenty-five year experience. Surgery 1977,81(11):413-420.
    45. O'Mara CS, Baker RR, Jeyasingham K. Pulmonary sequestration. Surg Gynecol Obstet 1978; 147(12):609-616.
    46. Thilenius OG, Rushihaupt DG, Reploge RL et al. Spectrum of pulmonary sequestration:association with anomalous pulmonary venous drainage in infants 36. Pediatr Cardiol 1983; 4(1):97-103.
    47. Choplin RH, Siegel MJ. Pulmonary sequestration:six unusual presentations. AJR Am J Roentgenol 1980; 13(4):695-700.
    48. Crushell E, Said A, Al-Hassan A, Walsh K. Occlusion of an aberrant artery to a pulmonary sequestration using a duct occluder. J Interv Cardiol 2002,15(1): 415-416.
    49. Tokel K, Boyvat F, Varan B. Coil embolization of pulmonary sequestration in two infants:a safe alternative to surgery. AJR Am J Roentgenol 2000,17(5): 993-995.
    50. Laurin S, Aronson S, Schuller H, Henrikson H. Spontaneous hemothoraz from bronchopulmonary sequestration:unusual angiographic and pathologic anatomic findings. Pediatr Radiol 1980,10(5):54-56.
    51. Sade RM, Clouse M, Ellis FH. The Spectrum of pulmonary sequestration. Ann Thorac Surg 1974,18(12):644-658.
    52. Arcomano JP, Azzoni AA. Intralobar pulmonary sequestration and intralobar enteric sequestration associated with vertebral anomalies. J Thorac Cardiovasc Surg 1976,3(12):470-476.
    53.张雷,丁嘉安,姜格宁,等.肺隔离症的诊断和治疗.中华结核和呼吸杂志,1998,21(11):675-676.
    54.吴常青.肺隔离症的外科治疗.医学研究杂志.2008,22(6):116-118.
    55. Saygi A. Intralobar Pulmonary sequestration. Chest,2001,14(3):186-187.
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    59.郭恩,周志忠,方卫民.13例肺隔离症的诊断和外科治疗探讨.福建医药杂志,2007,29(5):69-70.
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    61. Lehnhardt S,Winterer JT,Uhrmeister P, et al.Pulmonary sequestration: demonstration of blood supply with 2D and 3D MR angiography. Eur JRadio,2002,44(1):28-32.
    62.高思宗,雷复华.肺内隔离症误诊为肺癌3例.肿瘤研究与临床,1999,2(11),144-145.
    63. Saygi A. Intralobar Pulmonary sequestration. Chest,2001,14(3):186-187.
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    1. Sersar SI, Diasty M, Ibrahim HR,et al. Lower lobe segment and pulmonary sequestration. Thorac Cardiovase Surg,2004,127(3):898-899.
    2.陆明华,杨国仪.肺隔离症的诊疗体会.中国呼吸与危重监护杂志,2003,3:391.
    3. Peter BK,Didier S,Sven H, et al. Thoracoscopic treatment of pulmonary sequestation. European Journal of CardioThoracic Surgery,2006,29 (5) 815-818.
    4. Savic B, Brtiel FJ, Tholen W, et al. Lung sequestration:report of seven cases and review of 540 published cases. Thorax,1979,34(1):96-101.
    5. Zylak CJ, Eyler WR, Spizarny DL, et al. Developmental lung anomalies in the adult:radiologic pathologic correlation.RadioGraphics,2002,22(10):25-43.
    6. Halkic N,Cuenoud PF,Corthesy ME, et al. Pulmonary sequestration Eur Cardiothorac Surg,1998,14(13):127-133
    7. Thomas HB, Thomas BF. Congenital lesions of the lung and emphysema (pulmonary sequestration) in:David CS, Frank CS. Gibbon's surgery of the chest. Third Edition. Philadelphia:W. B. Saunders company,1976,62(6):62-91
    8. Carter R. Pulmonary sequestration. Ann Thorac Surg,1969,7(1):68-88.
    9. Smith RA. A theory of the origin of intralabar sequestration of lung.Thorax,1956, 11(7):102-110.
    10. Epping H, Shorenstein W. Krankheitender Lungen [inGerman]. Ergeb Allg Pathol 1902,8(10):267-285.
    11.张熙曾,吴德泰,白人华.肺隔离症(附2例报告).天津医药肿瘤学附刊,1982,9(2):49-52.
    12. Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lung:a report of seven case. Patho,1946,58(9):457-467.
    13.胡为伟,赵松.肺隔离症的外科诊疗.中国现代医学杂志,2004,4(12):135-136.
    14.黄孝迈.现代胸外科学.北京:人民军医出版社,1991:80
    15.陈灏珠.实用内科学下册12版.北京:人民卫生出版社,2006:1785.
    16. Cockayne EA,Gladstone RJ. A case of accessory lungs associated with hernia through acongenital defect of the diaphragm. J Anat 1947,52(12):64-96.
    17. Bolck F.Zur Frageder Entstechungvon Neben lungen [inGerman]. Virchows Arch 1950; 31(9):20-43.
    18. Cole FH, Alley FH, Jones RS. Aberrant systemic arteries to the lower lung. Surg Gynecol Obstet 1951,93(6):589-596.
    19. Boyden EA. Bronchogenic cysts and the theory of intralobar sequestrations. J Thorac Cardiovasc Surg 1958,35(11):604-616.
    20. Gebauer PW, Mason CB. Intralobar pulmonary sequestration associated with anomalous pulmonary vessels anonentity. DisChest 1959,30(2):282-288.
    21. Kyllonen KE. Intralobar pulmonary sequestration and a theory as to it saetiology. Acta Chir Scand 1964,127(13):307-310.
    22. Blesovsky A. Pulmonary sequestration:a report of an unusual case and review of the literature. Thorax 1967,22(12):351-357.
    23. Gerle RD, Jaretzki AM, Ashley CA, Berne AS.Congenital bronchopulmonary foregut malformation:pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968,27(85):1413-1419.
    24. Moscarella AA, Wyllie RH. Congenital communication between the esophagus and isolate dectopic pulmonary tissue. J thorac Caridovasc Surg 1968, 55(16):672-676.
    25. Albrenchtsen D. Pulmonary sequestration. Sc and J Thorac Cardiovasc Surg 1974, 8(1):64-66.
    26. Stocker JT,Malczak HT. A study of pulmonary ligament arteries:relationship to intralobar sequestration. Chest 1984,86(12):611-615.
    27. Nicolette LA,Kosloske AM,Bartow SA,Murphy S. Intrapulmonary sequestration: a clinical and pathological spectrum. J pediatr Surg 1993,28(6):802-805.
    28.李树本,何建行,杨运有,等.叶内型肺隔离症的诊断与外科治疗.中华心血管外科杂志,2006,22(4):271-272
    29. Shibli M, Connery C, Shapiro JM. Intralobar and extralobar bronchopulmonary sequestration complicated by nocardia asteroides infection. Southern Med
    J,2003,96(1):78-80.
    30. DeParedes CG, Pierce WS, Johnson DG. Pulmonary sequestration in infants and children:a 20-year experience and review of the literature. J Paediatr Surg 1970, 5(1):136-147.
    31. Sabiston S,著.石应康,译.胸外科学.6版.北京:人民卫生出版社,2000:581-583.
    32. Curtis MR, Mooney DP, Vaccaro TJ et al. Prenatal ultrasound characterization of the suprarenal mass:distinction between neuroblastoma and subdiaphragmatic extralobar pulmonary sequestration. J Ultrasound Med 1997, 16(1):75-83.
    33. White J, Chan YF, Neuberger S, Wilson T. Prenatal sonographic detection of intra-abdominal extralobar pulmonary sequestration:report of three cases and literature review. Prenat Diagn 1994,14(12):653-658.
    34. Chan YF,Oldfield R,Vogel S, Ferguson S. Pulmonary sequestration presenting as a prenatally detected suprarenal lesion in a neonate. Pediatr Surg 2000,35(16): 1367-1369.
    35. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol.1986,3(2):106-121.
    36. Savic B, Birtel FJ, Tholen W et al. Lung sequestration:report seven cases and review of 540 published cases. Thorax 1979,34(2):96-101.
    37. Dressler S. Massive pleural effusion and hypoplasia of the lung accompanying extrapulmonary sequestration. Hum Pathol 1981,12(10):862-864.
    38. Conran RM, Stocker ST. Extralobar sequestration with frequently associated congenital cystic adenoid malformation, type 2:Report of 50 cases. Pediatr Dev Pathol 1999,2(16):454-463.
    39. McMullin N, Doi O, Kent M. The spectrum of bronchopulomonary foregut malformations:a case of combined bronchogenic cyst and sequestration. Pediatr Surg Int 1987,2(10):304-306.
    40. Heithoff KB, Shashikant MS, Williams HJ et al.Bronchopulmonary foregut malformations:a unifying etiological concept. AJR Am Roentgenol 1976,12(6): 46-55.
    41. Buntain WL, Woolley MM, Mahom GH et al. Pulmonary sequestration in
    children:a twenty-five year experience. Surgery 1977,81 (6):413-420.
    42. O'Mara CS, Baker RR, Jeyasingham K. Pulmonary sequestration. Surg Gynecol Obstet 1978,147 (12):609-616.
    43. Thilenius OG, Rushihaupt DG, Reploge RL et al. Spectrum of pulmonary sequestration:association with anomalous pulmonary venous drainage in infants 36. Pediatr Cardiol 1983,4(10):97-103.
    44. White JJ, Donahoo JS, Ostrow PT, Murphy J, Haller JA. Cardiovas-cular and respiratory manifestations of pulmonary sequestration in childhood. Ann Thoarc Surg 1974,18(12):286-294.
    45. Choplin RH, Siegel MJ. Pulmonary sequestration:six unusual presentations. AJR Am J Roentgenol 1980,134(15):695-700.
    46. Crushell E, Said A, Al-Hassan A, Walsh K. Occlusion of an aberrant artery to a pulmonary sequestration using a duct occluder. J Interv Cardiol 2002,15(16): 415-416.
    47. Tokel K,Boyvat F,Varan B.Coil embolization of pulmonary sequestration in two infants:a safe alternative to surgery. AJR Am J Roentgenol 2000,17(5): 993-995.
    48. Ferretti GR,Jouvan FB,Coulomb M. MDCT demonstration of intralobar pulmonary sequestration of the rightupper lobe in an adult. AJR,2005, 185(6):1663-1664.
    49. Laurin S, Aronson S, Schuller H, Henrikson H. Spontaneous hemothoraz from bronchopulmonary sequestration:unusual angiographic and pathologic-anatomic findings. Pediatr Radiol 1980,10(1):54-56.
    50. Sade RM, Clouse M, Ellis FH. The Spectrum of pulmonary sequestration. Ann Thorac Surg 1974,18(12):644-658.
    51. Arcomano JP, Azzoni AA. Intralobar pulmonary sequestration and intralobar enteric sequestration associated with vertebral anomalies.Thorac Cardiovasc Surg 1976,3(20):470-476.
    52.张雷,丁嘉安,姜格宁,等.肺隔离症的诊断和治疗.中华结核和呼吸杂志,1998,21(11):675-676.
    53.钱可宝,向旭东,段林灿,等.肺隔离症的诊断和外科治疗.临床误诊误治,2009,(04)28:29.
    54.吴常青.肺隔离症的外科治疗.医学研究杂志.2008,22(5):116-118.
    55. Saygi A. Intralobar Pulmonary sequestration.Chest,2001,14(3):186-187-
    56. Sersar SI, Diasty M, Ibrahim HR,et al. Lower lobe segment and pulmonary sequestration.Thorac Cardiovase Surg,2004,127(3):898-899.
    57.刘伦旭,周清华,陈光富,等.肺隔离症的诊断和治疗.中国胸心血管外科临床杂志,1999,6(3):186-187.
    58.郭恩,周志忠,方卫民.13例肺隔离症的诊断和外科治疗探讨.福建医药杂志,2007,29(5):69-70.
    59.聂永康,赵绍宏,蔡祖龙,等.螺旋CT三维重建在肺隔离症诊断中的应用.中华放射学杂志,2003,37(11):997-1000.
    60. Lehnhardt S,Winterer JT,Uhrmeister P, et al. Pulmonary sequestration: demonstration of blood supply with 2D and 3D MR angiography. Eur J Radio, 2002,44(1):28-32.
    61.高思宗,雷复华.肺内隔离症误诊为肺癌3例.肿瘤研究与临床,1999,2(11),144-145.
    62. Juettner FM, Pinter HH, Friehs GB. Bronchial carcinoid arising in interlober bronchopulmonary sequestrationwith vascular supply from the lefe gastric artery. J Thorac Cardiovase Surg,1987,90(1):25.
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