Intralobar Bronchopulmonary sequestration in adults
over age 50: case series and review.
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Abstract: |
Introduction: Bronchopulmonary sequestration (BPS) is a rare
congenital lung malformation, comprising only 0.15 to 6.4% of all cases
of congenital lung malformation. It is characterized by an abnormal
segment of bronchopulmonary tissue supplied by an aberrant systemic
artery. Diagnostic delays are common in the adult patients since the
symptoms often mimic other common diseases such as pneumonia, emphysema,
and lung abscess. In 60% of cases, intralobar pulmonary sequestration
(ILS) is typically diagnosed at age 20 or younger and is rarely found in
adults older than 40 years. Since a heightened clinical suspicion needs
to be maintained to entertain this diagnosis in the adult population, we
conducted a retrospective chart review of all adult patients at our
institution over age 50. Materials and Methods: A retrospective chart review of all adult patients evaluated at our institution with a pathological proven diagnosis of BPS and subsequent surgical correction from January 1993 through December 2008 was conducted. Data obtained included demographics, clinical presentation, diagnostic procedures, location and origin of the lesion, operative therapy, histology of the surgical specimen, and postoperative complications. Results: Three patients had undergone surgical correction for BPS. All three patients at our institution were female. The average age was 59 years with a range from 57 to 62 years. All three patients had symptoms preoperatively including intermittent cough and recurrent pneumonia. Radiologic evaluation revealed an enlarging lung mass in one patient and lesions suggestive of BPS in two of the patients. Two patients had a right lower lobe sequestration and one patient had a left lower lobe sequestration. All three patients underwent lower lobectomies without any significant postoperative complications. Conclusion: BPS in patients older than 50 is very rare. Persistent lower lobe consolidation in medial or posterior basal segments accompanied by an aberrant artery in adults should heighten suspicion for it. Recurrent pneumonias, cough, chest pain occur in the majority of patients. Surgical excision after correctly identifying the aberrant vessel is curative and also diagnostic in some cases. |
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Article Type: | Report |
Subject: |
Bronchial diseases
(Diagnosis) Bronchial diseases (Care and treatment) Pneumonectomy (Methods) Aorta (Physiological aspects) |
Authors: |
Montjoy, Carol Hadique, Sarah Graeber, Geoffrey Ghamande, Shekhar |
Pub Date: | 09/01/2012 |
Publication: | Name: West Virginia Medical Journal Publisher: West Virginia State Medical Association Audience: Academic Format: Magazine/Journal Subject: Health Copyright: COPYRIGHT 2012 West Virginia State Medical Association ISSN: 0043-3284 |
Issue: | Date: Sept-Oct, 2012 Source Volume: 108 Source Issue: 5 |
Geographic: | Geographic Scope: West Virginia Geographic Code: 1U5WV West Virginia |
Accession Number: | 303449699 |
Full Text: |
Introduction BPS is a rare congenital lung malformation, comprising only 0.15 to 6.4% of all pulmonary malformation. It is characterized by an abnormal segment of bronchopulmonary tissue supplied by an aberrant systemic artery. (1) The diagnosis can easily be missed in adult patients since the symptoms often mimic other common diseases such as pneumonia, emphysema, and lung abscess. (2) Intralobar sequestration (ILS) is diagnosed at age 20 years or younger in approximately 50% to 60% of cases, and it is rarely found in patients older than 40 years.2 We report our experience over the last 15 years of adult patients over 50 years of age diagnosed with intralobar BPS and treated with surgical resection to further enhance our understanding of this rare disease in elderly patients. A review of the epidemiology, pathogenesis, clinical features, diagnostic procedures, and treatment is included in this discussion. Materials and Methods Records of all adult patients at our institution with a pathological proven diagnosis of BPS and subsequent surgical correction between January 1993 and December 2008 were reviewed retrospectively. Age, sex, symptoms, diagnostic procedures, location and origin of the lesion, operative therapy, histology of the surgical specimen, and postoperative complications were evaluated. This study was approved by our institutional review board. Three patients had undergone surgical correction for BPS. All three patients at our institution were female. The average age was 59 years with a range from 57 to 62 years. All three patients were symptomatic. Most common presentation was intermittent cough and recurrent pneumonia. Radiologic evaluation revealed an enlarging mass in one patient and lesions suggestive of BPS in two of the patients. Two patients had a right lower lobe sequestration and one patient had a left lower lobe sequestration. All three patients underwent a lower lobectomy without any significant postoperative complications. A summary of each case is given below. Discussion Pulmonary sequestration was first described by Rektorzik in 1861, as a nonfunctioning mass of lung tissue which lacks normal communication with the tracheobronchial tree and with an anomalous systemic arterial supply. (3) The systemic arterial supply is via the descending thoracic aorta (72%); abdominal aorta, celiac axis, or splenic artery (21%); intercostal artery (3.7%); and rarely via the subclavian, internal thoracic, and pericardiophrenic arteries. Most venous drainage (95%) is via the pulmonary veins. (4) Pryce first coined the term pulmonary sequestration in 1946 and further classified the lesion as intralobar or extralobar on the basis of the morphologic patterns of sequestration. (5) Epidemiology BPS is estimated to comprise 0.15 to 6.4% of all congenital pulmonary malformations for which even tertiary care centers only diagnose approximately one case per year. (1) The two types of bronchopulmonary sequestration are intralobar (ILS), as in our patients, and extralobar sequestration (ELS). ILS shares common visceral pleura within a normal lung lobe, whereas ELS has its own pleural lining and thus is separated from the remaining lung tissue. ILS is four times more common than ELS. ILS is more common in early adulthood while ELS is more commonly diagnosed in the fetal and neonatal period. Males and females are equally affected by ILS while ELS has a male predominance in most series. (1,2) Pathogenesis BPS has been postulated to occur due to the formation of an accessory lung bud inferior to the normal lung buds that develop from the primitive foregut as it migrates caudally. (6) During bronchial branching, the bronchial buds are supplied by a capillary plexus derived from the primitive aorta which typically regresses; however, growth arrest locally at the pulmonary artery during bronchial division, which may lead to persistence of the blood supply from the aorta. (7) Since this occurs during fetal development, it can explain the association of BPS, particularly ELS, with other congenital anomalies. Associated congenital anomalies in ELS include diaphragmatic hernia, congenital cystic adenoid malformation, bronchogenic cysts, cardiovascular malformation, and pectus excavatum. [FIGURE 1 OMITTED] [FIGURE 2 OMITTED] Some authors have proposed that ILS is an acquired defect related to bronchial obstruction, pneumonia, and pleuritis. According to this hypothesis, repeated infection causes angiogenic growth factors to be activated leading to angiogenesis and the development of a systemic arterial supply. This would be a plausible explanation for the prevalence of ILS in adult patients and the lack of association with other congenital anomalies. (8) Clinical Presentation and Complications Due to the association of congenital anomalies, ELS is typically diagnosed in childhood with signs and symptoms of respiratory distress. Although ILS is diagnosed in childhood, up to half of patients are diagnosed after the age of twenty. (6) Hirai recently reviewed BPS in patients over fifty and revealed only 10 reported cases since 1998 as seen in Table 1. (9) We found an additional nine cases since Hirai's series, including our cases (Table 2). Cough, sputum production, recurrent episodes of pneumonia, and symptoms related to associated anomalies are the most common symptoms of patients with BPS. Some patients with BPS remain asymptomatic and are incidentally discovered (15.5% of ILS patients and 10% of ELS patients). The risk of infection is counterintuitive since the sequestered lung lacks a direct connection to the tracheobronchial tree. BPS malformations, however, are not completely isolated from the native lung due to bacteria invasion through the pores of Kohn. Once the bacteria have colonized the sequestration, infection can progress because of the lack of normal bronchial drainage. (6) Pulmonary infections described in sequestered lung include Aspergillus fumigatus, Mycobacterium tuberculosis, kansasi and bacteremia due to Gordonia bronchialis infection. (10-15) Besides recurrent infections, both ELS and ILS have been reported to cause fatal hemoptysis, massive hemothorax, and cardiovascular complications. (16,17) Surgical excision remains the treatment of choice in known cases of BPS. When these complications occur in undiagnosed cases, it often leads to diagnostic delays but ultimately surgical excision is both diagnostic and curative. Radiographic Features Most common location of the ILSs are in the medial and posterior basal segments of the left lung. Overall, 98% occur in the lower lobes. (2) Plain chest radiograph is generally nonspecific and shows an ill-defined area of consolidation suggestive of pneumonia. (20) Chest CT usually shows a discrete mass in the medial or posterior basal segments of the left lower lobe with or without cystic changes. (6,20) Two out of three patients in our series showed a lesion in the right lower lobe which is less common than left lung lesions. Lesions present in other than the lower lobe should imply an alternative diagnosis given that sequestered lesions outside the lower lobe account for only 5% of cases reported in the literature. (2) Cystic changes, as seen in patient 3, are usually multiple in nature and are usually surrounded by emphysematous changes, presumably due to air trapping from the sequestration itself (Figure 3). (6,20,21) Other findings include pneumothorax due to cyst rupture into the pleural cavity. Lastly, focal bronchiectasis may also be found. (22) [FIGURE 3 OMITTED] Pre-operative radiographic identification of the aberrant artery is critical in preventing operative morbidity and mortality due to exsanguinations. (23) Due to recent advances in the diagnostic modalities of CT (helical CT, dynamic CT, and 3-D CT), CT chest with contrast may be sufficient in identifying the aberrant artery. (9) [FIGURE 4 OMITTED] [FIGURE 5 OMITTED] Some authors have reported the utility of contrast-enhanced MRI and MRA as a means to evaluate the thoracic and pulmonary vasculature. (2) However, the gold standard for identifying pulmonary sequestration is angiography as it confirms the anatomy, identifies the systemic supply, and shows the venous drainage. (2,21) Pathologic Features Grossly, intralobar sequestrations usually show the effects of chronic inflammation due to recurrent infection. The pleura is thickened with associated adhesions and the parenchyma shows fibrosis and cysts. The cysts often contain mucinous or frankly purulent material, as seen in our case (Figure 5). Histologically, the parenchyma shows the effects of inflammation and fibrosis as well. Bronchi are dilated and contain mucous or purulent material. The alveoli are filled with macrophages. Thick-walled vessels may be revealed with elastic tissue stains. (25) Similarly, extralobar sequestration may appear normal if there are no secondarily inflamed bronchi or may show the effects of chronic inflammation with a thick pleural surface covered by exudates. The cut surface may reveal cystic changes, fibrosis, and purulent secretions. In uninfected cases, dilated airways are lined by bronchiolar-type epithelium and dilated airspaces are lined with Type 1 and Type 2 pneumocytes. In infected cases, there is nonspecific acute and chronic inflammation, fibrosis, and purulent exudates. Treatment Surgical excision is the treatment of choice in both symptomatic and asymptomatic patients due to the risk of complications such as recurrent infections, congestive heart failure, and massive hemoptysis. (23) Surgery usually involves lobar resection via standard thoracotomy. (23) However, thoracoscopic techniques are now being reported more frequently in the literature. (26,27) Video-assisted thoracoscopic wedge resection has even been described in patients with localized pulmonary sequestration. (28,29) In cases where the risk of surgery is high, angiographic embolization of the feeding systemic vessel might be an option. (6) Despite the origin of ILS from the aorta, the aberrant artery to ILS malformation is not thick-walled like a bronchial artery, but thin-walled with a wide lumen like a pulmonary artery. (7,30) Surgical resection, therefore, requires careful identification and dissection of the systemic arterial supply and venous drainage of the BPS malformation in order to avoid the exsanguinating hemorrhage. (23) The prognosis following surgical excision of intralobar sequestration is excellent, and long-term follow-up suggests that these patients do well. Conclusion Because BPS in patients older than age 50 is very rare, we conducted a single institutional case series in patients over 50 with BPS along with a review of the literature in hopes of raising awareness of this entity. The constellation of recurrent pneumonia and lower-lobe lesions typically in medial and posterior basal segments fed by an aberrant systemic arterial vessel are hallmarks of this disease. The diagnosis of ILS should be considered in adults with these features. Complications such as unusual bacterial pneumonias, hemoptysis and hemothorax have been recognized in these patients. Symptomatic patients should be referred to surgery for definitive lobectomy or wedge resection. Given the potential risk of massive hemoptysis in asymptomatic patients, they should be referred for resection as well. The main and feared surgical complication, exsanguinating hemorrhage, can be prevented by preoperative radiographic identification of the arterial supply of the sequestration and appropriate early surgical isolation and management of the aberrant vessel. Acknowledgements Robert Tallaksen, MD, Department of Radiology, West Virginia University, Morgantown, WV David S. Harrer, MD, Department of Pathology, West Virginia University, Morgantown, WV Kristen Biggers, MD, Department of Pathology, West Virginia University, Morgantown, WV References (1.) Gezer S, Tastepe I, Sirmali M, et al. A single institutional series composed of 27 cases. Journal of thoracic and cardiovascular surgery. 2007. 133(4): 955-959. (2.) Petersen G, Martin U, Singhal A, et al. Intralobar sequestration in the middle aged and elderly adult: recognition and radiographic evaluation. Journal of thoracic and cardiovasc surgery. 2003. 126(6): 2086-2090 (3.) Rektorzik E. Ueber accessorische lungen lappen. Wochenbl Z Ges Aerzte (Wein) 1861;17:4-6. (4.) L.N. Turk, III and G.E. Lindskog, The importance of angiographic diagnosis in intralobar pulmonary sequestration. J Thorac Cardiovasc Surg 41 (1961), pp. 299-305. (5.) D.M. Pryce, Lower accessory pulmonary artery with intralobar sequestration of lung: a report of seven cases. J Pathol Bacteriol 58 (1946), pp. 457-467. (6.) Cooke C. Bronchopulmonary sequestration. Respiratory care. 2006. 51(6): 661-664. (7.) Murray JF, Nadel J. Textbook of Respiratory Medicine. Saunders Publications. 2005;Chapter 50. (8.) Palmowski M, Schreiner K, Hansmann J, et al. Bronchopulmonary sequestration: a differential diagnosis in young adults for recurrent pneumonia. 2007;369:1318. (9.) Hirai S, Hamanaka Y, Mitsui N, et al. Surgical treatment of infected intralobar pulmonary sequestration: a collective review of patients older than 50 years reported in the literature. Ann thorac cardiovasc surg. 2007;13(5): 331-334. (10.) Berna P, Lebied ED, Foucault C, et al. Pulmonary sequestration and aspergillosis. European journal of cardio-thoracic surgery. 2005; 27: 28-31. (11.) Lohani S, Varma R, Leahy B. A case of pulmonary sequestration with aspergillus species infection presenting as an enlarged right paratracheal mass. J thorac cardiovasc surg. 2005;129(6): 1459-1460. (11.) Lohani S, Varma R, Leahy B. A case of pulmonary sequestration with aspergillus species infection presenting as an enlarged right paratracheal mass. J thorac cardiovasc surg. 2005;129(6): 1459-1460. (12.) Sato H, Watanabe A, Yamaguchi T, et al. Pulmonary sequestration associated with asymptomatic aspergillosis. Ann thoracic cardiovasc surg. 2005;11(1): 41-43. (13.) Yatera K, Izumi M, Imai M, et al. Intralobar sequestration with tuberculous infection confined to the sequestrated lung. Respirology. 2005; 10: 685-688. (14.) Lin SH, Lee LN, Chang YL, et al. Infected pulmonary sequestration casued by mycobacterium kansasii. Thorax. 2005; 60: 355. (15.) Sng L, Koh TH, Toney SR, et al. Bacteremia caused by gordonia bronchialis in a patient with sequestered lung. Journal of clinical microbiology. 2004;42(6): 2870-2871. (16.) Wandschneider W, Illiasch H. Intralobar sequestration: a rare cause of severe hemothorax. J thorac cardiovasc surg. 2003; 126(3): 872-873. (17.) Hofman F, Pasker H, Speekenbrink G. Hemoptysis and massive hemothorax as presentation of intralobar sequestration. Ann thorac surg. 2005; 80: 2343-2344. (18.) Hunninghake GM, Kanarek DJ. Pulmonary sequestration supplied by a coronary artery. Thorax. 2005; 60: 792. (19.) Tsitouridis I, Tsinoglou K, Cheva A, et al. Intralobar pulmonary sequestration with arterial supply from the coronary circulation. J thorac imaging. 2005; 20(4): 313-315. (20.) Bolca N, Topal U, Bayram S. Bronchopulmonary sequestration: radiologic findings. 2004; 52: 185-191. (21.) Ahmed M, Jacobi V, Vogl T. Multislice CT and CT angiography for non-invasive evaluation of bronchopulmonary sequestration. Eur Radiol. 2004; 14: 2141-2143. (22.) Ko S, Ng S, Lee T, et al. Noninvasive imaging of bronchopulmonary sequestration. AJR. 2000; 175: 1005-1012. (23.) Sheilds T, LoCicero III J, Ponn R, et al. General thoracic surgery. Lipponcot Williams & Wilkins. 2005;1108-1117. (24.) Kang M, Khandelwal N, Vijayanadh O, et al. Multidetector CT angiography in pulmonary sequestration. J comput assist tomogr. 2006; 30(6): 926-932. Please contact the authors for additional references. Carol Montjoy, MD West Virginia University, Morgantown, WV Sarah Hadique, MD West Virginia University, Morgantown, WV Geoffrey Graeber, MD University of Massachusetts, Worcester, MA Shekhar Ghamande, MD, FCCP West Virginia University, Morgantown, WV Age/Sex Smoking Symptoms history Patient 1 62/F Non smoker Intermittent Figure 1 dry cough with anterior chest wall pain Patient 2 57/F Non smoker Four episodes Figure 2 of right lower lobe pneumonia Patient 3 58/F Smoker Recurrent left Figure 3, lower lobe 4, 5 pneumonias with left sided chest pain and dyspnea. Imaging Treatment Patient 1 Chest CT -enlarging mass CT guided biopsy Figure 1 in the basilar segment of of the mass was the right lower lobe with nondiagnostic. extension to the posterior Video assisted ribs and involvement of thoracoscopic of the diaphragm. the right lower lobectomy was done. Patient 2 CT chest--right lower lobe Right lower Figure 2 pulmonary sequestration. lobectomy A CT arteriogram showed a single aberrant artery arising from the anterior right lateral aspect of the thoracic aorta at the level of T10 supplying the right lower lobe pulmonary sequestration Patient 3 Chest X-ray--a vague Left lower Figure 3, opacity in the lower lobe lobectomy. 4, 5 of the left lung. Chest CT chest- intralobar sequestration with a prominent systemic artery arising from the descending thoracic aorta at the level of the gastroesophageal junction. Extensive cystic changes were also noted in the pulmonary parenchyma Pathology finding Patient 1 Intralobar Figure 1 pulmonary sequestration Patient 2 Intralobar Figure 2 pulmonary sequestration. Patient 3 Acute and chronic Figure 3, bronchiolitis with 4, 5 cystic dilation characteristic of intralobar pulmonary sequestration. Table 1: A review of the literature concerning the surgical case reports of pulmonary intralobar sequestration in patients older than age 50 since 1998. (6) (This information was originally published in Ann Thorac Cardiovasc Surg, reprinted with permission of ATCS Editorial Office) Author Age/sex Symptoms Location (year) Tsunezuka 75/F Asymptomatic Left posterior and Sato (abnormal basal segment 1998 shadow) Yamanaka 68/F Dyspnea, Left posterior et al. 1999 hemopytsis basal segment Sakiyama 60/F Lumbago Left posterior et al 2000 segment basal segment Lewis and 66/M Dyspnea Left lower Tsour 2000 lobe Singh and 53/M Hemoptysis Left posterior Nath 2001 basal segment Petersen 79/F Hemoptysis Left lower et al. 2003 lobe Sakuma 60/M Asymptomatic Right lower et al 2004 Abnormal basal segment shadow Sato 65/F Hemoptysis Left lower et al 2005 basal lesion Matsuoka and 62/M Asymptomatic Left lateral & Nohara 2006 Abnormal dorsal basal shadow segment Hirai 2006 56/M Pneumonia Left posterior basal segment Author Origin of aberrant Operation (year) artery (number/ diameter) Tsunezuka Descending thoracic Lower lobectomy and Sato aorta (3/2 mm, 1998 2mm, 2 mm) Yamanaka Descending thoracic Basalectomy et al. 1999 aorta (1/20 mm) Sakiyama Descending thoracic Basalectomy et al 2000 aorta (2/7 mm, 3 mm) Lewis and Left circumflex None Tsour 2000 artery (1/unknown) Singh and Descending thoracic Lower lobectomy Nath 2001 aorta (1/30 mm) Petersen descending thoracic lobectomy & graft et al. 2003 aorta (1/1mm) replacement of descending thoracic artery Sakuma Descending thoracic) Video-assisted et al 2004 aorta (1/unknown thoracoscopic wedge resection Sato Descending thoracic Video-assisted et al 2005 aorta (1/10 mm) thoracoscopic lower lobectomy Matsuoka and Descending thoracic Lower lobectomy Nohara 2006 aorta (1/ unknown) Hirai 2006 Descending thoracic Lower lobectomy aorta (1/10 mm) Table 2: A literature review of case reports of pulmonary intralobar sequestration in patients older than 50 since 2005 Author (year) Age/sex Symptoms Location Hofman 55/M Hemoptysis Right lower et al 2005 (7) and lobe hemothorax Okamoto 69/M Exertional Left lower et al 2005 (8) dypnea lobe Akhavan- 61 Exertional Right lower Heidari et al dyspnea lobe 2006 (9) Berna et 64/M Non-produc Left upper al 2008 (10) Cough lobe Masuko et 74/M Asymptomatic Right lower al 2008 (11) lobe, medial basal segment Parvathy et 58/F Massive Posterior al 2008 (12) hemoptysis basal segment of left lower lobe Montjoy 2010 62/F Intermittent Right lower cough lobe Montjoy 2010 57/F Recurrent Right lower pneumonia lobe Author (year) Origin of Operation aberrant artery Hofman Right inferior Right et al 2005 (7) pulmonary ligament, Pneumonectomy rest Unknown Okamoto Descending None et al 2005 (8) thoracic aorta Akhavan- Abdominal Lower Heidari et al aorta lobectomy 2006 (9) Berna et Bronchial Upper al 2008 (10) artery lobectomy Masuko et Descending Lower al 2008 (11) thoracic lobectomy aorta Parvathy et Descending Lower al 2008 (12) thoracic lobectomy aorta Montjoy 2010 Descending Video thoracic assisted aorta thoracoscopic lobectomy Montjoy 2010 Descending Lower thoracic lobectomy aorta |
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