Intralobar Bronchopulmonary sequestration in adults over age 50: case series and review.
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.
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.

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 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.

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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)

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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)

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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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