Bronchopulmonary sequestration (BPS) is a rare congenital foregut anomaly of the lung, consisting of nonfunctioning primitive tissue that does not communicate with the healthy tracheobronchial tree. The abnormal segment has its own anomalous systemic blood supply, and typically drains into the pulmonary venous system.
It is frequently asymptomatic and may be discovered incidentally. Symptoms may vary, with patients presenting with vague symptoms related to chronic respiratory infection. Early diagnosis and intervention decreases morbidity and mortality.
We present a case of intralobar pulmonary sequestration involving the right lower lobe in a male child.peer-reviewe

DeGiovanni, Joseph V.

Grech, Victor E.

Micallef, S.P.

Sammut, Patrick

English

PubMed

OAR@UM

Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 5  IMAGES  in PAEDIATRIC CARDIOLOGY  Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. Paediatric Dept, Mater Dei Hospital, Malta.     Introduction Bronchopulmonary sequestration (BPS) is a rare congenital foregut anomaly of the lung, consisting of nonfunctioning primitive tissue that does not communicate with the healthy tracheobronchial tree. The abnormal segment has its own anomalous systemic blood supply, and typically drains into the pulmonary venous system.   It is frequently asymptomatic and may be discovered incidentally. Symptoms may vary, with patients presenting with vague symptoms related to chronic respiratory infection. Early diagnosis and intervention decreases morbidity and mortality.3,4    We present a case of intralobar pulmonary sequestration involving the right lower lobe in a male child.  Case presentation A three year old male, known case of mild aortic stenosis, was referred to the paediatric respiratory physician with a seven month history of chronic productive cough and recurrent wheeze. The cough was resistant to regular inhaled corticosteroids and salbutamol. Physical examination was unremarkable (longstanding positive finding of a systolic murmur), with no respiratory compromise.    Of note, in the past history, the child had been admitted to the Neonatal Intensive Care Unit at 16 hours of age with mild respiratory distress, which settled after minimal support and intravenous antibiotics.   At three years of age, there was one presentation to hospital with wheezing, resolving on nebulised salbutamol and oral antibiotics. A plain chest radiograph at the time reported “signs of right sided basal pneumonia associated with a small right sided pleural effusion” (Figure 1).  Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 6    Figure 1: Plain chest radiograph showing blunting of cardiophrenic and costophrenic angles of the right lung.   A repeat chest radiograph requested by the respiratory physician showed persistence of the above-mentioned abnormalities. A high resolution contrast enhanced computed tomography (HRCT) scan of the chest was performed, showing an elevated right hemi-diaphragm and a smaller right lung. The right basal lung parenchyma received its blood supply from an anomalous artery arising from the abdominal aorta at the level of the coeliac axis. The veins of the affected lobe drained directly into the pulmonary veins, implying a right-to-left shunt.   The lobe involved appeared inseparable from surrounding  normal  pulmonary  parenchyma with a  discernible  reduction  in  the  number  of  bronchi  within  the  involved  lung.  The findings were suggestive of a diagnosis of intralobar pulmonary sequestration (Figure 2).   Figure 2: CT showing anomalous artery    Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 7     The patient was referred to the paediatric cardiology team for further management. A diagnostic and therapeutic cardiac catheterisation was performed which confirmed the presence of the anomalous vessel, measuring 9mm in diameter, arising close to the coeliac axis (Figures 3,4).  This vessel was embolised successfully using a 10 by 8 Amplatzer duct occluder device (Figure 5).   Figure 3: Angiogram showing aberrant artery measuring approx 9mm   Figure 4: Angiogram showing aberrant artery (white) supplying sequestered lobe (black). DA: Descending Aorta  Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 8   Figure 5: Device deployed in aberrant vessel supplying sequestered lobe    The patient had an uncomplicated clinical course, with his symptoms having improved significantly. He continues to receive regular outpatient follow-up, and remains well to date.  Discussion Intralobar pulmonary sequestration is a rare congenital anomaly, being responsible for 0.15-6.4% of all congenital pulmonary malformations.1,2 Asymptomatic patients can present with incidental pulmonary lesions on plain chest radiography. Ultrasound may show a hyperechoic chest mass with Doppler ultrasound indicating flow through arteries and veins.3 Recurrent pneumonia or pleural effusions may render patients symptomatic.  There are two predominant types of BPS: extralobar (ELS), where sequestered lung lies outside the visceral pleura and can be invested in its own external layer, and intralobar (ILS), where abnormal bronchopulmonary tissue shares common visceral pleura with neighbouring lung tissue (Table 1).  The final diagnosis is reached via histological examination of resected tissue.4 Both intrapulmonary and extrapulmonary BPS may be associated with other congenital anomalies.3  BPS usually presents at a young age, in particular ELS which can even present as antenatal fetal hydrops, although 10% of ELS and 15% of ILS are diagnosed in adulthood in view of paucity of symptoms.2,3    Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 9   Table 1. Comparison of intralobar and extralobar pulmonary sequestration  Pulmonary Sequestration Extralobar Intralobar Age of onset 61% in <6 months; 10% asymptomatic Rare before 2 years of age unless diagnosed antenatally, 20% by 20 years, 15% asymptomatic Gender (male:female) 3-4:1 1:1 Location Lower lobe: 63% Other: intra-abdominal, Mediastinum Lower lobe: 98% Affected lung 65% Left 55% Left Associated anomalies 65% 11% Symptoms Respiratory distress Feeding difficulties Fetal hydrops Congestive cardiac failure Recurrent infections (frequent) Pneumothorax, Haemothorax Haemoptysis Congestive cardiac failure  Diagnosing pulmonary sequestration is fraught with difficulty, depending on the location and type of pathology and the presence or absence of symptoms. Contrast enhanced HRCT is usually sufficient to provide a diagnosis, as exemplified in this case report, giving detailed anatomical information, clearly demonstrating pertinent anatomy. The gold standard for diagnosing pulmonary sequestration is CT angiography, delineating arterial supply and venous drainage, with the benefit of being less invasive than conventional angiography.  This modality allows for safer surgical planning with more detailed visualisation of vascularisation of sequestered tissue.4,5,6,7    BPS can be diagnosed on prenatal ultrasound, although it may be difficult to differentiate from other lung abnormalities such as congenital cystic adenomatoid malformation, bronchogenic cysts,  enteric duplication cysts and neuroblastoma.8 A precise antenatal diagnosis can only be achieved if a systemic feeding artery is visualised.8    There have been varying clinical techniques used to manage this pathology. BPS can be managed conservatively or operatively, depending on the clinical setting.  Many authors advocate no intervention in the case of asymptomatic patients as such lesions rarely cause clinical problems.3,8    In symptomatic patients, anomalous lung may be removed surgically or embolised with endovascular intervention. With regards to surgery, sequestrectomy or pulmonary lobectomy can be performed.2,5,7,9 Lobectomy is preferred when sequestered tissue cannot be distinguished from functioning parenchyma. Sequestrectomy allows the preservation of as much lung tissue as possible.2,10 In children, surgery may be associated with a high risk of skeletal and muscular deformities such as asymmetry of the chest wall, scoliosis caused by rib fusion or scapular winging.3,4  Endovascular intervention may therefore be preferred to avoid the morbidity associated with surgery.4,11,12 Embolisation, however, carries a significant risk of subsequent pulmonary infections.4      In conclusion, there is a wide range of manifestations associated with pulmonary sequestration, ranging from complete asymptomatology to those reporting various cardiac or pulmonary complaints. Diagnostic imaging must is dependent on the clinical scenario but must delineate Micallef SP, Grech V, Sammut P, DeGiovanni JV. 3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. 10  vascularity, which will guide subsequent treatment. In this case report, a child with chronic cough had pulmonary sequestration involving the right lower lobe, with early diagnosis and prompt treatment leading to resolution of symptoms, which should be the standard of care in all patients with this pathology.   References 1. Savic B, Bertel FJ, Tholen W, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979;34:96-101. 2. Andrade CF, Paiva da Costa Ferreira H, Bueno Fischer G. Congenital lung malformations.  J Bras Pneumol. 2011;37(2):259-271 3. Corbett HJ & Humphrey GM.  Pulmonary sequestration. Paediatr Respir Rev. 2014; 5(1):59-68  4. Marine LM, Valdes FE, Mertens RM,  Bergoeing MR, Kramer A. Endovascular treatment of symptomatic pulmonary sequestration.  Annals of Vascular Surgery 2011; 25(5):11-15  5. Gezer S, Tastepe I, Sirmali M, Findik G, Turut H, Kaya S, et al. Pulmonary sequestration: a single-institutional series composed of 27 cases. J Thorac Cardiovasc Surg. 2007;133(4):955-9. 6. Jesch NK, Leonhardt J, Sumpelmann R, Gluer S, Nustede R, Ure BM. Thoracoscopic resection of intra- and extralobar pulmonary sequestration in the first 3 months of life. J Pediatr Surg. 2005;40(9):1404-6. 7. Azizkhan RG, Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int. 2008;24(6):643-57. 8. Adzick NS, Harrison MR, Crombleholme TM et al. Fetal lung lesions: management and outcome. Am J Obstet Gynecol 1998; 179:884–889. 9. Shanmugam G, MacArthur K, Pollock JC. Congenital lung malformations, antenatal and postnatal evaluation and management. Eur J Cardiothorac Surg. 2005;27(1):45-52. 10. Hertzenberg C, Daon E, Kramer J. Intralobar pulmonary sequestration in adults: three case reports. J Thorac Dis 2012;4(5):516-519 11. Lee KH, Sung KB, Yoon HK, et al. Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results. J Vasc Interv Radiol 2003;14: 363-367. 12. Nayar PM, Thakral CL, Sajwani MJ. Congenital lobar emphysema and sequestration treatment by embolization. Pediatr Surg Int 2005;21:727-729      Contact Information  Victor Grech victor.e.grech@gov.mt  © Images in Paediatric Cardiology (1999-2016)    

3 year old with chronic wet cough: Intralobar Bronchopulmonary sequestration. Images Paediatr Cardiol 2016;18(2):5-10. complaints. Diagnostic imaging must is dependent on the clinical scenario but must delineate

Congenital lobar emphysema and sequestration treatment by embolization. Pediatr Surg Int

Congenital lung malformations, antenatal and postnatal evaluation and management.

Congenital lung malformations.

Crombleholme TM. Congenital cystic lung disease: contemporary antenatal and postnatal management. Pediatr Surg Int.

Endovascular treatment of symptomatic pulmonary sequestration. Annals of Vascular Surgery

Fetal lung lesions: management and outcome.

Intralobar pulmonary sequestration in adults: three case reports.

Lung sequestration: report of seven cases and review of 540 published cases.

Pulmonary sequestration: a single-institutional series composed of 27 cases.

Pulmonary sequestration. Paediatr Respir Rev.

Thoracoscopic resection of intra- and extralobar pulmonary sequestration in the first 3 months of life.

Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results.

3 year old with chronic wet cough : Intralobar Bronchopulmonary sequestration

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3 year old with chronic wet cough : Intralobar Bronchopulmonary sequestration

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