A Neglected Case of Intralobar Pulmonary Sequestration

Suresh Kumar D et al.: A Neglected Case of Intralobar Pulmonary Sequestration

A Neglected Case of Intralobar Pulmonary Sequestration

Suresh Kumar D¹,Somenathkundu²

¹Junior Resident, ²A2 Professor and HOD,
Department of Respiratory Medicine, IPGMER & SSKM Hospital, Kolkata -20

Introduction

Pulmonary sequestration is rare developmental anomaly of lungs. It is due to abnormal lung budding during embryogenesis. It is non functional lung parenchyma and often it lacks communication with normal bronchus1. Lung sequestration is more commonly located at medial basal and postero basal segment of left lower lobe. Often this disease is under diagnosed. This case is reported because of its rare occurrence and neglected for almost twelve years.

Case Report

33 years old male farmer, never smoker from Rural Kolkata, admitted for recurrent respiratory tract infections since childhood. patient is normotensive, normoglycemic and no significant
past history of tuberculosis. Patient was hospitalised in 2007 for same illness and patient was diagnosed as cystic lung disease in left lower lobe with infective exacerbations. Patients have frequent exacerbations (4 to 5 episodes per year) and treated in multiple hospital with multiple course of antibiotics.
N ow patient admitted with acute infective exacerbations and chest skiagrams shows left lower and mid zone multiple cyst with air-fluid level withmediastinal shift to right side. So we proceed with HRCT chest and it showed multiple thick walled cyst with air fluid level with adjacent mimimalcystic bronchiectasis in left lower lobe with infective pneumonic changes in right lower lobe.

we planned for surgical resection as curative options since patient is symptomatic with recurrent exacerbations and lesions confined to left lower lobe. since pathology located in left lower lobe we suspected likely high chance of any congenital anomalies and we proceed with CT angiogram and it showed ultiple cystic lesion12*12*13 cm in left lower lobe and it has no communication with left lower lobe bronchus and its received Systemic arterial supply from thoracic aorta and venous drainage to pulmonary veins. And thus angiography findings suggestive of intra lobar pulmonary sequestration. After getting rgical fitness, patient underwent left posterolateral thoracotomy. On table, multiple cystic cavity involving lateral and anteromedial basal segment of left lower lobe and rest of lungs appears healthy. After ligating systemic artery arising from thoracic aorta, segmentectomy done with lateral and anteromedial basal segments of left lower lobe. Biopsy samples send for histopathological examination and reports confirm the diagnosis of pulmonary
sequestration.

Discussion

The term “Sequestration “was first used in the medical literature by Pyre in 1946. Its origin from latin word sequestare, which means“ to separate”.

Bronchopulmonary sequestration are rare lung developmental anomalies. It is due to abnormal lung budding in embryogenesis. It is non functional lung parenchyma and often it lacks communication with normal bronchus. They are two types. Intra lobar sequestration[ILS] and extra lobar sequestration[ELS]2. Intra lobar sequestration is four times more common than extra lobar sequestration and it is more commonly seen in medial basal and postero basal segment of left lower lobe. ILS confined within normal lungparenc hyma whereas ELS have separate pleura. Intralobar sequestration usually presents in adolescence or adulthood in the form of recurrent pneumonia or hemoptysis. Other presentations like multiple cysts, cavities, lung abscess, consolidation and mass. Extra lobar sequestration usually presents in neonatal or early childhood period and is often associated with other congenital anomalies like congenital diaphragmatic hernia, CCAM, Cardiac anomalies.

Arterial supply of ILS is thoracic or abdominal aorta and ELS usually receives from abdominal aorta and the venous drainage is pulmonary veins in ILS and systemic veins in case of ELS3. since sequestrated lobe is incapable of complete drainage, recurrent infection supervenes and patient usually presented with recurrent pneumonia.

In our case patient is symptomatic since childhood and multiple thick walled cyst with air fluid level with mediastinal shift in chest skiagram mimicking encysted pyopneumothorax and diaphragmatic hernia. Patient is neglected and it takes several years to confirm diagnosis. Patient had multiple course of antibiotics because of recurrent pneumonia. Although angiography is done in olden days, nowadays, CT/MRI angiography is gold standard investigations to find the feeding vessel^4,5.
Treatment options for asymptomatic pulmonary sequestration is debatable. Since our patient is symptomatic with recurrent chest infections, patient was taken up for lung resection in our case. Recent emerging treatment options for pulmonary sequestration with recurrent hemoptysis is endovascular coil embolisation.

Conclusion

Intralobar pulmonary sequestrations are although rare anomaly and is often neglected .in our case it takes 12 years to diagnose the disease. Before doing any curative lung resection for sequestration, angiogram is essential to find out feeding vessels and thereby preventing torrential haemorrhage during intraoperative period. High index of clinical suspicion is warranted.

References

1. Cooke CR, Bronchopulmonary sequestration, resp care 2006:51:661-664
2. Andrade C.F. Ferreira H.P.D.C,J Bras pneumol. 2011:37(2) :259-271
3. AqrabawiH.E.pulmonary sequestration case report Clindiagresp 2007:1:73-75
4. Lyold Petty ,case reportpulmonary sequestration in a adult RadiocaseResp 2018 feb 13 :21-23
5. Xin Qian et al,Pulmonary sequestrationInt J ClinExp med 2015:8(11):21822-21825