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Case Study
Intralobar sequestration with situs inversus totalis
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314561097 aan.sagepub.com
Nainar Madhu Sankar, Sanath Kumar, Sugumar Natarajan, Subbaiyan Kumar, Ramaiah Krishnan and Yogesh Jawale
Abstract Pulmonary sequestration is a relatively rare congenital lesion of the lung. The occurrence of intralobar pulmonary sequestration coincidentally with situs inversus totalis is an interesting combination. We report a case of intralobar sequestration with situs inversus totalis a 30-year-old lady, which was treated successfully in our institution.
Keywords Bronchopulmonary sequestration, dextrocardia, pneumonectomy, situs inversus
Introduction Pulmonary sequestration is a congenital lesion of the lung, consisting of nonfunctional lung tissue inside healthy parenchyma, without any communication with the normal airway, often supplied by a branch from a systemic vessel and occasionally by the pulmonary artery. The venous drainage is to either the systemic or pulmonary veins. The presence of an intralobar pulmonary sequestration along with situs inversus totalis is a rare occurrence with a possibly undefined histogenetic association. Prompt clinical and radiological examination can help to diagnose this entity early and treat the patient appropriately. This report describes a case of intralobar sequestration with situs inversus totalis in a 30-year-old lady, which was treated successfully by lobectomy.
Case report A 30-year-old homemaker and mother of two healthy children, and a known case of situs inversus totalis diagnosed at the age of 5 years, presented with the complaint of recurrent respiratory tract infections since childhood. She had a history of anti-Koch’s treatment for pulmonary tuberculosis 6 years previously. In view of her recurrent respiratory symptoms, computed tomography had been performed a few months earlier, which revealed intralobar sequestration of the posterior and medial segment of the left lung lower lobe, with a
large septated fluid-filled cyst cavity measuring approximately 13 12 cm (Figure 1). An aberrant artery from the right-sided descending abdominal aorta was seen supplying the sequestration (Figure 2), and additional findings confirmed situs inversus totalis and dextrocardia. A preoperative pulmonary function test showed a restrictive pattern with a forced expiratory volume in 1 s of 1.5 L, consistent with a predicted value of 55%. The patient underwent a left lower lobectomy after the risks involved were explained. Under general anesthesia with a double-lumen endotracheal tube, a left posterolateral thoracotomy was performed. The anatomy of the left lung was consistent with that of the morphological right lung with 3 lobes, and the other structures (azygos vein, superior vena cava, inferior vena cava) were mirror images of the right side. Intraoperatively, the sequestration cyst was seen occupying the middle lobe as well, hence a middle and lower lobectomy was performed, and the aberrant artery supplying the sequestrum was ligated and divided. The surgery was carried out with minimal Department of Cardiothoracic Surgery, Meenakshi Mission Hospital and Research Centre, Madurai, India Corresponding author: Nainar Madhu Sankar, Department of Cardiothoracic Surgery, Meenakshi Mission Hospital and Research Centre, Lake Area, Melur Road, Madurai 625107, India. Email: [email protected]
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Asian Cardiovascular & Thoracic Annals 0(0) blood loss of 300 mL. The specimen was sent for histopathological examination, and the wound was closed with 2 chest drains. The patient was extubated on the table and moved to the intensive care unit with stable hemodynamics. Her postoperative course was uneventful and she was moved to the ward on the 2nd postoperative day. Daily chest radiographs showed residual air in the thoracic cavity with a minimal air leak in the chest drain. The drains were removed on the 7th day, and she was discharged symptom-free on the 9th postoperative day. Histopathology of the specimen showed an intralobar sequestration with organizing pneumonia. The cyst fluid was negative for any microorganisms on culture.
Discussion
Figure 1. Computed tomography showing an intralobar sequestration of the posterior and medial segment of left lung lower lobe, with a large septated fluid filled cyst cavity measuring approximately 13 12 cm.
Figure 2. The aberrant artery from the right-sided descending abdominal aorta, supplying the sequestration.
The common presentations of pulmonary sequestration include persistent cough, fever, and recurrent respiratory tract infections.1 It may even present as a coin lesion in the lung as an incidental finding during radiological imaging, without any symptoms. Massive hemoptysis due to aneurysm and erosion of the aberrant artery supplying the sequestration can be a dangerous presenting symptom.2,3 Gupta and colleagues4 reported a case of situs inversus in a patient with nevoid basal cell carcinoma, and suggested a histogenetic relationship between these two entities. A similar histogenetic relationship may explain the association of situs inversus totalis and intralobar sequestration, as seen in our patient. The prevalence of situs inversus totalis is reported to be 1:10,000. The combination of intralobar sequestration with dextrocardia but not with situs inversus totalis was reported by Ivanovi-Herceg and colleagues.5 Our review of the literature revealed a few cases of lobectomy being performed in situs inversus totalis for other pathologies such as lung carcinoma and Kartagener’s syndrome, but not for a sequestration.6 Several other cardiac malformations such as tetralogy of Fallot, ventricular septal defect, and hypoplastic left heart syndrome in association with situs inversus totalis have also been reported.7 Imaging studies such as computed tomography helps us to diagnose a pulmonary sequestration and delineate the aberrant vessel supplying the sequestrum. A sequestration that is left untreated after diagnosis may lead to serious life-threatening complications. Hence early diagnosis and surgical intervention with a lobectomy or pneumonectomy through a conventional thoracotomy or video-assisted approach is mandatory.8 The general principles of lobectomy in situs inversus apply to this case as well. The left lung appears anatomically as the mirror image of the morphological right lung with 3 lobes. Understanding the mirror image anatomy
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of the thoracic viscera helps the surgeon to perform a lobectomy in situs inversus very precisely. Intralobar sequestration is a relatively rare congenital anomaly that can be detected in early childhood with proper evaluation and radiological investigations. Recurrent pneumonia in the absence of a congenital cardiac defect should alert the physician to the possibility of a pulmonary sequestration. The coincidence of situs inversus totalis in this setting is rare. Surgical intervention in the form of lobectomy of a single or multiple lobes remains the mainstay of treatment, which can be performed through either thoracoscopy or open thoracotomy. Acknowledgement We thank the ethical committee for their timely assistance.
Funding
2.
3.
4.
5.
6.
7.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. 8.
Conflicts of interest statement None declared.
cases treated with lobectomy. Scand J Surg 2006; 95: 190–194. Janssen DP, Schilte PP, De Graaff CS and Van Dijk HA. Bronchopulmonary sequestration associated with an aneurysm of the aberrant artery. Ann Thorac Surg 1995; 60: 193–194. Rubin EM, Garcia H, Horowitz MD and Guerra JJ Jr. Fatal massive hemoptysis secondary to intralobar sequestration. Chest 1994; 106: 954–955. Gupta M, Das D, Mittal A, Kaur H and Malhotra A. Situs inversus in a patient with nevoid basal cell carcinoma syndrome: a histiogenetic relationship? J Oral Sci 2011; 53: 253–256. Ivanovi-Herceg Z, Majeric´-Kogler V, Mazuranic´ I, Neralic´-Meniga I and Puljic´ I. Bronchopulmonary sequestration and dextrocardia. Coll Antropol 1998; 22: 127–133. Wo´jcik J, Grodzki T, Bielewicz M, Wojtys´ M, et al. Lung cancer in situs inversus totalis (SIT)—literature review. Adv Med Sci 2013; 58: 1–8. Oppido G, Pace Napoleone C, Martano S and Gargiulo G. Hypoplastic left heart syndrome in situs inversus totalis. Eur J Cardiothorac Surg 2004; 26: 1052–1054. Gonzalez D, Garcia J, Fieira E and Paradela M. Videoassisted thoracoscopic lobectomy in the treatment of intralobar pulmonary sequestration. Interact Cardiovasc Thorac Surg 2011; 12: 77–79.
References 1. Pikwer A, Gyllstedt E, Lillo-Gil R, Jo¨nsson P and Gudbjartsson T. Pulmonary sequestration—a review of 8
Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 30, 2015
(AAN)
[1–3] [PREPRINTER stage]
Case Study
Intralobar sequestration with situs inversus totalis
Asian Cardiovascular & Thoracic Annals 0(0) 1–3 ß The Author(s) 2014 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492314561097 aan.sagepub.com
Nainar Madhu Sankar, Sanath Kumar, Sugumar Natarajan, Subbaiyan Kumar, Ramaiah Krishnan and Yogesh Jawale
Abstract Pulmonary sequestration is a relatively rare congenital lesion of the lung. The occurrence of intralobar pulmonary sequestration coincidentally with situs inversus totalis is an interesting combination. We report a case of intralobar sequestration with situs inversus totalis a 30-year-old lady, which was treated successfully in our institution.
Keywords Bronchopulmonary sequestration, dextrocardia, pneumonectomy, situs inversus
Introduction Pulmonary sequestration is a congenital lesion of the lung, consisting of nonfunctional lung tissue inside healthy parenchyma, without any communication with the normal airway, often supplied by a branch from a systemic vessel and occasionally by the pulmonary artery. The venous drainage is to either the systemic or pulmonary veins. The presence of an intralobar pulmonary sequestration along with situs inversus totalis is a rare occurrence with a possibly undefined histogenetic association. Prompt clinical and radiological examination can help to diagnose this entity early and treat the patient appropriately. This report describes a case of intralobar sequestration with situs inversus totalis in a 30-year-old lady, which was treated successfully by lobectomy.
Case report A 30-year-old homemaker and mother of two healthy children, and a known case of situs inversus totalis diagnosed at the age of 5 years, presented with the complaint of recurrent respiratory tract infections since childhood. She had a history of anti-Koch’s treatment for pulmonary tuberculosis 6 years previously. In view of her recurrent respiratory symptoms, computed tomography had been performed a few months earlier, which revealed intralobar sequestration of the posterior and medial segment of the left lung lower lobe, with a
large septated fluid-filled cyst cavity measuring approximately 13 12 cm (Figure 1). An aberrant artery from the right-sided descending abdominal aorta was seen supplying the sequestration (Figure 2), and additional findings confirmed situs inversus totalis and dextrocardia. A preoperative pulmonary function test showed a restrictive pattern with a forced expiratory volume in 1 s of 1.5 L, consistent with a predicted value of 55%. The patient underwent a left lower lobectomy after the risks involved were explained. Under general anesthesia with a double-lumen endotracheal tube, a left posterolateral thoracotomy was performed. The anatomy of the left lung was consistent with that of the morphological right lung with 3 lobes, and the other structures (azygos vein, superior vena cava, inferior vena cava) were mirror images of the right side. Intraoperatively, the sequestration cyst was seen occupying the middle lobe as well, hence a middle and lower lobectomy was performed, and the aberrant artery supplying the sequestrum was ligated and divided. The surgery was carried out with minimal Department of Cardiothoracic Surgery, Meenakshi Mission Hospital and Research Centre, Madurai, India Corresponding author: Nainar Madhu Sankar, Department of Cardiothoracic Surgery, Meenakshi Mission Hospital and Research Centre, Lake Area, Melur Road, Madurai 625107, India. Email: [email protected]
Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 30, 2015
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(AAN)
[1–3] [PREPRINTER stage]
2
Asian Cardiovascular & Thoracic Annals 0(0) blood loss of 300 mL. The specimen was sent for histopathological examination, and the wound was closed with 2 chest drains. The patient was extubated on the table and moved to the intensive care unit with stable hemodynamics. Her postoperative course was uneventful and she was moved to the ward on the 2nd postoperative day. Daily chest radiographs showed residual air in the thoracic cavity with a minimal air leak in the chest drain. The drains were removed on the 7th day, and she was discharged symptom-free on the 9th postoperative day. Histopathology of the specimen showed an intralobar sequestration with organizing pneumonia. The cyst fluid was negative for any microorganisms on culture.
Discussion
Figure 1. Computed tomography showing an intralobar sequestration of the posterior and medial segment of left lung lower lobe, with a large septated fluid filled cyst cavity measuring approximately 13 12 cm.
Figure 2. The aberrant artery from the right-sided descending abdominal aorta, supplying the sequestration.
The common presentations of pulmonary sequestration include persistent cough, fever, and recurrent respiratory tract infections.1 It may even present as a coin lesion in the lung as an incidental finding during radiological imaging, without any symptoms. Massive hemoptysis due to aneurysm and erosion of the aberrant artery supplying the sequestration can be a dangerous presenting symptom.2,3 Gupta and colleagues4 reported a case of situs inversus in a patient with nevoid basal cell carcinoma, and suggested a histogenetic relationship between these two entities. A similar histogenetic relationship may explain the association of situs inversus totalis and intralobar sequestration, as seen in our patient. The prevalence of situs inversus totalis is reported to be 1:10,000. The combination of intralobar sequestration with dextrocardia but not with situs inversus totalis was reported by Ivanovi-Herceg and colleagues.5 Our review of the literature revealed a few cases of lobectomy being performed in situs inversus totalis for other pathologies such as lung carcinoma and Kartagener’s syndrome, but not for a sequestration.6 Several other cardiac malformations such as tetralogy of Fallot, ventricular septal defect, and hypoplastic left heart syndrome in association with situs inversus totalis have also been reported.7 Imaging studies such as computed tomography helps us to diagnose a pulmonary sequestration and delineate the aberrant vessel supplying the sequestrum. A sequestration that is left untreated after diagnosis may lead to serious life-threatening complications. Hence early diagnosis and surgical intervention with a lobectomy or pneumonectomy through a conventional thoracotomy or video-assisted approach is mandatory.8 The general principles of lobectomy in situs inversus apply to this case as well. The left lung appears anatomically as the mirror image of the morphological right lung with 3 lobes. Understanding the mirror image anatomy
Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 30, 2015
XML Template (2014) [20.11.2014–8:47am] //blrnas3.glyph.com/cenpro/ApplicationFiles/Journals/SAGE/3B2/AANJ/Vol00000/140242/APPFile/SG-AANJ140242.3d
(AAN)
[1–3] [PREPRINTER stage]
Sankar et al.
3
of the thoracic viscera helps the surgeon to perform a lobectomy in situs inversus very precisely. Intralobar sequestration is a relatively rare congenital anomaly that can be detected in early childhood with proper evaluation and radiological investigations. Recurrent pneumonia in the absence of a congenital cardiac defect should alert the physician to the possibility of a pulmonary sequestration. The coincidence of situs inversus totalis in this setting is rare. Surgical intervention in the form of lobectomy of a single or multiple lobes remains the mainstay of treatment, which can be performed through either thoracoscopy or open thoracotomy. Acknowledgement We thank the ethical committee for their timely assistance.
Funding
2.
3.
4.
5.
6.
7.
This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors. 8.
Conflicts of interest statement None declared.
cases treated with lobectomy. Scand J Surg 2006; 95: 190–194. Janssen DP, Schilte PP, De Graaff CS and Van Dijk HA. Bronchopulmonary sequestration associated with an aneurysm of the aberrant artery. Ann Thorac Surg 1995; 60: 193–194. Rubin EM, Garcia H, Horowitz MD and Guerra JJ Jr. Fatal massive hemoptysis secondary to intralobar sequestration. Chest 1994; 106: 954–955. Gupta M, Das D, Mittal A, Kaur H and Malhotra A. Situs inversus in a patient with nevoid basal cell carcinoma syndrome: a histiogenetic relationship? J Oral Sci 2011; 53: 253–256. Ivanovi-Herceg Z, Majeric´-Kogler V, Mazuranic´ I, Neralic´-Meniga I and Puljic´ I. Bronchopulmonary sequestration and dextrocardia. Coll Antropol 1998; 22: 127–133. Wo´jcik J, Grodzki T, Bielewicz M, Wojtys´ M, et al. Lung cancer in situs inversus totalis (SIT)—literature review. Adv Med Sci 2013; 58: 1–8. Oppido G, Pace Napoleone C, Martano S and Gargiulo G. Hypoplastic left heart syndrome in situs inversus totalis. Eur J Cardiothorac Surg 2004; 26: 1052–1054. Gonzalez D, Garcia J, Fieira E and Paradela M. Videoassisted thoracoscopic lobectomy in the treatment of intralobar pulmonary sequestration. Interact Cardiovasc Thorac Surg 2011; 12: 77–79.
References 1. Pikwer A, Gyllstedt E, Lillo-Gil R, Jo¨nsson P and Gudbjartsson T. Pulmonary sequestration—a review of 8
Downloaded from aan.sagepub.com at NORTH DAKOTA STATE UNIV LIB on May 30, 2015
