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Bilateral Extralobar Pulmonary Sequestration Communicating With the Upper Gastrointestinal System in a Newborn With Duodenal Atresia
1380
CASE REPORT GUDBJARTSSON ET AL BILATERAL EXTRALOBAR PULMONARY SEQUESTRATION
Bilateral Extralobar Pulmonary Sequestration Communicating With the Upper Gastrointestinal System in a Newborn With Duodenal Atresia Tomas Gudbjartsson, MD, PhD, Saemundur J. Oddsson, MD, and Bjarni Torfason, MD Department of Cardiothoracic Surgery, Landspitali University Hospital, Faculty of Medicine, University of Iceland, Reykjavik, Iceland
FEATURE ARTICLES
We report a rare form of bilateral pulmonary sequestration (PS) that was directly connected to the gastroesophagal junction with a well-formed aberrant bronchus-like structure. An upper gastrointestinal contrast series was performed directly after birth, and a unique PS bronchogram was identified, along with duodenal atresia. The PS was successfully resected and the duodenal atresia was corrected. (Ann Thorac Surg 2007;84:1380 –1) © 2007 by The Society of Thoracic Surgeons
P
ulmonary sequestration (PS) is a rare malformation in which nonfunctioning lung tissue is separated from the normal bronchopulmonary tree and is vascularized by an aberrant systemic artery rather than a branch from the pulmonary artery [1]. The PS is defined as extralobar if the sequestrated lung mass possesses a distinct pleural covering, and it is defined as intrapleural if the pleura is shared with the adjacent normal lung [2]. Most cases present in the first 6 months of life; however, diagnosis of PS can be made by echocardiography in utero [3]. One quarter of all patients present shortly after birth with respiratory distress or feeding difficulties [3]. Older children can present with signs of pneumonia, whereas others present with respiratory symptoms or sometimes congestive heart failure due to a left-to-right shunt through the sequestration mass [4, 5]. Extralobar PS is often associated with coexisting congenital anomalies. Congenital diaphragmatic hernia and other congenital lung abnormalities, such as pulmonary hypoplasia, congenital cystic adenomatoid [6], congenital lobar emphysema [7], or bronchogenic cyst [8] are most common. Other associated anomalies include cardiac anomalies, chest deformities, pericardial defects, and cysts [7]. Rarely an aberrant bronchus connects the PS with the foregut, which is referred to as a communicating bronchopulmonary foregut malformation [9]. We present a case of this complex anomaly that was referred to our institution, which demonstrated a unique bronchogram of the PS.
Ann Thorac Surg 2007;84:1380 –1
A preterm female infant (36 weeks of gestation, 2,800 g) showed signs of major respiratory distress and intestinal obstruction shortly after birth. Cardiac resuscitation was needed and she was intubated to stabilize her condition. An upper gastrointestinal series was performed and revealed a duodenal atresia along with contrast forming a bronchogram in the right lower hemithorax. The contrast reached the lung through a direct passage from the gastroesophagal junction (Fig 1). The diagnosis of PS was confirmed by computed tomography of the chest and an angiography, in which both an aberrant PS artery and vein were noticed (Figs 2A and 2B, respectively). At the age of 5 days the patient underwent a thoracotomy, at which time an extralobar PS of 5.5 ⫻ 4.2 cm was identified in the right lung. It compressed the otherwise normal right lung and was connected to another, but smaller, extrapleural PS of 3.2 ⫻ 1.8 cm in the left hemithorax. Together with the aberrant PS artery (originating from the abdominal aorta), the bronchus and artery divided into branches to both sequestrations after penetrating the diaphragm. Otherwise, the two lung masses were not in direct conjunction, being separated by the bronchial structures and vessels of the PS. The venous return from the sequestrations was through a large vein that drained into the portal vein (Fig 2B). The sequestrations were resected through a unilateral thoracoabdominal incision, and the duodenal stricture was also corrected. Pathologic examination showed that the connection between the stomach and the sequestrations resembled a normal bronchus, both macroscopically and microscopically. There were no respiratory complications postoperatively. The patient was dis-
Accepted for publication May 1, 2007. Address correspondence to Dr Gudbjartsson, Department of Cardiothoracic Surgery, Landspitali University Hospital, IS-101 Reykjavik, 107 Iceland; e-mail: [email protected].
© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. An upper gastrointestinal series showing barium contrast extending from the stomach into the right hemithorax (arrow), forming a unique bronchogram of the pulmonary sequestration. 0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.05.012
Ann Thorac Surg 2007;84:1380 –1
CASE REPORT GUDBJARTSSON ET AL BILATERAL EXTRALOBAR PULMONARY SEQUESTRATION
1381
sidered the operation of choice [4]. However, transcatheter arterial embolization of PS in neonates is an alternative procedure [16]. In conclusion, the present case highlights the complexity of these malformations, clearly demonstrating the direct communication between the PS and the GI tract. Such foregut communication is rare in bilateral PS, especially when associated with duodenal atresia.
charged from the hospital after 2 months and is doing well 4 years later.
Comment Bronchopulmonary foregut malformations are rare. This is especially true for bilateral pulmonary sequestrations communicating with the upper gastrointestinal tract, and there have only been a few cases reported in the literature [10, 11]. We believe that communicating bilateral PS associated with a duodenal atresia has not been previously reported. The atresia in the duodenum caused early symptoms of severe intestinal obstruction and diverted the contrast administered for the upper gastrointestinal series into the PS, forming a unique bronchogram of the PS. Other concomitant anomalies are seen in the majority of patients with PS, and many of these are cardiac. These can have a profound effect on the surgical outcomes and survival of these children [7]. Except for the duodenal atresia, our patient did not have any other associated malformations. Several theories have been suggested to explain the genesis of PS and communicating bronchopulmonary foregut malformations. Earlier theories suggested an infectious origin for PS [12], but most recent evidence supports a congenital origin, including reports of cases that could be detected by antenatal sonography [3]. Today PS is believed to be a result of an abnormal accessory tracheobronchial bud evaginating from the foregut during the 3 to 4 weeks of gestation [13]. Therefore abnormalities in the development of the tracheoesophageal sulcus or septum may explain the persistence of pulmonary structures connected to the GI tract [14]. The cause of duodenal atresia is also complex. It is believed to be a result of failure in a vacuolation process that recanalizes the duodenum after epithelial cells have proliferated and completely plugged the lumen during weeks 7 and 8 of embryogenesis [15]. Both sequestrations were successfully removed through a thoracoabdominal incision, together with correction of the duodenal atresia. In symptomatic PS, surgery is usually recommended, and lobectomy is con-
We thank Adolf Thrainsson, MD, for help in preparing the images.
References 1. Gottrup F, Lund C. Intralobar pulmonary sequestration: a report of 12 cases. Scand J Respir Dis 1978;59:21–9. 2. Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev 2004;5:59 – 61. 3. Walford N, Htun K, Chen J, Liu YY, Teo H, Yeo GSH. Intralobar sequestration of the lung is a congenital anomaly: anatomopathological analysis of four cases diagnosed in fetal life. Pediatr Dev Pathol 2003;6:314 –21. 4. Pikwer A, Gyllstedt E, Lillo-Gil R, Jonsson P, Gudbjartsson T. Pulmonary sequestration–a review of 8 cases treated with lobectomy. Scand J Surg 2006;95:190 – 4. 5. Van Raemdonck D, De Boeck K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001;19:388 –95. 6. Conran RM, Stocker JT. Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 1999;2: 454 – 63. 7. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986;3:106 –21. 8. Freedom RM, Yoo SJ, Goo HW, Mikailian H, Anderson RH. The bronchopulmonary foregut malformation complex. Cardiol Young 2006;16:229 –51. 9. Stallworth J, Campbell B, Carpenter C, Adkins ES. Communicating bronchopulmonary foregut malformation: a case report. JSC Med Assoc 2004;100:278 – 80. 10. Singal AK, Kumar VRR, Rao M, Matthai J. Bilateral communicating bronchopulmonary foregut malformations in a child. Ann Thorac Surg 2006;82:330 –2. 11. Fowler CL, Pokorny WJ, Wanger ML, Kessler MS. Review of bronchopulmonary foregut malformations. J Pediatr Surg 1988;23:793–7. 12. Wilson RL, Lettieri C, Fitzpatrick T, Shorr A. Intralobular bronchopulmonary sequestrations associated with bronchogenic cysts. Respir Med 2005;99:508 –10. 13. Haller JA Jr, Golladay ES, Pickard LR, Tepas JJ 3rd, Shorter NA, Shermeta DW. Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration. Ann Thorac Surg 1979;28:33– 43. 14. Leithiser RE Jr, Capitanio MA, Macpherson RI, Wood BP. “Communicating” bronchopulmonary foregut malformations. AJR Am J Roentgenol 1986;146:227–31. 15. Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y. Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia. J Hepatobiliary Pancreat Surg 1999;6:50 – 4. 16. Lee KH, Sung KB, Yoon HK, Ko GY, Yoon CH, Goo HW. Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results. J Vasc Interv Radiol 2003;14:363–7.
FEATURE ARTICLES
Fig 2. (A) Angiogram showing the large aberrant sequestration artery (arrow) originating from the abdominal aorta. (B) The venous phase of the angiography. The venous blood returns into a large vein (arrow) that drains into the portal vein.
CASE REPORT GUDBJARTSSON ET AL BILATERAL EXTRALOBAR PULMONARY SEQUESTRATION
Bilateral Extralobar Pulmonary Sequestration Communicating With the Upper Gastrointestinal System in a Newborn With Duodenal Atresia Tomas Gudbjartsson, MD, PhD, Saemundur J. Oddsson, MD, and Bjarni Torfason, MD Department of Cardiothoracic Surgery, Landspitali University Hospital, Faculty of Medicine, University of Iceland, Reykjavik, Iceland
FEATURE ARTICLES
We report a rare form of bilateral pulmonary sequestration (PS) that was directly connected to the gastroesophagal junction with a well-formed aberrant bronchus-like structure. An upper gastrointestinal contrast series was performed directly after birth, and a unique PS bronchogram was identified, along with duodenal atresia. The PS was successfully resected and the duodenal atresia was corrected. (Ann Thorac Surg 2007;84:1380 –1) © 2007 by The Society of Thoracic Surgeons
P
ulmonary sequestration (PS) is a rare malformation in which nonfunctioning lung tissue is separated from the normal bronchopulmonary tree and is vascularized by an aberrant systemic artery rather than a branch from the pulmonary artery [1]. The PS is defined as extralobar if the sequestrated lung mass possesses a distinct pleural covering, and it is defined as intrapleural if the pleura is shared with the adjacent normal lung [2]. Most cases present in the first 6 months of life; however, diagnosis of PS can be made by echocardiography in utero [3]. One quarter of all patients present shortly after birth with respiratory distress or feeding difficulties [3]. Older children can present with signs of pneumonia, whereas others present with respiratory symptoms or sometimes congestive heart failure due to a left-to-right shunt through the sequestration mass [4, 5]. Extralobar PS is often associated with coexisting congenital anomalies. Congenital diaphragmatic hernia and other congenital lung abnormalities, such as pulmonary hypoplasia, congenital cystic adenomatoid [6], congenital lobar emphysema [7], or bronchogenic cyst [8] are most common. Other associated anomalies include cardiac anomalies, chest deformities, pericardial defects, and cysts [7]. Rarely an aberrant bronchus connects the PS with the foregut, which is referred to as a communicating bronchopulmonary foregut malformation [9]. We present a case of this complex anomaly that was referred to our institution, which demonstrated a unique bronchogram of the PS.
Ann Thorac Surg 2007;84:1380 –1
A preterm female infant (36 weeks of gestation, 2,800 g) showed signs of major respiratory distress and intestinal obstruction shortly after birth. Cardiac resuscitation was needed and she was intubated to stabilize her condition. An upper gastrointestinal series was performed and revealed a duodenal atresia along with contrast forming a bronchogram in the right lower hemithorax. The contrast reached the lung through a direct passage from the gastroesophagal junction (Fig 1). The diagnosis of PS was confirmed by computed tomography of the chest and an angiography, in which both an aberrant PS artery and vein were noticed (Figs 2A and 2B, respectively). At the age of 5 days the patient underwent a thoracotomy, at which time an extralobar PS of 5.5 ⫻ 4.2 cm was identified in the right lung. It compressed the otherwise normal right lung and was connected to another, but smaller, extrapleural PS of 3.2 ⫻ 1.8 cm in the left hemithorax. Together with the aberrant PS artery (originating from the abdominal aorta), the bronchus and artery divided into branches to both sequestrations after penetrating the diaphragm. Otherwise, the two lung masses were not in direct conjunction, being separated by the bronchial structures and vessels of the PS. The venous return from the sequestrations was through a large vein that drained into the portal vein (Fig 2B). The sequestrations were resected through a unilateral thoracoabdominal incision, and the duodenal stricture was also corrected. Pathologic examination showed that the connection between the stomach and the sequestrations resembled a normal bronchus, both macroscopically and microscopically. There were no respiratory complications postoperatively. The patient was dis-
Accepted for publication May 1, 2007. Address correspondence to Dr Gudbjartsson, Department of Cardiothoracic Surgery, Landspitali University Hospital, IS-101 Reykjavik, 107 Iceland; e-mail: [email protected].
© 2007 by The Society of Thoracic Surgeons Published by Elsevier Inc
Fig 1. An upper gastrointestinal series showing barium contrast extending from the stomach into the right hemithorax (arrow), forming a unique bronchogram of the pulmonary sequestration. 0003-4975/07/$32.00 doi:10.1016/j.athoracsur.2007.05.012
Ann Thorac Surg 2007;84:1380 –1
CASE REPORT GUDBJARTSSON ET AL BILATERAL EXTRALOBAR PULMONARY SEQUESTRATION
1381
sidered the operation of choice [4]. However, transcatheter arterial embolization of PS in neonates is an alternative procedure [16]. In conclusion, the present case highlights the complexity of these malformations, clearly demonstrating the direct communication between the PS and the GI tract. Such foregut communication is rare in bilateral PS, especially when associated with duodenal atresia.
charged from the hospital after 2 months and is doing well 4 years later.
Comment Bronchopulmonary foregut malformations are rare. This is especially true for bilateral pulmonary sequestrations communicating with the upper gastrointestinal tract, and there have only been a few cases reported in the literature [10, 11]. We believe that communicating bilateral PS associated with a duodenal atresia has not been previously reported. The atresia in the duodenum caused early symptoms of severe intestinal obstruction and diverted the contrast administered for the upper gastrointestinal series into the PS, forming a unique bronchogram of the PS. Other concomitant anomalies are seen in the majority of patients with PS, and many of these are cardiac. These can have a profound effect on the surgical outcomes and survival of these children [7]. Except for the duodenal atresia, our patient did not have any other associated malformations. Several theories have been suggested to explain the genesis of PS and communicating bronchopulmonary foregut malformations. Earlier theories suggested an infectious origin for PS [12], but most recent evidence supports a congenital origin, including reports of cases that could be detected by antenatal sonography [3]. Today PS is believed to be a result of an abnormal accessory tracheobronchial bud evaginating from the foregut during the 3 to 4 weeks of gestation [13]. Therefore abnormalities in the development of the tracheoesophageal sulcus or septum may explain the persistence of pulmonary structures connected to the GI tract [14]. The cause of duodenal atresia is also complex. It is believed to be a result of failure in a vacuolation process that recanalizes the duodenum after epithelial cells have proliferated and completely plugged the lumen during weeks 7 and 8 of embryogenesis [15]. Both sequestrations were successfully removed through a thoracoabdominal incision, together with correction of the duodenal atresia. In symptomatic PS, surgery is usually recommended, and lobectomy is con-
We thank Adolf Thrainsson, MD, for help in preparing the images.
References 1. Gottrup F, Lund C. Intralobar pulmonary sequestration: a report of 12 cases. Scand J Respir Dis 1978;59:21–9. 2. Corbett HJ, Humphrey GM. Pulmonary sequestration. Paediatr Respir Rev 2004;5:59 – 61. 3. Walford N, Htun K, Chen J, Liu YY, Teo H, Yeo GSH. Intralobar sequestration of the lung is a congenital anomaly: anatomopathological analysis of four cases diagnosed in fetal life. Pediatr Dev Pathol 2003;6:314 –21. 4. Pikwer A, Gyllstedt E, Lillo-Gil R, Jonsson P, Gudbjartsson T. Pulmonary sequestration–a review of 8 cases treated with lobectomy. Scand J Surg 2006;95:190 – 4. 5. Van Raemdonck D, De Boeck K, Devlieger H, et al. Pulmonary sequestration: a comparison between pediatric and adult patients. Eur J Cardiothorac Surg 2001;19:388 –95. 6. Conran RM, Stocker JT. Extralobar sequestration with frequently associated congenital cystic adenomatoid malformation, type 2: report of 50 cases. Pediatr Dev Pathol 1999;2: 454 – 63. 7. Stocker JT. Sequestrations of the lung. Semin Diagn Pathol 1986;3:106 –21. 8. Freedom RM, Yoo SJ, Goo HW, Mikailian H, Anderson RH. The bronchopulmonary foregut malformation complex. Cardiol Young 2006;16:229 –51. 9. Stallworth J, Campbell B, Carpenter C, Adkins ES. Communicating bronchopulmonary foregut malformation: a case report. JSC Med Assoc 2004;100:278 – 80. 10. Singal AK, Kumar VRR, Rao M, Matthai J. Bilateral communicating bronchopulmonary foregut malformations in a child. Ann Thorac Surg 2006;82:330 –2. 11. Fowler CL, Pokorny WJ, Wanger ML, Kessler MS. Review of bronchopulmonary foregut malformations. J Pediatr Surg 1988;23:793–7. 12. Wilson RL, Lettieri C, Fitzpatrick T, Shorr A. Intralobular bronchopulmonary sequestrations associated with bronchogenic cysts. Respir Med 2005;99:508 –10. 13. Haller JA Jr, Golladay ES, Pickard LR, Tepas JJ 3rd, Shorter NA, Shermeta DW. Surgical management of lung bud anomalies: lobar emphysema, bronchogenic cyst, cystic adenomatoid malformation, and intralobar pulmonary sequestration. Ann Thorac Surg 1979;28:33– 43. 14. Leithiser RE Jr, Capitanio MA, Macpherson RI, Wood BP. “Communicating” bronchopulmonary foregut malformations. AJR Am J Roentgenol 1986;146:227–31. 15. Ando H, Kaneko K, Ito F, Seo T, Harada T, Watanabe Y. Embryogenesis of pancreaticobiliary maljunction inferred from development of duodenal atresia. J Hepatobiliary Pancreat Surg 1999;6:50 – 4. 16. Lee KH, Sung KB, Yoon HK, Ko GY, Yoon CH, Goo HW. Transcatheter arterial embolization of pulmonary sequestration in neonates: long-term follow-up results. J Vasc Interv Radiol 2003;14:363–7.
FEATURE ARTICLES
Fig 2. (A) Angiogram showing the large aberrant sequestration artery (arrow) originating from the abdominal aorta. (B) The venous phase of the angiography. The venous blood returns into a large vein (arrow) that drains into the portal vein.