Expert Review of Respiratory Medicine
ISSN: 1747-6348 (Print) 1747-6356 (Online) Journal homepage: http://www.tandfonline.com/loi/ierx20
Early thoracoscopic resection of an atypical upper extralobar sequestration Virgile Omnès, Jean Stephane Valla, Catherine Desvignes, Frederic Blanc, André Maues de Paula & Pascal de Lagausie To cite this article: Virgile Omnès, Jean Stephane Valla, Catherine Desvignes, Frederic Blanc, André Maues de Paula & Pascal de Lagausie (2014) Early thoracoscopic resection of an atypical upper extralobar sequestration, Expert Review of Respiratory Medicine, 8:6, 673-675 To link to this article: http://dx.doi.org/10.1586/17476348.2014.960400
Published online: 14 Sep 2014.
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Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ierx20 Download by: [University of California, San Diego]
Date: 05 November 2015, At: 13:46
Special Report
Early thoracoscopic resection of an atypical upper extralobar sequestration Downloaded by [University of California, San Diego] at 13:46 05 November 2015
Expert Rev. Respir. Med. 8(6), 673–675 (2014)
Virgile Omne`s1, Jean Stephane Valla2, Catherine Desvignes3, Frederic Blanc4, Andre´ Maues de Paula5 and Pascal de Lagausie*1 1 Department of Paediatric Surgery, Timone Children Hospital, Aix-Marseille University, 264 rue saint apierre, 13385 Marseille, France 2 Department of Paediatric Surgery, Lenval Hospital, Nice, France 3 Department of Paediatric Radiology, Timone Children Hospital, Aix-Marseille University, Marseille, France 4 Department of Paediatric Anaesthesiology, Timone Children Hospital, Aix-Marseille University, Marseille, France 5 Department of Pathology, Timone Children Hospital, Aix-Marseille University, Marseille, France *Author for correspondence: Tel.: +33 491 386 679 Fax: +33 491 385 746 [email protected]
We report a very rare case of complex upper extralobar pulmonary sequestration, detected during pregnancy and resected early because the diagnosis was uncertain (possibility of a teratoma). At 2 months, the upper left pulmonary lesion was completely removed by thoracoscopy. Pathology concluded an extralobar sequestration associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful. The early thoracoscopic approach in this type of antenatally diagnosed lesion was a good option. KEYWORDS: antenatal diagnosis • bronchogenic cyst • oesophageal duplication • pulmonary sequestration • thoracoscopy
Pulmonary sequestrations (PSs) are defined by the presence of a non-functional pulmonary parenchyma with an abnormal vascularization. The incidence of PS is estimated to be between 0.15 and 1.8% [1,2] and represents 0.15–6.45% [3] of bronchopulmonary malformations. The extralobar PS is frequently associated with different congenital anomalies. Here, we describe an antenatal diagnosis of an extralobar PS associated with a duplication cyst of mixed bronchogenic and esophageal type. This lesion was treated by the thoracoscopic approach. Case report
A 2-month-old girl was transferred to our hospital for the treatment of a complex malformation, which was a hyperechogenic pulmonary lesion in the left thorax, discovered during the 33rd week of pregnancy on ultrasound. A fetal thoracic MRI was performed in the 34th week and revealed a polylobed cystic lesion of 24 20 13 mm in the left upper lobe compatible with a congenital pulmonary airway malformation, bronchogenic cyst or angiolymphoma. Pregnancy and delivery were without complication. At 1 month of life, the thoracic MRI found the same polylobed cystic lesion with mediastinal and parenchymal parts (24 35 mm).
informahealthcare.com
10.1586/17476348.2014.960400
One month later, a second preoperative MRI (FIGURE 1A & 1B) showed an increase in tumor size (39 34 34). The main diagnostic hypotheses were a teratocarcinoma because of the heterogeneity (a-feto-protein was normal for age) or an angiolymphoma; a congenital pulmonary airway malformation was unlikely because of the cyst component. A neuroblastoma was excluded by the absence of fixation on MIBG scintigraphy. The 2-month girl underwent a surgical excision of the lesion by thoracoscopy. We used three 5 mm ports. At the top of left thoracic cavity, we found an atypical extralobar PS, with a small systemic artery (2–3 mm) arising from the ascending aorta. This sequestration was associated with a cystic component and surrounded the aorta and extended into the esophagus but without communication. The whole tumor was removed in one piece using Endo Peanuts (Covidien, Courbevoie) and linking the aberrant artery. The pathological examination (FIGURE 2A–2C) showed a cystic unilocular formation with histology similar to that found in the bronchi and the esophagus. This is compatible with an extralobar PS associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful.
2014 Informa UK Ltd
ISSN 1747-6348
673
Special Report
Downloaded by [University of California, San Diego] at 13:46 05 November 2015
A
` s, Valla, Desvignes, Blanc, de Paula & de Lagausie Omne
B
Figure 1. Thoracic MRI. (A) Coronal and (B) transverse. Pulmonary lesion of the left upper lobe with solid and liquid contingent. It is near to the trachea and the left main bronchus, under the aortic arch.
Discussion
We report the fourth case of 2-month-old girl with three different pulmonary malformations in the left upper lobe. The extralobar PS is found in most cases (more than 75%) between the diaphragm and the lower lobe [4,5]. An upper PS is very rare [6].
A
B
C
Between the third and the sixth week of gestation, the foregut divides into dorsal and ventral diverticular buds that become respectively the esophagus and the respiratory tract. A bronchogenic cyst arises from an abnormal bud. Its position depends on the timing of the development of the defect: it will be in the mediastinum if the embryological anomaly occurs early or within the lung tissue if it occurs later [7]. An esophageal duplication also appears early in fetal development and often in close contact with the esophagus. An association of bronchogenic cyst and esophageal duplication is rare, associating two different epitheliums (digestive and pulmonary). Their fusion with sequestration is exceptional. The coexistence of these three malformations in the 33rd week of pregnancy supports a common embryological pathogenesis. It offers evidence for an early embryological anomaly and should follow Gerle’s nomenclature of congenital foregut malformations [8]. There are only three other cases in the literature that report the same association. The first one was that of a 58-year-old woman with a lung lesion found incidentally [9]. A similar lesion was discovered incidentally in a 13-year-old girl [10], treated by thoracoscopy. The last one was a 1-year-old boy with a mediastinal mass found during bronchitis. This lesion was resected by thoracotomy associated with cervicotomy [11]. We report the first case where this lesion was detected antenatally (33rd week of pregnancy) and treated early by thoracoscopy. Because it was impossible in our case to exclude a teratoma, we performed early surgery. Otherwise, thoracoscopic resection of an apical extrapulmonary sequestration in a small infant is feasible and may minimize the morbidity of this procedure [12,13]. The last one was a 1-year-old boy with a mediastinal mass found during bronchitis. This lesion was resected by thoracotomy associated with cervicotomy [11]. Financial & competing interests disclosure
Figure 2. Complex bronchopulmonary foregut malformation. Histologic microphotographs showing different morphologic aspects: (A) pseudostratified ciliated cylindrical epithelium; (B) malpighian epithelium; (C) edematous pulmonary tissue with back to back bronchiolar structures observed in the sequestrated component of the lesion.
674
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript.
Expert Rev. Respir. Med. 8(6), (2014)
Early thoracoscopic resection of an atypical upper extralobar sequestration
References 1.
Downloaded by [University of California, San Diego] at 13:46 05 November 2015
2.
Barbut J, Fernandez C, Blanc F, et al. Pulmonary sequestration of the left upper lobe associated with a bronchogenic cyst: case report of an exceptional association. Pediatr Pulmonol 2011;46(5):509-11
7.
Williams HJ, Johnson KJ. Imaging of congenital cystic lung lesions. Pediatric Respir Rev 2002;3:120-7
8.
Gerle RD, Jaretzki A III, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformation. Pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968;278(26):1413-19
Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lung; a report of seven cases. J Pathol Bacteriol 1946;58(3):457-67 Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979; 34(1):96-101
3.
Halkic N, Cue´noud PF, Corthe´sy ME, et al. Pulmonary sequestration: a review of 26 cases. Eur J Cardiothorac Surg 1998; 14(2):127-33
4.
Joyeux L, Mejean N, Rousseau T, et al. Ectopic extralobar pulmonary sequestrations in children: interest of the laparoscopic approach. J Pediatr Surg 2010;45(11): 2269-73
5.
6.
Corbett HJ, Humphrey GME. Pulmonary sequestration. Paediatr Respir Rev 2004; 5(1):59-68
informahealthcare.com
9.
10.
Lee S, Lee J, Park JH, et al. Extralobar pulmonary sequestration with an associated cyst of mixed bronchogenic and esophageal type–a case report. J Korean Med Sci 1997; 12(6):567-9
Special Report
malformation: extralobar pulmonary sequestration associated with a duplication cyst of mixed bronchogenic and oesophageal type. Pediatr Radiol 2001;31(4):265-8 11.
Yasufuku M, Hatakeyama T, Maeda K, et al. Bronchopulmonary foregut malformation: a large bronchogenic cyst communicating with an esophageal duplication cyst. J Pediatr Surg 2003;38(2):e2
12.
Reinke C, Kherani A, Rice HE. Thoracoscopic resection of an apical extralobar pulmonary sequestration in an infant. J Laparoendosc Adv Surg Tech A 2007;17(2):252-4
13.
Boubnova J, Peycelon M, Garbi O, et al. Thoracoscopy in the management of congenital lung diseases in infancy. Surg Endosc 2011;25(2):593-6
Kim KW, Kim WS, Cheon JE, et al. Complex bronchopulmonary foregut
675
ISSN: 1747-6348 (Print) 1747-6356 (Online) Journal homepage: http://www.tandfonline.com/loi/ierx20
Early thoracoscopic resection of an atypical upper extralobar sequestration Virgile Omnès, Jean Stephane Valla, Catherine Desvignes, Frederic Blanc, André Maues de Paula & Pascal de Lagausie To cite this article: Virgile Omnès, Jean Stephane Valla, Catherine Desvignes, Frederic Blanc, André Maues de Paula & Pascal de Lagausie (2014) Early thoracoscopic resection of an atypical upper extralobar sequestration, Expert Review of Respiratory Medicine, 8:6, 673-675 To link to this article: http://dx.doi.org/10.1586/17476348.2014.960400
Published online: 14 Sep 2014.
Submit your article to this journal
Article views: 11
View related articles
View Crossmark data
Full Terms & Conditions of access and use can be found at http://www.tandfonline.com/action/journalInformation?journalCode=ierx20 Download by: [University of California, San Diego]
Date: 05 November 2015, At: 13:46
Special Report
Early thoracoscopic resection of an atypical upper extralobar sequestration Downloaded by [University of California, San Diego] at 13:46 05 November 2015
Expert Rev. Respir. Med. 8(6), 673–675 (2014)
Virgile Omne`s1, Jean Stephane Valla2, Catherine Desvignes3, Frederic Blanc4, Andre´ Maues de Paula5 and Pascal de Lagausie*1 1 Department of Paediatric Surgery, Timone Children Hospital, Aix-Marseille University, 264 rue saint apierre, 13385 Marseille, France 2 Department of Paediatric Surgery, Lenval Hospital, Nice, France 3 Department of Paediatric Radiology, Timone Children Hospital, Aix-Marseille University, Marseille, France 4 Department of Paediatric Anaesthesiology, Timone Children Hospital, Aix-Marseille University, Marseille, France 5 Department of Pathology, Timone Children Hospital, Aix-Marseille University, Marseille, France *Author for correspondence: Tel.: +33 491 386 679 Fax: +33 491 385 746 [email protected]
We report a very rare case of complex upper extralobar pulmonary sequestration, detected during pregnancy and resected early because the diagnosis was uncertain (possibility of a teratoma). At 2 months, the upper left pulmonary lesion was completely removed by thoracoscopy. Pathology concluded an extralobar sequestration associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful. The early thoracoscopic approach in this type of antenatally diagnosed lesion was a good option. KEYWORDS: antenatal diagnosis • bronchogenic cyst • oesophageal duplication • pulmonary sequestration • thoracoscopy
Pulmonary sequestrations (PSs) are defined by the presence of a non-functional pulmonary parenchyma with an abnormal vascularization. The incidence of PS is estimated to be between 0.15 and 1.8% [1,2] and represents 0.15–6.45% [3] of bronchopulmonary malformations. The extralobar PS is frequently associated with different congenital anomalies. Here, we describe an antenatal diagnosis of an extralobar PS associated with a duplication cyst of mixed bronchogenic and esophageal type. This lesion was treated by the thoracoscopic approach. Case report
A 2-month-old girl was transferred to our hospital for the treatment of a complex malformation, which was a hyperechogenic pulmonary lesion in the left thorax, discovered during the 33rd week of pregnancy on ultrasound. A fetal thoracic MRI was performed in the 34th week and revealed a polylobed cystic lesion of 24 20 13 mm in the left upper lobe compatible with a congenital pulmonary airway malformation, bronchogenic cyst or angiolymphoma. Pregnancy and delivery were without complication. At 1 month of life, the thoracic MRI found the same polylobed cystic lesion with mediastinal and parenchymal parts (24 35 mm).
informahealthcare.com
10.1586/17476348.2014.960400
One month later, a second preoperative MRI (FIGURE 1A & 1B) showed an increase in tumor size (39 34 34). The main diagnostic hypotheses were a teratocarcinoma because of the heterogeneity (a-feto-protein was normal for age) or an angiolymphoma; a congenital pulmonary airway malformation was unlikely because of the cyst component. A neuroblastoma was excluded by the absence of fixation on MIBG scintigraphy. The 2-month girl underwent a surgical excision of the lesion by thoracoscopy. We used three 5 mm ports. At the top of left thoracic cavity, we found an atypical extralobar PS, with a small systemic artery (2–3 mm) arising from the ascending aorta. This sequestration was associated with a cystic component and surrounded the aorta and extended into the esophagus but without communication. The whole tumor was removed in one piece using Endo Peanuts (Covidien, Courbevoie) and linking the aberrant artery. The pathological examination (FIGURE 2A–2C) showed a cystic unilocular formation with histology similar to that found in the bronchi and the esophagus. This is compatible with an extralobar PS associated with a duplication cyst of mixed bronchogenic and esophageal type. The postoperative course was uneventful.
2014 Informa UK Ltd
ISSN 1747-6348
673
Special Report
Downloaded by [University of California, San Diego] at 13:46 05 November 2015
A
` s, Valla, Desvignes, Blanc, de Paula & de Lagausie Omne
B
Figure 1. Thoracic MRI. (A) Coronal and (B) transverse. Pulmonary lesion of the left upper lobe with solid and liquid contingent. It is near to the trachea and the left main bronchus, under the aortic arch.
Discussion
We report the fourth case of 2-month-old girl with three different pulmonary malformations in the left upper lobe. The extralobar PS is found in most cases (more than 75%) between the diaphragm and the lower lobe [4,5]. An upper PS is very rare [6].
A
B
C
Between the third and the sixth week of gestation, the foregut divides into dorsal and ventral diverticular buds that become respectively the esophagus and the respiratory tract. A bronchogenic cyst arises from an abnormal bud. Its position depends on the timing of the development of the defect: it will be in the mediastinum if the embryological anomaly occurs early or within the lung tissue if it occurs later [7]. An esophageal duplication also appears early in fetal development and often in close contact with the esophagus. An association of bronchogenic cyst and esophageal duplication is rare, associating two different epitheliums (digestive and pulmonary). Their fusion with sequestration is exceptional. The coexistence of these three malformations in the 33rd week of pregnancy supports a common embryological pathogenesis. It offers evidence for an early embryological anomaly and should follow Gerle’s nomenclature of congenital foregut malformations [8]. There are only three other cases in the literature that report the same association. The first one was that of a 58-year-old woman with a lung lesion found incidentally [9]. A similar lesion was discovered incidentally in a 13-year-old girl [10], treated by thoracoscopy. The last one was a 1-year-old boy with a mediastinal mass found during bronchitis. This lesion was resected by thoracotomy associated with cervicotomy [11]. We report the first case where this lesion was detected antenatally (33rd week of pregnancy) and treated early by thoracoscopy. Because it was impossible in our case to exclude a teratoma, we performed early surgery. Otherwise, thoracoscopic resection of an apical extrapulmonary sequestration in a small infant is feasible and may minimize the morbidity of this procedure [12,13]. The last one was a 1-year-old boy with a mediastinal mass found during bronchitis. This lesion was resected by thoracotomy associated with cervicotomy [11]. Financial & competing interests disclosure
Figure 2. Complex bronchopulmonary foregut malformation. Histologic microphotographs showing different morphologic aspects: (A) pseudostratified ciliated cylindrical epithelium; (B) malpighian epithelium; (C) edematous pulmonary tissue with back to back bronchiolar structures observed in the sequestrated component of the lesion.
674
The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties. No writing assistance was utilized in the production of this manuscript.
Expert Rev. Respir. Med. 8(6), (2014)
Early thoracoscopic resection of an atypical upper extralobar sequestration
References 1.
Downloaded by [University of California, San Diego] at 13:46 05 November 2015
2.
Barbut J, Fernandez C, Blanc F, et al. Pulmonary sequestration of the left upper lobe associated with a bronchogenic cyst: case report of an exceptional association. Pediatr Pulmonol 2011;46(5):509-11
7.
Williams HJ, Johnson KJ. Imaging of congenital cystic lung lesions. Pediatric Respir Rev 2002;3:120-7
8.
Gerle RD, Jaretzki A III, Ashley CA, Berne AS. Congenital bronchopulmonary-foregut malformation. Pulmonary sequestration communicating with the gastrointestinal tract. N Engl J Med 1968;278(26):1413-19
Pryce DM. Lower accessory pulmonary artery with intralobar sequestration of lung; a report of seven cases. J Pathol Bacteriol 1946;58(3):457-67 Savic B, Birtel FJ, Tholen W, et al. Lung sequestration: report of seven cases and review of 540 published cases. Thorax 1979; 34(1):96-101
3.
Halkic N, Cue´noud PF, Corthe´sy ME, et al. Pulmonary sequestration: a review of 26 cases. Eur J Cardiothorac Surg 1998; 14(2):127-33
4.
Joyeux L, Mejean N, Rousseau T, et al. Ectopic extralobar pulmonary sequestrations in children: interest of the laparoscopic approach. J Pediatr Surg 2010;45(11): 2269-73
5.
6.
Corbett HJ, Humphrey GME. Pulmonary sequestration. Paediatr Respir Rev 2004; 5(1):59-68
informahealthcare.com
9.
10.
Lee S, Lee J, Park JH, et al. Extralobar pulmonary sequestration with an associated cyst of mixed bronchogenic and esophageal type–a case report. J Korean Med Sci 1997; 12(6):567-9
Special Report
malformation: extralobar pulmonary sequestration associated with a duplication cyst of mixed bronchogenic and oesophageal type. Pediatr Radiol 2001;31(4):265-8 11.
Yasufuku M, Hatakeyama T, Maeda K, et al. Bronchopulmonary foregut malformation: a large bronchogenic cyst communicating with an esophageal duplication cyst. J Pediatr Surg 2003;38(2):e2
12.
Reinke C, Kherani A, Rice HE. Thoracoscopic resection of an apical extralobar pulmonary sequestration in an infant. J Laparoendosc Adv Surg Tech A 2007;17(2):252-4
13.
Boubnova J, Peycelon M, Garbi O, et al. Thoracoscopy in the management of congenital lung diseases in infancy. Surg Endosc 2011;25(2):593-6
Kim KW, Kim WS, Cheon JE, et al. Complex bronchopulmonary foregut
675
![[Intradiaphragmatic extralobar pulmonary sequestration].](/assets/img/hold.png)
