Surg Endosc DOI 10.1007/s00464-015-4082-4
and Other Interventional Techniques
DYNAMIC MANUSCRIPT
Esophageal bronchogenic cyst and review of the literature Maria S. Altieri • Richard Zheng • Aurora D. Pryor Alan Heimann • Soojin Ahn • Dana A. Telem
•
Received: 14 August 2014 / Accepted: 8 January 2015 Ó Springer Science+Business Media New York 2015
Abstract Background Bronchogenic cysts are rare foregut abnormalities that arise from aberrant budding of the tracheobronchial tree early in embryological development. These cysts predominantly appear in the mediastinum, where they may compress nearby structures. Intra-abdominal bronchogenic cysts are rare. We report an intra-abdominal bronchogenic cyst that was excised laparoscopically. Methods A 40-year old female with a history of gastritis presented for evaluation of recurrent abdominal pain. A previous ultrasound showed cholelithiasis and a presumed portal cyst. Physical examination and laboratory findings were unremarkable. A CT scan with pancreatic protocol was performed and an intra-abdominal mass adherent to the esophagus was visualized. A laparascopic enucleation of the mass was performed. A 3-cm myotomy was made after circumferential dissection of the cyst and the decision was made intraoperatively to reapproximate the muscularis layer. A PubMed literature search on surgical management of esophageal bronchogenic cysts was subsequently performed.
Results The literature search performed on the subject of esophageal bronchogenic cysts found one review article focusing on intramural esophageal bronchogenic cysts in the mediastinum and five case reports of esophageal bronchogenic cysts. Of these, only one was both intraabdominal and managed laparascopically with simple closure of the resulting myotomy. The majority of the bronchogenic cysts mentioned in the literature were located mediastinally and were managed via open thoracotomy. Our findings confirm the rarity of this particular presentation and the unique means by which this cyst was surgically excised. Conclusion This case highlights the management of a rare entity and advocates for enucleation of noncommunicating, extraluminal esophageal bronchogenic cysts and closure of the esophageal muscular layers over intact mucosa as a viable surgical approach to this unusual pathology. Other cases of laparascopic enucleation of bronchogenic cysts have shown similarly uneventful postoperative courses and rapid recovery with no apparent return of symptoms. Keywords
Electronic supplementary material The online version of this article (doi:10.1007/s00464-015-4082-4) contains supplementary material, which is available to authorized users. M. S. Altieri (&) R. Zheng A. D. Pryor S. Ahn D. A. Telem Division of Bariatric and Advanced Gastrointestinal Surgery, Department of Surgery, Stony Brook University Medical Center, 100 Nichols Road, HSC T18-040, Stony Brook, NY 11794, USA e-mail: [email protected]; [email protected] A. Heimann Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY, USA
Esophageal General Technical Surgical
Bronchogenic cyst is the most common cystic lesion of the mediastinum. It is a rare benign congenital malformation, which results from an abnormal budding of the primitive foregut. Assessment of demographics demonstrates these lesions to have a female predominance (65.2 %) [1]. The presentation of esophageal bronchogenic cysts in adults is variable. Common presentation includes symptoms such as chest pain, dysphagia, cough, and dyspnea [2]. Diagnosis is made by pathology, as their characteristics of having mucoid material in the cyst, respiratory epithelial lining, and bronchiolar structures in the cyst wall are the
123
Surg Endosc
pathological diagnostic criteria determining a respiratory or enteric origin [3]. The location of the cyst is dependent on the stage of embryogenesis at which budding occurs [4]. The most common location of these cysts is in the middle and superior mediastinum [1]. Completely intra-abdominal lesions are rare, and when they occur most often arise from the esophagus. Although this lesion is often benign, bronchogenic cysts can cause complications via compression of the surrounding structures and malignant degeneration has been described [5]. Surgical resection is curative and the recommended method of treatment. As the majority of cysts are within the mediastinum, most cases are safely performed by thoracotomy or thoracoscopy (see Table 1). The approach to completely intra-abdominal bronchogenic cysts is less defined. The purpose of this study is to describe our experience with a completely intra-abdominal bronchogenic cyst arising from the esophagus and review of the literature with an emphasis on surgical technique of resection. Patient characteristics, location of lesion, method of resection, and outcomes are described.
Case presentation This patient is a 40-year-old woman who presented to our office with complaints of diffuse abdominal pain located mainly in the right upper quadrant, epigastrium, and left upper quadrant. The abdominal pain was not accompanied by nausea or vomiting. She had symptoms of mild dysphagia and pain following meals. She also denied any fevers or weight loss. Her past medical history is significant for gastritis. Physical exams and laboratory values were unremarkable. An ultrasound obtained prior to presentation demonstrated cholelithiasis and a possible portal cyst. Due to financial constraints of the patient, further work-up was limited to computed tomography (CT) scan with pancreatic protocol for evaluation of the portal cyst. CT scan demonstrated a 3-cm low-density lesion along the anterior aspect of the gastroesophageal junction (Fig. 1), which was thought to represent a duplication cyst arising from the distal esophagus or proximal stomach. In addition, an enlarged gallbladder and cholelithiasis were noted. Prior to surgery, an esophagogastroduodenoscopy was performed. No intra-esophageal extension or abnormality was noted. Given the size of the lesion and symptomatology, a decision was made for cholecystectomy with excision of mass. The patient was taken to the operating room and underwent an uncomplicated laparoscopic cyst enucleation with primary closure of the resulting esophageal myotomy and cholecystectomy. For details of the laparoscopic
123
resection and enucleation of bronchogenic cyst and pathology, please refer to the multimedia file. The patient’s intra-operative and postoperative course was uneventful and she was discharged home on postoperative day 1. At the follow-up visit 2 weeks after surgery, the patient had no complaints and was tolerating a regular diet. No esophagogram was performed postoperatively. The final pathology revealed a cystic mass containing bronchial glands in the wall of the cyst and lined by respiratory epithelium. These findings coupled with the lack of communication with the esophageal lumen are consistent with a bronchogenic cyst (Figs. 2, 3).
Discussion Bronchogenic cysts are one of the most common foregut malformations. The lesions are most commonly encountered in the middle and superior mediastinum. Intraabdominal esophageal bronchogenic cysts are rare. A third of these lesions are intra-parenchymal [6]. Although these cysts are commonly encountered close to the esophagus, completely intramural cysts are rare, as only 23 cases have been described in the literature [1]. Our case describes a rare presentation of intra-abdominal intramural esophageal bronchogenic cyst that was safely removed via laparoscopic enucleation. As there is a risk of malignant transformation and increased complications in adulthood, these lesions, even if asymptomatic, are usually removed. Recent review [7] compared presentation in the pediatric versus adult population and showed that whereas in the pediatric population these cysts are usually asymptomatic, 80 % of the adult patients presented with complications, including bronchitis, pneumonia, pericarditis, sepsis, pain, dysphonia, hemoptysis, and dysphagia. In addition, the same review showed that adult patients undergoing resection had greater extent of resection, which translated into longer hospital stay [7]. Therefore, early treatment is recommended to minimize morbidity and cost. Resection in adults is usually performed by thoracotomy or video-assisted thoracoscopic surgery (VATS), although endoscopic and laparoscopic cases have also been described (Table 1). Very few cases are described in the literature that use laparoscopic methods of excision, as these lesions are mostly present in the thorax and are inaccessible during laparoscopy. Only four cases of laparoscopic resection were noted in the literature without accompanying details about the specifics of the procedure [8–11]. During our case, an important consideration had to be made: After enucleation of the mass, a 3-cm myotomy was created with intact underlying mucosa above the gastroesophageal junction. At this stage, various considerations for
1
1
2006
2005
2004
Ko et al. [14]
Melo et al. [11] Hallani et al. [15]
2013
2007
2009
2005
2004
2007
Vannucci et al. [20]
Grover et al. [21]
Diaz Nieto et al. [9]
Pages et al. [22]
Westerterp et al. [23]
Turkyilmaz et al. [1]
2011
2007
Chuang et al. [19]
Chafik et al. [24]
2002
1
1
3
1
1
1
1
1
1
1
2006
Sashiyama et al. [12]
1
2009
Rubin et al. [17] Yasunori et al. [18]
1
2008
Kiral et al. [16]
7
1
2012
Wang et al. [13]
Number patients
Year
Author
51 M
48 M
67 M; 49 F; 49 F
25 F
67 M
85 F
39 F
42 M
34 F
26 M
27F
44 M
64 M
39 F
29.9 (19–60) 6F/1 M
56/M
Age/sex
Thoracotomy and enucleation
R thoracotomy
Transthoracic esophagectomy; Endoscopic Mucosal resection; Local Enucleation
VATS with conversion to open posterolateral thoracotomy
Laparoscopy
R posterolateral thoracotomy
R thoracotomy
L posterolateral thoracotomy
Endoscopy
Thoracoscopy
Left thoracotomy
Left thoracotomy, lower lobectomy
Thoracotomy
Laparoscopy
Thoracotomy/VATS
Posterolateral thoracotomy
Procedure
Table 1 Esophageal bronchogenic cysts: review of the literature
Lower thorax
Distal esophagus
Intra-esophageal, submucosal
Centro-posterior mediastinum, paraesophageal cyst, compressing esophagus w/fistulization into esophageal lumen
Lower esophagus, intra-abdominal, attached to the GE junction
Subcarina, communicating with esophageal lumen
Thoracoabdominal
Middle and lower 1/3 of esophagus
Submucosal cyst in middle third of esophagus
Mediastinal bronchogenic cyst perforating into esophagus Lower esophagus
Paravertebral, communication between cyst and esophagus
Distal esophagus
Gastric fundus
Mid-thoracic/lower thoracic
Lower para-esophageal segment
Region
Enucleation
Enucleation
NS
Mass excised with VATS; converted to open for closure of esophageal wall and creation of an omental flap
NS
Esophageal muscle was approximated over the staple line
NS
NS
Endoscopic mucosal resection; saline w/epinephrine injected below lesion, snare loop ? monopolar cautery used for piecemeal excision
Closure using omentum; transposed through left hemidiaphragm. Closure of myotomy over endoscope
Mass excised, fistula was dissected, cut and oversewn by 3-0vicryl
NS
Wedge resection using stapling device
Total excision
Primary closure
Technique
2 years
6 months
1 year
3 weeks
NS
NS
3 years
2 years
1 month
NS
2 years
3 years
2 years
NS
1–14 years (mean 6 years)
2 years
Follow-up
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Diaphragmatic rupture Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Course
Surg Endosc
123
5 days Mass excised, muscularis reapproximated with 3–0 vicryl sutures Distal esophagus
Uneventful
Uneventful NS Excision Middle mediastinum connected to the esophagus by a tubular esophageal duplication
123
Bold words are highlighted as significant event
M male, F female, NS not specified, VATS video-assisted thoracoscopic surgery, GE gastroesophageal, R right, L Left
Fig. 1 CT scan with pancreatic protocol showing a cystic lesion. Red arrow Cystic lesion (Color figure online)
VATS 46 M 2011 Barbetakis et al. [27]
1
2007 Dae-Woon Eom et al. [26]
1
18 M
NS
Uneventful 6 months Enucleation GE junction 2013 Ballehaninna et al. [8]
1
40 F
Laparoscopy
Chronic chest wall pain NS Mass excised with simple laparoscopy Lesser sac near GE junction 2011 Fernandez et al. [10]
1
33 M
Laparoscopy
Uneventful NS Mass enucleated 2012 Ghobakhlou et al. [25]
1
23 F
Thoracotomy and enucleation
Lower third of esophagus
Course Technique Year Author
Table 1 continued
Number patients
Age/sex
Procedure
Region
Follow-up
Surg Endosc
Fig. 2 Cyst lining demonstrating pseudostratified ciliated columnar respiratory epithelium, respiratory type (hematoxylin and eosin, original magnification, 9600 magnification)
addressing the myotomy could be made. Options included primary closure over a bougie or endoscope, continuation of the myotomy onto the gastroesophageal junction with or without partial fundoplication, or no intervention. For this case, as the muscle was easily approximated over the endoscope without tension, primary repair was chosen. The role of a myotomy in disease process such as achalasia is to reduce the resistance to a food bolus passing through the esophagus into the stomach. However, if there is a complete elimination of pressure, the risk of developing reflux is present. In the case of a myotomy made during enucleation of an esophageal lesion, no current guidelines exist regarding whether the myotomy should be left alone, extended onto the gastroesophageal (GE) junction, or closed primarily. Individual patient consideration
Surg Endosc
complications and have a potential for malignant transformation, surgical resection is recommended. Laparoscopy is a safe approach when such lesions are encountered intra-abdominally. When a myotomy is encountered, patient factors in addition to myotomy length and location should be considered prior to operative decisions.
Disclosure Dr. Dana Telem receives speaking honoraria from Gore, consulting honoraria from Ethicon and has a research funding from Cook. Dr. Aurora Pryor receives speaking honoraria from Gore and consulting honoraria from Freehold Medical. Dr. Maria Altieri, Dr. Alan Heimann, Dr. Soojin Ahn, and Richard Zheng have no conflicts of interest or financial ties to disclose.
Fig. 3 Wall of cyst containing submucosal bronchial type glands (black arrow) and cyst lining composed of respiratory epithelium upper aspect of the photograph (yellow arrow) (hematoxylin and eosin, original magnification, 940 magnification) (Color figure online)
and assessment of length and location of myotomy should be assessed prior to deciding which to perform in order to preserve normal function. If myotomy is more extensive and the surgeon is concerned about lower esophageal sphincter (LES) function and subsequent reflux, the myotomy can be extended onto the GE junction and continued distally for 2–3 cm onto the anterior gastric wall, followed by some variant of partial (Touper or Dor) fundoplication to prevent reflux. Following the procedure, we recommend an endoscopic evaluation to rule out perforation and assess patency of repair. If the myotomy is small and there is no concern about the LES function, it can be left alone or closed primarily. In our case, there was a concern that leaving the myotomy can result in the formation of distal high-pressure zone from an otherwise intact LES, thus leading to an increase esophageal wall stress and diverticulum development, thus primary closure was performed. The postoperative course following resection in the majority of cases is uneventful (Table 1). Only one patient in the thoracotomy group had a diaphragmatic rupture, whereas one patient in the laparoscopy group had chronic chest wall pain postoperatively. Although thoracotomy or thoracoscopy is the therapy of choice, laparoscopic resection is a safe alternative if lesion is amenable to transabdominal excision. Endoscopic transoral resection has been reported as well [12], as those are appropriate for small and asymptomatic lesions.
Conclusion Intramural bronchogenic cyst of the esophagus is a rare malformation. As these lesions can lead to various
References 1. Turkyilmaz A, Eroglu A, Subasi M, Findik G (2007) Intramural esophageal bronchogenic cysts: a review of the literature. Dis Esophagus 20(6):461–465 2. Sarper A, Ayten A, Golbasi I, Demircan A, Isin E (2003) Bronchogenic cyst. Tex Heart Inst J 30(2):105–108 3. Sirivella S, Ford WB, Zikria EA et al (1985) Foregut cysts of the mediastinum. J Thorac Cardiovasc Surg 90(5):776–782 4. Ko S, Hsieh MJ, Lin JW et al (2006) Bronchogenic cyst of the esophagus: clinical and imaging features of seven cases. Clin Imaging 30(5):309–314 5. Okada Y, Mori H, Maeda T, Obashi A, Itoh Y, Doi K (1996) Congenital mediastinal bronchogenic cyst with malignant transformation: an autopsy report. Pathol Int 46(8):594–600 6. Aktogu S, Yuncu G, Halilcolar H et al (1996) Bronchogenic cysts: clinicopathological presentation and treatment. Eur Respir J 9(10):2017–2021 7. Fievet L, D’Journo XB, Guys JM, Thomas PA, De Lagausie P (2012) Bronchogenic cyst: best time for surgery? Ann Thorac Surg 94(5):1695–1700 8. Ballenahinna UK, Shaw JP, Brichkov I (2013) Subdiaphregmatic bronchogenic cyst at the gastroesophageal junction presenting with Dysphagia: a case report. Surg Laparosc Endosc Percutan Tech 23(4): e170–2 9. Diaz Nieto R, Naranjo Torres A, Gomez Alvarez M, Ruiz Rabelo JF et al (2010) Intraabdominal bronchogenic cyst. J Gastrointest Surg 14(4):756–758 10. Fernandez JL, Bauza G, McAneny DB (2011) Minimally invasive management of lesser sac bronchogenic cyst. JSLS 15(4):571–574 11. Melo N, Pitman MB, Rattner DW (2005) Bronchogenic cyst of the gastric fundus presenting as a gastrointestinal stromal tumor. J Laparoendosc Adv Surg Tech 15(2):163–165 12. Sashiyama H, Miyazaki S, Okazaki Y et al (2002) Esophageal bronchogenic cyst successfully excised by endoscopic mucosal resection. Gastrointest Endosc 56(1):141–145 13. Wang W, Ni Y, Zhang L, Li X, Ke C, Lu Q, Cheng Q (2012) A case report of para-esophageal bronchogenic cyst with esophageal communication. J Cardiothorac Surg 7:94 14. Ko SF, Hsieh MJ, Lin JW, Huang CC, Li CC, Cheung YC, Ng SH (2006) Bronchogenic cyst of the esophagus clinical and imagin features of seven cases. Clin Imaging 30(5):309–314 15. Hallani H, Eslick GD, Cox M, Wyatt JM, Lee CH (2004) Chest pain? cause. Lancet 363(9407):452
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Surg Endosc 16. Kiral H, Tezel CS, Kosar A, Keles M (2008) Clinicopathologic demonstration of complex bronchopulmonary foregut malformation. Ann Thoac Surg 85(6):2114–2116 17. Rubin S, Sandu S, Durand E, Baehrel B (2009) Diaphragmatic rupture during labour, two years after an intra-oesophageal rupture of a bronchogenic cyst treated by an omental wrapping. Interact CardioVasc Thorac Surg 9(2):374–376 18. Akutsu Y, Matsubara H, Hayashi H, Okazumi S, Aoki T, Kozu T, Ochiai T (2006) Endoscope-assisted thoracoscopic technique for esophageal bronchogenic cyst which presented elevetated CA 125. Dig Surg 23(4):209–214 19. Chuang KH, Huang TW, Cheng YL et al (2007) Esophageal bronchogenic cyst: a rare entity. Z Gastroenterol 45(9):958–960 20. Vennucci J, Pecoriello R, Tassi V, Ceccarelli S, Puma F (2013) Giant thoracoabdominal esophageal bronchogenic cyst. Dis Esophagus 26(3):340 21. Grover M, Gupta A, Wagner DP, Orringer MB (2007) Hard to swallow. Am J Med 120(12):1023–1025
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22. Pages ON, Rubin S, Baehrel B (2005) Intra-esophageal rupture of a bronchogenic cyst. Interact CardioVasc Thorac Surg 4(4):287–288 23. Westerterp M, van den Berg JG, van Lanschot JJ, Fockens P (2004) Intramural bronchogenic cysts mimicking solid tumors. Endoscopy 36(12):1119–1122 24. Chafik A, Benjelloun A, Qassif H, El Fikri A, El Barni R, Zrara I (2011) Intramural esophageal bronchogenic cysts. Asian Cardiovasc Thorac Ann 19(1):69–71 25. Ghobakhlou M, Fatemi SR, Dezfouli AA, Tirgary F, Zali MR (2012) Long-term dysphagia due to bronchogenic cyst of the esophagus. Endoscopy 44(Suppl 2):E129–E130 26. Eom DW, Kang GH, Kim HW, Ryu DS (2007) Unusual bronchopulmonary foregut malformation associated with pericardial defect: bronchogenic cyst communicating with tubular esophageal duplication. J Korean Med Sci 22(3):564–567 27. Barbetakis N, Asteriou C, Kleontas A, Papadopoulou F, Tsilikas C (2011) Video-assisted thoracoscopic resection of a bronchogenic esophageal cyst. J Minim Access Surg 7(4):249–252
and Other Interventional Techniques
DYNAMIC MANUSCRIPT
Esophageal bronchogenic cyst and review of the literature Maria S. Altieri • Richard Zheng • Aurora D. Pryor Alan Heimann • Soojin Ahn • Dana A. Telem
•
Received: 14 August 2014 / Accepted: 8 January 2015 Ó Springer Science+Business Media New York 2015
Abstract Background Bronchogenic cysts are rare foregut abnormalities that arise from aberrant budding of the tracheobronchial tree early in embryological development. These cysts predominantly appear in the mediastinum, where they may compress nearby structures. Intra-abdominal bronchogenic cysts are rare. We report an intra-abdominal bronchogenic cyst that was excised laparoscopically. Methods A 40-year old female with a history of gastritis presented for evaluation of recurrent abdominal pain. A previous ultrasound showed cholelithiasis and a presumed portal cyst. Physical examination and laboratory findings were unremarkable. A CT scan with pancreatic protocol was performed and an intra-abdominal mass adherent to the esophagus was visualized. A laparascopic enucleation of the mass was performed. A 3-cm myotomy was made after circumferential dissection of the cyst and the decision was made intraoperatively to reapproximate the muscularis layer. A PubMed literature search on surgical management of esophageal bronchogenic cysts was subsequently performed.
Results The literature search performed on the subject of esophageal bronchogenic cysts found one review article focusing on intramural esophageal bronchogenic cysts in the mediastinum and five case reports of esophageal bronchogenic cysts. Of these, only one was both intraabdominal and managed laparascopically with simple closure of the resulting myotomy. The majority of the bronchogenic cysts mentioned in the literature were located mediastinally and were managed via open thoracotomy. Our findings confirm the rarity of this particular presentation and the unique means by which this cyst was surgically excised. Conclusion This case highlights the management of a rare entity and advocates for enucleation of noncommunicating, extraluminal esophageal bronchogenic cysts and closure of the esophageal muscular layers over intact mucosa as a viable surgical approach to this unusual pathology. Other cases of laparascopic enucleation of bronchogenic cysts have shown similarly uneventful postoperative courses and rapid recovery with no apparent return of symptoms. Keywords
Electronic supplementary material The online version of this article (doi:10.1007/s00464-015-4082-4) contains supplementary material, which is available to authorized users. M. S. Altieri (&) R. Zheng A. D. Pryor S. Ahn D. A. Telem Division of Bariatric and Advanced Gastrointestinal Surgery, Department of Surgery, Stony Brook University Medical Center, 100 Nichols Road, HSC T18-040, Stony Brook, NY 11794, USA e-mail: [email protected]; [email protected] A. Heimann Department of Pathology, Stony Brook University Medical Center, Stony Brook, NY, USA
Esophageal General Technical Surgical
Bronchogenic cyst is the most common cystic lesion of the mediastinum. It is a rare benign congenital malformation, which results from an abnormal budding of the primitive foregut. Assessment of demographics demonstrates these lesions to have a female predominance (65.2 %) [1]. The presentation of esophageal bronchogenic cysts in adults is variable. Common presentation includes symptoms such as chest pain, dysphagia, cough, and dyspnea [2]. Diagnosis is made by pathology, as their characteristics of having mucoid material in the cyst, respiratory epithelial lining, and bronchiolar structures in the cyst wall are the
123
Surg Endosc
pathological diagnostic criteria determining a respiratory or enteric origin [3]. The location of the cyst is dependent on the stage of embryogenesis at which budding occurs [4]. The most common location of these cysts is in the middle and superior mediastinum [1]. Completely intra-abdominal lesions are rare, and when they occur most often arise from the esophagus. Although this lesion is often benign, bronchogenic cysts can cause complications via compression of the surrounding structures and malignant degeneration has been described [5]. Surgical resection is curative and the recommended method of treatment. As the majority of cysts are within the mediastinum, most cases are safely performed by thoracotomy or thoracoscopy (see Table 1). The approach to completely intra-abdominal bronchogenic cysts is less defined. The purpose of this study is to describe our experience with a completely intra-abdominal bronchogenic cyst arising from the esophagus and review of the literature with an emphasis on surgical technique of resection. Patient characteristics, location of lesion, method of resection, and outcomes are described.
Case presentation This patient is a 40-year-old woman who presented to our office with complaints of diffuse abdominal pain located mainly in the right upper quadrant, epigastrium, and left upper quadrant. The abdominal pain was not accompanied by nausea or vomiting. She had symptoms of mild dysphagia and pain following meals. She also denied any fevers or weight loss. Her past medical history is significant for gastritis. Physical exams and laboratory values were unremarkable. An ultrasound obtained prior to presentation demonstrated cholelithiasis and a possible portal cyst. Due to financial constraints of the patient, further work-up was limited to computed tomography (CT) scan with pancreatic protocol for evaluation of the portal cyst. CT scan demonstrated a 3-cm low-density lesion along the anterior aspect of the gastroesophageal junction (Fig. 1), which was thought to represent a duplication cyst arising from the distal esophagus or proximal stomach. In addition, an enlarged gallbladder and cholelithiasis were noted. Prior to surgery, an esophagogastroduodenoscopy was performed. No intra-esophageal extension or abnormality was noted. Given the size of the lesion and symptomatology, a decision was made for cholecystectomy with excision of mass. The patient was taken to the operating room and underwent an uncomplicated laparoscopic cyst enucleation with primary closure of the resulting esophageal myotomy and cholecystectomy. For details of the laparoscopic
123
resection and enucleation of bronchogenic cyst and pathology, please refer to the multimedia file. The patient’s intra-operative and postoperative course was uneventful and she was discharged home on postoperative day 1. At the follow-up visit 2 weeks after surgery, the patient had no complaints and was tolerating a regular diet. No esophagogram was performed postoperatively. The final pathology revealed a cystic mass containing bronchial glands in the wall of the cyst and lined by respiratory epithelium. These findings coupled with the lack of communication with the esophageal lumen are consistent with a bronchogenic cyst (Figs. 2, 3).
Discussion Bronchogenic cysts are one of the most common foregut malformations. The lesions are most commonly encountered in the middle and superior mediastinum. Intraabdominal esophageal bronchogenic cysts are rare. A third of these lesions are intra-parenchymal [6]. Although these cysts are commonly encountered close to the esophagus, completely intramural cysts are rare, as only 23 cases have been described in the literature [1]. Our case describes a rare presentation of intra-abdominal intramural esophageal bronchogenic cyst that was safely removed via laparoscopic enucleation. As there is a risk of malignant transformation and increased complications in adulthood, these lesions, even if asymptomatic, are usually removed. Recent review [7] compared presentation in the pediatric versus adult population and showed that whereas in the pediatric population these cysts are usually asymptomatic, 80 % of the adult patients presented with complications, including bronchitis, pneumonia, pericarditis, sepsis, pain, dysphonia, hemoptysis, and dysphagia. In addition, the same review showed that adult patients undergoing resection had greater extent of resection, which translated into longer hospital stay [7]. Therefore, early treatment is recommended to minimize morbidity and cost. Resection in adults is usually performed by thoracotomy or video-assisted thoracoscopic surgery (VATS), although endoscopic and laparoscopic cases have also been described (Table 1). Very few cases are described in the literature that use laparoscopic methods of excision, as these lesions are mostly present in the thorax and are inaccessible during laparoscopy. Only four cases of laparoscopic resection were noted in the literature without accompanying details about the specifics of the procedure [8–11]. During our case, an important consideration had to be made: After enucleation of the mass, a 3-cm myotomy was created with intact underlying mucosa above the gastroesophageal junction. At this stage, various considerations for
1
1
2006
2005
2004
Ko et al. [14]
Melo et al. [11] Hallani et al. [15]
2013
2007
2009
2005
2004
2007
Vannucci et al. [20]
Grover et al. [21]
Diaz Nieto et al. [9]
Pages et al. [22]
Westerterp et al. [23]
Turkyilmaz et al. [1]
2011
2007
Chuang et al. [19]
Chafik et al. [24]
2002
1
1
3
1
1
1
1
1
1
1
2006
Sashiyama et al. [12]
1
2009
Rubin et al. [17] Yasunori et al. [18]
1
2008
Kiral et al. [16]
7
1
2012
Wang et al. [13]
Number patients
Year
Author
51 M
48 M
67 M; 49 F; 49 F
25 F
67 M
85 F
39 F
42 M
34 F
26 M
27F
44 M
64 M
39 F
29.9 (19–60) 6F/1 M
56/M
Age/sex
Thoracotomy and enucleation
R thoracotomy
Transthoracic esophagectomy; Endoscopic Mucosal resection; Local Enucleation
VATS with conversion to open posterolateral thoracotomy
Laparoscopy
R posterolateral thoracotomy
R thoracotomy
L posterolateral thoracotomy
Endoscopy
Thoracoscopy
Left thoracotomy
Left thoracotomy, lower lobectomy
Thoracotomy
Laparoscopy
Thoracotomy/VATS
Posterolateral thoracotomy
Procedure
Table 1 Esophageal bronchogenic cysts: review of the literature
Lower thorax
Distal esophagus
Intra-esophageal, submucosal
Centro-posterior mediastinum, paraesophageal cyst, compressing esophagus w/fistulization into esophageal lumen
Lower esophagus, intra-abdominal, attached to the GE junction
Subcarina, communicating with esophageal lumen
Thoracoabdominal
Middle and lower 1/3 of esophagus
Submucosal cyst in middle third of esophagus
Mediastinal bronchogenic cyst perforating into esophagus Lower esophagus
Paravertebral, communication between cyst and esophagus
Distal esophagus
Gastric fundus
Mid-thoracic/lower thoracic
Lower para-esophageal segment
Region
Enucleation
Enucleation
NS
Mass excised with VATS; converted to open for closure of esophageal wall and creation of an omental flap
NS
Esophageal muscle was approximated over the staple line
NS
NS
Endoscopic mucosal resection; saline w/epinephrine injected below lesion, snare loop ? monopolar cautery used for piecemeal excision
Closure using omentum; transposed through left hemidiaphragm. Closure of myotomy over endoscope
Mass excised, fistula was dissected, cut and oversewn by 3-0vicryl
NS
Wedge resection using stapling device
Total excision
Primary closure
Technique
2 years
6 months
1 year
3 weeks
NS
NS
3 years
2 years
1 month
NS
2 years
3 years
2 years
NS
1–14 years (mean 6 years)
2 years
Follow-up
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Diaphragmatic rupture Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Uneventful
Course
Surg Endosc
123
5 days Mass excised, muscularis reapproximated with 3–0 vicryl sutures Distal esophagus
Uneventful
Uneventful NS Excision Middle mediastinum connected to the esophagus by a tubular esophageal duplication
123
Bold words are highlighted as significant event
M male, F female, NS not specified, VATS video-assisted thoracoscopic surgery, GE gastroesophageal, R right, L Left
Fig. 1 CT scan with pancreatic protocol showing a cystic lesion. Red arrow Cystic lesion (Color figure online)
VATS 46 M 2011 Barbetakis et al. [27]
1
2007 Dae-Woon Eom et al. [26]
1
18 M
NS
Uneventful 6 months Enucleation GE junction 2013 Ballehaninna et al. [8]
1
40 F
Laparoscopy
Chronic chest wall pain NS Mass excised with simple laparoscopy Lesser sac near GE junction 2011 Fernandez et al. [10]
1
33 M
Laparoscopy
Uneventful NS Mass enucleated 2012 Ghobakhlou et al. [25]
1
23 F
Thoracotomy and enucleation
Lower third of esophagus
Course Technique Year Author
Table 1 continued
Number patients
Age/sex
Procedure
Region
Follow-up
Surg Endosc
Fig. 2 Cyst lining demonstrating pseudostratified ciliated columnar respiratory epithelium, respiratory type (hematoxylin and eosin, original magnification, 9600 magnification)
addressing the myotomy could be made. Options included primary closure over a bougie or endoscope, continuation of the myotomy onto the gastroesophageal junction with or without partial fundoplication, or no intervention. For this case, as the muscle was easily approximated over the endoscope without tension, primary repair was chosen. The role of a myotomy in disease process such as achalasia is to reduce the resistance to a food bolus passing through the esophagus into the stomach. However, if there is a complete elimination of pressure, the risk of developing reflux is present. In the case of a myotomy made during enucleation of an esophageal lesion, no current guidelines exist regarding whether the myotomy should be left alone, extended onto the gastroesophageal (GE) junction, or closed primarily. Individual patient consideration
Surg Endosc
complications and have a potential for malignant transformation, surgical resection is recommended. Laparoscopy is a safe approach when such lesions are encountered intra-abdominally. When a myotomy is encountered, patient factors in addition to myotomy length and location should be considered prior to operative decisions.
Disclosure Dr. Dana Telem receives speaking honoraria from Gore, consulting honoraria from Ethicon and has a research funding from Cook. Dr. Aurora Pryor receives speaking honoraria from Gore and consulting honoraria from Freehold Medical. Dr. Maria Altieri, Dr. Alan Heimann, Dr. Soojin Ahn, and Richard Zheng have no conflicts of interest or financial ties to disclose.
Fig. 3 Wall of cyst containing submucosal bronchial type glands (black arrow) and cyst lining composed of respiratory epithelium upper aspect of the photograph (yellow arrow) (hematoxylin and eosin, original magnification, 940 magnification) (Color figure online)
and assessment of length and location of myotomy should be assessed prior to deciding which to perform in order to preserve normal function. If myotomy is more extensive and the surgeon is concerned about lower esophageal sphincter (LES) function and subsequent reflux, the myotomy can be extended onto the GE junction and continued distally for 2–3 cm onto the anterior gastric wall, followed by some variant of partial (Touper or Dor) fundoplication to prevent reflux. Following the procedure, we recommend an endoscopic evaluation to rule out perforation and assess patency of repair. If the myotomy is small and there is no concern about the LES function, it can be left alone or closed primarily. In our case, there was a concern that leaving the myotomy can result in the formation of distal high-pressure zone from an otherwise intact LES, thus leading to an increase esophageal wall stress and diverticulum development, thus primary closure was performed. The postoperative course following resection in the majority of cases is uneventful (Table 1). Only one patient in the thoracotomy group had a diaphragmatic rupture, whereas one patient in the laparoscopy group had chronic chest wall pain postoperatively. Although thoracotomy or thoracoscopy is the therapy of choice, laparoscopic resection is a safe alternative if lesion is amenable to transabdominal excision. Endoscopic transoral resection has been reported as well [12], as those are appropriate for small and asymptomatic lesions.
Conclusion Intramural bronchogenic cyst of the esophagus is a rare malformation. As these lesions can lead to various
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