Presentation and Surgical Management of Bronchogenic and Esophageal Duplication Cysts in Adults

Presentation and Surgical Management of Bronchogenic and Esophageal Duplication Cysts in Adults* Ugo Cioffi , MD; Luigi Bonavina, MD; Matilde De Simone, MD; Luigi Santambrogio, MD; Gianni Pavoni, MD; Alberto Testori, MD; and Alberto Peracchia, MD

Objective: Bronchogenic and esophageal duplication cysts are congenital anomalies of the tracheobronchial tree and foregut that are often asymptomatic at initial presentation in adults. Surgery is always recommended , even for patients with asymptomatic disease, because of the possible development of symptoms and complications during the natuml course of the disease and because definitive diagnosis can be established only on surgical specimen. Method.
B

ronchogenic and esophageal duplication cysts are congenital anomalies of the bronchial tree and foregut that are often asymptomatic a t presentation in adults. We consider these conditions together because they develop from th e primitive foregut around the fourth to eighth week of gestation and usually present with sim il ar diagnostic and therapeutic problems.l-5 The bronchogenic-type cyst occurs more frequently; it is usually located in the mediastinum around the tracheobronchial tree or within the pulmo naJy parenchyma. Intrapericardial and subdiaPhragmatic locations are also reported. 1•6-8 Most *From the Department of General and Oncologic Surge1y (Drs. Cioffi, Bonavina, an d Peracchia) and the Departm ent of General and Thoracic Surge1y (Drs. De Simone, Santambrogio, Pavoni, and Testo ri ), Ospedale Maggio re Policlini co VIACCS , Un ive rsity of Milan, Italy. Manuscript received April 28, 1997; revision accepted October 31, 1997. Beprint requests: Matilde de Simone, MD, via M. BOI-sa 14, 20151 Milan, Italy 1492

often, the cysts are unilocular rather than multilocular, although they may contain internal trabeculations. The bronchogenic cysts a re c haracterized by a lining of ciliated columnar epithelium and by the presence of cartilage; they are filled with mucoid material. Mediastinal cysts can be classified as esophageal duplications if th ey are close to the esophageal wall, are covered by two muscle layers, and if the lining is squamous, columnar, cuboid, pse udostratified, or ciliated epithelium . Bronchogenic and esophageal duplication cysts can be complicated by intracystic hemorrhage, perforation , and infection, especially those with bronchial and esophageal communication. 9 - 11 Squamous metaplasia may also be presentJ.6.7 ,9- J 1 CT and endoscopic ultrasonography (EUS) allow an accurate study of these lesions. MRI may also play a role when the differential diagnosis from other mediastinal tumors is difficult. Complete surgical excision by thoracotomy or thoracoscopy is the therapy of choice. We present a Clinical Investigations

retrospective review of bronchogenic and esophageal duplication cysts surgically treated in our institution over the last 2 decades.

MATERIALS AND METHODS Between 1976 and 1996, 27 patients with bronchogenic cysts (group 1) and esophageal duplication cys ts (group 2) were admitted to the hospital for surgical therapy. Twenty-two of 27 patients (81.4%) were contacted by telephone intetview, and most of the m agreed to have an office visit and a chest radiograph. Five patients were unavailable for follow-up after 8 to 10 months. Group 1 consisted of 16 patients, 8 male and 8 female, with a mean age of 43 years (range, 24 to 67 years ). Nine patients (56.2%) were symptomatic and complained of chest pain (n=8) and epigastric pain (n = 1). Two patients complained of chest pain and chronic cough. The remaining seven patients were asymptomatic, and the mediastinal mass was incidentally discovered on a routine chest radiograph. Diagnostic studies included standard chest radiograph, barium swallow, and esophagoscopy in all patients. CT scan (Fig 1) and EUS were perform ed in the last 16 and 9 patients, respectively. Fiberoptic bronchoscopy was perform ed in five patients and was normal. MRI was performed in one patient to confirm th e diagnosis and to better characterize the cystic content (Fig 2). Group 2 consisted of 11 patients, 6 male and 5 female, with a mean age of 46 years (range, 16 to 53 years ). Eight patients (72.7%) were symptomatic and complained of dysphagia (n =4), chest pain (n =2), and epigastiic pain (n = 2). The remaining three patients were asymptomatic, and the mass was incidentally discovered on a routine chest radiograph in two patients and on a barium swallow study in one patient. The diagnostic workup included chest radiograph, barium swallow, and esophagoscopy in all cases. CT (Fig 3) was perform ed in all patients. EUS (Fig 4) was performed in the seven most recently observed patients.

RESULTS

Most bronchogenic cysts were located in the right posterior middle mediastinum (10/16), whereas most FIGURE 2. MRI. Top: coronal T 1-weighted image showing a 1ight medi astinal bilobated bronchogenic cyst (10 X5 em) (white arrows) with high signal intensity consistent with high protein content. The black arrow shows the sept of the cyst. Bottom: axial T 2 -weighted image of th e same bronchogenic cys t (arrow) showing decreased signal intensity.

FIGURE l. CT scan showing a retrocardiac hypodense mass in a patient affected by bronchogenic cyst close to the right bronchus .

esophageal duplication cysts were located in the 1ight posterior inferior mediastinum (9/11 ) (Table 1). Table 2 summarizes the results of the chest radiograph, barium swallow, endoscopy, CT, and EUS of bronchogenic and esophageal duplication cysts. The preoperative diagnosis of bronchogenic cysts and esophageal duplication was quite obvious in all 16 patients who where investigated by EUS and CT scan. In two patients and in two additional patients who underwent CT scan alone, the diagnosis of leiomyoma was made because of the high density of CHEST / 113 / 6 / JUNE, 1998

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Table !-Characteristics of 27 Patients with Bronchogenic and Esophageal Duplication Cysts Bronchogenic Esophageal Cysts Duplication Cysts

FIGURE 3. CT scan showing a

retrocardiac hypodense mass arising From th e submucosa of th e e sophagus.

the cyst contents. No patient had transbronchial or fine-needle aspiration biopsy. A complete excision of the cyst was done in 26 cases, whereas a right upper lobectomy was performed in one case with intrapulmonmy cyst (Table 3). A posterolateral thoracotomy was performed in 23 patients, and a video-assisted right thoracoscopy using three port technique was performed in the last 4 patients. All esophageal duplication cysts were excised without opening the esophageal mucosa: the muscle layers of the esophagus were bluntly dissected and in most patients, the edges were approximated by interrupted sutures after removal of the cysts. The diameter of the cysts varied from 2 to 10 em and most contained a clear, jelly-like fluid. Typical histologic findings included a ciliated columnar epithelial lining and a connective tissue wall. Often,

Sex Female Male Age, yr < 30 30-39 40-49 50-59 > 60 Symptoms Epigastri c pain Chest pain Dysphagia Cough o s ymptoms Mass location Upper mediastinum Poste1ior middle mediastinum Postc•io r lower mediastin um Pulmomuy parenchyma

1494

5 6

3 3 6 3

2 3 5 l

0 l 8 0 2 7

2 2 4 0 3

2 8 5

l l

9 0

l

Maximum size, <.:m

<3 3-5 >5

6 9

4 7 0

l

these were infiltrated by inflammatmy cells. Cartilage was always present in the cysts that were external to the esophageal wall.

Table 2-Diagnostic Results Patients With Diagnostic Technique

FIGURE 4. EUS showing a lar ge hypoechoic mass (arrows ) of the wall of th e esophagus in a patient with esophageal duplicati on cyst. A= aorta.

8 8

Characteristics

Chest radiograph E nl arge ment of the mediastinum Pulmonary opacity No abnormalities Bulge with ove rlying Endoscopy normal mucosa Barium swaUow Fi Uing d efect Well-de fin ed CT hypodense mass Not perform ed EUS Homogenous hypoechoic mass in the submucosa Hypechoi c mass adjaee nt to the esophageal wall MRI Coronal : high signal intensity Axial: decreased intensity

Esophageal Bronchogenic Duplication Cysts Cysts

15

4

0 3

7

11

3 16

11 9

0 0

2

9

0

l

0

0

7

Clinical Investigations

Table 3-Surgical Treatment in 27 Patients With Bronchogenic and Esophageal Duplication Cysts

Treatm ent

Approach

Cyst excision

Posterolateral thoracotomy Videothoracoscopy Posterolateral thoracotomy

Right upper lobectomy

Bronchogenic Cysts

Esophageal Duplication Cysts

12

10

3 0

No postoperative morbidity was recorded. The patients operated on with thoracoscopy had a shorter hospital stay compared with those who underwent the thoracotomy. Twenty-six patients were asymptomatic at median follow-up time of 4 years (range, 8 to 77 months ). The chest radiograph, performed in 21 patients, was unremarkable. One patient in group 2 complained of dyspepsia and occasional regurgitation or vomiting. A barium esophagogram showed a pseudodiverticular defect in the distal esophagus corresponding to the site of the excised cyst. The esophagoscopy was normal. Manometry, 24-h esophageal monitoring, and scintigraphy showed an incompetent lower esophageal sphincter with pathologic gastroesophageal reflux and delayed gastric emptying. The patient was treated with histamine-2 blockers and cisapride with good results.

DISCUSSION

The diagnosis of bronchogenic and esophageal duplication cysts was incidental in 37% of our patients. Chest pain was more common in patients with bronchogenic cyst, whereas dysphagia was more common in patients affected by esophageal duplication cyst. In our series, according to previously reported data,2·4 ·.'5· 11 · 12 the more frequent location of the esophageal duplication cysts was the right posteroinferior mediastinum, whereas the bronchogenic cysts were more frequently located in the middle mediastinum. As for the risk of symptomatic compression, the topography of the cysts appears to be more important than their volume; in fact , those located in the upper m ediastinum can produce more compression than those located in the middle and inferior mediastinum .9 However, dysphagia occurs only if the esophageal lumen is appreciably compressed by the cyst. Modern imaging techniques, such as CT, EUS, and MRI may help in excluding malignancy and in evaluating the topographic relationship of the mass in order to plan the most appropriate surgical approach. The CT density of bronchogenic and esoph-

ageal duplication cysts can vary from typical water density (0 to 20 Hounsefield units) to high-density 80 to 90 Hounsefield units. EUS appears to be the best method to diagnose the mediastinal cysts and to verify if an esophageal impression is intramural or extrinsic to the esophageal wall.l·13 We performed EUS with an instrument (Olympus GF3-EUM 3; Huntington Station, NY) utilizing a 7.5 to 12-MHz echoprobe. The high-resolution images obtained with this instrument can determine if the lesion is cystic and can demonstrate its relationship to adjacent structures . MRI can provide additional information that is not available by other noninvasive techniques. The MRI appearance is dependent on the content of the cyst, especially the presence and amount of mucus or other proteinaceus material. 4 · 12 However, CT scan and EUS remain the investigations of choice in most patients. Once a mediastinal mass is suspected based on the chest radiograph or the barium swallow examination, endoscopy should be performed to evaluate the esophageal mucosa. If the mucosa overlying the mass is normal, no biopsy specimens should be taken because they would complicate surgical removal. 14 Although transbronchial and transesophageal needle aspiration have been suggested for both diagnosis and treatment of mediastinal bronchogenic and esophageal duplication cysts in adults,3. 7 ,15,I6 they do not provide useful information for diagnosis and have the potential of infecting the mass. 4 ·12,17 All presumed cysts should be resected because an operation can be hazardous when the cyst becomes symptomatic and because definitive diagnosis can be established only on the surgical specimen . Moreover, recent series3 suggest that most adults with mediastinal cysts develop symptoms and/or complications. In a r eported s eries of 86 patients, St. Georges et aJ1 8 noted that, with prolonged observation, 72% of patients became symptomatic and/or experienced complications. Complete surgical excision is the treatment of choice for bronchogenic and esophageal duplication cysts.l,8,I2,17 .19. 20 Recurrence of mediastinal cysts has been reported after incomplete surgical removaJ.3·7 The conventional surgical approach for removal of the cyst is a posterolateral thoracotomy. Esophageal duplication cysts should be excised by carefully prese1ving the muscle layer. Both vagal nerves should be identified and preserved. Mucosal integrity should be checked intraoperatively by air insufflation through the nasogastric tube. We emphasize the need to approximate the muscle edges of the esophagus after enucleation of the mass. A pseudodiverticulum has occurred once in our experience in an CHEST I 113 I 6 I JUNE, 1998

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area of disorganized muscular anatomy. This event may require surgical correction for relief of dysphagia.2I Recent advances in minimally invasive surgery have led to a less traumatic approach for the treatment of benign mediastinal lesions. The videothoracoscopic technique is effective and as safe as the open surgeiy, 22 -2.5 providing excellent visualization inside the chest without spreading the ribs. Thoracoscopic resection of bronchogenic and esophageal duplication cysts is usually easy because of the hypovascular nature of these masses. Care must be taken to identify both the vagal nerves and the phrenic nerve during the dissection. We believe that during thoracoscopic excision of the mass, as in open surgery, permanent transillumination through the esophagoscope is very useful because it allows the control of the integrity of the esophageal mucosa. The main advantages of the thoracoscopic procedure compared with the open approach are reduced postoperative pain, earlier recovery and hospital discharge of the patient, and a superior cosmetic result. Follow-up is mandatory, especially in patients with esophageal duplication cysts and histmy of gastroesophageal reflux disease. We believe that preservation of the vagal nerves and suturing the muscle edges after complete removal of the esophageal duplication cyst improve the long-term outcome of the operation by preserving the propulsive activity of the esophagus.

CONCLUSIONS

With the introduction of modern imaging techniques, the accuracy of the preoperative differential diagnosis is high but not always possible. Surge1y is mandatory, especially after the introduction of minimally invasive procedures. Observation should be reserved for patients who refuse or are not suitable for thoracic surgery.

REFERENCES

1 Van Dam J, Rice TW, Sivak MV. Endoscopic ultrasonography and endoscopically guided needle aspiration for the diagnosis of upper gastrointestinal tract foregut cysts. Am J Gastroenterol 1992; 87:762-65 2 Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J Thorac Cardiovasc Surg 1995; 109:1003-10 3 Patel SR, Meeker DP, Biscotti CV, et a!. Presentation and manage ment of bronchogenic cysts in the adult. Chest 1994; 106:79-8.5

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4 Suen H-C, Mathisen DJ, Grillo HC, et al. Surgical management and radiological characteristics of bronchogenic cysts. Ann Thorac Surg 1993; 55:476-81 5 Hole mans JA, Rankin SC. Case report: oesophageal duplication cyst causing left lung collapse and hypoperfusion. J Radio! 1995; 68:82-84 6 Bolton JWR , Shahian OM . Asymptomatic bronchogenic cysts: what is the best management ? Ann Thorac Surg 1992; 53:1134-37 7 Gharagozloo F, Dausmann MJ, McReynolds SO, et a!. Recurrent bronchogenic pseudocyst 24 years after incomplete excision: report of a case . Chest 1995; 108:880-83 8 Dagenais F, Nassif E , De1y R, e tal. Bronchogenic cyst of the right he midiaphragm. Ann Thorac Surg 1995; 59:1235-37 9 Ribet ME, Copin MC, Gosselin BH . Bronchogenic cysts of the lung. Ann Thorac Surg 1996; 61:1636-40 10 Coran AG, Drongowski R. Congenital cystic disease of the tracheobronchial tree in infants and children. Arch Surg 1994; 129:521-27 ll Bondestam S, Salo JA, Salonen OLM , et a!. Imaging of congenital esophageal cysts in adults. Gastrointest Radio! 1990; 15:279-81 12 Rafal RB , Markisz JA. Magnetic resonance imaging of an esophageal duplication cyst. Am J Gastroenterol 1991; 86: 1809-ll 13 Ott DJ, Wolfman NT, Wu WC, eta!. Endoscopic ultrasonography of benign esophageal cyst simulating leiomyoma. J Clin Gastroenle rol 1992; 15:85-87 14 Bonavina L, Segalin A, Incarbone H, et a!. Surgical management of leiomyoma and extramucosal cysts of the esophagus. In: Bremner CG , DeMeester TR, Peracchia A, eds. Modem approach to benign esophageal disease. St. Louis: Quality Medical Publishing, 1995: 165-71 15 Ginsberg RJ , Atkins RW, Paulson DL. A bronchogenic cyst successfully treated by mediastinoscopy. Ann Thorac Surg 1972; 13:266-68 16 Carle ns E. Mediastinoscopy: a m ethod for inspection and tissue biopsy in th e superior mediastinum. Dis Chest 19.59; 36:343-52 17 Ursche l JD , Horan TA. Mediastinoscopic treatment of mediastinal cysts. Ann Thorac Surg 1994; 58:1698-1701 18 St.Georges R, Deslauriers J, Duranceau A, e t al. Clinical spectrum of bronchogenic cysts of th e mediastinum and lung in the adult. Ann Thorac Surg 1991; .52:6-13 19 Tarpy SP, Kornfeld H, Moroz K, e t al. Unusual presentation of a large tension bronchogenic cyst in an adult. Thorax 1993; 48:951-52 20 Pierson RN III, Mathisen OJ. Pedicled pericardia! patch repair of a carina! bronchogenic cyst. Ann Thorac Surg 1995; 60:1419-21 21 Bonavina L, Segalin A, Rosati H, et al. Surgical therapy of esophageal leiomyoma. J Am Coli Surg 1995; 181:257-62 22 Sugarbaker OJ. Thoracoscopy in the management of anterior mediastinal masses. Ann Thorac Surg 1993; 56:653-56 23 Halzerigg SR, Landreneau RJ, Mack MJ, e t al. Thoracoscopic resection of mediastinal cysts. Ann Thorac Surg 1993; 56: 659-60 24 Levvis RJ, Caccavale RJ, Sisler GE. Jmaged thoracoscopic surge1y: a n ewthoracic technique for resection of mediastinal cysts. Ann Thorac Surg 1992; 53:318-20 25 AcuffTE, Mack MJ, Ryan WH, eta!. Thoracoscopic excision of bronchogenic cysts. Ann Thorac Surg 1993; 55:196-200

Clinical Investigations