- Email: [email protected]
Hard to Swallow
The American Journal of Medicine (2007) 120, 1023-1025
DIAGNOSTIC DILEMMA: THORACIC MEDICINE Charles M. Wiener, MD, Section Editor
Hard to Swallow Madhusudan Grover, MD,a Amit Gupta, MD,a Dianne P. Wagner, MD,a Mark B. Orringer, MD,b a Department of Internal Medicine, Michigan State University, East Lansing, Mich, bSection of Thoracic Surgery, University of Michigan, Ann Arbor, Mich.
PRESENTATION Days after her symptoms began, an 85-year-old woman coughed up the diagnosis. She was in her usual state of health when she presented to her primary care physician with a 2-day history of dysphagia that began with solid food and rapidly progressed to an inability to swallow her own saliva. She had no nausea, vomiting, chest or abdominal pain, or history of reflux. Although her appetite was good, she had lost 7-pounds in the previous 3 months. She recalled being quite ill with fever and chest discomfort 50 years before, at which time she was told that a “lump” in the chest was seen on an x-ray. At that time, she had opted against surgery, and the fever and chest discomfort resolved without recurrence. A medical history also was significant for essential hypertension, hypothyroidism following partial thyroidectomy, osteoporosis, and a benign colon polyp. The patient’s mother and sister had a history of colon cancer. Medications included low-dose aspirin, levothyroxine, an angiotensinconverting enzyme inhibitor, a multivitamin, vitamin D, calcium, and alendronate. The patient had no history of cigarette smoking or alcoholism.
ASSESSMENT A physical examination was normal, as were a complete blood count and serum chemistry panel. An esophagogastroduodenoscopy revealed dilatation of the proximal esophagus and a small amount of retained fluid; narrowing secondary to extrinsic compression in the mid-esophagus; ulceration at the gastroesophageal junction; a small hiatal hernia; and diffuse erythema in the gastric antrum. Biopsies showed esophagitis and antral gastritis with no dysplasia or metaplasia. A chest radiograph showed small bilateral pleural effusions and a possible small retrocardiac hiatal hernia. Requests for reprints should be addressed to Madhusudan Grover, MD, Department of Internal Medicine, B 301, Clinical Center, Michigan State University, East Lansing, MI 48842. E-mail address: [email protected]
0002-9343/$ -see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.amjmed.2007.08.022
A barium esophagogram showed extrinsic compression of the mid-esophagus and what was interpreted as an esophageal perforation that directly communicated with a posterior mediastinal mass (Figure 1). Contrast enhanced computed tomography of the chest confirmed an 8-cm, fluid-filled, subcarinal mass in the posterior mediastinum that communicated with the esophagus through a fistulous tract in the anterior wall (Figure 2).
DIAGNOSIS Shortly after her hospitalization, the patient had a coughing episode, felt something “pop” in her high anterior chest, and regurgitated a brownish liquid. After this, she was able to swallow her saliva again. The imaging findings combined with the remote medical history—retrosternal discomfort associated with febrile illness, chest discomfort, and a chest radiographic abnormality—suggested a long-standing bronchogenic cyst that communicated with the esophageal lumen. It was assumed that the coughing, regurgitation of brownish fluid, and recovered ability to swallow saliva represented sudden decompression of the cyst into the esophagus. Consequently, esophageal compression by the cyst was reduced, and swallowing improved. The patient was advised to resume a mechanical soft diet, which she tolerated well. Eight weeks after the patient’s initial presentation, an elective right posterolateral thoracotomy disclosed an inflamed cystic mass, 7-8 cm in diameter, adhering to the subcarinal soft tissues and communicating with the esophageal lumen through a 4-mm fistulous tract. The fistula was stapled closed, and adjacent esophageal muscle was approximated over the staple line. Pathology on the resected specimen indicated ciliated epithelium of respiratory type that was surrounded with small particles of cartilage and focal thick lymphoid aggregates consistent with a bronchogenic cyst (Figures 3a and 3b). Mediastinal cysts are rare developmental anomalies of the foregut and are usually classified as duplication, bronchogenic, or neurenteric cysts, based primarily on histolog-
1024
The American Journal of Medicine, Vol 120, No 12, December 2007
Figure 1 A barium esophagogram revealed the source of the patient’s symptoms; it is identified with a white arrow.
ical evaluation.1 These anomalies develop during the fourth week of gestation when the foregut divides into the tracheobronchial tree and esophagus. Abnormal sequestration and further multiplication of cells during the process of division gives rise to cystic malformations.2 Bronchogenic cysts are one of the most common bronchopulmonary malformations, accounting for 6% to 15% of mediastinal masses.2 They originate from abnormal budding of the tracheobronchial tree, most commonly developing at
Figure 2 Computed tomography of the chest provided another view of the culprit; the white arrow indicates a fistulous tract.
Figure 3 Histology of the mass showed (A) respiratory epithelium and cartilage (hematoxylin and eosin staining, 4⫻) and (B) respiratory mucosa and mucus glands (hematoxylin and eosin staining, 20⫻).
the level of the tracheal bifurcation or main bronchi. The cyst wall is lined by ciliated pseudostratified columnar epithelium and often contains bronchial mucus glands, smooth muscle, and cartilage.3 Unusual sites are the lung parenchyma, presternal tissues, diaphragm, spine, skin, neck or abdomen.3,4 These cysts can be paraesophageal or intraesophageal. Paraesophageal bronchogenic cysts with esophageal communication are extremely rare.5 Clinical presentations vary widely, depending upon the location of the cyst and the age of the patient. Symptoms, which more commonly afflict children, result from mechanical effects of the cyst and include stridor, cough, and respiratory distress.3,4 In the pediatric population, 75% of bronchogenic cysts are located at or above the hilum, whereas among adults, only 60% of cysts are above the level of the hilum.2 Bronchogenic cysts have generally been thought to be asymptomatic in adults, since they frequently present as incidental radiological findings. However, recent reports suggest that they are, in fact, often symptomatic in adults.6 They may present as recurrent pulmonary infections
Grover et al
Hard To Swallow
due to partial bronchial obstruction or as dysphagia due to compression of the esophagus. Complications, such as infection or hemorrhage within the cyst, can occur.3 Pulmonary artery stenosis, superior vena caval obstruction, pericardial tamponade, arrhythmias, unilateral pulmonary edema, bronchial atresia, and carcinomatous or sarcomatous changes have been reported in adults.4 It is likely that in our patient, pressure necrosis between the cyst and esophageal wall occurred, and the cyst partially decompressed as it contents were regurgitated. The diagnosis is usually made with a combination of imaging and endoscopic modalities. A barium esophagogram can detect a nonspecific, often smooth, rounded, intramural or paraesophageal process that narrows the lumen of the esophagus. A fistulous connection between the cyst and the esophagus might be discovered as well. Esophagogastroduodenoscopy usually reveals a bulge in the esophagus with overlying normal mucosa. The procedure helps to distinguish between submucosal, intramural, and intraluminal tumors. Computed tomography and endoscopic ultrasonography are the imaging tests of choice. Although computed tomography is helpful in evaluating the topographical relationship of the mass in order to plan the most appropriate surgical approach, it cannot definitely exclude a malignancy, as the computed tomography density of a bronchogenic cyst varies from typical water (0-20 Hounsfield units) to soft tissue density (80-90 Hounsfield units).7 Endoscopic ultrasonography accurately differentiates solid and cystic lesions, and helps in determining their relationship with the adjacent structures.8 Nonetheless, definitive diagnosis of a bronchogenic cyst requires excision and pathological evaluation. The differential diagnosis includes esophageal duplication cyst, esophageal leiomyoma, pleural fibroma, granuloma, and lymphadenopathy.9
MANAGEMENT Our patient’s postoperative course was uneventful, and she resumed normal oral intake. Resection, even in asymptomatic patients, has been recommended as the treatment of choice for bronchogenic cysts.10 This eliminates the likelihood of future complications—as occurred in our patient— and rules out malignancy.11 In elderly patients with high surgical risk or asymptomatic patients who refuse surgery, close follow-up with computed tomography or endoscopic ultrasonography might be appropriate, but the risk of complications continues to exist.4,12 Minimally invasive surgery with videothoracosopic technique is less traumatic and
1025 might be as effective as open thoracotomy, but intense inflammatory reaction surrounding a chronically infected bronchogenic cyst might preclude thoracoscopic removal.13 Postoperative outcome is generally favorable, and recurrence after complete resection is exceedingly unusual. Reflux symptoms might occur after resection of distal cysts if their mobilization results in disruption of the lower esophageal sphincter mechanism. When extensive dissection of the musculature is required for cyst removal, esophageal dysmotility might follow, but this is seldom of clinical significance.14 Long-term follow-up is recommended to monitor for these complications.
References 1. Foker EJ, Boyle EMJ. Pediatric disorders: congenital anomalies. In: Pearson FG, Cooper JD, Deslauriers J, et al, eds. Esophageal Surgery. 2nd ed. New York: Churchill Livingstone; 1995:184-199. 2. Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J ThoracCardiovasc Surg. 1995;109:1003-1010. 3. Fraser RB, Pare JAP, Pare PD, et al. Pulmonary abnormalities of developmental origin. In: Diagnosis of diseases of the chest. Vol 2. Philadelphia: WB Saunders; 1989:675-773. 4. Patel SR, Meeker DP, Biscotti CV, et al. Presentation and management of bronchogenic cysts in the adult. Chest. 1994;106:79-85. 5. Knezevic J, Radovanovic N, Simic A, et al. A paraesophageal bronchogenic cyst with esophageal communication. Dis Esophagus. 1999; 12:321-323. 6. St. Georges R, Deslauriers J, Duranceau A, et al. Clinical spectrum of bronchogenic cysts of the mediastinum and lung in the adult. Ann Thorac Surg. 1991;52:6-13. 7. Haddon MJ, Bowen A. Bronchopulmonary and neuroenteric forms of foregut anomalies. Imaging for diagnosis and management. Radiol Clin North Am. 1991;29:241-254. 8. Geller A, Wang KK, DiMagno EP. Diagnosis of foregut duplication cysts by endoscopic ultrasonography. Gastroenterology. 1995;109: 838-842. 9. Cartmill JA, Hughes CF. Bronchogenic cysts: a persistent dilemma. Aust N Z J Surg. 1989;59:253-256. 10. Postlethwait RW, Lowe JE. Benign tumors and cysts of the esophagus. In Zuidema GD, Yeo CJ, Orringer MB, et al, eds. Shackelford’s surgery of the Alimentary Tract. Vol 1.4th ed. Philadelphia: WB Saunders; 1996:369-386. 11. Miralles Lozano F, Gonzalez-Martínez B, Luna More S, Valencia Rodríguez A. Carcinoma arising in a calcified bronchogenic cyst. Respiration. 1981;42:135-137. 12. Kuhlman JE, Fishman EK, Wang KP, et al. Mediastinal cysts: diagnosis by CT and needle aspiration. AJR Am J Roentgenol. 1988;150: 75-78. 13. Watson DI, Britten-Jones R. Thoracoscopic excision of bronchogenic cyst of the esophagus. Surg Endosc. 1995;9:824-825. 14. Van Dam J, Rice TW, Sivak MV Jr. Endoscopic ultrasonography and endoscopically guided needle aspiration for the diagnosis of upper gastrointestinal tract foregut cysts. Am J Gastroenterol. 1992;87(6); 762-765.
DIAGNOSTIC DILEMMA: THORACIC MEDICINE Charles M. Wiener, MD, Section Editor
Hard to Swallow Madhusudan Grover, MD,a Amit Gupta, MD,a Dianne P. Wagner, MD,a Mark B. Orringer, MD,b a Department of Internal Medicine, Michigan State University, East Lansing, Mich, bSection of Thoracic Surgery, University of Michigan, Ann Arbor, Mich.
PRESENTATION Days after her symptoms began, an 85-year-old woman coughed up the diagnosis. She was in her usual state of health when she presented to her primary care physician with a 2-day history of dysphagia that began with solid food and rapidly progressed to an inability to swallow her own saliva. She had no nausea, vomiting, chest or abdominal pain, or history of reflux. Although her appetite was good, she had lost 7-pounds in the previous 3 months. She recalled being quite ill with fever and chest discomfort 50 years before, at which time she was told that a “lump” in the chest was seen on an x-ray. At that time, she had opted against surgery, and the fever and chest discomfort resolved without recurrence. A medical history also was significant for essential hypertension, hypothyroidism following partial thyroidectomy, osteoporosis, and a benign colon polyp. The patient’s mother and sister had a history of colon cancer. Medications included low-dose aspirin, levothyroxine, an angiotensinconverting enzyme inhibitor, a multivitamin, vitamin D, calcium, and alendronate. The patient had no history of cigarette smoking or alcoholism.
ASSESSMENT A physical examination was normal, as were a complete blood count and serum chemistry panel. An esophagogastroduodenoscopy revealed dilatation of the proximal esophagus and a small amount of retained fluid; narrowing secondary to extrinsic compression in the mid-esophagus; ulceration at the gastroesophageal junction; a small hiatal hernia; and diffuse erythema in the gastric antrum. Biopsies showed esophagitis and antral gastritis with no dysplasia or metaplasia. A chest radiograph showed small bilateral pleural effusions and a possible small retrocardiac hiatal hernia. Requests for reprints should be addressed to Madhusudan Grover, MD, Department of Internal Medicine, B 301, Clinical Center, Michigan State University, East Lansing, MI 48842. E-mail address: [email protected]
0002-9343/$ -see front matter © 2007 Elsevier Inc. All rights reserved. doi:10.1016/j.amjmed.2007.08.022
A barium esophagogram showed extrinsic compression of the mid-esophagus and what was interpreted as an esophageal perforation that directly communicated with a posterior mediastinal mass (Figure 1). Contrast enhanced computed tomography of the chest confirmed an 8-cm, fluid-filled, subcarinal mass in the posterior mediastinum that communicated with the esophagus through a fistulous tract in the anterior wall (Figure 2).
DIAGNOSIS Shortly after her hospitalization, the patient had a coughing episode, felt something “pop” in her high anterior chest, and regurgitated a brownish liquid. After this, she was able to swallow her saliva again. The imaging findings combined with the remote medical history—retrosternal discomfort associated with febrile illness, chest discomfort, and a chest radiographic abnormality—suggested a long-standing bronchogenic cyst that communicated with the esophageal lumen. It was assumed that the coughing, regurgitation of brownish fluid, and recovered ability to swallow saliva represented sudden decompression of the cyst into the esophagus. Consequently, esophageal compression by the cyst was reduced, and swallowing improved. The patient was advised to resume a mechanical soft diet, which she tolerated well. Eight weeks after the patient’s initial presentation, an elective right posterolateral thoracotomy disclosed an inflamed cystic mass, 7-8 cm in diameter, adhering to the subcarinal soft tissues and communicating with the esophageal lumen through a 4-mm fistulous tract. The fistula was stapled closed, and adjacent esophageal muscle was approximated over the staple line. Pathology on the resected specimen indicated ciliated epithelium of respiratory type that was surrounded with small particles of cartilage and focal thick lymphoid aggregates consistent with a bronchogenic cyst (Figures 3a and 3b). Mediastinal cysts are rare developmental anomalies of the foregut and are usually classified as duplication, bronchogenic, or neurenteric cysts, based primarily on histolog-
1024
The American Journal of Medicine, Vol 120, No 12, December 2007
Figure 1 A barium esophagogram revealed the source of the patient’s symptoms; it is identified with a white arrow.
ical evaluation.1 These anomalies develop during the fourth week of gestation when the foregut divides into the tracheobronchial tree and esophagus. Abnormal sequestration and further multiplication of cells during the process of division gives rise to cystic malformations.2 Bronchogenic cysts are one of the most common bronchopulmonary malformations, accounting for 6% to 15% of mediastinal masses.2 They originate from abnormal budding of the tracheobronchial tree, most commonly developing at
Figure 2 Computed tomography of the chest provided another view of the culprit; the white arrow indicates a fistulous tract.
Figure 3 Histology of the mass showed (A) respiratory epithelium and cartilage (hematoxylin and eosin staining, 4⫻) and (B) respiratory mucosa and mucus glands (hematoxylin and eosin staining, 20⫻).
the level of the tracheal bifurcation or main bronchi. The cyst wall is lined by ciliated pseudostratified columnar epithelium and often contains bronchial mucus glands, smooth muscle, and cartilage.3 Unusual sites are the lung parenchyma, presternal tissues, diaphragm, spine, skin, neck or abdomen.3,4 These cysts can be paraesophageal or intraesophageal. Paraesophageal bronchogenic cysts with esophageal communication are extremely rare.5 Clinical presentations vary widely, depending upon the location of the cyst and the age of the patient. Symptoms, which more commonly afflict children, result from mechanical effects of the cyst and include stridor, cough, and respiratory distress.3,4 In the pediatric population, 75% of bronchogenic cysts are located at or above the hilum, whereas among adults, only 60% of cysts are above the level of the hilum.2 Bronchogenic cysts have generally been thought to be asymptomatic in adults, since they frequently present as incidental radiological findings. However, recent reports suggest that they are, in fact, often symptomatic in adults.6 They may present as recurrent pulmonary infections
Grover et al
Hard To Swallow
due to partial bronchial obstruction or as dysphagia due to compression of the esophagus. Complications, such as infection or hemorrhage within the cyst, can occur.3 Pulmonary artery stenosis, superior vena caval obstruction, pericardial tamponade, arrhythmias, unilateral pulmonary edema, bronchial atresia, and carcinomatous or sarcomatous changes have been reported in adults.4 It is likely that in our patient, pressure necrosis between the cyst and esophageal wall occurred, and the cyst partially decompressed as it contents were regurgitated. The diagnosis is usually made with a combination of imaging and endoscopic modalities. A barium esophagogram can detect a nonspecific, often smooth, rounded, intramural or paraesophageal process that narrows the lumen of the esophagus. A fistulous connection between the cyst and the esophagus might be discovered as well. Esophagogastroduodenoscopy usually reveals a bulge in the esophagus with overlying normal mucosa. The procedure helps to distinguish between submucosal, intramural, and intraluminal tumors. Computed tomography and endoscopic ultrasonography are the imaging tests of choice. Although computed tomography is helpful in evaluating the topographical relationship of the mass in order to plan the most appropriate surgical approach, it cannot definitely exclude a malignancy, as the computed tomography density of a bronchogenic cyst varies from typical water (0-20 Hounsfield units) to soft tissue density (80-90 Hounsfield units).7 Endoscopic ultrasonography accurately differentiates solid and cystic lesions, and helps in determining their relationship with the adjacent structures.8 Nonetheless, definitive diagnosis of a bronchogenic cyst requires excision and pathological evaluation. The differential diagnosis includes esophageal duplication cyst, esophageal leiomyoma, pleural fibroma, granuloma, and lymphadenopathy.9
MANAGEMENT Our patient’s postoperative course was uneventful, and she resumed normal oral intake. Resection, even in asymptomatic patients, has been recommended as the treatment of choice for bronchogenic cysts.10 This eliminates the likelihood of future complications—as occurred in our patient— and rules out malignancy.11 In elderly patients with high surgical risk or asymptomatic patients who refuse surgery, close follow-up with computed tomography or endoscopic ultrasonography might be appropriate, but the risk of complications continues to exist.4,12 Minimally invasive surgery with videothoracosopic technique is less traumatic and
1025 might be as effective as open thoracotomy, but intense inflammatory reaction surrounding a chronically infected bronchogenic cyst might preclude thoracoscopic removal.13 Postoperative outcome is generally favorable, and recurrence after complete resection is exceedingly unusual. Reflux symptoms might occur after resection of distal cysts if their mobilization results in disruption of the lower esophageal sphincter mechanism. When extensive dissection of the musculature is required for cyst removal, esophageal dysmotility might follow, but this is seldom of clinical significance.14 Long-term follow-up is recommended to monitor for these complications.
References 1. Foker EJ, Boyle EMJ. Pediatric disorders: congenital anomalies. In: Pearson FG, Cooper JD, Deslauriers J, et al, eds. Esophageal Surgery. 2nd ed. New York: Churchill Livingstone; 1995:184-199. 2. Ribet ME, Copin MC, Gosselin B. Bronchogenic cysts of the mediastinum. J ThoracCardiovasc Surg. 1995;109:1003-1010. 3. Fraser RB, Pare JAP, Pare PD, et al. Pulmonary abnormalities of developmental origin. In: Diagnosis of diseases of the chest. Vol 2. Philadelphia: WB Saunders; 1989:675-773. 4. Patel SR, Meeker DP, Biscotti CV, et al. Presentation and management of bronchogenic cysts in the adult. Chest. 1994;106:79-85. 5. Knezevic J, Radovanovic N, Simic A, et al. A paraesophageal bronchogenic cyst with esophageal communication. Dis Esophagus. 1999; 12:321-323. 6. St. Georges R, Deslauriers J, Duranceau A, et al. Clinical spectrum of bronchogenic cysts of the mediastinum and lung in the adult. Ann Thorac Surg. 1991;52:6-13. 7. Haddon MJ, Bowen A. Bronchopulmonary and neuroenteric forms of foregut anomalies. Imaging for diagnosis and management. Radiol Clin North Am. 1991;29:241-254. 8. Geller A, Wang KK, DiMagno EP. Diagnosis of foregut duplication cysts by endoscopic ultrasonography. Gastroenterology. 1995;109: 838-842. 9. Cartmill JA, Hughes CF. Bronchogenic cysts: a persistent dilemma. Aust N Z J Surg. 1989;59:253-256. 10. Postlethwait RW, Lowe JE. Benign tumors and cysts of the esophagus. In Zuidema GD, Yeo CJ, Orringer MB, et al, eds. Shackelford’s surgery of the Alimentary Tract. Vol 1.4th ed. Philadelphia: WB Saunders; 1996:369-386. 11. Miralles Lozano F, Gonzalez-Martínez B, Luna More S, Valencia Rodríguez A. Carcinoma arising in a calcified bronchogenic cyst. Respiration. 1981;42:135-137. 12. Kuhlman JE, Fishman EK, Wang KP, et al. Mediastinal cysts: diagnosis by CT and needle aspiration. AJR Am J Roentgenol. 1988;150: 75-78. 13. Watson DI, Britten-Jones R. Thoracoscopic excision of bronchogenic cyst of the esophagus. Surg Endosc. 1995;9:824-825. 14. Van Dam J, Rice TW, Sivak MV Jr. Endoscopic ultrasonography and endoscopically guided needle aspiration for the diagnosis of upper gastrointestinal tract foregut cysts. Am J Gastroenterol. 1992;87(6); 762-765.