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Congenital Bronchoesophageal Fistula in a Patient With Crohn's Disease: A Cautionary Tale
1776
CASE REPORT DEVBHANDARI ET AL CONGENTIAL BEF IN CROHN’S DISEASE
5. Trastek VF, Pairolero PC, Ceithaml EL, Piehler JM, Payne WS, Bernatz PE. Surgical management of broncholithiasis. J Thorac Cardiovasc Surg 1985;90:842–8. 6. Faber LP, Jensik RJ, Chawla SK, Kittle CF. The surgical implication of broncholithiasis. J Thorac Cardiovasc Surg 1975;70:779 –89. 7. Arrigoni MG, Bernatz PE, Donoghue FE. Broncholithiasis. J Thorac Cardiovasc Surg 1971;62:231–7. 8. Snyder RW, Unger M, Sawicki RW. Bilateral partial bronchial obstruction due to broncholithiasis treated with laser therapy. Chest 1998;113:240 –2.
Congenital Bronchoesophageal Fistula in a Patient With Crohn’s Disease: A Cautionary Tale FEATURE ARTICLES
Mohan P. Devbhandari, FRCS, Lucia Raco, MD, Mark T. Hendrickse, FRCP, and Andrew J. Duncan, FRCS CTh Departments of Cardiothoracic Surgery and Medicine, Gastroenterology Unit, Blackpool Victoria Hospital, Blackpool, United Kingdom
We present the case of a young lady suffering from Crohn’s disease who was diagnosed as having a disabling bronchoesophageal fistula. This was mistakenly thought to be due to the Crohn’s disease, and surgery was declined for fear of further complications. She required multiple admissions for frequent chest infections. At review, 5 years later for a second opinion, the fistula was deemed congenital in origin. The fistula was successfully resected, and the histology confirmed the diagnosis. (Ann Thorac Surg 2005;79:1776 –7) © 2005 by The Society of Thoracic Surgeons
C
ongenital bronchoesophageal fistula (BEF) is a rare congenital anomaly first described by Morton and associates [1] in 1952. Braimbridge and Keith [2] subsequently proposed a system of classification into four types. There are only a few cases of esophageal Crohn’s disease reported in world literature, none causing fistulation into the bronchial tree. We report a case of BEF of congenital origin, which was mistakenly thought to be due to Crohn’s disease. The delay in proper diagnosis led to significant morbidity. A 35-year-old Asian woman was referred to our center in 1998 from a district hospital for surgical repair of a BEF after prolonged illness with frequent respiratory symptoms. Accepted for publication Oct 28, 2003. Address reprint requests to Dr Devbhandari, Department of Cardiothoracic Surgery, Blackpool Victoria Hospital, Blackpool FY3 8NR, UK; e-mail: [email protected].
© 2005 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2005;79:1776 –7
She had undergone an emergency laparotomy and ilio-colectomy in 1987 for severe right iliac fossa pain. She was found to have Crohn’s disease and had been on chronic steroid treatment since then. She presented in 1994 to another hospital with increasing cough associated with eating and lying on the left side. A barium swallow showed a diverticulum in the midesophagus with a fistulous tract to the left bronchial tree. Esophagoscopy confirmed a midesophageal diverticulum with a fistula. Bronchoscopy failed to visualize the opening of the fistula and revealed mucosal irregularity only. The fistula was thought to be due to the Crohn’s disease. She was referred to two esophageal centers for consideration of surgical excision. The attending surgeons believed that the risk of recurrence and further complication in the presence of Crohn’s disease was too high to perform a resection and repair at that stage. She was advised to pursue medical treatment. During the next 5 years, she continued to have frequent symptoms requiring numerous consultations and admissions to the hospital for chest infections. Repeated endoscopy during these visits showed intermittent features of healing and exacerbations of fistula. She became gravely ill toward the end of 1998 with severe sepsis, and hence the treating physician referred her to our center for a second opinion. Repeat esophagoscopy showed a diverticulum passing laterally to the left side at 26 cm with a small orifice at the tip going quite deeply into the mediastinum. On bronchoscopic examination, although the left bronchial tree was erythematous and inflamed, we could not see the site of the fistula. A contrast computed tomographic scan (Fig 1) demonstrated that the fistula was connected to the apical segment of the left lower lobe. Multiple biopsies taken from the esophagus, diverticulum, and stomach showed nonspecific chronic inflammation with no features of Crohn’s disease. Steroids were tapered off, she recovered from the chest infection, and surgery was planned. In January 1999, the patient underwent an operation through a left posterolateral thoracotomy. A 6-mm wide band of tissue running from the esophagus to the area of the apical segment of the left lower lobe was found. On opening this band there was a fistula about 5 mm in diameter lined by epithelium, which was more suggestive of congenital origin than of Crohn’s disease. The left lower lobe was destroyed due to extensive bronchiectatic infective changes. The fistula was divided close to the esophagus and was closed primarily with interrupted 3-0 Vicryl stitches (Ethicon Ltd, Edinburgh, UK). The badly damaged lower lobe was resected. She made an uneventful recovery from the operation. Contrast study with barium swallow showed no leak and she was discharged home on postoperative day 14. She has been followed-up for more than 4 years now, and she has remained very well with no further symptoms. 0003-4975/05/$30.00 doi:10.1016/j.athoracsur.2003.10.088
Fig 1. Computed tomographic scan of the patient. The arrow shows the contrast leaking from the esophagus into the left lower lobe through the bronchoesophageal fistula. Normally there should be no contrast passing in front of the aorta.
Comment The commonest cause of BEF in adults is malignancy arising from the bronchus or esophagus [3]. Benign BEFs are quite rare and the cause for them may be traumatic (ie, penetrating injury or ingestion of corrosives, Boerhaave’s syndrome), infective (ie, tuberculosis, histoplasmosis), inflammatory (ie, Crohn’s disease), or congenital. Congenital BEF results from failed separation of the bronchial tree that forms the esophagus in the initial stages of embryonic development [4]. However, the final site of the fistula in the bronchial tree can be variable depending on the degree of tracheoesophageal separation that occurs before a caudal elongation of the trachea. A fistula can be considered to be of congenital origin if there is no inflammation around the fistula and esophagus, and if there are no infected lymph nodes in the proximity. This should be further supported histologically by the presence of a normal esophageal mucosa and muscularis propria in the fistula. Braimbridge and Keith’s [2] classification of a congenital BEF is widely accepted. Type I fistula is a BEF connected to the tip of an esophageal diverticulum as in our case. Type II is a simple fistula. Type III is connected to a bronchogenic cyst, whereas type IV is connected to a sequestrated lung segment. We believe there has only been one previously reported case of a congenital BEF occurring in a patient with Crohn’s disease [5]. That patient had a mild form of
CASE REPORT DEVBHANDARI ET AL CONGENTIAL BEF IN CROHN’S DISEASE
1777
Crohn’s disease. He was identified to have a BEF of congenital origin before repeated episodes of chest infection occurred and thoracoscopic surgery was possible. Our case had quite severe Crohn’s disease requiring surgery and chronic steroid treatment. It was assumed to be the cause of BEF, and surgery was not offered to the patient. Consequently she presented at a much later stage and required open operation with a formal lobectomy. As a result of diagnostic misdirection she failed to benefit from the correct treatment and had to suffer for 5 years in spite of the fistula being discovered much earlier. This case serves to illustrate the fact that the obvious disease should not always be assumed to be the cause of the patient’s troubles and consideration of other possibilities should be made on the basis of objective evidence. Most congenital BEFs present in adulthood with a persistent unexplained cough. Congenital BEFs are often misdiagnosed as chronic bronchitis or adult onset of asthma. Coughing with meals tends to be a more specific symptom leading to the suspicion of this condition. This is frequently accompanied by productive sputum, fever, and hemoptysis due to recurrent chest infection. If untreated, this culminates in destruction of airway and lung tissue as illustrated in our case. Isolated esophageal diverticula in the absence of fistula tend to remain asymptomatic or presents with pain, dysphagia, hemorrhage, or middle lobe syndrome [6]. Crohn’s disease of the esophagus is quite rare and has been reported in only approximately 20 patients, with only 4 of these patients resulting in BEFs [7]. Most of these patients presented with nonspecific symptoms of heartburn, substernal pain, progressive dysphagia and weight loss. The BEF of congenital origin can occur in a patient with Crohn’s disease, but this is exceedingly rare. We offer a cautionary tale that the obvious disease should not be assumed to be the cause of the patient’s symptoms. This should be supported by objective evidence.
References 1. Morton DR, Osborne JF, Klassen KP. An apparently congenital broncho-esophageal fistula persistent to adult life. J Thorac Surg 1952;19:811. 2. Braimbridge MV, Keith HI. Oesophago-bronchial fistula in the adult. Thorax 1965;20:226 –33. 3. Mangi A, Gaissert H, Wright C, et al. Benign bronchoesophageal fistula in the adult. Ann Thorac Surg 2002;73: 911–5. 4. Smith BD Jr, Mikaelian DO, Cohn HE. Congenital bronchoesophageal fistula in the adult. Ann Otol Thinol Laryngol 1987;96:65–7. 5. Kato H, Yoshikawa M, Saito T, Fukuchi M, Kato R, Kuwano H. Congenital bronchoesophageal fistula with Crohn’s disease in an adult: report of a case. Surg Today 2001;31:446 –9. 6. Reddy ER, Smith I, Clarke H. Esophageal diverticula. J Can Assoc Radiol 1989;40:306 –7. 7. Ghahremani GG, Gore RM, Breuer RI, Larson RH. Esophageal manifestation of Crohn’s disease. Gastrointest Radiol 1982;7:199 –203.
FEATURE ARTICLES
Ann Thorac Surg 2005;79:1776 –7
CASE REPORT DEVBHANDARI ET AL CONGENTIAL BEF IN CROHN’S DISEASE
5. Trastek VF, Pairolero PC, Ceithaml EL, Piehler JM, Payne WS, Bernatz PE. Surgical management of broncholithiasis. J Thorac Cardiovasc Surg 1985;90:842–8. 6. Faber LP, Jensik RJ, Chawla SK, Kittle CF. The surgical implication of broncholithiasis. J Thorac Cardiovasc Surg 1975;70:779 –89. 7. Arrigoni MG, Bernatz PE, Donoghue FE. Broncholithiasis. J Thorac Cardiovasc Surg 1971;62:231–7. 8. Snyder RW, Unger M, Sawicki RW. Bilateral partial bronchial obstruction due to broncholithiasis treated with laser therapy. Chest 1998;113:240 –2.
Congenital Bronchoesophageal Fistula in a Patient With Crohn’s Disease: A Cautionary Tale FEATURE ARTICLES
Mohan P. Devbhandari, FRCS, Lucia Raco, MD, Mark T. Hendrickse, FRCP, and Andrew J. Duncan, FRCS CTh Departments of Cardiothoracic Surgery and Medicine, Gastroenterology Unit, Blackpool Victoria Hospital, Blackpool, United Kingdom
We present the case of a young lady suffering from Crohn’s disease who was diagnosed as having a disabling bronchoesophageal fistula. This was mistakenly thought to be due to the Crohn’s disease, and surgery was declined for fear of further complications. She required multiple admissions for frequent chest infections. At review, 5 years later for a second opinion, the fistula was deemed congenital in origin. The fistula was successfully resected, and the histology confirmed the diagnosis. (Ann Thorac Surg 2005;79:1776 –7) © 2005 by The Society of Thoracic Surgeons
C
ongenital bronchoesophageal fistula (BEF) is a rare congenital anomaly first described by Morton and associates [1] in 1952. Braimbridge and Keith [2] subsequently proposed a system of classification into four types. There are only a few cases of esophageal Crohn’s disease reported in world literature, none causing fistulation into the bronchial tree. We report a case of BEF of congenital origin, which was mistakenly thought to be due to Crohn’s disease. The delay in proper diagnosis led to significant morbidity. A 35-year-old Asian woman was referred to our center in 1998 from a district hospital for surgical repair of a BEF after prolonged illness with frequent respiratory symptoms. Accepted for publication Oct 28, 2003. Address reprint requests to Dr Devbhandari, Department of Cardiothoracic Surgery, Blackpool Victoria Hospital, Blackpool FY3 8NR, UK; e-mail: [email protected].
© 2005 by The Society of Thoracic Surgeons Published by Elsevier Inc
Ann Thorac Surg 2005;79:1776 –7
She had undergone an emergency laparotomy and ilio-colectomy in 1987 for severe right iliac fossa pain. She was found to have Crohn’s disease and had been on chronic steroid treatment since then. She presented in 1994 to another hospital with increasing cough associated with eating and lying on the left side. A barium swallow showed a diverticulum in the midesophagus with a fistulous tract to the left bronchial tree. Esophagoscopy confirmed a midesophageal diverticulum with a fistula. Bronchoscopy failed to visualize the opening of the fistula and revealed mucosal irregularity only. The fistula was thought to be due to the Crohn’s disease. She was referred to two esophageal centers for consideration of surgical excision. The attending surgeons believed that the risk of recurrence and further complication in the presence of Crohn’s disease was too high to perform a resection and repair at that stage. She was advised to pursue medical treatment. During the next 5 years, she continued to have frequent symptoms requiring numerous consultations and admissions to the hospital for chest infections. Repeated endoscopy during these visits showed intermittent features of healing and exacerbations of fistula. She became gravely ill toward the end of 1998 with severe sepsis, and hence the treating physician referred her to our center for a second opinion. Repeat esophagoscopy showed a diverticulum passing laterally to the left side at 26 cm with a small orifice at the tip going quite deeply into the mediastinum. On bronchoscopic examination, although the left bronchial tree was erythematous and inflamed, we could not see the site of the fistula. A contrast computed tomographic scan (Fig 1) demonstrated that the fistula was connected to the apical segment of the left lower lobe. Multiple biopsies taken from the esophagus, diverticulum, and stomach showed nonspecific chronic inflammation with no features of Crohn’s disease. Steroids were tapered off, she recovered from the chest infection, and surgery was planned. In January 1999, the patient underwent an operation through a left posterolateral thoracotomy. A 6-mm wide band of tissue running from the esophagus to the area of the apical segment of the left lower lobe was found. On opening this band there was a fistula about 5 mm in diameter lined by epithelium, which was more suggestive of congenital origin than of Crohn’s disease. The left lower lobe was destroyed due to extensive bronchiectatic infective changes. The fistula was divided close to the esophagus and was closed primarily with interrupted 3-0 Vicryl stitches (Ethicon Ltd, Edinburgh, UK). The badly damaged lower lobe was resected. She made an uneventful recovery from the operation. Contrast study with barium swallow showed no leak and she was discharged home on postoperative day 14. She has been followed-up for more than 4 years now, and she has remained very well with no further symptoms. 0003-4975/05/$30.00 doi:10.1016/j.athoracsur.2003.10.088
Fig 1. Computed tomographic scan of the patient. The arrow shows the contrast leaking from the esophagus into the left lower lobe through the bronchoesophageal fistula. Normally there should be no contrast passing in front of the aorta.
Comment The commonest cause of BEF in adults is malignancy arising from the bronchus or esophagus [3]. Benign BEFs are quite rare and the cause for them may be traumatic (ie, penetrating injury or ingestion of corrosives, Boerhaave’s syndrome), infective (ie, tuberculosis, histoplasmosis), inflammatory (ie, Crohn’s disease), or congenital. Congenital BEF results from failed separation of the bronchial tree that forms the esophagus in the initial stages of embryonic development [4]. However, the final site of the fistula in the bronchial tree can be variable depending on the degree of tracheoesophageal separation that occurs before a caudal elongation of the trachea. A fistula can be considered to be of congenital origin if there is no inflammation around the fistula and esophagus, and if there are no infected lymph nodes in the proximity. This should be further supported histologically by the presence of a normal esophageal mucosa and muscularis propria in the fistula. Braimbridge and Keith’s [2] classification of a congenital BEF is widely accepted. Type I fistula is a BEF connected to the tip of an esophageal diverticulum as in our case. Type II is a simple fistula. Type III is connected to a bronchogenic cyst, whereas type IV is connected to a sequestrated lung segment. We believe there has only been one previously reported case of a congenital BEF occurring in a patient with Crohn’s disease [5]. That patient had a mild form of
CASE REPORT DEVBHANDARI ET AL CONGENTIAL BEF IN CROHN’S DISEASE
1777
Crohn’s disease. He was identified to have a BEF of congenital origin before repeated episodes of chest infection occurred and thoracoscopic surgery was possible. Our case had quite severe Crohn’s disease requiring surgery and chronic steroid treatment. It was assumed to be the cause of BEF, and surgery was not offered to the patient. Consequently she presented at a much later stage and required open operation with a formal lobectomy. As a result of diagnostic misdirection she failed to benefit from the correct treatment and had to suffer for 5 years in spite of the fistula being discovered much earlier. This case serves to illustrate the fact that the obvious disease should not always be assumed to be the cause of the patient’s troubles and consideration of other possibilities should be made on the basis of objective evidence. Most congenital BEFs present in adulthood with a persistent unexplained cough. Congenital BEFs are often misdiagnosed as chronic bronchitis or adult onset of asthma. Coughing with meals tends to be a more specific symptom leading to the suspicion of this condition. This is frequently accompanied by productive sputum, fever, and hemoptysis due to recurrent chest infection. If untreated, this culminates in destruction of airway and lung tissue as illustrated in our case. Isolated esophageal diverticula in the absence of fistula tend to remain asymptomatic or presents with pain, dysphagia, hemorrhage, or middle lobe syndrome [6]. Crohn’s disease of the esophagus is quite rare and has been reported in only approximately 20 patients, with only 4 of these patients resulting in BEFs [7]. Most of these patients presented with nonspecific symptoms of heartburn, substernal pain, progressive dysphagia and weight loss. The BEF of congenital origin can occur in a patient with Crohn’s disease, but this is exceedingly rare. We offer a cautionary tale that the obvious disease should not be assumed to be the cause of the patient’s symptoms. This should be supported by objective evidence.
References 1. Morton DR, Osborne JF, Klassen KP. An apparently congenital broncho-esophageal fistula persistent to adult life. J Thorac Surg 1952;19:811. 2. Braimbridge MV, Keith HI. Oesophago-bronchial fistula in the adult. Thorax 1965;20:226 –33. 3. Mangi A, Gaissert H, Wright C, et al. Benign bronchoesophageal fistula in the adult. Ann Thorac Surg 2002;73: 911–5. 4. Smith BD Jr, Mikaelian DO, Cohn HE. Congenital bronchoesophageal fistula in the adult. Ann Otol Thinol Laryngol 1987;96:65–7. 5. Kato H, Yoshikawa M, Saito T, Fukuchi M, Kato R, Kuwano H. Congenital bronchoesophageal fistula with Crohn’s disease in an adult: report of a case. Surg Today 2001;31:446 –9. 6. Reddy ER, Smith I, Clarke H. Esophageal diverticula. J Can Assoc Radiol 1989;40:306 –7. 7. Ghahremani GG, Gore RM, Breuer RI, Larson RH. Esophageal manifestation of Crohn’s disease. Gastrointest Radiol 1982;7:199 –203.
FEATURE ARTICLES
Ann Thorac Surg 2005;79:1776 –7