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Dysphagia in a Patient With Recurrent Small-Cell Lung Cancer
Clinical Challenges and Images in GI, continued
Dysphagia in a Patient With Recurrent Small-Cell Lung Cancer William R. Brown1,2 and Elizabeth Dee2 1Department
of Medicine, University of Colorado School of Medicine, and 2Department of Radiology, Denver Health Medical Center, Denver, Colorado
Question: A 67-yearold woman with a neuroendocrine small-cell lung cancer (SCLC), recurrent after chemotherapy and radiation therapy, complained of recent-onset dysphagia. She has noticed regurgitation of liquids and some solid-food dysphagia. She has lost 20 pounds in the past 2 months. She has no past history of gastroesophageal reflux symptoms or esophagitis. Physical examination reveals no significant abnormalities, but she had had a peripheral sensory neuropathy that improved with treatment of her SCLC. Laboratory tests are unremarkable except for a positive Hu immunoglobulin (Ig)G serum anti-neuronal nuclear antibody test, an antibody that is associated with SCLC. On serial computed tomography chest examinations, the esophagus had appeared normal (November, 2010; Figure A, arrow). Two months later, the esophagus was slightly dilated and contained contrast (Figure B; computed tomography). On examinations 5 and 7 months later, the esophagus had become even more dilated and was fluid or gas filled (Figure C, D). At esophagogastroduodenoscopy, the esophagus was diffusely dilated, without contraction, and with retained food and secretions. The endoscope passed into the stomach without much resistance, and no lesion was seen on forward view or retroflexion at the gastroesophageal junction. What is the diagnosis? Look on page 252 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2013 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2012.08.007
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high mortality rate, physicians should be alert to these fatal fistula. If fistula is suspected, a contrast radiological study and direct endoscopic visualization can be employed to establish a diagnosis. Gastrocardiac fistula is a rare cause of upper gastrointestinal bleeding. The majority of diagnoses were made at autopsy. Only aggressive and emergent operative intervention can offer patients a chance of survival because they tend to deteriorate rapidly.1 This case of gastrocardiac fistula occurred after esophagectomy with gastric conduit reconstruction and a pericardiectomy. Immediate surgery is required for life-threatening upper gastrointestinal bleeding if gastrocardiac fistula is suspected. Patient survival is likely after immediate operation. References 1. Pentiak P, Seder CW, Chmielewski GW, et al. Benign postesophagectomy gastrocardiac fistula. Interact Cardiovasc Thorac Surg 2011;13:447– 449. 2. Schouten van der Velden AP, Ruers TJ, Bonenkamp JJ. A cardiogastric fistula after gastric tube interposition. A case report and review of literature. J Surg Oncol 2007;95:79 – 82. 3. Rana ZA, Hosmane VR, Rana NR, et al. Gastro-right ventricular fistula: a deadly complication of a gastric pull-through. Ann Thorac Surg 2010;90:297–299. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 34): Pseudochalasia in Paraneoplastic Syndrome, With Radiographic Documentation of Onset and Evolution A barium esophagogram revealed an esophagus with characteristic features of achalasia: dilatation, retention of air and fluid, and a “bird’s beak” configuration distally (Figure E). Botulinum injection into the distal esophagus provided the patient with partial relief of her swallowing symptoms. Evidence supporting the diagnosis of pseudoachalasia associated with paraneoplastic syndrome in this patient is the (1) development of characteristic features of achalasia in association with SCLC, the cancer that is most often associated with paraneoplastic achalasia1; (2) a serum antibody characteristic of SCLCassociated paraneoplastic syndrome; (3) peripheral neuropathy attributed to paraneoplastic syndrome; and (4) absence of an obstructive neoplasm at the gastroesophageal junction. Pseudoachalasia associated with malignancy may occur by 1 of 3 mechanisms1-3: (1) Primary or secondary carcinoma located at or near the gastroesophageal junction, (2) neural invasion of the esophagus, or (3) as a component of the paraneoplastic syndrome. Pseudoachalasia associated with a paraneoplastic syndrome is rare (an estimated 1 in 750,000), although it may be becoming more common.1 The relatively rapid onset of dysphagia is a reported feature of pseudoachalasia, in contrast with the more gradual onset in primary achalasia. Our report documents radiographically the progression within a few months from a normal-diameter esophagus to a very dilated, poorly functioning esophagus. We know of no similar report. Botulinum toxin injection has been reported effective in a few cases.1 References 1. Katzka DA, Farrugia G, Arora AS. Achalasia secondary to neoplasia: a disease with a changing differential diagnosis. Dis Esophagus 2012;25:331–336.
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2. Liu W, Fackler W, Rice TW, et al. The pathogenesis of pseudoachalasia. A clinicopathological study of 13 cases of a rare entity. Am J Surg Pathol 2002;26:784 –788. 3. Gockel J, Eckardt VF, Scmitt T, et al. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol 2005;40:378 –385. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 4 (page 35): Arteriovenous Fistulas Arising From the Subclavian and Coronary Arteries. We performed aortography along with coronary angiography to find the feeding vessels for the vascular bundle (Supplementary Video 1). There was an arteriovenous fistula that arose from the left subclavian artery, ran over the left mediastinum with the complex plexus, and emptied into the venous system of the left thorax. Multiple coronary artery fistulas originated in the left coronary artery, traversed the left and right mediastinum, and eventually emptied into the venous system of the mediastinum. The left anterior oblique view revealed a coronary artery fistula that arose from the distal right coronary artery and drained into the venous system of the thorax. In transthoracic echocardiography, the sizes of the left atrium and left ventricle were mildly dilated, but left ventricular systolic functions were preserved with an ejection fraction of 61%. We recommended surgery to the patient, but he refused invasive treatment. He will be followed with close observation. A coronary artery fistula is usually of congenital origin, and connects a major coronary artery directly with the cardiac chamber, coronary sinus, superior vena cava, or pulmonary artery. However, its connection with a systemic venous system is extremely rare. Congenital subclavian arteriovenous fistulas are rare because they usually occur as a complication of previous trauma, percutaneous catheterization, or surgery.1 Complications include “steal” from the adjacent myocardium causing myocardial ischemia, thrombosis/ embolism, cardiac failure, atrial fibrillation, rupture, endocarditis/endarteritis, and arrhythmia.2 Treatment options include close medical observation, surgical ligation, and catheter embolization.3
Supplementary Material Note: To access the supplementary material accompanying this article, visit the online version of Gastroenterology at www.gastrojournal.org, and at http://dx.doi.org/10.1053/j.gastro.2012.08.005. References 1. Brountzos EN, Kelekis NL, Danassi-Afentaki D, et al. Congenital subclavian artery-to-subclavian vein fistula in an adult: treatment with transcatheter embolization. Cardiovasc Intervent Radiol 2004;27:675– 677. 2. Wilde P, Watt I. Congenital coronary artery fistulae: six new cases with a collective review. Clin Radiol 1980;31:301–311. 3. Mangukia CV. Coronary artery fistula. Ann Thorac Surg 2012;93:2084 –2092. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Dysphagia in a Patient With Recurrent Small-Cell Lung Cancer William R. Brown1,2 and Elizabeth Dee2 1Department
of Medicine, University of Colorado School of Medicine, and 2Department of Radiology, Denver Health Medical Center, Denver, Colorado
Question: A 67-yearold woman with a neuroendocrine small-cell lung cancer (SCLC), recurrent after chemotherapy and radiation therapy, complained of recent-onset dysphagia. She has noticed regurgitation of liquids and some solid-food dysphagia. She has lost 20 pounds in the past 2 months. She has no past history of gastroesophageal reflux symptoms or esophagitis. Physical examination reveals no significant abnormalities, but she had had a peripheral sensory neuropathy that improved with treatment of her SCLC. Laboratory tests are unremarkable except for a positive Hu immunoglobulin (Ig)G serum anti-neuronal nuclear antibody test, an antibody that is associated with SCLC. On serial computed tomography chest examinations, the esophagus had appeared normal (November, 2010; Figure A, arrow). Two months later, the esophagus was slightly dilated and contained contrast (Figure B; computed tomography). On examinations 5 and 7 months later, the esophagus had become even more dilated and was fluid or gas filled (Figure C, D). At esophagogastroduodenoscopy, the esophagus was diffusely dilated, without contraction, and with retained food and secretions. The endoscope passed into the stomach without much resistance, and no lesion was seen on forward view or retroflexion at the gastroesophageal junction. What is the diagnosis? Look on page 252 for the answer and see the GASTROENTEROLOGY web site (www.gastrojournal.org) for more information on submitting your favorite image to Clinical Challenges and Images in GI. Conflicts of interest: The authors disclose no conflicts. © 2013 by the AGA Institute 0016-5085/$36.00 http://dx.doi.org/10.1053/j.gastro.2012.08.007
34
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high mortality rate, physicians should be alert to these fatal fistula. If fistula is suspected, a contrast radiological study and direct endoscopic visualization can be employed to establish a diagnosis. Gastrocardiac fistula is a rare cause of upper gastrointestinal bleeding. The majority of diagnoses were made at autopsy. Only aggressive and emergent operative intervention can offer patients a chance of survival because they tend to deteriorate rapidly.1 This case of gastrocardiac fistula occurred after esophagectomy with gastric conduit reconstruction and a pericardiectomy. Immediate surgery is required for life-threatening upper gastrointestinal bleeding if gastrocardiac fistula is suspected. Patient survival is likely after immediate operation. References 1. Pentiak P, Seder CW, Chmielewski GW, et al. Benign postesophagectomy gastrocardiac fistula. Interact Cardiovasc Thorac Surg 2011;13:447– 449. 2. Schouten van der Velden AP, Ruers TJ, Bonenkamp JJ. A cardiogastric fistula after gastric tube interposition. A case report and review of literature. J Surg Oncol 2007;95:79 – 82. 3. Rana ZA, Hosmane VR, Rana NR, et al. Gastro-right ventricular fistula: a deadly complication of a gastric pull-through. Ann Thorac Surg 2010;90:297–299. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 3 (page 34): Pseudochalasia in Paraneoplastic Syndrome, With Radiographic Documentation of Onset and Evolution A barium esophagogram revealed an esophagus with characteristic features of achalasia: dilatation, retention of air and fluid, and a “bird’s beak” configuration distally (Figure E). Botulinum injection into the distal esophagus provided the patient with partial relief of her swallowing symptoms. Evidence supporting the diagnosis of pseudoachalasia associated with paraneoplastic syndrome in this patient is the (1) development of characteristic features of achalasia in association with SCLC, the cancer that is most often associated with paraneoplastic achalasia1; (2) a serum antibody characteristic of SCLCassociated paraneoplastic syndrome; (3) peripheral neuropathy attributed to paraneoplastic syndrome; and (4) absence of an obstructive neoplasm at the gastroesophageal junction. Pseudoachalasia associated with malignancy may occur by 1 of 3 mechanisms1-3: (1) Primary or secondary carcinoma located at or near the gastroesophageal junction, (2) neural invasion of the esophagus, or (3) as a component of the paraneoplastic syndrome. Pseudoachalasia associated with a paraneoplastic syndrome is rare (an estimated 1 in 750,000), although it may be becoming more common.1 The relatively rapid onset of dysphagia is a reported feature of pseudoachalasia, in contrast with the more gradual onset in primary achalasia. Our report documents radiographically the progression within a few months from a normal-diameter esophagus to a very dilated, poorly functioning esophagus. We know of no similar report. Botulinum toxin injection has been reported effective in a few cases.1 References 1. Katzka DA, Farrugia G, Arora AS. Achalasia secondary to neoplasia: a disease with a changing differential diagnosis. Dis Esophagus 2012;25:331–336.
January 2013
CLINICAL CHALLENGES AND IMAGES IN GI
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2. Liu W, Fackler W, Rice TW, et al. The pathogenesis of pseudoachalasia. A clinicopathological study of 13 cases of a rare entity. Am J Surg Pathol 2002;26:784 –788. 3. Gockel J, Eckardt VF, Scmitt T, et al. Pseudoachalasia: a case series and analysis of the literature. Scand J Gastroenterol 2005;40:378 –385. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).
Answer to the Clinical Challenges and Images in GI Question: Image 4 (page 35): Arteriovenous Fistulas Arising From the Subclavian and Coronary Arteries. We performed aortography along with coronary angiography to find the feeding vessels for the vascular bundle (Supplementary Video 1). There was an arteriovenous fistula that arose from the left subclavian artery, ran over the left mediastinum with the complex plexus, and emptied into the venous system of the left thorax. Multiple coronary artery fistulas originated in the left coronary artery, traversed the left and right mediastinum, and eventually emptied into the venous system of the mediastinum. The left anterior oblique view revealed a coronary artery fistula that arose from the distal right coronary artery and drained into the venous system of the thorax. In transthoracic echocardiography, the sizes of the left atrium and left ventricle were mildly dilated, but left ventricular systolic functions were preserved with an ejection fraction of 61%. We recommended surgery to the patient, but he refused invasive treatment. He will be followed with close observation. A coronary artery fistula is usually of congenital origin, and connects a major coronary artery directly with the cardiac chamber, coronary sinus, superior vena cava, or pulmonary artery. However, its connection with a systemic venous system is extremely rare. Congenital subclavian arteriovenous fistulas are rare because they usually occur as a complication of previous trauma, percutaneous catheterization, or surgery.1 Complications include “steal” from the adjacent myocardium causing myocardial ischemia, thrombosis/ embolism, cardiac failure, atrial fibrillation, rupture, endocarditis/endarteritis, and arrhythmia.2 Treatment options include close medical observation, surgical ligation, and catheter embolization.3
Supplementary Material Note: To access the supplementary material accompanying this article, visit the online version of Gastroenterology at www.gastrojournal.org, and at http://dx.doi.org/10.1053/j.gastro.2012.08.005. References 1. Brountzos EN, Kelekis NL, Danassi-Afentaki D, et al. Congenital subclavian artery-to-subclavian vein fistula in an adult: treatment with transcatheter embolization. Cardiovasc Intervent Radiol 2004;27:675– 677. 2. Wilde P, Watt I. Congenital coronary artery fistulae: six new cases with a collective review. Clin Radiol 1980;31:301–311. 3. Mangukia CV. Coronary artery fistula. Ann Thorac Surg 2012;93:2084 –2092. For submission instructions, please see the GASTROENTEROLOGY web site (www.gastrojournal.org).