Unusual presentation of more common disease/injury
CASE REPORT
Dysphagia aortica: a rare cause of dysphagia Siti Rozana Abdul Haziz,1 Ian Bickle,2 Vui Heng Chong1 1
Department of Medicine, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan, Brunei Darussalam 2 Department of Radiology, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan, Brunei Darussalam Correspondence to Dr Vui Heng Chong, [email protected] Accepted 1 September 2015
SUMMARY Dysphagia is uncommon; however, it is considered a warning symptom, especially in the elderly population, and should prompt consideration of a neoplasm of the oesophagus. Dysphagia aortica is rare and often not considered in clinical practice. It is caused by any abnormality of the aorta that results in dysphagia. These include thoracic aortic aneurysm, aortic dissection and even a tortuous aorta. Our case is of an elderly woman, in her early 70s, with a history of treated colorectal carcinoma and hypertension, who presented with progressive dysphagia to solid foods, which, on investigations, showed a tortuous aorta causing compressions of the oesophagus at multiple levels. She was managed with dietary adjustment and optimal control of blood pressure.
BACKGROUND Dysphagia is uncommon; however, it is considered a warning symptom, especially in the elderly population, and should prompt consideration of a neoplasm of the oesophagus. Dysphagia aortica is a rare condition, largely affecting the elderly population, and is often associated with thoracic aorta aneurysm or dissecting aorta, two conditions that are associated with high mortality with or without intervention. Our case highlights the fact that dysphagia aortica can also be due to a tortuous thoracic aorta, thus not requiring surgical intervention, as it can be managed with dietary adjustments and optimal control of blood pressure.
recurrence. Colonoscopy carried out then did not show evidence of recurrence either. Complete physical examination was unremarkable apart from an in situ porta-cath and an ejection systolic murmur without radiation to the carotids. There was no lymphadenopathy or abnormal peripheral pulses, and chest, cardiovascular and abdominal examinations were normal.
INVESTIGATIONS Upper gastrointestinal endoscopy showed prominent impressions with lumen compromised in the mid and lower oesophagus, at the levels of the aortic arch and lower half of the thoracic descending aorta. Despite full insufflation during endoscopy, the impression at the arch persisted. A barium swallow (figure 1) showed compressions of the oesophagus at several levels, including at the arch of the aorta and where the descending aorta crosses over the oesophagus. The findings were consistent with dysphagia aortica. There were no abnormal contractions and no contrast reflux was noted.
CASE PRESENTATION
To cite: Abdul Haziz SR, Bickle I, Chong VH. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2015211726
A woman in her early 70s was referred for evaluation of a 2-month history of dysphagia. She also reported some weight loss. She had a history of hypertension, chronic hepatitis B and colorectal cancer, which was resected followed by adjuvant chemotherapy in 2009. Her dysphagia had been progressive with intermittent worsening, and was only to solid food. The dysphagia was located mainly in the mid and lower chest. By the time she was seen in our clinic, she had already adjusted her diet to semi-solids, which had helped to ease her dysphagia. She did not report any history of gastrooesophageal reflux disorder and denied any associated heartburn or back pain with this current illness. She had been followed up in the general clinic and the National Cancer Centre. Her last appointment with the National Cancer Centre was several months before her symptom started, and a routine surveillance CT scan performed at that time showed no evidence of colorectal cancer
Figure 1 A barium swallow showing compressions of the oesophagus at multiple levels; at the aortic arch and lower third of the oesophagus.
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
1
Unusual presentation of more common disease/injury Blood investigation showed mild anaemia (haemoglobin 10.1 g/dL; normal range 10.9–14.3) with mildly elevated mean corpuscular volume (95.7 fL, normal range 75.5–95.3) with mild leucopenia (3.6×109/L; normal 3.8–11.8), normal platelets count (187×109/L; normal 150–450), mild chronic kidney injury with serum creatinine of 119.2 μmol/L (normal 50.4– 98.1) and urea of 8.5 mmol/L (normal 3.5–7.2). Thyroid profile was normal. A repeat CT scan did not show any mediastinal lymphadenopathies, aneurysm or dissection of the aorta, but showed the aorta taking a left para midline route resulting in prominent impressions seen on barium swallow. There was no aneurysm or dissection. When the patient re-presented several months later with worsening of her dysphagia, a repeat barium swallow was performed. In addition to barium contrast, a bolus food swallow (a piece of bread soaked in contrast) was given and this demonstrated the food bolus being held up at the stricture at the level of the arch of the aorta with precipitation of the dysphagia. The food bolus passed after several swallows with relief of symptom. Again, there were no abnormal contractions and no contrast reflux was noted.
DIFFERENTIAL DIAGNOSIS An upper gastrointestinal endoscopy excluded a diagnosis of oesophageal neoplasm, an important consideration in elderly patients presenting with dysphagia. Barium swallow studies showed several levels of compression of the oesophagus, most marked at the level of the aortic arch. Although not the optimal test, two barium swallow studies did not show any features of motility disorder. A manometry study, not available in our centre, would have been preferred. A CT of the thorax excluded any mediastinal or pulmonary causes of oesophageal compression.
among the elderly, women, those of short statue, and among patients with hypertension or kyphosis.2 Patients with dysphagia aortica usually present with dysphagia to solids but not to liquids, unless the lumen is severely compromised. Dysphagia to liquids is more in keeping with dysmotility ailments. However, as the compression progresses, dysphagia to liquids will follow. Other manifestations secondary to the effect of aortic pathology (ie, aneurysm) such as compression on the phrenic nerve causing diaphragmatic paralysis and gastrointestinal bleeding from an aorto-oesophageal fistula, have also been reported.4 5 Management includes treating the underlying causes. However, underlying causes of such dissecting aortas or aneurysms are associated with the risk of catastrophic events, and treatment options are often also high risk. Management of the underlying pathologies includes surgical repairs, which will relieve the dysphagia or intravascular stenting but will not relieve the dysphagia. Fortunately, in our patient, the dysphagia was due to a tortuous thoracic aorta, which was managed conservatively with diet modification in the form of a soft diet with control of blood pressure. However, in the earlier follow-up after diagnosis of aortica dysphagia, our patient had intermittent worsening of dysphagia, most likely due to self-limiting food bolus impactions. This was clearly demonstrated in her second barium study, when she was also given contrast soaked bread, which brought up her dysphagia when the solid bolus was held up in the aortic arch compression. Since then, the patient largely kept to a soft diet and took adequate fluid with each meal; this has led to marked reduction in her dysphagia. In conclusion, our case highlights the need to consider dysphagia aortica in elderly patient with a history of hypertension, presenting with dysphagia. Dysphagia aortica has often been associated with significant pathology such as dissection or aneurysm of the aorta. A tortuous thoracic aorta can also cause dysphagia aortica.
TREATMENT The patient was managed with a soft diet and optimal control of blood pressure (nifedipine modified release 90 mg daily, bendrofluazide 2.5 mg daily, doxazosin 4 mg nocte and candesartan 12 mg daily), and she has remained well without worsening of her dysphagia. She was also given intermittent courses of oral prokinetic (domperidone 10 mg three times a day).
Learning points ▸ Dysphagia is an important warning symptom especially in the elderly; not all cases are due to underlying neoplasms. ▸ Dysphagia aortica needs to be considered in patients with a long history of hypertension and, in most cases, has been reported to be associated with aortic aneurysm or dissecting aorta. ▸ Dysphagia aortica associated with a non-aneurysmal tortuous aorta can be managed conservatively with optimal blood pressure control and adaptation of diet.
OUTCOME AND FOLLOW-UP The patient’s symptoms improved significantly but she continued to have intermittent transient dysphagia, especially when taking large boluses of solid food.
DISCUSSION In clinical practice, dysphagia is an uncommon symptom compared with other gastrointestinal symptoms in the elderly population; however, when dysphagia occurs, a neoplasm of the oesophagus needs to be considered. Other common causes of dysphagia in the elderly are motility disorder or gastrooesophageal reflux. Dysphagia aortica is a rare condition caused by extrinsic compressions of the oesophagus by the aorta; it can be ectatic, an aneurysm (true or pseudo), or a dissecting or even tortuous aorta.1 2 Aortic dissection and aneurysm are serious causes of dysphagia aortica.2 Another less common vascular cause of dysphagia is dysphagia lusoria, which is due to abnormal subclavian take off resulting in a vascular ring around the oesophagus, typically at the proximal third.3 Dysphagia aortica is more common 2
Contributors SRAH wrote the first draft and approved the final draft. IB was involved in the care of the patient, provided radiological imaging and legend, and approved the final draft. VHC looked after the patient, conceived the idea of the case report, and co-wrote and approved the final draft. Competing interests None declared. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2
Mittal RK, Siskind BN, Hongo M, et al. Dysphagia aortica. Clinical, radiological, and manometric findings. Dig Dis Sci 1986;31:379–84. Cao DB, Gao Y, Sun XY, et al. Dysphagia aortica secondary to descending thoracic aortic pseudoaneurysm. Ann Thorac Surg 2012;94:656.
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
Unusual presentation of more common disease/injury 3
de Araújo G, Junqueira Bizzi JW, Muller J, et al. “Dysphagia lusoria”—Right subclavian retroesophageal artery causing intermitent esophageal compression and eventual dysphagia—a case report and literature review. Int J Surg Case Rep 2015;10:32–4.
4 5
Haliloglu N, Ozkavukcu E, Erden A. Dysphagia aortica co-existing with unilateral diaphragm paralysis. Dig Liver Dis 2010;42:74–5. Contini S, Corrente V, Nervi G, et al. Dysphagia aortica: a neglected symptom of aortoesophageal fistula. Dig Liver Dis 2006;38:51–4.
Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
3
CASE REPORT
Dysphagia aortica: a rare cause of dysphagia Siti Rozana Abdul Haziz,1 Ian Bickle,2 Vui Heng Chong1 1
Department of Medicine, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan, Brunei Darussalam 2 Department of Radiology, Raja Isteri Pengiran Anak Saleha (RIPAS) Hospital, Bandar Seri Begawan, Brunei Darussalam Correspondence to Dr Vui Heng Chong, [email protected] Accepted 1 September 2015
SUMMARY Dysphagia is uncommon; however, it is considered a warning symptom, especially in the elderly population, and should prompt consideration of a neoplasm of the oesophagus. Dysphagia aortica is rare and often not considered in clinical practice. It is caused by any abnormality of the aorta that results in dysphagia. These include thoracic aortic aneurysm, aortic dissection and even a tortuous aorta. Our case is of an elderly woman, in her early 70s, with a history of treated colorectal carcinoma and hypertension, who presented with progressive dysphagia to solid foods, which, on investigations, showed a tortuous aorta causing compressions of the oesophagus at multiple levels. She was managed with dietary adjustment and optimal control of blood pressure.
BACKGROUND Dysphagia is uncommon; however, it is considered a warning symptom, especially in the elderly population, and should prompt consideration of a neoplasm of the oesophagus. Dysphagia aortica is a rare condition, largely affecting the elderly population, and is often associated with thoracic aorta aneurysm or dissecting aorta, two conditions that are associated with high mortality with or without intervention. Our case highlights the fact that dysphagia aortica can also be due to a tortuous thoracic aorta, thus not requiring surgical intervention, as it can be managed with dietary adjustments and optimal control of blood pressure.
recurrence. Colonoscopy carried out then did not show evidence of recurrence either. Complete physical examination was unremarkable apart from an in situ porta-cath and an ejection systolic murmur without radiation to the carotids. There was no lymphadenopathy or abnormal peripheral pulses, and chest, cardiovascular and abdominal examinations were normal.
INVESTIGATIONS Upper gastrointestinal endoscopy showed prominent impressions with lumen compromised in the mid and lower oesophagus, at the levels of the aortic arch and lower half of the thoracic descending aorta. Despite full insufflation during endoscopy, the impression at the arch persisted. A barium swallow (figure 1) showed compressions of the oesophagus at several levels, including at the arch of the aorta and where the descending aorta crosses over the oesophagus. The findings were consistent with dysphagia aortica. There were no abnormal contractions and no contrast reflux was noted.
CASE PRESENTATION
To cite: Abdul Haziz SR, Bickle I, Chong VH. BMJ Case Rep Published online: [please include Day Month Year] doi:10.1136/bcr-2015211726
A woman in her early 70s was referred for evaluation of a 2-month history of dysphagia. She also reported some weight loss. She had a history of hypertension, chronic hepatitis B and colorectal cancer, which was resected followed by adjuvant chemotherapy in 2009. Her dysphagia had been progressive with intermittent worsening, and was only to solid food. The dysphagia was located mainly in the mid and lower chest. By the time she was seen in our clinic, she had already adjusted her diet to semi-solids, which had helped to ease her dysphagia. She did not report any history of gastrooesophageal reflux disorder and denied any associated heartburn or back pain with this current illness. She had been followed up in the general clinic and the National Cancer Centre. Her last appointment with the National Cancer Centre was several months before her symptom started, and a routine surveillance CT scan performed at that time showed no evidence of colorectal cancer
Figure 1 A barium swallow showing compressions of the oesophagus at multiple levels; at the aortic arch and lower third of the oesophagus.
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
1
Unusual presentation of more common disease/injury Blood investigation showed mild anaemia (haemoglobin 10.1 g/dL; normal range 10.9–14.3) with mildly elevated mean corpuscular volume (95.7 fL, normal range 75.5–95.3) with mild leucopenia (3.6×109/L; normal 3.8–11.8), normal platelets count (187×109/L; normal 150–450), mild chronic kidney injury with serum creatinine of 119.2 μmol/L (normal 50.4– 98.1) and urea of 8.5 mmol/L (normal 3.5–7.2). Thyroid profile was normal. A repeat CT scan did not show any mediastinal lymphadenopathies, aneurysm or dissection of the aorta, but showed the aorta taking a left para midline route resulting in prominent impressions seen on barium swallow. There was no aneurysm or dissection. When the patient re-presented several months later with worsening of her dysphagia, a repeat barium swallow was performed. In addition to barium contrast, a bolus food swallow (a piece of bread soaked in contrast) was given and this demonstrated the food bolus being held up at the stricture at the level of the arch of the aorta with precipitation of the dysphagia. The food bolus passed after several swallows with relief of symptom. Again, there were no abnormal contractions and no contrast reflux was noted.
DIFFERENTIAL DIAGNOSIS An upper gastrointestinal endoscopy excluded a diagnosis of oesophageal neoplasm, an important consideration in elderly patients presenting with dysphagia. Barium swallow studies showed several levels of compression of the oesophagus, most marked at the level of the aortic arch. Although not the optimal test, two barium swallow studies did not show any features of motility disorder. A manometry study, not available in our centre, would have been preferred. A CT of the thorax excluded any mediastinal or pulmonary causes of oesophageal compression.
among the elderly, women, those of short statue, and among patients with hypertension or kyphosis.2 Patients with dysphagia aortica usually present with dysphagia to solids but not to liquids, unless the lumen is severely compromised. Dysphagia to liquids is more in keeping with dysmotility ailments. However, as the compression progresses, dysphagia to liquids will follow. Other manifestations secondary to the effect of aortic pathology (ie, aneurysm) such as compression on the phrenic nerve causing diaphragmatic paralysis and gastrointestinal bleeding from an aorto-oesophageal fistula, have also been reported.4 5 Management includes treating the underlying causes. However, underlying causes of such dissecting aortas or aneurysms are associated with the risk of catastrophic events, and treatment options are often also high risk. Management of the underlying pathologies includes surgical repairs, which will relieve the dysphagia or intravascular stenting but will not relieve the dysphagia. Fortunately, in our patient, the dysphagia was due to a tortuous thoracic aorta, which was managed conservatively with diet modification in the form of a soft diet with control of blood pressure. However, in the earlier follow-up after diagnosis of aortica dysphagia, our patient had intermittent worsening of dysphagia, most likely due to self-limiting food bolus impactions. This was clearly demonstrated in her second barium study, when she was also given contrast soaked bread, which brought up her dysphagia when the solid bolus was held up in the aortic arch compression. Since then, the patient largely kept to a soft diet and took adequate fluid with each meal; this has led to marked reduction in her dysphagia. In conclusion, our case highlights the need to consider dysphagia aortica in elderly patient with a history of hypertension, presenting with dysphagia. Dysphagia aortica has often been associated with significant pathology such as dissection or aneurysm of the aorta. A tortuous thoracic aorta can also cause dysphagia aortica.
TREATMENT The patient was managed with a soft diet and optimal control of blood pressure (nifedipine modified release 90 mg daily, bendrofluazide 2.5 mg daily, doxazosin 4 mg nocte and candesartan 12 mg daily), and she has remained well without worsening of her dysphagia. She was also given intermittent courses of oral prokinetic (domperidone 10 mg three times a day).
Learning points ▸ Dysphagia is an important warning symptom especially in the elderly; not all cases are due to underlying neoplasms. ▸ Dysphagia aortica needs to be considered in patients with a long history of hypertension and, in most cases, has been reported to be associated with aortic aneurysm or dissecting aorta. ▸ Dysphagia aortica associated with a non-aneurysmal tortuous aorta can be managed conservatively with optimal blood pressure control and adaptation of diet.
OUTCOME AND FOLLOW-UP The patient’s symptoms improved significantly but she continued to have intermittent transient dysphagia, especially when taking large boluses of solid food.
DISCUSSION In clinical practice, dysphagia is an uncommon symptom compared with other gastrointestinal symptoms in the elderly population; however, when dysphagia occurs, a neoplasm of the oesophagus needs to be considered. Other common causes of dysphagia in the elderly are motility disorder or gastrooesophageal reflux. Dysphagia aortica is a rare condition caused by extrinsic compressions of the oesophagus by the aorta; it can be ectatic, an aneurysm (true or pseudo), or a dissecting or even tortuous aorta.1 2 Aortic dissection and aneurysm are serious causes of dysphagia aortica.2 Another less common vascular cause of dysphagia is dysphagia lusoria, which is due to abnormal subclavian take off resulting in a vascular ring around the oesophagus, typically at the proximal third.3 Dysphagia aortica is more common 2
Contributors SRAH wrote the first draft and approved the final draft. IB was involved in the care of the patient, provided radiological imaging and legend, and approved the final draft. VHC looked after the patient, conceived the idea of the case report, and co-wrote and approved the final draft. Competing interests None declared. Patient consent Obtained. Provenance and peer review Not commissioned; externally peer reviewed.
REFERENCES 1 2
Mittal RK, Siskind BN, Hongo M, et al. Dysphagia aortica. Clinical, radiological, and manometric findings. Dig Dis Sci 1986;31:379–84. Cao DB, Gao Y, Sun XY, et al. Dysphagia aortica secondary to descending thoracic aortic pseudoaneurysm. Ann Thorac Surg 2012;94:656.
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
Unusual presentation of more common disease/injury 3
de Araújo G, Junqueira Bizzi JW, Muller J, et al. “Dysphagia lusoria”—Right subclavian retroesophageal artery causing intermitent esophageal compression and eventual dysphagia—a case report and literature review. Int J Surg Case Rep 2015;10:32–4.
4 5
Haliloglu N, Ozkavukcu E, Erden A. Dysphagia aortica co-existing with unilateral diaphragm paralysis. Dig Liver Dis 2010;42:74–5. Contini S, Corrente V, Nervi G, et al. Dysphagia aortica: a neglected symptom of aortoesophageal fistula. Dig Liver Dis 2006;38:51–4.
Copyright 2015 BMJ Publishing Group. All rights reserved. For permission to reuse any of this content visit http://group.bmj.com/group/rights-licensing/permissions. BMJ Case Report Fellows may re-use this article for personal use and teaching without any further permission. Become a Fellow of BMJ Case Reports today and you can: ▸ Submit as many cases as you like ▸ Enjoy fast sympathetic peer review and rapid publication of accepted articles ▸ Access all the published articles ▸ Re-use any of the published material for personal use and teaching without further permission For information on Institutional Fellowships contact [email protected] Visit casereports.bmj.com for more articles like this and to become a Fellow
Abdul Haziz SR, et al. BMJ Case Rep 2015. doi:10.1136/bcr-2015-211726
3
