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Case 12979
Dysphagia Lusoria
Muhammad Asim Rana Randy Maraj Ahmed Mady Mohammed Odat Waleed Aletreby1 1 2 2 2
1.King's Mill Hospital Sutton-in-Ashfield Nottinghamshire United Kingdom
2.King Saud Medical City, Riyadh Saudi Arabia
Email:drasimrana@yahoo.com
King Saud Medical City, Riyadh Saudi Arabia
Chest ImagingSection:
2015, Aug. 31Published:
61 year(s), femalePatient:
Clinical History
A middle aged lady presented with 2 years history of progressive dysphagia to solids. Her physical
examination was unremarkable except evidence of recent weight loss. All routine laboratory
investigations, chest x-ray and ECG were normal. Her endoscopy did not reveal anything. Contrast
enhanced CT chest revealed an unusual finding.
Imaging Findings
The upper oesophagus is dilated and fluid. There is an aberrant right subclavian artery passing
behind the oesophagus which could be contributing to the apparent dilatation. No mediastinal or
hilar lymphadenopathy. The heart is enlarged. There isatelectasis in both lower and the right middle
lobes and smooth fatty pleural thickening at the left lung base.
Discussion
Among different congenital anomalies of aortic arch, aberrant right subclavian artery is considered
most common and occurs in 0.5% to 1.8% of cases [1].
The anomaly can be explained on Edward's hypothesis which describes involution of fourth
vascular arch with the right dorsal aorta. The seventh intersegmental artery remains attached to the
descending aorta and this persisting intersegmental artery becomes the right subclavian artery.
Differential growth shifts the origin cranially and it lies close to the origin of the left subclavian
artery. It originates dorsally and therefore has a retro-oesophageal course [2].
Although most cases of this anomaly are asymptomatic, but symptoms may appear when a ring
completely encircles the trachea or oesophagus. Extrinsic compression of oesophagus may lead to
dysphagia. This phenomenon was first described in 1794 by a London physician David Bayford as a
post-mortem finding in a woman who had lifelong dysphagia and eventually died of starvation. He
named it Dysphagia Lusoria" which means dysphagia by freak of nature [3].
Klink Hamer [4] observed that in symptomatic cases aberrant right subclavian artery was associated
with a bicarotid trunk (common origin of right and left carotid arteries).
The diagnosis of Dysphagia Lusoria is difficult as the symptoms are nonspecific. Endoscopy may
miss the diagnosis in 50% of cases although a pulsating mass may be visualized. Barium swallow
may demonstrate a diagonal impression at the level of T3-T4. Digital subtraction angiogram,
contrast enhanced CT or MRI of thorax is perhaps the best diagnostic test that could pick up the
abnormality. Angiogram is needed for pre-operative assessment. [5]
Management Of dysphagia lusoria depends on the severity of the condition and it ranges from
dietary adjustments only to surgical resection.[6]
First successful repair was reported by Robert Gross[7]. Initial reports revealed that simple division
without blood flow restoration can cause ischemia of the arm. Bailey et al in 1965[8] attached distal
end of divided vessel to ascending aorta. Cooley anastomosed distal end to right common carotid
[9] This method was favoured by others but there is a possibility of cerebral ischemia with this
approach. Mok et al [10] recommended to anastomose the end to aortic arch with or without a graft.
Since then numerous alternatives have been described. Some recommend an endovascular or hybrid
approach to this anomaly. Endoluminal grafts have also been reported especially in presence of
aneurysm. [11]
Our patient was managed conservatively with dietary readjustments and showed improvement with
significant weight gain in 6 months.
Final Diagnosis
Dysphagia Lusoria due to aberrant right subclavian artery
Differential Diagnosis List
Achalasia, Neuromauscular, Interinsic lesions, Extrinsic compression
Figures
Figure 1 Dysphagia Lusoria
Coronal Section CT Chest. Aberrant right subclavian artery is seen crossing behind
oesophagus (marked by yellow arrow)
© Muhammad Asim Rana, King's Mill Hospital, Notts, UK.
Area of Interest: Thorax;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Coronal Section CT Chest. Aberrant right subclavian artery is seen behind oesophagus
(marked by yellow arrow)
© Muhammad Asim Rana, King's Mill Hospital, Notts, UK.
Area of Interest: Thorax;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
Figure 2 Dysphagia Lusoria
CT Chest transverse Section. Aberrant right subclavian artery is seen crossing behind
oesophagus and arching towards infraclavicular region (yellow arrows). Dilated oesophagus
is apparent in front of the crossing vessel.
© Muhammad Asim Rana, King's Mill Hospital, Notts, UK.
Area of Interest: Thorax;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
CT Chest Transverse Section. Aberrant right subclavian artery is seen crossing towards
infraclavicular region (yellow arrows). Dilated oesophagus is apparent in front of the
crossing vessel.
© Muhammad Asim Rana, King's Mill Hospital, Notts, UK.
Area of Interest: Thorax;
Imaging Technique: CT;
Procedure: Diagnostic procedure;
Special Focus: Congenital;
References
[1] Stewart JR, Kincaid OW, Edwards JE (1964) An atlas of vascular rings and related
malformations of the aortic arch system. p.53
[2] Edwards JE (1960) Congenital malformations of the heart and great vessels Pathology of the
heart 2nd ed. p.391-462.
[3] Bayford D (1794) An account of singular case of obstructed deglutition Memoirs Med Soc
London 2:275-286
[4] A.C. Klinkhamer (1996) Aberrant right subclavian artery. Clinical and roentgenologic aspects
American Journal of Roentgenology, Radium therapy and Nuclear Medicine 97(2)438-446
[5] Janssen M, Baggen MG, Veen HF, Smout AJ, Bekkers JA, Jonkman GJ, Ouwendijk RJ (2000)
Dysphagia lusoria: clinical aspects, manometric findings, diagnosis, and therapy Am J
Gastroenterol. 95(6):1411-6
[6] Hallman GL, Cooley DA (1964) Congenital aortic vascular ring. Surgical consideration Arch
Surg. 88(4)666-675
[7] Gross RE (1946) Surgical treatment for dysphagia lusoria Ann Surg 124(3)532-534
[8] Bailey C P, Hinrose T, Alba J (1965) Re-establishment of the continuity of the anomalous right
subclavian artery after operation for dysphagia lusoria Angiology 16(9)509-513
[9] Cooley D A (1986) Surgical treatment of aortic aneurysms. Philadelphia W B Saunders 174-184
[10] Mok C K, Cheung K L, Kong S M, Ong G B (1979) translocating the right subclavian artery in
dysphagia lusoria British Journal of Surgery 66(2)113-116
[11] Shennib H, Diethrich E B (2008) Novel approaches for the treatment of the aberrant right
subclavian artery and its aneurysm Journal of Vascular Surgery 47(5)1066-1070
Citation
Muhammad Asim Rana Randy Maraj Ahmed Mady Mohammed Odat Waleed Aletreby1 1 2 2 2
1.King's Mill Hospital Sutton-in-Ashfield Nottinghamshire United Kingdom
2.King Saud Medical City, Riyadh Saudi Arabia
Email:drasimrana@yahoo.com (2015, Aug. 31)
Dysphagia Lusoria {Online}
URL: http://www.eurorad.org/case.php?id=12979

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Dysphagia lusoria

  • 1. Case 12979 Dysphagia Lusoria Muhammad Asim Rana Randy Maraj Ahmed Mady Mohammed Odat Waleed Aletreby1 1 2 2 2 1.King's Mill Hospital Sutton-in-Ashfield Nottinghamshire United Kingdom 2.King Saud Medical City, Riyadh Saudi Arabia Email:drasimrana@yahoo.com King Saud Medical City, Riyadh Saudi Arabia Chest ImagingSection: 2015, Aug. 31Published: 61 year(s), femalePatient: Clinical History A middle aged lady presented with 2 years history of progressive dysphagia to solids. Her physical examination was unremarkable except evidence of recent weight loss. All routine laboratory investigations, chest x-ray and ECG were normal. Her endoscopy did not reveal anything. Contrast enhanced CT chest revealed an unusual finding. Imaging Findings The upper oesophagus is dilated and fluid. There is an aberrant right subclavian artery passing behind the oesophagus which could be contributing to the apparent dilatation. No mediastinal or hilar lymphadenopathy. The heart is enlarged. There isatelectasis in both lower and the right middle lobes and smooth fatty pleural thickening at the left lung base. Discussion Among different congenital anomalies of aortic arch, aberrant right subclavian artery is considered most common and occurs in 0.5% to 1.8% of cases [1]. The anomaly can be explained on Edward's hypothesis which describes involution of fourth
  • 2. vascular arch with the right dorsal aorta. The seventh intersegmental artery remains attached to the descending aorta and this persisting intersegmental artery becomes the right subclavian artery. Differential growth shifts the origin cranially and it lies close to the origin of the left subclavian artery. It originates dorsally and therefore has a retro-oesophageal course [2]. Although most cases of this anomaly are asymptomatic, but symptoms may appear when a ring completely encircles the trachea or oesophagus. Extrinsic compression of oesophagus may lead to dysphagia. This phenomenon was first described in 1794 by a London physician David Bayford as a post-mortem finding in a woman who had lifelong dysphagia and eventually died of starvation. He named it Dysphagia Lusoria" which means dysphagia by freak of nature [3]. Klink Hamer [4] observed that in symptomatic cases aberrant right subclavian artery was associated with a bicarotid trunk (common origin of right and left carotid arteries). The diagnosis of Dysphagia Lusoria is difficult as the symptoms are nonspecific. Endoscopy may miss the diagnosis in 50% of cases although a pulsating mass may be visualized. Barium swallow may demonstrate a diagonal impression at the level of T3-T4. Digital subtraction angiogram, contrast enhanced CT or MRI of thorax is perhaps the best diagnostic test that could pick up the abnormality. Angiogram is needed for pre-operative assessment. [5] Management Of dysphagia lusoria depends on the severity of the condition and it ranges from dietary adjustments only to surgical resection.[6] First successful repair was reported by Robert Gross[7]. Initial reports revealed that simple division without blood flow restoration can cause ischemia of the arm. Bailey et al in 1965[8] attached distal end of divided vessel to ascending aorta. Cooley anastomosed distal end to right common carotid [9] This method was favoured by others but there is a possibility of cerebral ischemia with this approach. Mok et al [10] recommended to anastomose the end to aortic arch with or without a graft. Since then numerous alternatives have been described. Some recommend an endovascular or hybrid approach to this anomaly. Endoluminal grafts have also been reported especially in presence of aneurysm. [11] Our patient was managed conservatively with dietary readjustments and showed improvement with significant weight gain in 6 months. Final Diagnosis Dysphagia Lusoria due to aberrant right subclavian artery Differential Diagnosis List Achalasia, Neuromauscular, Interinsic lesions, Extrinsic compression Figures Figure 1 Dysphagia Lusoria
  • 3. Coronal Section CT Chest. Aberrant right subclavian artery is seen crossing behind oesophagus (marked by yellow arrow) © Muhammad Asim Rana, King's Mill Hospital, Notts, UK. Area of Interest: Thorax; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital; Coronal Section CT Chest. Aberrant right subclavian artery is seen behind oesophagus (marked by yellow arrow) © Muhammad Asim Rana, King's Mill Hospital, Notts, UK. Area of Interest: Thorax; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital; Figure 2 Dysphagia Lusoria
  • 4. CT Chest transverse Section. Aberrant right subclavian artery is seen crossing behind oesophagus and arching towards infraclavicular region (yellow arrows). Dilated oesophagus is apparent in front of the crossing vessel. © Muhammad Asim Rana, King's Mill Hospital, Notts, UK. Area of Interest: Thorax; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital; CT Chest Transverse Section. Aberrant right subclavian artery is seen crossing towards infraclavicular region (yellow arrows). Dilated oesophagus is apparent in front of the crossing vessel. © Muhammad Asim Rana, King's Mill Hospital, Notts, UK. Area of Interest: Thorax; Imaging Technique: CT; Procedure: Diagnostic procedure; Special Focus: Congenital; References [1] Stewart JR, Kincaid OW, Edwards JE (1964) An atlas of vascular rings and related
  • 5. malformations of the aortic arch system. p.53 [2] Edwards JE (1960) Congenital malformations of the heart and great vessels Pathology of the heart 2nd ed. p.391-462. [3] Bayford D (1794) An account of singular case of obstructed deglutition Memoirs Med Soc London 2:275-286 [4] A.C. Klinkhamer (1996) Aberrant right subclavian artery. Clinical and roentgenologic aspects American Journal of Roentgenology, Radium therapy and Nuclear Medicine 97(2)438-446 [5] Janssen M, Baggen MG, Veen HF, Smout AJ, Bekkers JA, Jonkman GJ, Ouwendijk RJ (2000) Dysphagia lusoria: clinical aspects, manometric findings, diagnosis, and therapy Am J Gastroenterol. 95(6):1411-6 [6] Hallman GL, Cooley DA (1964) Congenital aortic vascular ring. Surgical consideration Arch Surg. 88(4)666-675 [7] Gross RE (1946) Surgical treatment for dysphagia lusoria Ann Surg 124(3)532-534 [8] Bailey C P, Hinrose T, Alba J (1965) Re-establishment of the continuity of the anomalous right subclavian artery after operation for dysphagia lusoria Angiology 16(9)509-513 [9] Cooley D A (1986) Surgical treatment of aortic aneurysms. Philadelphia W B Saunders 174-184 [10] Mok C K, Cheung K L, Kong S M, Ong G B (1979) translocating the right subclavian artery in dysphagia lusoria British Journal of Surgery 66(2)113-116 [11] Shennib H, Diethrich E B (2008) Novel approaches for the treatment of the aberrant right subclavian artery and its aneurysm Journal of Vascular Surgery 47(5)1066-1070 Citation Muhammad Asim Rana Randy Maraj Ahmed Mady Mohammed Odat Waleed Aletreby1 1 2 2 2 1.King's Mill Hospital Sutton-in-Ashfield Nottinghamshire United Kingdom 2.King Saud Medical City, Riyadh Saudi Arabia Email:drasimrana@yahoo.com (2015, Aug. 31) Dysphagia Lusoria {Online} URL: http://www.eurorad.org/case.php?id=12979