Dysphagia lusoria

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:[email protected]

King Saud Medical City, Riyadh Saudi Arabia

Chest Imaging Section: 2015, Aug. 31 Published:

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 behindoesophagus (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)




Figure 2 Dysphagia Lusoria

CT Chest transverse Section. Aberrant right subclavian artery is seen crossing behindoesophagus and arching towards infraclavicular region (yellow arrows). Dilated oesophagusis apparent in front of the crossing vessel.




CT Chest Transverse Section. Aberrant right subclavian artery is seen crossing towardsinfraclavicular region (yellow arrows). Dilated oesophagus is apparent in front of thecrossing vessel.




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:[email protected] (2015, Aug. 31)

Dysphagia Lusoria {Online}URL: http://www.eurorad.org/case.php?id=12979

Health & Medicine

Dysphagia lusoria