Case Report
Ortner’s Syndrome: A Rare Cause of Hoarseness.
Authors
Alok Kumar, Senior Registrar,
KP Dubey, Senior Specialist & Head of Dept.
Ajay Gupta, Senior Specialist,
Binayak Baruah, Associate Specialist,
Ashish Rai, Junior Resident),
Tata Main Hospital, Jamshedpur, India.
Address for Correspondence
Dr. Alok Kumar,
34, Old Professional Flat,
IC Road, Bistupur,
Jamshedpur-831001,
India.
E-mail:
alokjsrent@gmail.com
Citation
Kumar A, Dubey KP, Gupta A, Baruah B, Rai A. Ortner’s Syndrome: A Rare Cause of Hoarseness. Online J Health Allied Scs.
2014;13(3):6. Available at URL:
http://www.ojhas.org/issue51/2014-3-6.html
Open Access Archives
http://cogprints.org/view/subjects/OJHAS.html
http://openmed.nic.in/view/subjects/ojhas.html
Submitted: July 16,
2014; Accepted: Sep 28, 2014; Published: Oct 30, 2014 |
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Introduction:
Cardiovocal syndrome or Ortner’s syndrome is a clinical condition with hoarseness attributable to left recurrent laryngeal nerve palsy in variety cardiovascular diseases like aortic aneurysm, patent ductus arteriosus, primary pulmonary hypertension, atrial and ventricular septal defect, Eisenmenger’s syndrome and recurrent pulmonary embolism and mitral stenosis.
Case Report
A 72 years old lady, a home maker, and a hypertensive on treatment, presented with change in voice (Hoarseness) for 3 months which was gradually progressive. There was no history of difficulty in breathing or difficulty in swallowing. There were no history of trauma and nor of any addiction.
Cardiac examination revealed normal heart sounds S1 and S2 with ejection systolic murmur of grade IV/VI radiating to the carotids. There was no neurological deficit. All blood investigations were within normal limits. ECG showed frequent ectopics. 2D echo showed a normal mitral, aortic, pulmonary and tricuspid valve with trace mitral and tricuspid regurgitation, and mild concentric left ventricular hypertrophy.
Patient was subjected for flexible nasopharyngoscopy which showed paralysis of the left vocal cord paralysis with phonatory gap. X-Ray chest showed mild cardiomegaly with clear lung fields. CT scan of neck showed a normal appearance of the soft tissue structure of the neck. CT thorax showed pseudo aneurysm of the arch of aorta with surrounding thrombus and left recurrent laryngeal nerve compression. CT chest angiography showed a saccular aneurysm of size 4.0X3.5 with peripheral thrombus. Patent lumen showed a size of 2.7X2.0 cm. It was seen protruding inferiorly and to the left side. Aneurysm was seen arising 2.5 cm distal to the origin of left subclavian artery. The Descending aorta and right brachiocephalic artery was mildly sclerosed. Myocardial perfusion SPECT-Post DPM Stress and rest study stress protocol (post dipyridamole) showed normal myocardial perfusion scan and normal resting LVEF.
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Figure 1: 4.0X3.5 cm saccular aneurysm with peripheral thrombus as marked by yellow arrow. |
Discussion
Cardiovocal syndrome is a clinical entity manifested by hoarseness caused by an impaired ability of the left recurrent laryngeal nerve to transmit impulse to the laryngeal musculature because of stretching or impingement of the nerve from the disease induced changes in cardiac or greater vessel anatomy. Hoarseness of voice due to paralysis of the left recurrent laryngeal nerve caused by a dilated left atrium in mitral stenosis was first discussed by Nobert Ortner in 1897. A variety of cardiac problems can lead to paralysis of the left recurrent laryngeal nerve, these include thoracic aortic aneurysm, patent ductus arteriosus, primary pulmonary hypertension, trial and ventricular septal defect, Eisenmenger’s syndrome and recurrent pulmonary embolism.(1) In mitral stenosis it ranges from .6 to 5%.(2) Leonetti reported that 8 of 168 patients (4.8%) with thoracic aortic aneurysm presented with hoarseness and all of these had type I aneurysms (de bakey classification) involving the ascending root and aortic arch.(3)
The onset of hoarseness is usually insidious and, in the beginning it may be intermittent, but in due course, it progresses to complete aphonia. To understand how cardiovocal syndrome arises, one has to consider the anatomical position of the recurrent laryngeal nerve and the left atrium. The lengthy course of the recurrent laryngeal nerve in the thoracic cavity especially around the aortic arch makes it vulnerable to compression, traction and erosion by enlarged or displaced cardiac chambers, dilated pulmonary arteries and a dilated aorta.(4,5) Contrary to its name, the left atrium does not lie on the left side but forms the most posterior chamber of the heart. It is closely related to the esophagus, spine, left recurrent laryngeal nerve, pulmonary vessels, lung parenchyma and bronchi. Therefore, when the left atrium enlarges, it causes complication by compressing the adjacent structures.
Treatment and prognosis for this syndrome depends on the possibilities of managing the underlying cause. It is not necessary to perform any additional surgical procedure on the vocal fold if the patient shows sufficient improvement during speech therapy. Defintive treatment should be considered if aspiration is severe or if no improvement is visible after alleviation of the cardiac problem.(6)
Conclusion
Cardiovocal syndrome is rare in the general population but associated with causes such as aneurysms and mitral valve stenosis. A high index of suspicion is needed to make an early diagnosis which can lead to surgical correction of the potentially life threatening, underlying cardiovascular disease. Following such treatment, the cardiovocal syndrome usually resolves spontaneously as vocal cord function returns, often without a need for additional invasive treatment.
Acknowledgement
We are grateful to our GM (Medical Services) for allowing us to carry the publication work for this article
References
- Sengupta A, Dubey SP, Chaudhuri D, Sinha AK, Chakravarti P. Ortner’s syndrome revisited. Laryngol Otol.1998 Apr;112:377-9.
- Loughran S, Alves C, MacGregor FB. Current etiology of unilateral vocal fold paralysis in teaching hospital in west of Scotland. Laryngol Otol. 2002 Nov;116(11):907-10.
- Teixido MT, Leonetti JP. Recurrent laryngeal nerve paralysis associated with thoracic aortic aneurysm. Otolaryngol Head Neck Surg.1990 Feb;102(2):140-4.
- Thirwall AS. Ortner’s syndrome: a centenary review of unilateral recurrent laryngeal nerve palsy secondary to cardiothoracic diseases. Laryngol Otol. 1997 Sept;111:869-71.
- Mulpuru SK, Vasavada BC, Punukollu GK, Patel AG. Cardiovocal syndrome: a systematic review. Heart Lung Cir. 2008 Feb;17(1):1-4
- Lee SL, Pyun SB, Jang DH. Dysphagia and hoarseness associated with painless aortic dissection: a rare case of cardiovocal syndrome. Dysphagia. 2006 April;21(2):129-32.
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