Ortner's Syndrome

Clinical History

A 55-year-old male with known rheumatic heart disease and severe mitral regurgitation presented to ENT outpatients complaining of hoarseness of voice for 15 days. Indirect laryngoscopy was performed, which revealed ulcers on both vocal cords with a fixed left true vocal cord.

Imaging Findings

Computed Tomography findings reveal cardiomegaly with a grossly dilated left atrium and prominent ascending aorta (Figure 1). No size significant hilar/mediastinal lymphadenopathy was noted. There was dilatation of the left pyriform sinus and laryngeal ventricle (Figures 2, 4). Over adduction of the right vocal cord with convex medial margins was noted (Figure 3).
On coronal CT sections there was flattening of the subglottic arch with dilatation of the left pyriform sinus (Figures 2 and 4).

Discussion

Ortner's syndrome, also known as cardio vocal syndrome [1], is a rare entity characterized by left recurrent laryngeal nerve palsy resulting from cardiovascular pathologies. Such diseases include mitral valve disease, septal defects, thoracic aortic aneurysm, aneurysm of the ductus arteriosus or patent ductus arteriosus and pulmonary artery enlargement [2-4]. Left atrial enlargement due to any underlying cardiovascular disease is the most common cause.

The left recurrent laryngeal nerve is a branch of the vagus nerve arising at the level of the aortic arch and supplies all the intrinsic muscles of larynx except cricothyroid. It hooks around the ligamentum arteriosum at the level of aortopulmonary window and ascends upwards in the groove between trachea and esophagus. Left atrial enlargement causes stretching or compression of the nerve in the aortopulmonary window. It is therefore necessary to include the mediastinum to this level on computed tomography studies in order to analyze the entire course of left recurrent laryngeal nerve.

CT findings suggestive of a vocal cord palsy are dilatation of the ipsilateral pyriform sinus and laryngeal ventricle and medial rotation and thickening of aryepiglottic fold [5]. During breath holding there is failure of adduction of paralyzed vocal cord with compensatory over adduction of the normal vocal cord giving a bowed appearance with convex margins medially.

Ortner’s syndrome should be suspected clinically in cases of vocal cord palsy without clinically evident laryngeal lesions; though the final diagnosis is made by radiological investigations including CT from the base of the skull to the diaphragm following contrast-agent injection. In case a vascular cause or potentially hypervascular lesion is suspected, CT-angiography should be performed. In our case, contrast enhanced CT was not performed as mediastinal vessels were normal in calibre and left atrial enlargement was obvious on precontrast images.

Differential Diagnosis List

Final Diagnosis

Left recurrent laryngeal nerve palsy due to left atrial enlargement (Ortner’s syndrome).

URL:	https://www.eurorad.org/case/13873
DOI:	10.1594/EURORAD/CASE.13873
ISSN:	1563-4086