CASE 12609 Published on 04.05.2015

An unusual case of dysphagia presenting in an 8-month-old child



Case Type

Clinical Cases


J. De Groote, E. Ghariq, D. Lahuis, H. Lamb

UZ Gent, Radiology
De Pintelaan 185
9000 Gent

8 months, male

Area of Interest Oesophagus, Cardiovascular system ; Imaging Technique Fluoroscopy, CT-Angiography
Clinical History
Since the introduction of solid nutrition, an eighth-month-old boy presented with dysphagia. In addition, he suffered repetitive and abnormally frequent swallowing manoeuvres and regurgitation. The child’s weight and length curve showed significantly lower-than-average normal age-related values.
Imaging Findings
The child was known to have a developmental anomaly of the aorta with right-sided arch and right-sided descending aorta, diagnosed on prenatal ultrasound. To exclude possible gastro-oesophageal reflux, known as a frequent cause of dysphagia in young children, a iodine contrast oesophagogram was performed illustrating extrinsic compression with anterior and leftward displacement of the oesophagus, stasis and delayed secondary passage of the contrast bolus (Fig. 1).
CT angiography (CTA) confirmed a right-sided aortic arch with aberrant left subclavian artery, crossing the midline posterior of the oesophagus and trachea (Fig. 2, 3).
This aberrant left subclavian artery originates from a vascular diverticulum, also know as diverticulum of Kommerell, representing a remnant of the distal left dorsal aortic root. The diverticulum generates extrinsic compression and anterior-leftward displacement of the oesophagus at supracarinal level.
Background and Clinical perspective:
In normal embryogenesis the aortic arch develops from five pairs of pharyngeal arch arteries with left-to-right symmetry. Normal development involves regression of the right ductus arteriosus, right aortic arch and right dorsal aorta, with persistence of all primary vascular structures left of the oesophagus and trachea [1]. A right aortic arch with right descending aorta occurs in 0.05 – 0.1 % of the general population [1]. Approximately 50% of these cases are associated with an aberrant left-sided subclavian artery (arteria lusoria) originating as the last aortic arch branch and crossing the mid line, posterior of trachea and oesophagus [2]. This left-sided artery of lusoria usually arises from a vascular outpouching or diverticulum, representing the distal part of the primitive left-sided aortic arch. This outpoutching is known as "Kommerell’s diverticulum" [1]. However, the term originally referred to a vascular diverticulum arising from a right-sided artery of lusoria [2-3]. A right aortic arch with aberrant left-sided artery completes a vascular ring around the trachea and the oesophagus together with the ligamentum arteriosum. If associated with swallowing disorders, this vascular ring is considered causative for the clinical concept of ‘dysphagia lusoria’ [2].
Imaging Perspective:
In cases of suspected developmental anomaly of the aorta, chest radiography should be the primary imaging modality [3]. Crossing of the aortic arch over the right main bronchus instead of the left associated with right instead of left tracheal buckling suggests a right aortic arch [3]. If gastrointestinal symptoms predominate, contrast oesophagogram may be considered as a second line imaging modality of choice. A horizontal indentation of the posterior oesophageal wall may be seen as the result of the Kommerell’s diverticulum. MRA or CTA should be the next imaging step [1-3]. Since current dose reduction protocols on high-end CT devices make it possible to reduce doses to chest X-ray ranges, CTA can be considered as an imaging alternative to MRA. CTA is also considered superior to MRA in pre-operative planning and evaluation of secondary tracheal and/or oesophageal compression [4].
The child was successfully treated with surgical ligation of the ligamentum arteriosum resulting in oesophageal decompression.
Take Home Message:
Aortic arch developmental anomalies may present with symptoms of dysphagia and/or stridor in young children. Imaging work-up should initially start with conventional chest X-ray followed by contrast oesophagogram, and if a developmental anomaly is still suspected, CTA or MRA may be considered for final diagnosis and pre-operative planning.
Differential Diagnosis List
Right-sided aortic arch, Kommerell's diverticulum, left artery of lusoria
Gastro-oesophageal reflux
Right-sided aortic arch with left sided descending aorta
Double aortic arch
Final Diagnosis
Right-sided aortic arch, Kommerell's diverticulum, left artery of lusoria
Case information
DOI: 10.1594/EURORAD/CASE.12609
ISSN: 1563-4086