Encircling double aortic arch

Orpha code: 99075OMIM code:

Definition

Encircling double aortic arch is a very rare congenital anomaly of the great arteries characterized by the presence of two aortic arches (right and left) which encircle and compress the trachea and esophagus, resulting in various respiratory and gastrointestinal symptoms (e.g. harsh breathing, stridor, dyspnea, cyanotic and choking episodes, chronic cough, recurrent respiratory tract infections, dysphagia and reflux). Esophageal atresia and tracheoesophageal fistula have also been reported. It usually occurs isolated, but, on occasion, may be associated with other congenital heart anomalies and chromosomal aberations.

Disease data
Classification

Morphological anomaly

ORPHA code
99075
OMIM code
-
ICD10 code
Q25.4
ICD11 code
LA8B.Y

No additional description.

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