Vascular rings.
The term vascular ring refers to congenital vascular anomalies of the aortic arch system that compress the esophagus and trachea, causing symptoms related to those two structures. The most common vascular rings are double aortic arch and right aortic arch with left ligamentum. Pulmonary artery sling is rare and these patients need to be carefully evaluated for frequently associated tracheal stenosis. Another cause of tracheal compression occurring only in infants is the innominate artery compression syndrome. In the current era, the diagnosis of a vascular ring is best established by CT imaging that can accurately delineate the anatomy of the vascular ring and associated tracheal pathology. For patients with a right aortic arch there recently has been an increased recognition of a structure called a Kommerell diverticulum which may require resection and transfer of the left subclavian artery to the left carotid artery. A very rare vascular ring is the circumflex aorta that is now treated with the aortic uncrossing operation. Patients with vascular rings should all have an echocardiogram because of the incidence of associated congenital heart disease. We also recommend bronchoscopy to assess for additional tracheal pathology and provide an assessment of the degree of tracheomalacia and bronchomalacia. The outcomes of surgical intervention are excellent and most patients have complete resolution of symptoms over a period of time.
Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Box 22, 225 E. Chicago Ave, Chicago, Illinois 60611; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois. Electronic address: cbacker@luriechildrens.org.
Carl L
CL
Backer
Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Box 22, 225 E. Chicago Ave, Chicago, Illinois 60611; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Michael C
MC
Mongé
Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois; Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Andrada R
AR
Popescu
Division of Cardiovascular-Thoracic Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Box 22, 225 E. Chicago Ave, Chicago, Illinois 60611; Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Osama M
OM
Eltayeb
Department of Surgery, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Division of Otolaryngology, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
Jeffrey C
JC
Rastatter
Department of Medical Imaging, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois; Department of Radiology, Northwestern University Feinberg School of Medicine, Chicago, Illinois; Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
Cynthia K
CK
Rigsby
D001013:Aorta, Thoracic / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000601:surgery; D001018:Aortic Diseases / Q000151:congenital / Q000175:diagnosis / Q000196:embryology / Q000601:surgery; D016122:Brachiocephalic Trunk / Q000002:abnormalities; D001999:Bronchoscopy; D002648:Child; D004452:Echocardiography; D004940:Esophageal Stenosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D006801:Humans; D007223:Infant; D011651:Pulmonary Artery / Q000002:abnormalities; D013348:Subclavian Artery / Q000002:abnormalities; D014057:Tomography, X-Ray Computed; D014135:Tracheal Stenosis / Q000175:diagnosis / Q000209:etiology* / Q000601:surgery; D054079:Vascular Malformations / Q000175:diagnosis / Q000196:embryology / Q000601:surgery; D014656:Vascular Surgical Procedures
Double aortic arch; Kommerell diverticulum; Pulmonary artery sling; Right aortic arch; Slide tracheoplasty; Vascular rings