title: Not all symptoms disappear after vascular ring division: a pathophysiological interpretation.
abstract: Vascular rings are a group of cardiovascular lesions due to faulty embryological development derived from an abnormality of neural crest cells (cephalic neurocristopathy). In addition to peculiar symptoms due to compression on the trachea and/or the esophagus, patients with vascular rings might present further features of neural crest-related defects. We report on a case of a child with complete vascular ring, affected both by compressive symptoms and by autonomic disturbances and minor facial anomalies. The autonomic disturbances are clinical features of maturational dysautonomia and tend to disappear with aging, whereas major compressive symptoms are solved by surgery.
authors.affiliation: Pediatric Surgery Unit, University of Rome La Sapienza, Rome, Italy.
authors.forename: Augusto
authors.initials: A
authors.lastname: Zani
authors.forename: Francesco
authors.initials: F
authors.lastname: Morini
authors.forename: Pasquale
authors.initials: P
authors.lastname: Paolantonio
authors.forename: Denis A
authors.initials: DA
authors.lastname: Cozzi
mesh_terms: D018376:Cardiovascular Abnormalities / Q000503:physiopathology* / Q000601:surgery; D002675:Child, Preschool; D004373:Ductus Arteriosus / Q000601:surgery; D004940:Esophageal Stenosis / Q000503:physiopathology* / Q000601:surgery*; D019066:Facies; D006801:Humans; D008022:Ligaments / Q000601:surgery; D008026:Ligation; D008297:Male; D013577:Syndrome; D014135:Tracheal Stenosis / Q000503:physiopathology*