CASE REPORT Not All Symptoms Disappear After Vascular Ring Division A Pathophysiological Interpretation Augusto Zani Æ Francesco Morini Æ Pasquale Paolantonio Æ Denis A. Cozzi Received: 18 June 2007 / Accepted: 18 June 2007 / Published online: 13 September 2007 Ó Springer Science+Business Media, LLC 2007 Abstract Vascular rings are a group of cardiovascular lesions due to faulty embryological development derived from an abnormality of neural crest cells (cephalic neu- rocristopathy). 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 fea- tures of maturational dysautonomia and tend to disappear with aging, whereas major compressive symptoms are solved by surgery. Keywords Bark cough Á Clinical manifestation Á Ligamentum arteriosum Á Maturational dysautonomia Á Neural crest Á Vascular ring Introduction Vascular rings are a group of relatively rare congenital cardiovascular lesions that result from faulty embryologi- cal development of the aortic arch. This developmental defect is related to an abnormality of neural crest cell migration, colonization, or cytodifferentiation (cephalic neurocristopathies) [6]. Classically, symptoms of vascular ring are compression on the trachea and/or the esophagus, even when incomplete, and usually they appear in the first 6 months of life [2]. We present on a case of a child with a complete vascular ring, who presented, in addition to compressive symptoms, other autonomic disturbances. Case Report A 3-year-old boy presented with recurrent vomiting from the age of 2 months. Due to persisting vomiting and regurgita- tion, the boy had been medically treated at other institutions for gastro-esophageal reflux, without success. He continued to experience dysphagia, vomiting, a seallike bark during feeding, bronchopneumonia without fever, and several apparent life-threatening events. Other presenting symptoms were hyperhydrosis, hypersialosis, hyperthermia without signs of infection, and bitonal voice. On admission, the boy, weighing 14 kg (25th centile), had low-set ears, bilateral epicanthic folds, and a flat and broad nasal bridge. The remainder of the clinical examination was unremarkable. A barium swallow showed a sharp narrowing of the upper third of the esophagus, suggesting a vascular compression. On this suspicion, we performed an echocardiography, which was compatible with a double aortic arch without additional intracardiac anomalies. A subsequent contrast computed tomography (CT) scan revealed a right-sided aortic arch with retro-esophageal aberrant left subclavian artery. Three-dimensional image reconstruction detected an incomplete vascular ring composed of the aortic arch, the aberrant left subclavian artery, and the left pulmonary artery (Fig. 1a), compressing both the trachea and the esophagus. Therefore, a left thoracotomy was performed. At operation, a A. Zani Á F. Morini Á D. A. Cozzi (&) Pediatric Surgery Unit, University of Rome ‘‘La Sapienza’’, Rome, Italy e-mail: da.cozzi@uniroma1.it P. Paolantonio Department of Radiology, University of Rome ‘‘La Sapienza’’, Rome, Italy 123 Pediatr Cardiol (2008) 29:676–678 DOI 10.1007/s00246-007-9041-y