Vascular ring abnormalities: A retrospective study of 62 cases☆,☆☆
Section snippets
Materials and methods
We reviewed the files of 62 children with vascular ring abnormalities who were treated at Necker Hospital between 1990 and 2000, focusing on age at clinical onset, complementary examinations and their results, the type of abnormality, associated abnormalities, the type of surgical treatment, preoperative and postoperative complications, and outcome.
The study population was divided into 2 groups on the basis of age at diagnosis (during or after the first week of life), because clinical signs
Results
Sixty-two files were reviewed. There were 33 boys and 29 girls. Clinical onset occurred neonatally in 28% of patients. The average term at delivery was 37.7 weeks, and the average birth weight was 2,885 g. Clinical onset occurred during the first year of life in 68% of cases, and later in 4% (2 patients, 2 and 10 years old). Ten children (16%) also had other malformations, which were cardiac (2 atrial septal defects, one ventricular septal defect, one case of pulmonary hypertension with a
Discussion
Vascular ring abnormalities are caused by developmental failure of parts of the paired aortic arches (fourth and fifth arches) during embryonic life. A genetic origin has not been ruled out.3, 4 In our series, one patient had the Rubinstein-Taybi syndrome caused by microdeletion of chromosome arm 16p13.3.5
Aortic arches abnormalities must be suspected in infants with breathing noises, often during inspiration only but sometimes also during expiration (inspiratory stridor, expiratory wheezing),
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Address reprint requests to Y. Révillon, MD, Department de Chirurgie Pédiatrique, Hôpital Necker-Enfants Malades, 149 rue de Sèvres, 75015 Paris, France.
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0022-3468/03/3804-0003$30.00/0