Vascular ring abnormalities: A retrospective study of 62 cases,☆☆

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Abstract

Background: The authors analyzed clinical signs of vascular ring anomalies together with appropriate complementary examinations and factors predictive of outcome after surgical treatment. Methods: The authors reviewed the files of 62 patients with vascular ring abnormalities treated at Necker-Enfants Malades Hospital between January 1990 and January 2000, to analyze age at symptom onset, results of paraclinical examinations, the type of vascular ring abnormality, the surgical indications and type of surgery, and postoperative outcome. Outcomes were divided into 3 categories: cure, partial improvement, and no improvement. The χ2 test corrected with Fischer's Exact test was used for statistical analysis. Results: Vascular ring abnormalities were diagnosed at birth in 28% of cases and during the first year of life in 68%. Sixteen percent of patients had associated abnormalities. Recurrent pulmonary and bronchial infections occurred after one year of age. An esophagogram was done in 76% of cases and showed impression images. Endoscopy was done in 63% of cases and showed malacia in 41% of patients and stenosis in 51%. Fifty-eight children were treated surgically. The average hospital stay was 7.4 days. The average follow-up was 37.4 months (12 to 159 months). Complete improvement was obtained in 68% of cases, partial improvement in 17%, and no improvement in 15%. Preoperative malacia was more frequent in patients with partial improvement or no improvement. Conclusions: Surgical treatment of congenital vascular ring abnormalities is effective and safe. Complications are rare. Prognostic factors include the quality of preoperative preparation of respiratory function, the type of abnormality, and the degree of malacia. J Pediatr Surg 38:539-543. Copyright 2003, Elsevier Science (USA). All rights reserved.

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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

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