Clinical, radiological and functional follow-up after surgical decompression of double aortic arch.
BACKGROUND
Double aortic arch (DAA) is a congenital vascular anomaly that causes tracheal and oesophageal compression. It requires surgical intervention in patients with severe symptoms.


OBJECTIVE
To evaluate the clinical, radiological and functional follow-up after surgical relief of the compression.


METHODS
Ten children (seven boys) with DAA were operated on at a mean age of 1.3 years (range 0.2-7.5). At a mean age of 10.1 years (range 5-18 years), a follow-up study was performed that included clinical, radiological and functional parameters.


RESULTS
Seven children reported only mild respiratory symptoms and some trouble with swallowing. Preoperative fluoroscopy with spot images showed the mean tracheal diameter at the level of stenosis to be 37+/-23% of the maximal diameter. At the time of follow-up, this was 70+/-13%. The mean of the oesophageal diameter was 39+/-20% preoperatively and 47+/-16% postoperatively. Maximal expiratory flow volume (MEFV) curves of seven children showed typical characteristics of intrathoracic upper airway obstruction. Mean peak expiratory flow was significantly reduced (77+/-10% of predicted, P<0.0001). Bronchial hyper-reactivity, tested by methacholine challenge, was found in two patients.


CONCLUSIONS
There was marked relief of clinical symptoms after surgical decompression of DAA in all patients. In spite of this, radiological narrowing of trachea and oesophagus persisted and lung function results were abnormal at long-term follow-up.
Department of Respiratory Diseases, Wilhelmina Children's Hospital, Lundlaan 6, 3584 EA Utrecht, The Netherlands.
Maartje
M
ten Berge
Johan
J
van der Laag
Cornelis K
CK
van der Ent
Frederik J A
FJ
Beek
D000293:Adolescent; D001013:Aorta, Thoracic / Q000002:abnormalities*; D016535:Bronchial Hyperreactivity; D002648:Child; D002675:Child, Preschool; D019299:Decompression, Surgical; D004940:Esophageal Stenosis / Q000000981:diagnostic imaging / Q000209:etiology; D004947:Esophagus / Q000000981:diagnostic imaging; D005260:Female; D005500:Follow-Up Studies; D005540:Forced Expiratory Flow Rates; D006801:Humans; D007223:Infant; D016013:Likelihood Functions; D008297:Male; D011859:Radiography; D014132:Trachea / Q000000981:diagnostic imaging; D014135:Tracheal Stenosis / Q000209:etiology