Targeting Fanconi anemia/BRCA2 pathway defects in cancer: the significance of preclinical pharmacogenomic models.

Double aortic arch (DAA) is a complete form of vascular ring causing tracheoesophageal compression. We analyzed long-term results of a series of DAAs, over a period of 16 years. Between 1987 and 2003, 29 children underwent surgery for airway and/or esophageal compression secondary to a DAA. Dominant symptoms were stridor, dysphagia, choking episodes, and life-threatening apneic spells (n = 7). Diagnosis was established by barium studies, bronchoscopy, echocardiogram, angiogram, computed tomography (CT), and magnetic resonance imaging (MRI). Seven patients had concurrent cardiac anomalies. Two children had an associated tracheoesophageal fistula. Surgery was accomplished by left thoracotomy (n = 25), right thoracotomy (n = 2) or median sternotomy (n = 2). The operative mortality was zero. There was one late death due to respiratory failure. Four (13.8%) patients had a surgical complication (chylothorax, 3 cases; acute renal failure, 1 case). Follow-up (mean 7.1 years; range 6 months to 16 years) was complete in all patients, and showed complete improvement in 22 patients and partial improvement in 6 patients. Early surgical repair of DAA is associated with low mortality, and results in marked symptomatic relief in most patients. Patients with tracheomalacia or associated asthma, constitute a high-risk group and may manifest persistent symptoms and require adjunctive procedures.