Diagnostic Delay, Bronchoscopic Airway Phenotype, and Early Surgical Outcomes in Children With Vascular Rings and Pulmonary Artery Sling: A 10-Year Palestinian Referral-Center cohort.

Objective

To describe presentation, diagnostic delay, bronchoscopy-defined airway phenotype, and surgical outcomes in children with vascular rings or pulmonary artery sling at a Palestinian tertiary referral center.

Methods

Retrospective review of consecutive children with imaging- or operative-confirmed vascular rings/slings treated from 2013 to 2023. All string dates were parsed as dd/mm/yy, and ambiguous native Excel dates were checked for chronology. The primary postoperative outcome was persistent symptoms at the latest documented postoperative medical-record assessment; follow-up duration was not standardized. Continuous variables were summarized as medians (IQR) and compared with nonparametric tests; categorical variables used exact tests.

Results

Seventy-three children were analyzed: 33/73 (45.2%) had double aortic arch and 20/73 (27.4%) had right aortic arch with aberrant left subclavian artery. Median age at symptom onset was 14 days, and 67/73 (91.8%) became symptomatic within 1 year. Misdiagnosis occurred in 31/73 (42.5%) and was associated with longer diagnostic delay (10.0 vs 1.1 months, p<0.001). Among the 59 operated patients, 57 (96.6%) underwent paired flexible bronchoscopy before and after vascular decompression during the same operative session. Initial bronchoscopy showed pulsatile compression in 51/57 (89.5%), severe compression in 12/57 (21.1%), and tracheomalacia in 14/57 (24.6%). Surgery was performed in 59/73 (80.8%). Complete symptom resolution occurred in 38/59 (64.4%), persistent symptoms in 21/59 (35.6%), redo surgery in 5/59 (8.5%), postoperative complications in 16/59 (27.1%), and mortality in 2/59 (3.4%). Persistent symptoms were associated with greater presenting symptom burden (median 5 vs 3 symptoms, p=0.008).

Conclusions

Vascular rings/slings presented early but were frequently misdiagnosed. CT angiography defined anatomy, while bronchoscopy characterized airway compression and tracheomalacia. Most children improved after repair, but persistent symptoms remained common, especially in those with heavier presenting symptom burden.