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Comprehensive Endovascular and Open Surgical Management of Cerebral Arteriovenous Malformations
Published on: October 20, 2017
Pediatric vascular ring outcomes for surgically repaired vs. unoperated children: a single-center experience
Houdaïfa Ajdaa1, Karlien Carbonez2, Jelena Hubrechts2
1Department of Cardiovascular and Thoracic Surgery, Université Catholique de Louvain (UCLouvain)-Cliniques Universitaires Saint-Luc, Brussels, Belgium.
Insights
Most vascular ring patients are symptomatic, requiring prompt surgical repair. While surgery improves outcomes, 24% remain symptomatic, often due to asthma, highlighting the need for comprehensive management.
Area of Science:
- Pediatric Cardiology
- Congenital Cardiovascular Abnormalities
- Thoracic Surgery
Background:
- Vascular rings are rare congenital cardiovascular abnormalities (1% of cases).
- Symptoms range from asymptomatic to severe esophageal or tracheal compression.
- Optimal management for asymptomatic infants remains debated despite increased prenatal diagnosis.
Purpose of the Study:
- To review a three-decade experience in managing vascular rings.
- To analyze outcomes of surgical repair in symptomatic and asymptomatic patients.
Main Methods:
- Single-center retrospective study of 82 patients.
- Data collected from medical records, pediatric clinic notes, and questionnaires.
- Diagnosis via echocardiography, barium swallow, and CT scans.
Main Results:
- 69% of patients were symptomatic, presenting with respiratory or feeding issues.
- Surgical repair was performed in 96% of patients, with no mortality.
- 18% experienced minor complications; 24% remained symptomatic post-surgery.
Conclusions:
- Vascular rings, particularly those causing compression, necessitate timely intervention.
- While surgery is effective, long-term symptoms persist in some patients.
- Early surgical repair in asymptomatic cases shows low morbidity, supporting current recommendations.
Background:
Vascular rings represent 1% of congenital cardiovascular abnormalities. Phenotypic expression varies from asymptomatic to severe forms related to either oesophageal or tracheal compression. While refinement in prenatal screening led to an increase in fetal diagnosis, optimal management in asymptomatic neonates and infants is currently a matter of debate. We report our center experience of vascular ring management over three decades.
Methods:
In this single-center retrospective study, data were extracted from patient medical records. To obtain information on symptoms/medication at follow-up, clinical records from pediatric clinics were reviewed. For patients followed in other institutions, a web-based questionnaire was sent to referring pediatricians.
Results:
Out of 82 patients, 69 were symptomatic (84%). Common symptoms included recurrent respiratory tract infections (43%), stridor (32%), gastro-esophageal reflux (33%), and dysphagia (26%). Diagnosis relied on cardiac ultrasound, barium swallow studies, and chest computerized tomography scan. Surgical repair (thoracotomy 91%) was performed in 79 patients (96%). Median age at repair was 13 [interquartile range (IQR), 4.4-48] months. There was no mortality. Minor complications occurred in 14 patients (18%). Median hospital length of stay was 7 (IQR, 6-9) days. In total, 24% of patients remained symptomatic (median follow-up 54 months). Half of those were asthma-related, with nearly 90% freedom from ring-related symptoms.
Conclusions:
Most patients were symptomatic at the time of diagnosis. Vascular rings such as pulmonary slings or tracheal compression syndromes require prompt management. Despite surgery, 24% of patients were not symptom-free at follow-up. Finally, surgery in asymptomatic patients resulted in low morbidity supporting the current recommendation of early surgical repair.
Level Of Evidence:
III.
