CRP5 CASE REPORT: VASCULAR RING AND CONGENITAL TRACHEAL STENOSIS – DOES IT RING A BELL?

Xiao Ying YAP1, Hui Chean E1, Eu Ann GAN1, Angeline Seng Lian WAN1, Asiah KASSIM2
1.Department of Paediatrics, Hospital Melaka, Malaysia.
2.Department of Paediatrics, Hospital Tunku Azizah, Kuala Lumpur, Malaysia.

Background

Vascular ring anomalies are a broad category of embryologic vascular anatomic variant. Left pulmonary artery sling (LPAS) is characterized by abnormal origin and course of left pulmonary artery which often leads to tracheal and bronchial compression. This results in varying degrees of respiratory distress, stridor and feeding difficulties.

Case report

A late preterm baby was delivered via emergency lower segment Caesarean section for fetal distress and admitted for anorectal malformation. He was asymptomatic at birth with no stridor or respiratory distress. However, during elective intubation at day 2 of life for left transverse colostomy, there was difficulty in advancing the endotracheal tube. Post extubation, he had persistent inspiratory stridor and hoarseness of voice. Flexible laryngoscope revealed normal upper airway assessment. 

At day 12 of life, he was re-intubated for worsening respiratory distress. Subsequently there was difficulty in ventilation. Serial chest x-rays showed persistent right upper lobe collapse consolidation with hyperinflation. Contrast enhanced computed tomography of thorax showed features of pulmonary sling resulting in distal tracheal and proximal left main bronchus stenosis, which was confirmed with echocardiography.

Multidisciplinary team discussions on further surgical intervention were held. His condition was also complicated with persistent bacteraemia. Preoperative bronchoscopy showed severe tracheal stenosis with massive granulation tissue at stenosis site, hence not suitable for slide tracheoplasty. No further surgical intervention was deemed suitable, therefore withdrawal of care was offered.

Conclusion

Left pulmonary artery sling in neonates is difficult to diagnose. Early recognition, specialized imaging, and timely intervention can alleviate tracheal compression and ensure optimal outcomes.