Bifurcation of Aorta: A Diagnostic Challenge and Associated Infectious and Congenital Anomalies

Abstract

Double aortic arch is a rare cardiovascular anomaly. It represents about 61% of vascular rings, found in both gender equally, and results from persistence of the embryonic fourth arch. It is usually discovered at postmortem or at autopsy in the dissecting room. Most of the recorded cases had symptoms of constriction of the esophagus and tracheas, resulting in death from broncho-pneumonia at an early age, but sometimes patients with this condition have survived till adulthood. It must be considered if an infant presents with noisy breathing, persistent respiratory symptoms of infection, emesis, choking, and dysphagia or acute apneic spells. Here, we document a rare case, a 6-month-old male patient with a history of repeated admissions and referrals since birth. The characteristic complaints included attacks of respiratory distress, lower respiratory tract infections, stridor, feeding problems, vomiting, and sepsis. With a persistent right aortic arch that constricts esophagus and trachea, repeated infections, hypocalcemia, and umbilical hernia supporting 22q11 deletions disorder is being presented for the first time in Pakistan pediatric population.