To the Editor: Breath-holding spell (BHS) is a self-limiting, age-dependent common condition in children. Here, we describe a child presenting with BHS and, on evaluation, diagnosed with an unusual etiology.

A 14-mo-old girl presented with a history of frequent episodes of bluish discoloration of lips and extremities during crying since 8 mo of age. She was diagnosed with BHS locally. She had a history of noisy breathing and chest indrawing. These episodes also occurred in lying supine and during ingestion of solid foods. She was afebrile, pale, tachypneic, and had normal anthropometry but did not have clubbing or cyanosis. There were suprasternal, substernal chest retractions and biphasic stridor. Other systemic examinations were normal. Blood investigations were normal except for iron deficiency anemia. Chest X-ray showed homogenous linear opacity in the right paratracheal region. Flexible bronchoscopy revealed an asymmetrical tracheomalacia predominantly on the right side with visible pulsations. Echocardiography showed a double aortic arch (DAA) with a prominent right aortic arch. CT angiography revealed a full vascular ring encircling the trachea and esophagus at the T3-T4 level (Supplementary Fig. S1). The child was planned for surgical repair on follow-up.

Vascular anomaly is a rare congenital anomaly with an incidence of 1% and is more common in males [1]. It can be complete (ring) or incomplete (sling). DAA is the most common vascular anomaly. It occurs due to the persisting fourth aortic arches on both sides [2]. It can present as recurrent apnea, cyanotic spells, noisy breathing, dysphagia, recurrent wheezing, and recurrent respiratory infections [3]. The surgical division of vascular rings has an excellent long-term prognosis [4].

In conclusion, one should suspect vascular anomaly in any child with a cyanotic spell, stridor, and co-existing feeding difficulty.