A 2800-gm infant boy was born at term to a gravida 2, para 1 mother after an uncomplicated pregnancy. The child had persistent cough, spitting and sneezing for the first 2 weeks of life. On day 16 of life, the child was admitted with a fever and a right lower lobe pneumonia. A barium swallow (Figure 1) was performed. A renal sonogram was normal. The child had no detectable facial or genital anomalies.
DENOUEMENT AND DISCUSSIONCongenital laryngotracheoesophageal cleft (CLTEC) is an uncommon malformation resulting from variable degrees of incomplete closure of the tracheoesophageal septum, incomplete fusion of the cricoid cartilage and incomplete formation of the interarytenoid muscle.1 CLTEC usually presents as a toneless or hoarse cry, stridor, cyanosis, choking, coughing, and recurrent pneumonia.2 The most frequently used classification of CLTEC is as follows: type 1, a supraglottic cleft from failure of development of the interarytenoid muscles not extending into the cricoid cartilage; 1,2 type 2, a cleft extending into the cricoid cartilage but not beyond the inferior cricoid plate; type 3, extending to the cervical and thoracic esophagus; and type 4, a cleft throughout the trachea and extending into the right mainstem bronchus.3 Associated anomalies are common in patients with CLTEC. Anorectal malformation (21%), malrotation (13%), hypospadias (7% to 13%), cardiovascular anomalies (16% to 33%), and esophageal atresia/tracheoesophageal fistula (20% to 37%) 1,4 are the most common. The frequency of CLTEC in patients with esophageal atresia/tracheoesophageal fistula is $6%. 2 BBBG syndrome is an autosomal dominant condition with high male penetrance characterized by hypertelorism, hypospadias, and swallowing