Type IV Laryngotracheoesophageal Cleft Associated with Microgastria and Left Lung Hypoplasia: Report of a Long-term Survivor

Abstract

Laryngotracheoesophageal clefts (LTEC) are extremely rare congenital anomalies associated with a high attendant morbidity and mortality. LTEC are characterized by a midline communication between the esophagus and trachea extending from the larynx over a variable distance down to the bifurcation. The common proximal aerodigestive channel is a result of an incomplete separation of the primitive foregut into trachea and esophagus during fetal development. Clinical severity strongly depends on the length of the cleft. Many classifications have been proposed in order to distinguish the various clefts. Pettersson's anatomy-based classification divided laryngotracheoesophageal clefts into three groups according to their length. Later the classification was modified by Armitage [1] and Evans [9]. A clinically more relevant classification was offered by Benjamin and Inglis [10]: type I involves the supraglottic larynx, type II extends beyond the level of the vocal cords, type III is a total cricoid cleft with or without extension to cervical trachea, type IV is characterized by a complete extension of the cleft to the level of the carina with a common esophageal and tracheal lumen. Minor clefts account for the majority and only 3 % of all clefts are type IV. While type I may be repaired endoscopically or even treated conservatively, type II–IV clefts are fatal conditions and require surgical repair [3], [7], [21]. A review of the literature revealed a few reported cases of successful surgical repair of type IV LTEC but only a handful of long-term survivors [4], [13], [15], [27].