Congenital laryngotracheal (LT) cleft: Report of a successful repair in an adult

Abstract

Abstract. Congenital laryngotracheal (LT) cleft isan uncommon and highly lethal deformity unless corrected early in life. An unusual case of a patient whose cleft was diagnosed and repaired at age 24 years is described. The embryodysgenesis, clinical staging, and the diagnostic and management considerations are reviewed. A limited cleft (Evans Type I) may be more prevalent than is recognized and may be the cause of some presently unaccountable throat symptoms.Key words: Laryngotracheal cleft -Aspiration pneumonia -Embryodysgenesis.Laryngeal cleft is a rare anomaly of failed posterior closure of the larynx and trachea resulting in a freely communicating laryngotracheoesophageal space. The severity of symptoms is proportional to the length of the abnormally communicating cleft. The symptoms usually occur at first feeding in the newborn and, in large clefts, cause choking, aspiration, pulmonary infection, and a high death rate.The symptom complex of cough with feedings, aspiration with pneumonia, and upper airway obstruction of variable severity is identical to that observed in choanal atresia, tracheoesophageal fistula, and neuromuscular dysfunction of the pharyngolarynx. Laryngeal cleft is infrequently diagnosed early, and the majority of cases described in the medical literature were first detected at postmortem examination. This case report and clinical review describes an unusual instance of lar- Case DescriptionA 24-year-old male, a restaurant busboy, was evaluated for a life long history of cough associated with swallowing, chronic shortness of breath, and "over 100" bouts of pneumonia. His first episode of pneumonia occurred at 3 days of age, and he has had bouts of pneumonia at 6-8 week intervals in subsequent years. He required hospitalization at least once annually beginning in 1959, when he was 1 year old.In subsequent years, examinations for sweat chlorides, tuberculosis, alpha-l-antitrypsin, quantitative immunoglobins, thyroid function, and creatinine phosphokinase were all normal; these normal findings were obtained on many repeated determinations. Barium swallow and endoscopic examinations, also repeated on several occasions, were not diagnostic.A cinepharyngoesophagram in September 1982 identified a large defect of the posterior portion of the upper trachea communicating with the esophagus. Contrast entered the trachea through a 1 cm defect interpreted to be a tracheoesophageal (TE) fistula (Fig. 1). Initial otolaryngologic examination disclosed a "wet, hoarse quality voice and intact vocal cord function", findings that are consistent with the presumptive diagnosis of TE fistula. Operative endoscopy disclosed a normal-appearing larynx upon initial inspection and intubation. However, during esophagoscopy the endotracheal tube was visualized in the esophagus, and it was determined that a cleft deformity, rather than a fistula, was present (Fig. 2). The entire length of the cricoid lamina was absent in the posterior midline, and the cleft extended 2 em distal to the lower border of the cricoid; the mu...