Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature.

Laryngotracheoesophageal cleft (LTEC) is a congenital midline defect of the posterior larynx and trachea and the anterior wall of the esophagus. The classification scheme of LTEC that is most widely used classification, and the one that will be reference here, is that of Benjamin and Inglis that divides the clefts into 4 types based on function. Type I involves interarytenoid musculature, Type II involves the cricoid only, Type III involves the proximal LTE and Type IV involves the thoracic TE septum.

Embryogenesis of the tracheoesophageal septumis are important in the development of LTEC. Ridges of endodermal cells develop from the lateral walls of foregut. They unite within the lumen of the foregut dividing into ventral respiratory and dorsal digestive portion. The TE septum develops in a caudal to rostral fashion and should be complete up to the level of the first ring of the trachea by the fifth intrauterine week. LTEC develops as a result of failure of the TE septum to fully develop. The phase at interruption determines the length of the cleft.

LTEC is a rare disorder that occurs in less than 0.1% of the population. Evans found an incidence of 0.4% in all patients with laryngeal abnormalities. There is a slight male preponderance. Both Phelam and Finlay noted a familial occurrence after examining sibships with this diagnosis. The pattern of inheritance is that of autosomal dominant. However, most cases are sporadic. Prenatal hydramnios and prematurity are associated with approximately 33% of all clefts.

There are many anomalies associated with LTEC. The most common association is that of the tracheoesophageal fistula. This is seen in approximately 20 % of the LTEC and increased for Types II through IV. GI tract abnormalities overall are the most common anomalies associated and include anal atresia, Meckel's diverticulum, and hiatal hernia. GER is a condition that is commonly associated and causes significant morbidity when coexisting with LTEC. Other anomalies are laryngomalacia, cleft lip and palate, tracheomalacia, bronchial and tracheal stenosis, cardiovascular defects and genitourinary abnormalities.

Two syndromes in which laryngeal cleft occurs are G syndrome and Pallister Hall Syndrome. G syndrome is a constellation of congenital anomalies with a tendency toward midline defects. The manifestations include ocular hypertelorism, hypospadias, cleft lip and palate as well as cleft larynx. Pallister Hall syndrome is an autosomal dominant inheritance and includes congenital hypothalamia, hamartoblastoma, hypopituitarism, imperforate anus and postaxial polydactyly.

The symptoms of LTEC include aspiration and cyanosis with feeds, stridor, excessive oral secretions, recurrent aspiration pneumonia, respiratory compromise, and dysphonia or aphonia. The most common symptoms are aspiration and cyanosis with feeds, which are nonspecific.

The differential diagnosis for the newborn with aspiration and cyanosis consists of anatomical defects, such as choanal atresia; CNS lesions secondary to birth trauma; prematurity; congenital infections or maternal drug use causing swallowing difficulties; and esophageal disorders such as patulous cricopharyngeal sphincter or cricopharyngeal spasm.

Proper and quick diagnosis is helpful to avoid continued damage to lungs and esophagus. If the diagnosis of laryngotracheoesophageal cleft is entertained a PA and lateral chest x-ray, cine esophagogram followed by laryngoscopy should be performed. The esophagogram may be bypassed while proceeding directly to laryngoscopy if suspicion for LTEC is high.

The most helpful tool in diagnosis is endoscopy despite numerous case reports of missed diagnosis at direct laryngoscopy. Direct laryngoscopy should be performed in all patients suspected of having a cleft, under general anesthesia and breathing spontaneously. The diagnosis is often missed at endoscopy if attention to the interarytenoid region is not given. This includes palpation of the arytenoids and placement of the patient in suspension if bimanual palpation is necessary. Accurate measurement of the defect is necessary in order to plan the reparative stage.

Management begins with stabilization of the airway. If evaluations reveal a healthy patient with LTEC, the patient may only need to be intubated and early repair may be possible. Type IV clefts may need a double arm ETT that extends past the carina into both the mainstem bronchi.

With respect to the issue of tracheotomy, most patients will require one prior to repair of the cleft. Type I clefts generally have an adequate airway and will not require tracheotomy. Due to the high incidence of association with tracheomalacia and other pulmonary anomalies, tracheotomy is usually required in cleft Types II through IV. In his review of the literature, Leape showed that all patients required tracheotomy for great periods of time, usually 1 to 4 years. Geiduschek reported the use of ECMO to aid in the repair of a Type IV cleft and required no intubation or tracheotomy to repair the cleft. This allowed improved surgical exposure and minimized postoperative tracheal trauma.

As most patients have gastroesophageal reflux it is necessary to control this prior to repair. Simple medical management may be all that is indicated. This however is rare and the patient often requires surgical intervention. Insertion of a gastrostomy tube and fundoplication may be performed in order to avoid continued aspiration and to decompress the stomach. Cotton et al advocate gastric division in all patients with LTEC, because is prevents damage to the repair and further damage to the esophagus.

Surgical repair of the lesion is attempted once the patient has been evaluated for any related anomalies and is clinically stable. Repair should be performed as soon as possible. When repair is delayed, life-threatening pulmonary complications may ensue that prohibit surgical repair and lead to the ultimate demise of the patient. The approach utilized depends on the type of cleft involved and the surgeon's preference.

Endoscopic repair of cleft larynges is best used for Type I clefts. Koltai demonstrated beneficial results of this approach, with 9 or 11 patients having a successful outcome. Thus endoscopic repair is reasonable for patients with symptomatic Type I clefts.

The least utilized approach is posterior via a posterior pharyngotomy. Pracy and Stell contend that it avoids the problem of an unstable larynx as well as avoiding possible damage to the laryngeal nerves and surrounding vasculature. However, exposure with this method is limited and extensive dissection is necessary.

The anterior approach through a laryngofissure is practiced widely. This approach is satisfactory in all LTEC, offering the advantage of excellent exposure that provides for precise trimming of the esophageal mucosa and minimizes the risk to the recurrent and superior laryngeal nerves. Adverse effects on the laryngeal development and stability have not been a problem. Careful attention to alignment of the vocal cords during closure is necessary to avoid permanent hoarseness.

Another common approach is through a lateral pharyngotomy. Most feel it affords a good view of both the laryngeal and esophageal defects for adequate two layer closure, without the questionable risk of an unstable larynx. However the risk of damage to the superior and recurrent laryngeal nerves increases as well as possible damage to the vessels. Most authors feel that it should be used for small defects above the third tracheal ring.

The repair of Type IV LTE clefts is best approached through a lateral pharyngotomy combined with a thoracotomy. The right thoracotomy approach allows access to the lower end of the cleft and to the left esophageal wall with repair being performed in the cephalad direction. Several have recommended interposition of an esophageal flap to reinforce the repair.

LTEC is associated with a high mortality rate that is directly related to the severity of the defect. In a review by Roth et al of 85 cases in 1983, an overall mortality rate of 46% was found for this condition. When looking at each cleft type, there was a mortality rate of 43 % for Types I and II, 42 % for Type III and 93% for Type IV clefts. Of the patients who died, 67% had other serious congenital malformations. There are very few patients in the literature with Type IV clefts that have survived.

Case Presentation

A 48-day-old former term white female born July 1993, to a 31-year-old G3p1Ab2 whose pregnancy was complicated by polyhydramnios. After birth, she was noted to be dusky and pale with increased oral secretions. An orogastric tube was placed and met with obstruction. The infant was diagnosed with esophageal atresia and a distal tracheoesophageal fistula, which was repaired on day of life #1. In addition, she was noted to have an atrial septal defect and significant gastroesophageal reflux, diagnosed by upper gastrointestinal series, for which a gastrostomy tube and jejunostomy tube were placed. The esophageal anastomosis healed and the patient was begun on oral feeds. Subsequently she was noted to have episodes of bradycardia and apnea with her feeds, and intermittent stridor. The pediatric otolaryngology service was consulted. Additionally, on examination she was noted to have sternal retractions with expiratory grunting. Direct laryngoscopy and bronchoscopy were performed, which revealed severe tracheomalacia as well as a Type II laryngotracheoesophageal cleft. Due to the severe distal tracheomalacia and the cleft of the larynx, tracheotomy was performed. Over the ensuing months she had several admissions to the hospital secondary to complications with her feeding tubes, which eventually resolved. In July 1994, at 11 months of age, she underwent repair of the laryngotracheoesophageal cleft. The repair was performed via an anterior laryngofissure with freshening of the cleft edges and closure in multiple layers. Postoperatively she required aggressive pulmonary toilet but steadily improved and was discharged on the ninth postoperative day. Her repair will be examined six weeks postoperatively before allowing her to feed. Because of the significant tracheomalacia, aortopexy will probably be required before tracheostomy tube decannulation.

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