[size=20px]A four-kg baby with type C esophageal atresia and tracheoesophageal atresia has sputtering associated with significant desaturation and occasional apnea wirth each oral feeding. He has an intact anastomosis and minimal penetration of contrast into the airway on his postoperative esophagram. On bronchoscopy, he has mild tracheomalacia and a type II laryngotracheal cleft. There is no vocal cord paralysis.

 

The next best step for this baby with a laryngotracheal cleft is

 

A fundoplication.

 

B tracheopexy.

 

C tracheostomy.

 

D endoscopic repair of the cleft.

 

E sternotomy with laryngotracheal reconstruction.[/size]

D

(Untitled)

 

D

correct answer

D endoscopic repair of the cleft.

 

Laryngeal and laryngotracheoesophageal clefts (hereafter referred to as laryngeal clefts) result from failed fusion of the tracheoesophageal septum or lateral growth centers of the posterior cricoid cartilage during embryologic development. The incidence of laryngeal clefts is approximately one in 15,000 live births, with a slight male preponderance. Although the majority of cases are nonsyndromic, associated congenital anomalies (e.g. gastrointestinal, cardiac, urinary, craniofacial) are seen in over half of all patients.

 

Laryngeal clefts are classified by the Benjamin and Inglis classification.

• type I extends through the interarytenoid muscle but ends above the vocal cords
  
• type II extends through the posterior cricoid cartilage
  
• type III extends into the tracheoesophageal septum
  
• type IV extends through the septum into the thorax and sometimes to the level of the carina or into a mainstem bronchus
  

Respiratory symptoms predominate with severity dependent on cleft depth and the presence of associated malformations. Type I clefts may be minimally symptomatic and the diagnosis may not be established until beyond the first year of life. Type III and IV clefts often present with hypoxia and apnea within the first few days of life and demand earlier investigation. The diagnostic algorithm includes a chest radiograph, a modified barium swallow conducted with an occupational therapist and flexible fiberoptic laryngoscopy.

 

The standard of care for Type II clefts is endoscopic repair - usually performed without an endotracheal tube during spontaneous ventilation. Because of the high association of gastroesophageal reflux, empiric antireflux therapy should be considered and persistence of respiratory symptoms should prompt evaluation for other conditions including gastroesophageal reflux, tracheomalacia and swallowing dysfunction.