Laryngeal Cleft

A laryngeal cleft is a congenital condition in which the posterior wall of the laryngotracheal tract is open and food or liquids can pass from the esophagus into the trachea, leading to aspirations.

Type 1 laryngeal cleft means the opening is above the vocal cords.

Type 2 laryngeal cleft means it extends below the vocal cords.

Type 3 laryngeal cleft means it extends down into the trachea.

The Benjamin Inglis classification should be modified to include a type 4 long because type 4 could be proximal above the carina, at the carina, or go straight through the carina.

Flexible bronchoscopy is not adequate for diagnosing a posterior laryngeal cleft.

The mass closure technique developed in Cincinnati uses the same concept as endoscopic tracheoesophageal fistula repair: you want raw against raw, with wide strips of raw tissue opposed after removing the non-stick mucosal surface.

KTP laser or CO2 laser can be used for broad mucosal removal in laryngeal cleft repair, and using laser is easier to use and easier to teach with.

Open approach for laryngeal clefts is reserved for failed endoscopic repair cases, some type 2s and type 3s, using a laryngofissure approach.

For type 4 clefts, a cervical approach is used.

Type 4 long clefts present anesthetic challenges requiring double lumen tube, single lung ventilation, ECMO or bypass, and often have associated microgastria and multiple congenital anomalies.

Type 4 long clefts have a very high mortality rate of approximately 50%.

The Cincinnati team does not distinguish between anatomical type 1 cleft versus deep notch for treatment decisions; they care whether the patient is aspirating (physiological cleft).

Most type 3 clefts are attempted endoscopically unless there is a reason to go open.

The transtracheal technique for type 4 clefts involves forming layers between trachea and esophagus, sewing the esophageal layer with knots in lumen, sewing the tracheal layer with knots in lumen, with optional interposition graft.

The novel surgical technique for type 4 long clefts involves transecting the trachea at the lower border of the cricoid, peeling the trachea off the esophagus beyond the cleft while keeping the patient intubated into one bronchus, repairing the esophagus with optional second imbricating layer, placing an interposition graft (typically sternal or tibial periosteum), reconnecting the trachea to the cricoid, and placing a tracheostomy relatively late at two or three weeks.

For the type 4 long cleft case, the team waited three months until the child was greater than 5 kilograms based on outcomes data from previous research.

When placing an endotracheal tube for type 4 long cleft repair, think big, like 4.5 size.

The biggest risk with laryngotracheal esophageal clefts is that the distal end turns into a tracheoesophageal fistula.

Residual tracheoesophageal fistula can be repaired endoscopically using bugbee cautery to demucosalize the tract followed by endoscopic suture placement with P2 needle on 4-0 PDS to create raw-against-raw closure.

A type 1 laryngeal cleft can appear normal on flexible bronchoscopy but be visible on rigid bronchoscopy using less sophisticated identification techniques.

Combined flexible and rigid bronchoscopy evaluations are complementary and necessary to avoid missing laryngeal clefts.

Surgical indications for laryngeal cleft repair include ongoing respiratory symptoms, aspiration, failure to thrive, recurrent pulmonary infections, and failure of conservative measures (or in some instances before trying conservative measures).

The goal of endoscopic cleft repair is to remove the interarytenoid mucosa to create two raw surfaces and then suture the edges together.

Mass closure or layered closure technique can be used for laryngeal cleft repair, but the key requirement is that edges must be inverted when finished.

If suture bites are taken too deeply during cleft repair, edges will be inverted and the child will continue to have problems.

Interarytenoid mucosa can be resected using laser or cold steel (knife), and the choice does not matter clinically.

Cold steel is preferred over laser for minor cleft resection because it is simpler to set up, faster, and eliminates the risk of laser fire.

Complete demucosalization of the apex is essential; if incomplete, a hole will remain at the apex and the child will continue to aspirate.

Closure is performed using interrupted sutures, typically PDS on an RB1 or P3 needle depending on child size.