Endoscopic Repair of Type IIIB Posterior Laryngeal Cleft

Procedure: Direct laryngoscopy and bronchoscopy with endoscopic repair of type IIIB posterior laryngeal cleft. Introduction: Posterior laryngeal clefts (PLCs) result from incomplete fusion of the posterior glottic laryngeal, and/or tracheal structures and are graded as types I through IV depending on depth [1].{Pezzettigotta, 2008 #12} Type IIIB clefts extend from the interarytenoid area, through the cricoid, and down to the cervical tracheoesophageal wall. Type III clefts are considered difficult to repair endoscopically and are traditionally repaired through an open approach [2]. The ability to perform an endoscopic repair is surgeon and patient dependent. Materials and Methods: Patients with clinical symptoms suspicious for a PLC--such as aspiration, recurrent pneumonias, feeding difficulties, or upper airway obstruction--undergo flexible laryngoscopy and direct laryngoscopy with bronchoscopy for the purposes of diagnosis. The interarytenoid area must be palpated with a rigid instrument to confirm the presence and determine the depth of a cleft. Endoscopic repair begins with removal of redundant mucosa along the edges of the cleft. Mucosa must be removed all the way to the apex of the cleft. Interrupted sutures, through the tracheal and esophageal mucosa on each side, are then placed starting at the apex and proceeding superiorly toward the interarytenoid area. The aryepiglottic folds are released to take tension off of the repair. Patients undergo postoperative surveillance bronchoscopies to monitor healing. Results: A 3-month old female with a type IIIB posterior laryngeal cleft underwent endoscopic repair with interrupted sutures. Surgery and postoperative care were uncomplicated resulting in a well-healed repair without airway stenosis. 8 months after the procedure, the patient was tolerating a normal diet and had an excellent voice outcome. Conclusion: Endoscopic repair can be performed in patients with major laryngeal clefts, such as a type IIIB.

Laryngeal clefts, which result from failure of fusion of the tracheoesophageal septum [3] resulting in the incomplete formation of posterior glottic, laryngeal, and/or tracheal structures, can present with a wide array of clinical symptoms depending on the depth of the cleft and patient factors. The interarytenoid muscle--along with all of the intrinsic laryngeal muscles--and the cricoid cartilage derive from the sixth branchial arch between the fifth and sixth week of embryologic development [4]. Laryngeal clefts are most commonly categorized according to the Benjamin and Inglis classification [1]. According to this classification, clefts are graded by depth as follows: a type I cleft—which is embryologically thought to derive from incomplete formation of the interarytenoid muscles [5]—does not extend past the vocal cords; a type II cleft extends into the cricoid cartilage; a type III cleft extends to the cervical portion of the tracheoesophageal septum; and a type IV cleft extends to the thoracic portion of the tracheoesophageal septum. Types III can be further divided into subclasses: type IIIa extends through the cricoid while type IIIb continues more inferiorly into part of the cervical tracheoesophageal wall [1]. Patients with type I and II laryngeal clefts often present with clinical and radiographic features that tend to mimic reactive airway disease (RAD). Recurrent aspiration can cause asthma-like symptoms that show some response to bronchodilators. In addition, radiologic findings of asthma are often similar to that of a respiratory tract infection [5]. Patients with type III or IV clefts tend to present with more severe clinical signs and symptoms including apneic episodes, hypoxia, significant aspiration, and respiratory failure. Endoscopy should be performed in any patient with unexplained respiratory symptoms, recurrent pneumonias, or feeding difficulty with signs of aspiration. Laryngeal clefts are commonly under-diagnosed or misdiagnosed [6]. All laryngeal clefts require operative direct laryngoscopy with palpation of the inter-arytenoid area for appropriate diagnosis as they are not readily visible on flexible laryngoscopy due to collapsibility of the larynx and redundant mucosa [1]. The diagnosis of type I and II laryngeal clefts tends to be more delayed, due to their more indolent nature. In contrast, type III and IV clefts are often diagnosed in the first few days of life [5]. Clinicians must have a high index of suspicion for a laryngeal cleft in any pediatric patient with unexplained respiratory symptoms, aspiration, failure to thrive, or recurrent pneumonias. Some physicians are proponents of contrast based radiographic studies, such as a modified barium swallow, prior to endoscopy. However, others believe that the risk of aspiration of contrast material outweighs the benefit and prefer to proceed with endoscopy as the initial evaluation in a patient with a suspected cleft. The treatment of clefts ranges from conservative management--such as feeding therapy, reflux medication, and optimizing respiratory status--to surgical intervention [3].  In general, clefts that are type II or deeper are always treated with surgical repair. Surgical options include an open or endoscopic approach. Open repairs utilize an anterior laryngofissure, lateral pharyngotomy, or thoracotomy approach and may require cardiopulmonary bypass [3]. Type I and II clefts are amenable to an endoscopic approach, while type IV clefts necessitate an open approach. Type III clefts are traditionally repaired through an open approach [2]; however, in some instances, they can be repaired endoscopically. The ability to do so is surgeon and patient dependent. Endoscopic repair requires that spontaneous ventilation be maintained throughout the procedure and that there is sufficient posterior glottic exposure [2]. Contraindications to endoscopic repair include 1) Insufficient exposure of the posterior glottis via direct laryngoscopy 2) the inability to reach the apex of the cleft for endoscopic suture placement and 3) the inability to maintain an adequate level of anesthesia from a cardiopulmonary perspective.

Preoperative management of patients includes reflux medication, placement of a gastrostomy-jejunostomy (G-J) tube, and Speech and Language Pathology (SLP) evaluation including a videofluoroscopic swallowing study (VFSS). This video demonstrates an endoscopic repair of a type III b posterior laryngeal cleft. After suspension with a Lyndholm laryngoscope, a right angle probe is used to confirm the presence and grade of the cleft. This patient has a type III B laryngeal cleft extending into the second tracheal ring. A Lyndholm laryngeal spreader is placed in an inverted fashion and secured to the suspension apparatus with two rubber bands. Bronchoscopy is performed with a telescope to visualize the extent of the cleft and the distal airway. Under spontaneous ventilation, surgery begins with trimming the redundant laryngeal mucosa from the laryngeal cleft edges. The cleft edge is grasped with a Bouchayer forcep and is retracted medially to determine the extent of laryngeal cleft edge to resect. Using a straight microlaryngeal scissor, the laryngeal (or tracheal) surface of the laryngeal cleft edge is incised. The esophageal surface of the laryngeal cleft is then incised with a straight microlaryngeal scissor, to complete the resection. The same is done on the contralateral side. It is important to remove all redundant mucosa along both edges but especially including the apex of the cleft.   If too much mucosa is excised there will be tension on the closure. If not enough mucosa is excised, there with be posterior tracheomalacia and airway obstruction. Hemostasis is achieved using an oxymetazoline soaked pledget prior to placement of sutures Closure should start at the distal most aspect of the cleft. A 6-0 PDS suture on a BV-1 needle is bent and used for the first stitch. It is placed from the esophageal side through to the tracheal mucosa on the left, and from the tracheal to the esophageal side on the right. The first knot should be a surgeon’s knot and a total of 4 to 5 knots should be tied down with the assistance of a knot pusher. When possible, switch to a larger suture, such as a 5-0 PDS on a bent P-2 needle, or an SS-14 polysorb violet double armed suture on a taper needle (L-179K). Interrupted stitches are placed sequentially from distal to proximal in the same fashion as the first stitch. The aryepiglottic folds are then divided to reduce tension on the laryngeal cleft closure. If necessary during the procedure, patients can be intubated with a small Microcuff endotracheal tube. However, it is ideal for patients to be breathing spontaneously throughout. Postoperatively, patients are left extubated and monitored in an intensive care unit (ICU) setting. We routinely administer nebulized steroids, proton pump inhibitors (PPI), and histamine-2 (H2) blockers. Antibiotics are given for a total of 7 days. Patients remain nil per os (NPO) for 7 days as well and received tube feeds via a G-J tube. CPAP can be used if needed. Chest physical therapy (PT) is routinely administered.

This video demonstrates successful closure of a type IIIB posterior laryngeal cleft. Surveillance bronchoscopy is performed one week post operatively to reveal that the mucosal incisions are healing and the glottic and tracheal airways are patent. One month later, the repair continues to have a healthy appearance with normal mucosa and normal airway caliber. On the day of G-tube stoma closure (eight months postoperatively), the airway has a similar healthy appearance.  The patient is currently tolerating a full oral diet and has an excellent voice outcome.

The largest published series of endoscopically repaired type III clefts includes 6 patients [2]. The same case series now reportedly includes 10 patients [3], all of which were repaired successfully. However, other results in the literature are variable: Sandu et al [7] successfully repaired 4 patients via an endoscopic approach; Garabedian et al [8] endoscopically repaired 4 patients, 2 of which required a second endoscopic procedure; Broomfield et al [9] attempted to endoscopically repair 4 children with type III clefts, 2 of which required revision endoscopic procedures and 2 of which required an open revision; Thiel et al published a case report of a patient with a type IIIB laryngeal cleft who failed both an initial endoscopic and a revision open procedure. Fukumoto et al [10] reported a successful endoscopic approach to a type IV cleft; however, the apex of the cleft was closed via an open approach and the remaining portion (a type III cleft) was then repaired endoscopically. Although some surgeons advocate a two layer closure [7, 8, 11, 12] for all laryngeal clefts, recent studies have shown that a single-layer, or “mass-closure” technique, performed endoscopically is equally as effective [5, 13, 14]. While the endoscopic repair of deep type III clefts remains challenging, it has several advantages over an open approach. Endoscopic repair avoids risks such as wound complications and potential damage to the recurrent laryngeal nerve, which can be seen with lateral pharyngotomy approaches [2]. In addition, patients who undergo endoscopic repairs may avoid the need for tracheostomy or endotracheal intubation [7]. Overall, there is a trend toward endoscopic management for airway pathology. As individual surgeons gain experience, the endoscopic repair of type III clefts will likely become more prevalent and has the potential to be a safe and effective procedure.

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Thank you to Vidal Maurrasse for providing the voice-over material.

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