As cited earlier, type I clefts are uncommon and may be overdiagnosed. They do not necessarily require operative intervention, but surgery is warranted when associated symptoms are present. Repair may be either endoscopic or open. In our experience, type III and IV clefts require open repair. In most cases, surgical intervention reveals that a cleft is more extensive than previously suspected on endoscopy. Although a lateral pharyngotomy was the mainstay of operative intervention for many years, it carried significant risks to the recurrent laryngeal nerves. In addition, access is suboptimal with a longer cleft.
We recommend a transtracheal approach for repair of type III, IV, and some type II clefts. Figures 21-4 to 21-13 illustrate a type III cleft repair. Anesthetic is provided through a shortened oral RAE endotracheal tube placed through the tracheal stoma in a child with a preexisting tracheotomy. In a single-stage procedure, after induction with an oral or nasal endotracheal tube, a temporary low tracheotomy can be placed and a shortened oral RAE tube introduced through the low tracheotomy to maintain anesthesia. An esophageal bougie is then placed. With the patient’s neck well extended, a transverse neck incision is made in a skin crease close to the level of the cricoid cartilage. Subplatysmal skin flaps are raised, and the strap muscles and thyroid isthmus divided and carried laterally. This exposes the anterior trachea. A beaver blade is used to enter the airway, and great care is taken to stay strictly in the midline. The anterior trachea is opened from the second or third tracheal ring up through the cricoid cartilage and cricothyroid membrane. The airway is held retracted with Prolene stay sutures, and the extent of the posterior cleft is ascertained. In children with a long laryngotracheoesophageal cleft, the incision in the anterior trachea is carried as inferiorly as is necessary to gain good exposure to the posterior cleft.
FIGURE 21-4 Type III posterior laryngeal cleft repair—an anterior laryngotomy and a tracheotomy.
Type III posterior laryngeal cleft repair—developing a plane between the tracheal and esophageal mucosal layers of the cleft.
Type III posterior laryngeal cleft repair—esophageal mucosal layer.
Type III posterior laryngeal cleft repair—tracheal mucosal layer.
Posterior laryngeal cleft—axial view.
Posterior laryngeal cleft—planned mucosal incisions in edges of the cleft. A. Short esophageal flap; (A1) long esophageal flap; (B) long tracheal flap; (B1) short tracheal flap.
Posterior laryngeal cleft—developing a dissection plane between the esophageal and tracheal mucosal layers.
Posterior laryngeal cleft—aligning mucosal flaps.
Posterior laryngeal cleft—closure with noncontiguous suture lines.
Posterior laryngeal cleft—view immediately following repair.
The larynx must then be split. To avoid damage to the vocal cords, this procedure should be done exactly in the midline, through the anterior commissure. In young children, the level of the anterior commissure lies one third of the way between the superior and inferior notches of the thyroid cartilage, closer to its inferior border. This procedure is most accurately performed with an assistant providing a televised view of the glottis with a rigid 30 Hopkins rod telescope. This endoscopic view enables the surgeon to place a pair of Jake forceps between the true vocal cords (from below). Opening these forceps distracts the cords, allowing an excellent view of the anterior commissure. A beaver blade is then visualized by the endoscope as an anterior laryngofissure is performed.
The larynx is distracted with Prolene sutures and the full extent of the cleft can be observed (Fig. 21-4). A significant amount of redundant mucosa is typically present. This requires removal to prevent airway compromise while still maintaining a tension-free 2-layer repair. The edges of the cleft are infiltrated with 1% lidocaine and 1:100,000 epinephrine, and 3-0 Monocryl stay sutures are placed along the edge of the cleft to provide traction. The mucosa along the edge of the cleft is then incised or excised, depending on the amount of redundant mucosa present. Particular care should be taken at the apex of the cleft distally and over the arytenoid cartilages proximally. The aim is to repair the cleft 3 to 4 mm above the level of the true vocal folds.
Both sharp and blunt dissection are used to create a plane between the tracheal and esophageal mucosal layers of the edges of the cleft (Fig. 21-5). This procedure should ideally be performed so that additional tracheal mucosa is preserved on one side of the cleft and additional esophageal mucosa is preserved on the other side (Figs. 21-8 to 21-13). This method allows for noncontiguous suture lines upon 2-layer closure.
Once this procedure has been performed, the esophageal mucosa is closed with interrupted 4-0 or 5-0 sutures over the esophageal bougie, with the knots lying in the esophageal lumen (Figs. 21-6 and 21-12). This closure commences distally, and the suture at the apex of the cleft should be tied like a purse string. Each suture can act as a retractor to allow placement of the following suture before being cut. After the esophageal mucosa is closed, we place a thin layer of fibrin glue on the suture line prior to closing the tracheal mucosa. The tracheal mucosa is then closed distally to proximally in an identical fashion to the esophageal mucosa. Sutures are placed with the knots lying in the tracheal lumen (Figs. 21-7 and 21-12), and particular care is taken over the most distal and proximal sutures. This layer is further reinforced with a very thin film of fibrin glue. Figures 21-14 to 21-19 illustrate this operative technique in a 1-year-old child.
Endoscopic view of a type III cleft—preoperative glottic level.
Endoscopic view of a type III cleft—preoperative posterior glottis.
Intraoperative view of a type III cleft. Open trachea with redundant mucosa.
Intraoperative view of a type III cleft. The trachea is open, and an esophageal bougie is displayed.
Intraoperative view of a type III cleft. The trachea is open, and the cleft has been repaired.
Endoscopic view of a repaired type III cleft 10 days postoperatively.
In a single-stage procedure (ie, without a tracheotomy), the patient is then nasally intubated. The tip of the tracheotomy tube should lie distal to the repair site. The anterior trachea is then closed using 4-0 PDS (polydioxanone) sutures through the tracheal rings and cricoid and 5-0 Vicryl sutures in the cricothyroid membrane if required (Figs. 21-20 and 21-21). The larynx must be closed with particular care so as not to overlap the vocal cords at the anterior commissure. This technique is best performed with figure-of-8 or mattress sutures at the level of the anterior commissure and further figure-of-8 or mattress sutures above and below the anterior commissure. This suture line is also reinforced with fibrin glue, and the neck is then closed in layers over a Penrose drain. If closure of the cricoid over an age-appropriate endotracheal tube cannot be accomplished without tension, a small anterior cartilage graft will allow tension-free closure. The graft may be costal cartilage or thyroid alar cartilage.
Endoscopic preoperative view of a type III cleft in a 1-month-old infant.
Endoscopic view of a type III cleft in a 1-month-old infant 1 week postoperatively (single stage).
Type I and type II laryngeal clefts are best repaired endoscopically, as this approach does not require a complete laryngofissure and therefore does not place the vocal cords or voice at risk. However, if laryngeal exposure is inadequate, access may be a problem; in such cases, an open approach may therefore be required. Similarly, an open approach may be preferable in children with an associated subglottic stenosis, as this allows both lesions to be addressed concurrently.
The original descriptions of endoscopic laryngeal cleft repairs involve creating tracheal and esophageal mucosal flaps and performing a painstaking 2-layer closure with very fine (6-0) PDS sutures on a BV-1 needle. This is a technically challenging procedure with a significant risk of repair breakdown. In recent years, we have changed our philosophy regarding endoscopic cleft repairs, and now prefer a 1-layer mass closure technique with only 1 or 2 heavy sutures placed in a mattress fashion. Our rationale is that an endoscopic repair is most likely to break down starting at the proximal aspect of the repair, and that a heavy proximal suture lessens the risk of dehiscence. The most proximal suture should pass through the cuneiform cartilage bilaterally, as this further lessens the risk of dehiscence.
Our technique is to position the patient with the larynx suspended on a suitable laryngoscope (eg, medium Lindholme) and exposed with vocal cord spreaders. Using an operating microscope and microlaryngeal instruments, we denude the mucosa of the inner aspect of the cleft, with particular attention to the apex of the cleft. Rather than removing a narrow strip of mucosa, the mucosal excision is more aggressive in order to maximize the demucosalized surface area on either side of the cleft. The raw surfaces are opposed by the placement of 4-0 PDS sutures on a taper needle. These sutures are placed in a horizontal mattress fashion. The first throw of the distal suture commences at the mucosal edge of the esophageal side and exits at the mucosal edge of the tracheal aspect on the same side of the larynx, with the suture having incorporated a large “bite” of tissue. The second throw of the needle is on the opposite side of the larynx from close to the mucosal edge on the tracheal side, exiting close to the mucosal edge on the esophageal side. The suture may be tied down with at least 6 throws, as PDS is notoriously slippery. If required, additional sutures are then placed more proximally, with the most proximal suture involving the cuneiform cartilage if possible to minimize the risk of the suture pulling through.
The use of interposition grafts is reserved for cases in which the risk of failure is either higher or potentially catastrophic. The relative risk of failure is higher in revision cases, children with a history of TEF repair, and children with Opitz-Frias syndrome. Failure is potentially catastrophic in children with type IV clefts. In all of these cases, consideration should be given to the use of interposition grafts. Suggested graft material includes temporal fascia and tibial periosteum, both of which are used as free grafts. However, in our institution, if a small graft is needed, clavicular periosteum is easily harvested from within the surgical field. If a larger graft is desired, sternal periosteum may also be obtained from the surgical field and is abundant. Periosteum is robust, but exceedingly difficult to place sutures through; thus, once the esophagus is repaired, the periosteum is laid over the esophageal suture line and stabilized with a small amount of fibrin glue. Tracheal mucosa is then closed over the top of the periosteum.
Laryngeal cleft repairs have a recognized failure rate. The repair is at the greatest risk of breakdown either at the distal end of the repair with TEF formation, or at the proximal end with cleft recurrence. Laryngeal cleft failures usually occur within weeks of the initial repair; however, late occurrences have been described several years after a successful initial repair.
Infants born with a long type IV cleft are challenging to manage for a variety of reasons. Severe aspiration is inevitable. Airway maintenance may be problematic, as an endotracheal tube may reside in the esophagus or trachea, with the “tracheoesophagus” being a common cavity. Other congenital anomalies are common and the closer the cleft is to the carina, the greater the likelihood of other major congenital anomalies; these may be classified as airway and nonairway anomalies. Airway anomalies associated with long laryngeal clefts include bronchial stenosis and bronchomalacia, which is usually left-sided. Cartilage abnormalities of the laryngotracheal complex are common and may include areas of cartilage aplasia and conjoined tracheal rings.
Nonairway anomalies include microgastria, polysplenia, and annular pancreas. Microgastria may result in uncontrollable gastroesophageal reflux, and is not amenable to a fundoplication. Prior to embarking on surgical repair of the laryngeal cleft, a decision must be made as to whether the child is salvageable.
In a child who has a highly abnormal left bronchus and associated tracheal cartilage abnormalities or in a child who has a sustained significant anoxic brain injury, the prospects of an acceptable long-term outcome are remote. If a child is deemed worth salvaging, controlling gastroesophageal reflux is highly desirable. Although an esophagogastric separation with a Roux-en-Y approach is attractive, subsequent cleft repair is more prone to dehiscence, possibly due to a compromise of the esophageal blood supply. Our current approach in a child with microgastria who cannot undergo a fundoplication is to cross-staple the stomach with a vented gastrostomy tube proximal to the staple line and a feeding gastrostomy tube distal to the staple line.
There are many methods to repair a type IV cleft, primarily because none of the approaches is ideal. The dilemmas fundamentally relate to issues of access and issues of oxygenation. Access may be through a lateral thoracotomy approach, a transsternal approach, or a cervical approach, either transtracheally or from an extended lateral pharyngotomy. Oxygenation may be achieved with the use of intubation, extracorporeal membrane oxygenation, or cardiopulmonary bypass. Having had experience with all of these methods, our current preference is to perform a transcervical repair whereby the upper trachea is exposed and transected at the lower border of the cricoid cartilage. The trachea distal to the transection is then “peeled up” off the esophagus. This is achieved by dividing the mucosa on either side of the cleft at the apex of the tracheoesophageal mucosal junction. The dissection is continued down to the apex of the cleft and, ideally, is continued a few millimeters into the tracheoesophageal groove distal to the apex of the cleft. In most children with a long laryngeal cleft, the trachea is relatively short, and this can therefore be achieved through a transcervical approach. However, if access is cramped, a limited sternotomy improves access. During this procedure, oxygenation may be obtained by an endotracheal tube placed in the trachea with the tip of the tube lying in the right mainstem bronchus. The left lung is partially ventilated through the Murphy eye of the endotracheal tube, which should lie at the carinal level. The proximal aspect of the laryngeal cleft can then be dissected, commencing at the lower border of the cricoid and progressing proximally to the arytenoids. Performing a complete laryngofissure is an option to improve access if required. The esophageal aspect of the cleft is then repaired with interrupted sutures, with the knots placed in the esophageal lumen. At this point, an interposition graft may be placed from beyond the carina up to the arytenoids; we recommend sternal periosteum as an ideal graft material because it is abundant and readily available. The posterior trachea may then be repaired in a distal to proximal fashion, with the repair being performed from the posterior aspect of the trachea. This approach permits oxygenation, as the endotracheal tube remains in position while the posterior trachea is repaired. Once the distal trachea has been repaired, the proximal tracheal mucosa may be repaired from the lower border of the cricoid cartilage to the arytenoids. The transected trachea may then be reconnected to the cricoid cartilage. The problem with this approach is that the posterior tracheal mucosa will have a 4-point anastomosis at the lower border of the cricoid, and is therefore at a higher risk of dehiscence. However, if the interposition graft and the esophageal mucosa are intact, the tracheal dehiscence eventually heals. In the event that a TEF redevelops at this site, it is easier to manage and repair than a dehiscence close to the carina.
To minimize the pressure on the suture line, we recommend managing the postoperative airway with a transnasal endotracheal tube. Although placement of a tracheotomy tube is inevitable, if this can be delayed for 2 or 3 weeks following surgery, the tip of the tracheotomy tube will not be as great a threat to the integrity of the distal repair. A stormy postoperative course is routine in these children.