The tongue-lip adhesion

Before surgical intervention is considered, nonsurgical management should be attempted. Management algorithms begin by evaluating the effectiveness of prone positioning.^{,  ,  ,  ,  ,  ,}  Nasopharyngeal airway (NPA) placement can also be attempted should positioning fail.^{,  ,  ,}  Sher has reported that at least 50% patients treated with NPAs did not require definitive surgical intervention to correct airway obstruction. Wagener et al and Anderson et al had no failures with their nonsurgical management protocol using an NPA in the Birmingham unit.

It is critical that glossoptosis be determined as the definitive etiology for airway obstruction in a PRS infant before a TLA is performed. Sher described 4 different mechanisms of airway obstruction in patients with craniofacial anomalies, including PRS (Figure 2). The TLA has been shown to be effective in addressing Type 1 obstructions defined as posterior movement of the dorsum of the tongue to the posterior pharyngeal wall so that most of the obstruction is anteroposterior.^,  Flexible fiberoptic nasopharyngoscopy can be used to elucidate this as the underlying etiology of airway obstruction and this determination can therefore guide surgical management.^, 

The TLA is performed under general anesthesia. Local anesthesia is infiltrated (0.5% lidocaine with 1:200,000 epinephrine). A transverse incision is made on the ventral surface of the tongue just above the floor of the mouth. Short perpendicular releases are made at either end of the transverse incision and are directed toward the floor of the mouth. Care must be taken to visualize the openings of the submandibular and sublingual gland ducts. It is through this incision that the genioglossus can be stripped, though not all authors advocate this approach. A transverse incision is then made in the mucosa of the lower lip at the base of the labial vestibule and the incision is released perpendicularly at either end toward the lip (Figure 3). Both flaps should be deep to muscle. The flaps are then brought together such that the transverse incision on the ventral surface of the tongue is brought forward and sutured to the transverse incision at the base of the labial vestibule of the labial flap. This creates a continuous raw surface for adhesion (Figure 4). Once the inner flap of the adhesion is sutured, deep sutures are used to bring together the intrinsic muscles of the tongue and the orbicularis oris. It is universally agreed that this is a necessary step in the procedure to avoid wound dehiscence.^{,  ,  ,}  As mentioned earlier, several variations on the technique have been reported. Kirschner et al advocate the use of a retention button on the dorsum of the tongue that is anchored below the anterior chin (Figure 5), and this variation has been successful, reporting few complications. Argamaso has reported similar results, advocating the use of a suture looped around the anterior mandible and tied to the intrinsic muscles of the tongue. It is advisable that after the procedure is complete, endoscopic evaluation be undertaken to observe relief of glossoptosis while the infant is supine.^,  Most authors recommend a nasopharyngeal tube during the immediate postoperative period, to be left in place for 2-3 days.^{,  ,  ,}  The patient may then remain in the semirecumbent position while awake. Adequate gas exchange must be verified with continuous pulse oximetry or formal sleep study as mandated clinically. Nasogastric feeding should continue for approximately 1 week to allow healing and to avoid compromising the wound by vigorous suckling. Once oral feeding begins, and caloric intake is appropriate for daily requirements, the patient may be discharged. Frequent follow-ups to monitor growth and development and nasopharyngoscopic examinations to determine airway patency are warranted.

There is no general consensus as to when the tongue should be released. Most authors agree that it should be done at or before repair of the palate.^{,  ,  ,  ,}  Proponents of earlier takedown argue that it is essential to determine if the infant can maintain a patent airway after palatal repair. Takedown of the adhesion earlier also facilitates proper hygiene of lower central incisors. Proponents of the simultaneous takedown and palate repair appear to base their decisions solely on avoiding multiple exposures to anesthesia and hospitalization.

Largely because of modifications in incision design by Routledge in 1960, few complications have been reported in the literature, all of which are fairly uncommon. Dehiscence is the most commonly noted complication occurring in 4.2%-17.2% patients.^{,  ,  ,}  Abnormal eruption of deciduous incisors has also been reported. Though not a complication, some authors have reported a failure rate as high as 20% in the procedure. This is likely attributable to inaccurate diagnosis of the cause of airway obstruction. Contrary to popular belief, given the young age of adhesion takedown, speech development is generally not adversely affected by the procedure.^{,  ,}  Scarring that results from the TLA is usually minor and of no esthetic or functional consequence.

Although limited in its applicability, the TLA has a definitive role in the management of upper airway obstruction in infants with PRS. The evolution of the procedure over the last 60 years is a function of ingenuity in minimizing the complications of earlier techniques and, more importantly, in addressing the etiology for which the procedure is effective at circumventing—glossoptosis. It should be emphasized that a thorough history and physical examination, including genetic testing, is essential to rule out a syndromic diagnosis in children with PRS, which is found in isolation in only 20% of cases. Syndromic infants have poorer outcomes and are more likely to have persistent apnea after TLA despite nasopharyngoscopic confirmation of glossoptosis.^,  This is likely a reflection of the heterogeneous nature of upper airway obstruction in PRS. Hypotonia secondary to neurologic impairment will work synergistically to impair posterior pharyngeal obstruction. That said, the TLA is a relatively easy, reversible procedure with fewer long-term complications than either mandibular distraction or tracheostomy.