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Address reprint requests and correspondence: Thomas D. Samson, MD, Division of Plastic Surgery, Penn State Hershey Medical Center, 500 University Dr, Hershey, PA 17033.
The management of Pierre Robin sequence is challenging and requires a multidisciplinary approach. A thorough workup must be done to exclude sites of airway obstruction other than the base of the tongue and to exclude central sleep apnea. Airway security is critical and can be managed nonoperatively in most children. When surgical management is necessary, the tongue-lip adhesion is the first step in many institutional algorithms. Multiple modifications have been described, but the basic premise is an anterior advancement of the tongue base and suture fixation of the tongue to the lip. This increases the diameter of the airway and results in safe and successful management in the overwhelming majority of patients. There are few sequelae with the most common being dehiscence. This can be prevented by intermuscular sutures, a circummandibular stitch, and release of the genioglossus muscle. Once mandibular “catch-up” growth has been achieved, the tongue-lip adhesion may be reversed.
The clinical triad of micrognathia, glossoptosis, and airway obstruction is referred to as Pierre Robin sequence. Although our understanding of the etiology is still incomplete, it is generally accepted that Pierre Robin represents a sequence and not a distinct syndrome as the clinical associations are the result of a prior developmental anomaly. In Pierre Robin sequence, a symmetric and hypoplastic mandible (micrognathia) forces the tongue to be posteriorly displaced which creates glossoptosis resulting in airway obstruction (Figure 1).
Figure 1Preoperative view demonstrating glossoptosis and cleft palate. (Color version of figure is available online.)
Pierre Robin sequence may occur in isolation or in conjunction with other syndromes such as Stickler, 22q11 deletion, fetal alcohol, Nager syndrome, or Treacher Collins syndrome. The incidence of isolated Pierre Robin sequence is reported to range from 17%-63%.
U-shaped clefting of the secondary palate occurs in approximately 90% of patients with Pierre Robin sequence owing to malposition of the tongue which creates a mechanical obstruction during fusion of the palatal shelves. Although there is debate in the current literature whether cleft palate is a universally defining feature, airway obstruction is always present to a degree and is the primary determinant of morbidity and mortality.
Early recognition and management of Pierre Robin sequence is essential to preventing devastating complications associated with airway obstruction including feeding difficulty, aspiration, failure to thrive, chronic hypoxemia, elevated pulmonary vascular resistance, cor pulmonale, and death.
When treating a child with Pierre Robin sequence, the first priority is properly addressing the airway. Prone positioning employs gravity to relieve the posterior displacement of the tongue which can obstruct the oropharynx. This simple nonsurgical modality has been reported to successfully treat airway obstruction in 50%-70% of patients with Pierre Robin sequence.
In a recent study by Meyer, 48% of children who required an airway intervention were successfully treated with a nasopharyngeal airway. In 1 series, those treated with nasopharyngeal airways required continuous pulse oximetry and a prolonged hospital stay (mean = 60 days, range: 25-162 days).
Tongue-lip adhesion, or glossopexy, is a well-established part of the surgical algorithm for airway obstruction in Pierre Robin sequence. The first rudimentary description of the tongue-lip adhesion is credited to Shukowsky
formally described the tongue-lip adhesion by suturing the anterior portion of the tongue in a forward position in 1946. Since that time, there have been modifications to the technique and debate regarding the role of tongue-lip adhesion in the management of Pierre Robin sequence. Modifications have included intermuscular sutures, an anchoring stitch around the anterior mandible, a retention button over the base of the tongue, and division of the genioglossus muscle for anterior mobilization of the tongue. Independent of the technique, tongue-lip adhesion has a reported success rate ranging from 83%-100%.
Mandibular distraction osteogenesis has also been advocated for the treatment of Pierre Robin sequence. In this technique, bilateral osteotomies are made in the mandible and an external or internal distraction device gradually lengthens the mandible, which increases the pharyngeal airway size.
Although the procedure is safe, the distraction progresses at a rate of 0.5-2 mm per day. As such, the airway must be secured during the process with an endotracheal tube, tracheostomy, or nasopharyngeal airway. Potential complications include damage to the marginal mandibular and inferior alveolar nerves, injury to premolar tooth buds, infection, and dislodgement of the distractor.
The decision to use distraction osteogenesis vs tongue-lip adhesion appears to be based on surgical training and philosophy.
Regardless of the philosophy, there is agreement that tracheostomy is considered a last resort. Tracheostomy may be indicated in patients who have failed to respond to other measures or when there are multiple levels to the airway obstruction.
Indications
Surgical treatment of Pierre Robin sequence has the potential for severe morbidity and should only be explored when conservative methods have been exhausted. There is significant heterogeneity between the anatomy, clinical presentation, and severity of patients with Pierre Robin sequence, even when comparing isolated or syndromic patients. As such, it is imperative that a thorough workup of airway obstruction is undertaken to isolate the etiology.
A complete flexible nasopharyngoscopy and bronchoscopy evaluation is mandatory. There may be multiple levels of obstruction or other pathologic anomalies such as tracheomalacia, acute angulation of the basicranium, subglottic anomalies, or hypotonia. Anterior mobilization and adhesion of the tongue will not correct these other underlying pathologies and is not indicated in these mixed clinical presentations.
Polysomnography is an important study used to differentiate between central and obstructive sleep apnea. A polysomnogram consists of a 24-hour recording of a nasal thermistor, oral thermistor, end-tidal carbon dioxide, thoracic and abdominal strain gauges to monitor mechanical respiratory effort, electromyography of the phrenic nerve, pulse oximetry, electrocardiography, and tachography. Central sleep apnea is diagnosed when no muscle effort is associated with apnea, and conversely obstructive sleep apnea is diagnosed when there is muscle effort in the absence of detected airflow.
Technique
Tongue-lip adhesion is performed under general anesthesia preferably via nasotracheal intubation. In our institution, patients are given a preoperative weight-based dose of intravenous clindamycin for bacterial prophylaxis as well as dexamethasone to minimize postoperative edema. Deep venous thromboembolism prophylaxis is not indicated.
Once the patient is properly anesthetized, local anesthesia (1:1 mix of 0.25% bupivacaine and 0.5% lidocaine with 1:200,000 epinephrine) is infiltrated at the base of the tongue, submentally and along the lingual and buccal aspect of the mandible.
After adequate time for the epinephrine to take effect, a stab incision is made on the lingual aspect of the symphyseal region of the mandible. Sharp dissection is used to release the floor of the mouth by dissecting the origin of the genioglossus muscle. Although not all authors advocate this step, the release allows for anterior advancement of the tip of the tongue.
A small skin incision is made at the submental position to introduce an Obwegeser awl. A circummandibular 3-0 nylon suture is placed through the base of the tongue at the junction of the circumvallate papillae (Figure 3). The stitch is then tagged and later tied down once the mucosal flaps have been inset.
Figure 3Circummandibular nylon suture (different patient). (Color version of figure is available online.)
Inferiorly based, triangular mucosal flaps are raised on the ventral surface of the tongue approximately 5 mm posterior to the tongue tip. Care must be taken to avoid the submandibular and sublingual gland ducts. Reciprocal inferiorly based triangular mucosal flaps are raised on the lingual aspect of the lower lip at the base of the labial vestibule (Figure 2). Once the mucosal flaps are raised, the genioglossus and orbicularis oris muscles are approximated with interrupted 4-0 Vicryl sutures that are tagged and set aside (Figure 4). This step is crucial to avoid wound dehiscence. The circummandibular 3-0 nylon stitch is then tied down. The tongue must be closely inspected to assure vascularity of the tip after tying this suture. The muscular sutures are then tied down. Next, the mucosal flaps on the base of the tongue are then approximated to reciprocal mucosal flaps on the lower lip with interrupted 5-0 Vicryl stitches (Figure 5).
Figure 2Mucosal flap markings on ventral tongue and lower lip. Note: Stay sutures in anterolateral tongue. (Color version of figure is available online.)
After closure, an appropriately sized nasopharyngeal airway is placed at the patientʼs bedside. The patient is extubated.
Postoperative care
The patient is monitored in the postanesthesia care unit overnight for postoperative lingual edema. Most patients can be discharged home the next day. Continuous pulse oximetry is monitored to ensure adequate gas exchange and relief of the obstruction. Strict head-of-bed elevation is maintained. If a nasogastric feeding tube was previously placed, it is continued until adequate oral caloric intake is achieved. If the patient is feeding orally, specialized nipples, such as the Haberman or Pigeon, are used to avoid vigorous suckling, which could compromise the adhesion in the initial few weeks. Postoperative polysomnography is obtained as clinically indicated.
Release of the tongue-lip adhesion
As mandibular growth occurs over the first year of life, the tongue base is “pulled” anteriorly thereby increasing the upper airway diameter. This leads to stability of the airway and serves as an indication for takedown of the tongue-lip adhesion. This is typically performed at approximately 15 months of age. When a cleft palate is present, the cleft should be repaired before release of the tongue-lip adhesion. Approximately 2-3 months following cleft palate repair, the tongue-lip adhesion may be released if the airway is felt to be secure.
Takedown of the adhesion is performed under general anesthesia via nasotracheal intubation. Once the patient is properly anesthetized, local anesthesia with epinephrine is infiltrated at the tongue-lip junction. After adequate time for the epinephrine to take effect, the interface is incised and the intermuscular plane is dissected. The circummandibular suture is identified and removed. Next, the mucosal flaps are rotated back into position and affixed with 4-0 Vicryl sutures.
After closure, a nasopharyngeal airway is sized and placed at the patientʼs bedside. The patient is then extubated and admitted for 23-hour observation (Figure 5).
Figure 5Immediate postoperative photograph showing tongue-lip adhesion, and correction of mechanical airway obstruction from posterior tongue positioning. (Color version of figure is available online.)
Complications from tongue-lip adhesion are primarily related to postoperative lingual edema. These are best managed preemptively with perioperative steroids, continued postoperative nasotracheal intubation if the airway is not felt to be secure, and having a sized nasopharyngeal airway available for emergency purposes at the patientʼs bedside.
The most common complication is dehiscence of the adhesion. In a study by Kirschner et al,
the overall dehiscence rate was 17%, but a subset analysis noted a 41.6% dehiscence rate with a mucosal adhesion only; however, none were observed when the adhesion included intermuscular sutures. The combination of a circummandibular suture and an intermuscular adhesion has drastically reduced the dehiscence rate in our institution. Careful attention to the use of specialty feeding nipples in the immediate postoperative period is also important to protect the adhesion.
Conclusion
The management of Pierre Robin sequence is challenging and requires a multidisciplinary approach. In our institution, all children who present with micrognathia, glossoptosis, and airway compromise undergo a thorough evaluation. The initial management algorithm begins with prone or lateral positioning. The majority of our patients have been able to control their airway with positioning alone.
A thorough endoscopic and bronchoscopic assessment is performed to delineate the site of obstruction. Laryngotracheal anomalies are appropriately treated with tracheostomy as needed. Patients also undergo polysomnography to assess for central or obstructive sleep apnea. Genetic testing is obtained as clinically indicated.
When positioning is insufficient and the workup has not revealed any other pathologic processes, the childʼs airway is secured with nasopharyngeal or orotracheal intubation as a temporary measure until a tongue-lip adhesion is performed. By combining a circummandibular stitch with an intermuscular adhesion and release of the genioglossus origin, we have had success with the tongue-lip adhesion. To date, we have not noted any long-term compromise of mandibular growth, eruption or growth of mandibular incisors, adhesion failure, feeding difficulties, or effects on speech among our patients.
Although we favor tongue-lip adhesion for patients, there are surgeons who favor mandibular distraction as the first-line surgical treatment. We would use mandibular distraction secondarily such as when there was a failure of a tongue-lip adhesion.
References
Shprintzen R.J.
The implications of the diagnosis of Robin sequence.
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