Management of sleep-related breathing disorders in children

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Chapter 63 Management of sleep-related breathing disorders in children

David H. Darrow, Kaalan E. Johnson

1 INTRODUCTION

Sleep-related breathing disorders (SRBD) are a common occurrence in children. While such events may be central in etiology, most affected children experience obstructive sleep disorders, caused by anatomic variations and complicated by the diminished pharyngeal muscle tone and neurophysiologic changes that typically accompany sleep. Hyperplasia of the tonsils and adenoid is the most common cause of such obstruction, but infants and toddlers may be affected at a variety of sites in the upper respiratory tract. Causes of SRBD in children may be grouped on the basis of age, simplifying the differential diagnosis for a given patient (Table 63.1). In small children, caliber of the airway may be reduced due to nasal obstruction, compromised skeletal anatomy, hyperplastic pharyngeal or laryngeal soft tissues or neuromuscular compromise. In addition, in older children, obstruction due to obesity has been encountered with increasing frequency over the last two decades.^1,²

Table 63.1 Causes of sleep-related breathing disorders in children

Neonates and infants

Nasal aplasia, stenosis, or atresia

Nasal or nasopharyngeal masses

Craniofacial anomalies:

Hypoplastic mandible (Pierre Robin, Nager’s, Treacher Collins)

Hypoplastic maxilla (Apert’s, Crouzon’s)

Macroglossia (Beckwith–Wiedemann)

Vascular malformations of tongue and pharynx

Congenital cysts of the vallecula and tongue

Neuromuscular disorders

Toddlers and older children

Rhinitis, nasal polyposis, septal deviation

Syndromic narrowing of nasopharynx (Hunter’s, Hurler’s, Down’s, achondroplasia)

Adenotonsillar hyperplasia

Obesity

Macroglossia (Down’s)

Vascular malformations of tongue and pharynx

Neuromuscular disorders

Iatrogenic

Nasopharyngeal stenosis

Successful management of SRBD in children depends on accurate identification of the site of obstruction and assessment of the severity of obstruction. Only then can appropriate surgical and non-surgical remedies be considered.

2 DIAGNOSIS OF SRBD IN CHILDREN

In most children with SRBD, the presenting symptom is stertor, a term used to describe sonorous breathing in the upper airways, including snoring. The prevalence of snoring during sleep has been reported as 3–12% in children,³ although some studies suggest the rate may be as high as 27%.⁴ Primary snoring is generally not considered a form of SRBD, although some studies do suggest the possibility of associated morbidity such as elevated blood pressure and reduced arterial distensibility.⁵

In its mildest form, SRBD presents as upper airway resistance syndrome (UARS). Affected children demonstrate episodic arousals resulting from partial obstruction of the upper airway, associated with symptoms of heroic snoring, mouth-breathing, sleep pauses or breath-holding, gasping, perspiration, and enuresis. Daytime manifestations of sleep disturbance include morning headache, dry mouth, halitosis, and most significantly behavioral and neurocognitive disorders.6 Hypersomnolence may occur in older children and adolescents. Other signs and symptoms include audible breathing with open mouth posture, hyponasal speech, and chronic nasal obstruction with or without rhinorrhea. Approximately 40% of children who snore demonstrate more significant degrees of obstruction characteristic of obstructive hypopnea syndrome (OHS) or obstructive sleep apnea syndrome (OSAS) as defined below.⁶ The most severely affected patients may develop cor pulmonale, right ventricular hypertrophy, congestive heart failure, alveolar hypoventilation, pulmonary hypertension, pulmonary edema, or failure to thrive, and are at risk for permanent neurological damage and even death.

Physical examination is critical in establishing the site of obstruction and will ultimately determine the most appropriate surgical intervention. The examination should include assessment of the patient’s weight and body habitus, a complete examination of the head and neck with attention to potential sites of obstruction, and auscultation of the patient’s heart and lungs. Friedman tongue position (FTP), described in Chapter 16 on clinical staging, should be assessed. Children with FTP III or IV have significant retrolingual obstruction in addition to possible tonsillar and adenoid obstruction. Postoperative PSL and follow-up is essential in those patients since they are likely to have residual or recurrent OSAHS after tonsillectomy and adenoidectomy. Findings of nasal dyspnea or mouth-breathing, hyponasal speech, mandibular hypoplasia, drooling, neuromuscular deficit, and tonsillar hyperplasia all suggest some degree of upper airway obstruction. In many cases, such as infants in whom the jaw is extremely micrognathic or toddlers in whom the tonsils are massively enlarged, office examination alone may suffice. Ancillary studies such as high kilovoltage radiography of the adenoid pad and/or lingual tonsils and direct fiberoptic assessment of the nasal vault, adenoid pad, and distal pharynx and larynx may be useful in selected cases. In severely obstructed children, chest radiography and electrocardiography should be considered as well. In cases of nasal obstruction in infants and young children, CT scanning may be desirable in order to define the bony anatomy and to assess the relationship of nasal masses to the sinonasal tract and the central nervous system. At some institutions, cross-table lateral fluoroscopy, drug-induced sleep endoscopy, or cine MRI may be performed during sleep to aid in localizing the site of obstruction.

When a history of severe symptoms of sleep disturbance correlates with obvious physical findings of airway obstruction, additional studies to establish a patient’s candidacy for surgical intervention may be superfluous. However, studies suggest that in most cases accurate diagnosis of SRBD cannot be established solely on the basis of a history and physical examination.⁷ SRBD occurs primarily during REM sleep when children are less likely to be observed by their parents,⁸ and in many cases of UARS and OHS parents may misinterpret the symptoms only as snoring in the absence of obstruction. In addition, airway dynamics during sleep cannot be determined by static examination in the office setting, and fiberoptic assessment of the airway is often deceptive due to the distorted wide-angle view. Similarly, radiographic assessment of the adenoid tissue and tongue base may be difficult to interpret. In such cases, polysomnography (PSG) remains the gold standard for objective correlation of ventilatory abnormalities with sleep disordered breathing.⁷ However, the severity of obstruction for which intervention is recommended remains controversial, with most authors advocating aggressive management of children whose Respiratory Distress Index (RDI; obstructive apneas+hypopneas per hour) is between 1 and 5. Furthermore, the expense and scheduling difficulties associated with PSG make this a cumbersome method of assessment in many otolaryngology practices. Other techniques of assessment including audiotaping, videotaping, and home and abbreviated PSG have demonstrated favorable results, but require further study.

3 MANAGEMENT OF SRBD IN CHILDREN

3.1 NON-SURGICAL MANAGEMENT

Treatment of SRBD in children is tailored to the etiology of the airway obstruction. Medical management, such as thioxanthines and methylphenidate, may be useful in cases of central apnea. Pharmacotherapy may also be considered in less severe cases of obstructive apnea, or when surgical intervention does not address the pathology. Examples of such disorders include neonatal rhinitis, allergic rhinitis, and acute tonsillitis. Systemic steroids are effective in reducing lymphoid hyperplasia, but the results are often transient. In cases of chronic upper airway obstruction, positive airway pressure has demonstrated high efficacy in treating SRBD in children; however, the dropout rate is high and the rate and duration of nightly use are relatively low even among compliant children.⁹ Other alternatives, such as mechanical correction by prostheses, orthodontia, or weight loss, may be worth consideration as well, although these interventions are rarely tolerated in children and are often ineffective. As a result, even in cases of SRBD due to obesity and neuromuscular impairment, surgical management is generally considered as first-line therapy and other modalities are entertained postoperatively to address residual obstruction.

3.2 SURGICAL MANAGEMENT

3.2.1 PREOPERATIVE PLANNING

Preoperative planning is an essential component of the surgical management of patients with SRBD. Such individuals are prone to airway embarrassment during induction of anesthesia, and the surgeon and anesthetist should discuss emergency intervention for the airway should the patient become acutely obstructed. In most cases, dynamic airway collapse may be overcome by bag/mask ventilation used with an oral airway. In patients with severe micrognathia, macroglossia, or lingual tonsil hyperplasia, visualization of the larynx may be compromised. In such cases, the operating team should preoperatively assemble those items necessary for intubation with a Bullard laryngoscope, flexible fiberoptic bronchoscope, or fiberoptic intubation wand, and patients should be intubated under conditions of maximal topical and minimal systemic anesthesia, preferably in the sitting position.

Postoperative respiratory distress is common after surgery for SRBD due to effects of anesthesia, bleeding, edema, and residual airway compromise. Patients at greatest risk include those with severe OSAS, diminished neuromuscular tone (i.e. cerebral palsy), morbid obesity, skeletal and craniofacial abnormalities such as hypoplasia of the midface and/or mandible or nasopharyngeal vault, and very young children (under age 2–3).^10,¹¹ As a result, high-risk individuals who are undergoing even routine procedures such as adenotonsillectomy should be admitted to a high-visibility bed in the hospital with continuous cardiac and oxygen saturation monitoring. Intraoperative use of steroids and postoperative placement of nasopharyngeal airways and tongue sutures for anterior traction may reduce the risk of airway compromise after surgery. Narcotics and sedatives should be used sparingly in severely obstructed children. Obese patients and those with reduced neuromuscular tone may benefit from airway support with positive airway pressure. In the most extreme cases, overnight endotracheal intubation may be desirable. If it is suspected that the airway may be difficult to reintubate should obstruction occur after extubation, the tube should be remove in the operating room setting.

3.2.2 NASAL AND NASOPHARYNGEAL OBSTRUCTION

SRBD due to nasal and nasopharyngeal masses is best addressed by removal of the mass. Depending on the pathology, the procedure may be as simple as a transoral, retropalatal approach for adenoidectomy or marsupialization of nasolacrimal duct cysts, or as complex as an anterior craniofacial approach for encephalocele. In most cases, microdebriders fitted with blades of appropriate size can be used to resect such lesions (see Adenotonsillectomy section below). Some nasal and nasopharyngeal neoplasms may require aggressive resection, as well as preoperative embolization (juvenile nasopharyngeal angiofibroma) or postoperative radiation therapy or chemotherapy (malignancies).

Choanal stenosis or atresia and stenosis of the piriform aperture are common causes of obstructive apnea in neonates. While unilateral disease is often well tolerated until school age years, bilateral cases usually require early intervention due to the dependence of neonates on nasal breathing. Affected infants present with cyclic cyanosis relieved by crying, and the diagnosis is further established by inability to pass a 6-French suction catheter through the nasopharynx. Respiratory distress can typically be relieved using an oropharyngeal airway; the diagnosis is then confirmed and characterized by CT scanning. Such babies should not otherwise leave an intensive care setting until a secure airway is established by tracheotomy or repair of the bony defect. Timing of surgery is controversial; early repair with avoidance of tracheotomy is always desirable, but children several weeks to several months old will better tolerate bleeding and will better accommodate instruments used in the nasal cavity.

While choanal atresia may be approached by either the transpalatal or the transnasal route, improvements in endoscopic and powered instrumentation have made the transnasal approach the first choice for most otolaryngologists. Our preferred approach is as follows.

1. Anatomy of the nasal cavity is confirmed endoscopically using a small rigid nasal endoscope. The mucosa overlying the atresia plate is injected with 1% lidocaine with 1:100,000 epinephrine and the nose is briefly packed with pledgets of oxymetazoline.

2. The patient’s head is placed in extension and a Malek mouth gag (Stryker-Leibinger; Kalamazoo, MI) suspended on a stack of towels is used to facilitate exposure of the nasopharynx. A 3-0 silk suture placed through the uvula is secured cranially outside the patient to improve the exposure.

3. A 120° rod lens telescope (Karl Storz; Culver City, CA) is placed in the oral cavity with the palate retracted to directly visualize the atresia plate(s) via the nasopharynx.

4. A 6-French Van Buren urethral sound is passed into the nasal cavity on one side. Contact between the sound and the atresia plate is confirmed endoscopically by visualizing the sound through the thin plate, or by observing mucosal movement if the plate is thicker.

5. The atresia plate is perforated with the sound (Fig. 63.1A), and the perforation is dilated as much as possible using progressively larger sounds. This procedure is duplicated on the opposite side.

6. A backbiting forceps is introduced into one side of the nose and passed closed through the perforated atresia plate. The forceps are then opened and used to excise the posterior portion of the vomer (Fig. 63.1B).

7. Once the vomer has been considerably reduced, the mouth gag is removed and the procedure is completed transnasally using a 0° telescope. A 2.9 mm or 3.5 mm microdebrider blade is used in oscillating mode to remove any loose or undesirable soft tissue or cartilage. If necessary, the microdebrider may also be fitted with a 2.9 mm round burr used at high speed to drill down any remaining bony prominences.

8. Mitomycin C, which has been reported to reduce the risk of restenosis, may be applied topically to the choanal mucosa at a concentration of 0.4 mg/ml.

9. Stenting of the choanae is not usually necessary, but may be considered in some cases. Our preference is to use individual endotracheal tube stents for each side of the nose, trimmed to keep one end just inside the nasal introitus and the other just beyond the choana. The tube length is measured and recorded so caregivers can suction the appropriate distance. The tubes are rotated to place the beveled end facing anteriorly; a 2-0 silk sutures is then placed transeptally between the tubes and an additional suture is placed through the inferior tip on each side, securing the tube sublabially.

Fig. 63.1 Endoscopic transnasal repair of choanal atresia. Viewed through a 120° telescope in the oropharynx, the atretic plate is perforated with a Van Buren sound (A). After dilating the perforation to the largest sound, backbiting forceps are introduced transnasally and used to resect the nasopharyngeal end of the nasal septum (B). After initial resection of bone, the endoscope may be placed transnasally to remove bone with greater precision.

In cases of piriform aperture stenosis, the offending bone may be approached through a sublabial approach (Fig. 63.2), and the aperture is widened using the 2.9 mm round burr at high speed. Stents may be placed as in choanal atresia repair if desired.

Fig. 63.2 Transoral repair of piriform aperture stenosis. Piriform aperture is exposed by a sublabial approach. Intranasal degloving incisions may be added if necessary. A drill or microdebrider fitted with a small cutting burr is used to widen the aperture bilaterally.

Patients return to the office 2–4 weeks postoperatively for reassessment and stent removal if placed, and again 8 weeks postoperatively. Steroid-containing ototopical drops may be used to reduce the risk of infection and granulation tissue. Should respiratory distress return, a trip to the operating room for dilation and/or revision should be considered.

Restenosis is the most common complication of these procedures. Success rates (permanent patency) in recent studies are quoted as 78% to 95% with 1–3 year follow-up; number of required revisions is very variable.^12,¹³

Nasopharyngeal stenosis, once a common complication of syphilis, may result as a complication of adenotonsillectomy, uvulopalatopharyngoplasty, or surgery for cleft palate or velopharyngeal insufficiency. This disorder often causes obstruction of the upper airway that is even more significant than the disorder the original surgery was intended to correct. Typically, cicatrix forms circumferentially in the velopharynx as a result of excessive removal of mucosa from opposing surfaces.

In mild cases, lysis of the scar with some combination of steroid injection, topical application of mitomycin C, and nasopharyngeal stenting may be sufficient to re-establish adequacy of the nasopharyngeal airway. However, frequently simple release of the scarred area results in recurrence, and treatment must therefore include the movement of fresh, well-vascularized tissue to cover the denuded bed. A variety of techniques has been recommended, including Z-plasty, laterally based pharyngeal flaps, other advancement and rotation flaps, and radial forearm and jejunal free flaps. In most cases, the unilateral laterally based pharyngeal flap described by Cotton ¹⁴ results in an adequate nasopharyngeal airway without restenosis. The procedure is as follows (Fig. 63.3).

1. The pharynx is exposed using a tonsil/adenoid mouth gag of the surgeon’s choosing (Fig. 63.3A). A 3-0 silk suture is placed through the uvula for purposes of retraction. The pharyngeal wall is injected with 1% lidocaine with 1:100,000 epinephrine lateral to the remaining velopharyngeal port.

2. The stenosis is released using a scalpel and scissors if necessary (Fig. 63.3B). The incision is carried through the underlying muscle and as far lateral as possible.

3. Flaps are developed submuscularly superior, inferior, and lateral to the incision (Fig. 63.3C).

4. A laterally based posterior pharyngeal flap is incised including an inferior back cut. The flap is elevated with the underlying muscle (Fig. 63.3D and E).

5. The area denuded by release of the stenosis is covered by advancing the pharyngeal flap. Closure is performed with 4-0 Vicryl and chromic gut sutures (Fig. 63.3F).

6. The entire area is injected with triamcinolone 40 mg/ml to reduce the risk of restenosis. Application of mitomycin C may also be considered.

7. Stents are placed, consisting of soft nasopharyngeal airways, endotracheal tubes, or prefabricated prostheses. We prefer to leave the stents for 8 weeks or as long as tolerated, although the necessary duration of such stenting is controversial.

Fig. 63.3 Laterally based pharyngeal flap for correction of nasopharyngeal stenosis. A, The velopharyngeal port is circumferentially narrowed. B and C, A retraction suture is placed in the uvula and a lateral incision is made from velopharyngeal opening into lateral scar on one side and deepened. D, The mucosa is elevated from the scar inferolaterally; excess scar may be excised. A laterally based posterior pharyngeal flap is incised incorporating a back cut. E, The flap is elevated along with the underlying muscle. F, Points (A₁) and (B₁) are closed to points (A) and (B) respectively, covering the denuded area. (After Cotton RT, Nasopharyngeal stenosis. Arch Otolaryngol 1985;111:146–8, Copyrighted 1985, American Medical Association.)

In refractory cases, more distal flaps such as the facial artery myomucosal (FAMM) flap or the sternocleidomastoid myocutaneous flap may be necessary considerations. Patients should be followed for at least 12 months postoperatively for surveillance of restenosis and compromise of vocal resonance.

3.2.3 ADENOTONSILLAR HYPERPLASIA AND OROPHARYNGEAL OBSTRUCTION

Adenotonsillectomy is generally the first intervention considered for most patients with SRBD, providing they have at least mild adenotonsillar hyperplasia. Improvements in snoring and polysomnography may be anticipated postoperatively in such patients, including those in whom obesity exacerbates their airway obstruction.15–18 Improvement following adenotonsillar surgery has also been demonstrated in children with preoperative enuresis, orthodontic abnormalities, and behavioral issues prior to surgery. Validated surveys suggest an overall improvement in quality of life after adenotonsillectomy.^19,²⁰

Over the past century, there has been an evolution in tonsillectomy and adenoidectomy indications, techniques, and devices. In the age of ether anesthesia, clinicians emphasized expedience and efficiency at the expense of complete dissection of the tissues. As improvements in anesthesia permitted more methodical dissection, new techniques were developed in an effort to reduce pain and bleeding associated with the procedure. Several new devices for tonsillectomy and adenoidectomy have been proposed in recent years as technology has evolved.

With the report by Krespi and Ling of serial tonsillectomy with CO₂ laser in the outpatient setting came the notion that partial tonsillectomy was safe and less painful than traditional tonsillectomy for patients with tonsil hyperplasia.²¹ It is theorized that the exposure of muscle resulting from removal of the tonsil capsule is the cause of pain associated with tonsillectomy, and that leaving some small portion of the tonsil behind may vastly diminish this sequela.²² Proposed many years ago, the technique had been abandoned due to the risk of tonsil regrowth at a time when most such procedures were performed for recurring infection. Initially studied using the CO₂ laser, intracapsular tonsillectomy is now more commonly performed using the microdebrider given its greater efficiency and lower cost (Fig. 63.4). Randomized studies comparing microdebrider intracapsular tonsillectomy to complete electrocautery tonsillectomy generally suggest a modest advantage in pain reduction, particularly otalgia, and return to normal activity; other outcomes yielded less consistent results.^23–26 The procedure involves a slight increase in duration and blood loss. Devices for traditional tonsillectomy developed over the last 20 years, such as the harmonic scalpel (Ethicon Endo-Surgery; Cincinnati, OH), radiofrequency devices (Somnoplasty, Somnus Medical Technologies, Sunnyvale, CA), and Coblation® (Fig. 63.5; ArthroCare Corp., Sunnyvale, CA) have been studied in small trials that suggest modest reduction in pain compared with electrocautery, with further decrease in postoperative pain when the tonsil is reduced rather than excised.

Fig. 63.4 Power-assisted intracapsular tonsillectomy using a microdebrider. The microdebrider is used at 2000 rpm.

Fig. 63.5 Tonsillectomy performed using Coblation® (ArthroCare Corp., Sunnyvale, CA). The procedure may be used to excise the entire tonsil along the capsule or in a tonsil-sparing, intracapsular technique.

Techniques of adenoidectomy include curettage, suction electrocautery ablation (Fig. 63.6), Coblation® (Fig. 63.7) and removal by power-assisted devices (Fig. 63.8). Traditional curettage is inexpensive but is the least precise of these techniques and is associated with hemorrhage that must be controlled before leaving the operating room. Electrocautery dissection, by definition, is associated with less bleeding and is also a precise and inexpensive device. However, high settings are required on the cautery device, with the potential for thermal injury to deep structures, and surgical times have been variable. Studies of power-assisted (microdebrider) techniques have demonstrated excellent precision with rapid removal of tissue and minimal additional time for cautery, but the disposable blades add significant expense.

Fig. 63.6 Suction cautery adenoidectomy. High cautery settings (40 watts or more) are used to fulgurate the tissue, and suction facilitates its removal.

Fig. 63.7 Adenoidectomy performed using Coblation® (ArthroCare Corp., Sunnyvale, CA). The wand is bent for application in the nasopharynx.

Fig. 63.8 Power-assisted adenoidectomy using the Straight Shot® microdebrider (Medtronic/Xomed; Jacksonville, FL) fitted with the RADenoid® blade. The microdebrider is used at 2000 rpm.

Uvulopalatopharyngoplasty is not commonly performed in children, perhaps since most children with sleep apnea do not demonstrate the redundant tissue found in adults with similar symptoms. Several studies have demonstrated that the procedure is efficacious in the most difficult-to-treat patients with SRBD, particularly those with obesity, neurological impairment, or Down syndrome. However, these reports are retrospective, and it remains unclear whether resection of the palate and uvula adds significantly to tonsillectomy with plication of the tonsil pillars. In addition, nasopharyngeal stenosis remains a significant risk when the procedure is performed at the same time as adenoidectomy.

3.2.4 MACROGLOSSIA AND THE PTOTIC TONGUE

Children with macroglossia generally have Beckwith–Wiedemann syndrome (macroglossia, omphalocele, visceromegaly, cytomegaly of the adrenal cortex), Down syndrome, or a vascular malformation of the tongue. Complications of macroglossia include aberrant dental eruption and malocclusion, maldevelopment of the maxilla and mandible, excessive drying of the tongue with ulceration, and airway obstruction. Unfortunately, surgical reduction of the tongue is generally effective only for the first three indications, and less so for airway obstruction. The procedure usually consists of a resection of the lingual margin or a wedge resection with or without aggressive resection at the foramen cecum, and therefore fails to address obstruction at the distal oropharynx and tongue base. As a result, airway obstruction persists in many children undergoing tongue reduction for macroglossia.

Other methods of managing macroglossia include suture suspension of the tongue, genioglossus advancement, and radiofrequency ablation. The tongue suspension suture technique (Repose®, InfluENT, Concord, NH) in children has been reported only in individual cases, and recently in a series of 16 patients refractory to treatment with adenotonsillectomy.²⁷ The procedure is performed as follows (Fig. 63.9).

1. The procedure is performed under general anesthesia with nasotracheal intubation. The patient is placed in tonsillectomy position, with the oral cavity exposed using a Molt mouth prop. A 2-0 silk retraction suture is placed at the tip of the tongue.

2. After injection of 1% lidocaine with 1:100,000 epinephrine, a small midline incision is made in the floor of the mouth medial and posterior to the submandibular ducts. Through this incision, a disposable battery-operated screw inserter is used to place a 3 mm mini-screw attached to a #1 polypropylene suture in the midline of the mandible below the level of the incisor roots (Fig. 63.9A). Firm placement of the screw is confirmed by gently pulling on the suture.

3. A preloaded proprietary suture passer with a suture loop is passed through the floor of mouth incision posteriorly toward the base of tongue. The suture is passed out the tongue base approximately 1.0–1.5 cm lateral to the midline (to the right of the midline if the surgeon is right handed), caudal to the foramen cecum (Fig. 63.9B). Care is taken not to injure the neurovascular bundle of the tongue laterally. The suture loop is then grasped as the suture passer is removed.

4. The suture passer is loaded with a strand of the polypropylene suture attached to the screw and passed through the floor of mouth and out the tongue base symmetrically on the opposite (left) side.

5. The polypropylene suture exiting at the tongue base is loaded on a large curved Mayo needle. The suture is inserted and tunneled submuscularly from its origin (left) to the exit point of the contralateral (right) suture loop. After removing the needle the single polypropylene strand is passed through the loop.

6. The loop is pulled anteriorly through the floor of mouth and into the incision, dragging along the polypropylene suture from the screw (Fig. 63.9C). The suture is then knotted to a second anchor suture attached to the screw, pulling the tongue base anteriorly. Care must be taken not to break the suture or cause necrosis of the tissue at the tongue base. Executed correctly, tightening the suture creates an indentation in the tongue base. The knot is then buried submucosally in the floor of mouth, and the incision site is closed with chromic suture.

Fig. 63.9 Tongue base suspension using the Repose® system (InfluENT, Concord, NH). A, A disposable battery-operated screw inserter is used to place a 3 mm mini-screw attached to a #1 polypropylene suture in the midline of the lingual mandibular surface below the roots of the incisor teeth. B, After passing a suture loop through the floor of mouth and out the tongue base on one side, the polypropylene suture attached to the screw is passed through the floor of mouth and out the tongue base symmetrically on the opposite side. The polypropylene suture is loaded on a large curved Mayo needle and passed submuscularly into the contralateral suture loop.

The loop is then pulled anteriorly through the floor of mouth and into the incision, dragging along the polypropylene suture and pulling the tongue base anteriorly. The suture is then tied to a second anchor suture pre-attached to the screw.

Overall success of tongue suspension was reported as 63%, but only 50% among children with Down syndrome.²⁷

Genioglossus advancement through advancement of the genial tubercle is rarely performed in young children due to the risk of injury to the developing mandibular incisors. However, the procedure may be considered for older children whose mandibles are well developed (Fig. 63.10).

1. A preoperative panoramic/tomographic radiograph of the mandible is performed to establish the anatomy of the mandibular incisors and the anterior mandible. The patient is anesthetized and intubated nasotracheally or orotracheally. The anterior vestibule (gingivobuccal sulcus) is infiltrated with 1% lidocaine with 1:100,000 epinephrine.

2. An incision is made in the sulcus, leaving a cuff of mucosa on the mandibular side to facilitate closure. The incision is carried subperiosteally until the inferior border of the mandible is exposed. The location of the genial tubercle is estimated based on the preoperative radiograph and confirmed by palpation of the lingual surface of the mandible.

3. Using a sagittal saw, a rectangular osteotomy is performed through the outer cortex such that the genial tubercle is included. In the fully developed mandible, the center of the osteotomy is approximately 5 mm below the incisor root tips. An outer cortex screw placed in the center of the osteotomy assists in manipulation of the bone segment.

4. Cuts are made through the inner cortex, freeing the osteotomy segment. Once hemostasis is obtained, the segment is advanced and rotated 90° (Fig. 63.10A), pulling the base of tongue anteriorly (Fig. 63.10B).

5. The outer cortex and bone marrow are removed from the segment and the bone is contoured with fluted and diamond burrs.

6. The segment is fixed in position using a screw placed through the inferior aspect of the osteotomy segment into the inferior mandibular border. The mucosa is reapproximated using absorbable suture.

Fig. 63.10 Genioglossus advancement. A, Through a mandibular sulcus incision, a rectangular osteotomy is performed 5 mm below the incisor root tips. Initial cuts are made through the outer cortex so as to include the genial tubercle. After placing an outer cortex screw in the center of the osteotomy, cuts are made through the inner cortex freeing the osteotomy segment which is then rotated 90°, pulling the base of tongue anteriorly (B).

Patients may generally be discharged within 24 hours after surgery. Complications of genioglossus advancement are unusual, but include dental injury, hematoma, and hypesthesia of the teeth and chin. The procedure has not been reported in any large series of children; however, adults usually undergo simultaneous procedures such as uvulopalatopharyngoplasty and hyoid advancement since genioglossus advancement alone will rarely correct severe sleep apnea. When performed with adjunctive procedures, studies demonstrate a widened retrolingual airway, improvement in oxygen saturation, and a 70% reduction in Respiratory Distress Index.²⁸

Radiofrequency ablation of the tongue has not been reported in children except for those with lymphatic malformations.²⁹ In adults with sleep apnea, only modest results have been obtained, and the improvement has been shown to deteriorate over time.³⁰ The device (Somnoplasty; Somnus Medical Technologies, Sunnyvale, CA) uses radio-frequency to create a submucosal coagulum that results in tissue contracture and reduction in tissue volume. The tongue handpiece consists of two prongs with retractable needles. After infiltration of local anesthesia, the handpiece is placed in contact with the mucosa and the needles are then advanced into the muscle. The needles are placed no further than 2 cm from the midline to avoid injury to the neurovascular pedicles. Complications include postoperative edema, abscess formation and tissue necrosis.

Submucosal minimally invasive lingual excision (SMILE) has recently been described as a novel method of reducing tissue of both the anterior tongue and the tongue base.³¹ The procedure uses ultrasound and endoscopically guided Coblation® reduction of lingual tissue through a small incision in the anterior tongue (Fig. 63.11).

1. General anesthesia is administered through either a nasotracheal tube or a previously placed tracheotomy. Preoperative administration of intravenous steroids and antibiotics is recommended. The oral cavity is gently brushed with antiseptic oral rinse and a retraction suture is placed through the anterior midline tongue tip.

2. A hand-held ultrasound probe is used to delineate the course of the lingual arteries bilaterally and the vessels are marked. A midline incision is made approximately 2 cm from the tongue tip with blunt dissection to open the incision.

3. The EVAC 70 T&A Plasma Wand attached to the Coblator II Surgery System (ArthroCare Corporation, Austin, TX) is inserted into the incision and programmed for ablation at a setting of 9. The wand is advanced in the posterior direction with care to stay medial to the marked courses of the lingual arteries. Tissue is excised in a dorsal to ventral fashion by alternating anterior and posterior movements of the wand. The non-operating hand palpates the tip of the wand through the tongue mucosa. Progress may be monitored by intermittently removing the wand and inserting a 0° irrigating endoscope into the surgical cavity.

4. The anterior tongue incision is left open to reduce the risk of hematoma.

Fig. 63.11 SMILE technique. After delineating the course of the lingual arteries with a hand-held ultrasound probe, a midline incision is made ending 2 cm from the tongue tip and opened bluntly. The Coblator® wand (ArthroCare Corporation, Austin, Texas) is inserted into the incision and advanced in the posterior direction. Tissue is excised in a dorsal to ventral fashion by alternating anterior and posterior movements of the wand.

After surgery, the patient is either extubated and monitored or intubated for less than 24 hours. Intravenous steroid and antibiotics are continued. Once extubated, the patient is started on a regular, soft diet. Children are observed for less than 72 hours and discharged home with 7 days of oral antibiotics, steroids, and analgesics. We suggest the procedure is better tolerated and more effective than other means of tongue reduction.

Vascular malformations of the tongue are generally of lymphatic or venous origin. Microcystic lymphatic malformation causing the tongue to be both wide and thick is extremely difficult to treat. Surgical resection may be performed transorally or transcervically and is tailored to sites of obstruction and dentofacial deformity. Regardless of approach or technique, recurrence is exceedingly common. Limited success has been reported using radiofrequency and Coblation® technology. Many such patients retain a tracheotomy indefinitely. Conversely, lymphatic malformations that are macrocystic may be virtually eliminated by sclerotherapy with OK-432, alcohol, bleomycin, or tetracycline.³² All of these sclerosants are known to cause significant inflammation; hence all patients with involvement of the neck, pharynx, or larynx who are not already tracheotomized should be monitored for postoperative airway obstruction. Venous malformations of the tongue may be reduced considerably using a combination of superficial and intralesional Nd:YAG laser therapy, alcohol sclerosis, and/or excision.³³

Ductal cysts of the vallecula may present with sleep disordered breathing in neonates. Occasionally, the presentation may also be consistent with laryngomalacia. These lesions are thought to result from mucous gland obstruction, but the etiology has not been definitively elucidated. The diagnosis may be difficult to make endoscopically due to the position of the mass, and lateral radiograph of the upper airway may be useful when the diagnosis is suspected. The lesion is managed surgically as follows (Fig. 63.12).

1. The patient is nasotracheally intubated and the head is positioned for suspension laryngoscopy. A Parsons or other slotted laryngoscope facilitates instrumentation, but any operating laryngoscope may be utilized. The tongue is suspended and the cyst visualized.

2. The dome of the cyst is grasped with small cup forceps and a laryngeal scissors is used to open the cyst cavity.

3. Once the cyst has been decompressed, the cyst wall is removed using the microdebrider (Fig. 63.12). The blade is inserted into the cyst cavity, and initial excision of the cyst wall is performed on the side opposite the forceps. The remaining cyst wall is then regrasped so as to permit completion of the excision.

4. CO₂ laser applied to the base helps to control hemorrhage and theoretically reduces the risk of recurrence.

Fig. 63.12 Excision of vallecular cyst. After nasotracheal intubation and suspension laryngoscopy, the cyst is aspirated and/or opened with microscissors. The cyst wall may then be grasped with microcup forceps and excised using the microdebrider. We prefer to treat the cyst base with CO₂ laser to lessen the risk of recurrence.

In most cases, results are excellent immediately after surgery. Recurrence and complications are rare and extended follow-up is not necessary.

Hyperplastic lingual tonsil tissue can also occupy the tongue base and vallecula, causing obstruction and retroflexion of the epiglottis. Excision of this tissue may occasionally be necessary in patients with diffuse lymphoid hyperplasia, such as those with Down syndrome or post-transplantation lymphoproliferative disorder, if symptoms are not relieved by adenotonsillectomy alone. Candidacy for the procedure is established by direct and fiberoptic assessment of lingual tonsil size and by lateral neck radiography. The procedure is performed as follows.

1. After induction of general anesthesia, the patient is nasotracheally intubated. In the most severe cases of lingual tonsil hyperplasia, fiberoptic intubation may be necessary. Preoperative steroid medication is administered.

2. With the table rotated 90° and the head in the Rose position, a 2-0 silk retraction suture is placed in the anterior tongue. The tongue base is infiltrated with 1% lidocaine with 1:100,000 epinephrine.

3. An operating laryngoscope is introduced transorally and suspended with the tip just above the vallecula (Fig. 63.13). We prefer the Parsons laryngoscope for this so that instruments can easily be introduced through the slide slot; a Lindholm scope can also be used if the procedure is to be performed with a CO₂ laser. The operating microscope is fitted with a 400 mm lens and focused on the surgical field. Alternatively, particularly when hand-held instruments are used, a Jennings mouth gag may be used to keep the mouth open while the field is exposed with a 70° telescope.

4. Lingual tonsil tissue may be excised using a variety of techniques. Coblation performed using the EVAC 70 T&A Plasma Wand attached to the Coblator II Surgery System (ArthroCare Corporation, Austin TX) causes minimal bleeding and results in efficient removal of tissue. The microdebrider fitted with a 3.5 mm tricut straight sinus blade can be used in the oscillating mode at 2000 revolutions per minute with even greater efficiency but with increased bleeding (Fig. 63.13). CO₂ laser may also be used for the dissection, offering the advantage of less bleeding and the disadvantage of less efficiency. Settings depend on the laser used; if a flash-scanner is available, excellent results are obtained using the laser in this mode at 6–10 watts with a spot size of 3 or 4 mm. When a scanner is not used, the beam should be defocused and the power increased. Suction cautery is also an alternative modality.

Fig. 63.13 Lingual tonsillectomy. After nasotracheal intubation, an operating laryngoscope is suspended with the tip just cranial to the vallecula. Lingual tonsil tissue may then be excised using the microdebrider with a straight sinus blade as shown; alternatively, the CO₂ laser or Coblator® may also be used.

In all cases, the dissection is kept above the muscle layer, intermittently controlling bleeding with oxymetazoline packs and suction electrocautery. The telescope or laryngoscope is periodically relocated to keep the level of dissection even.

Patients are monitored overnight on the floor or in the intensive care unit depending on their preoperative degree of obstruction. Two to three doses of steroid are given postoperatively and pain is managed appropriately. A regular diet may be taken. Complications include taste disturbance, scarring or injury to the neurovascular bundle if the dissection is excessive, and bleeding. Patients should be monitored for at least a year for evidence of tissue regrowth. There are no large-scale studies of the efficacy of the procedure in children or adults.

3.2.5 HYPOPLASIA OF THE MIDFACE AND MANDIBLE

Upper airway obstruction due to hypoplasia of the midface and mandible is usually associated with craniofacial syndromes. Micrognathia due to Pierre Robin sequence often improves within the first 2 years of life without surgical intervention for the mandible. In cases of mild airway obstruction, children with competent caretakers may be managed by prone positioning and nasopharyngeal stenting via nasal trumpet or similar device. When symptoms are more severe, airway obstruction may be relieved by temporary repositioning of the ptotic tongue through labioglossopexy. The surgical technique is as follows (Fig. 63.14).

1. If the patient is not already intubated, preparation is made for intubation by flexible fiberoptic bronchoscopy or Bullard laryngoscopy in the event the larynx cannot be visualized using a standard anesthesia laryngoscope. After being taken to the operating room, the patient is anesthetized via mask and subsequently intubated by one of these techniques with an age-appropriate tube. Nasal intubation can be performed; however, our experience has been that an oral endotracheal tube does not interfere with the surgery. A weight-appropriate dose of cefazolin or clindamycin is given. A small amount of 1% lidocaine with 1:100,000 epinephrine is then injected for hemostasis into the soft tissues of the ventral tongue, the anterior floor of mouth, and the lingual surface of the anterior mandibular alveolus. The patient’s lower face and chin are sterilely prepared and draped.

2. A Molt mouth prop is opened laterally between the upper and lower alveolus to expose the anterior oral cavity. A 2-0 silk suture placed through the dorsal tongue facilitates manipulation of the tongue during the procedure. The lingual frenulum may need to be released by transection or Z-plasty. A long T-shaped incision is made on the anterior ventral tongue and carried vertically from the tongue tip to the frenulum. An opposing incision is made in similar fashion along the lower lip to the anterior mandibular alveolus. The wound edges on both incisions are undermined to create mucosal flaps and the underlying tongue and orbicularis oris muscles are exposed.

3. The vertical lip-to-alveolus incision is carried down to the bone and a small periosteal elevator is used to tunnel along the anterior and posterior aspects of the mandible. The genioglossus muscle is gently detached from the genial tubercle using blunt dissection.

4. An 18 or 20-gauge spinal needle is passed through the soft tissue along the posterior surface of the mandible and through the skin of the mentum; a small skin incision is made at the exit site. Dissection of the subcutaneous tissue deep to the incision allows the suture to pass close to the mandible and reduces slippage. A non-absorbable suture without needle (i.e. 0 or 2-0 Prolene) is placed through the spinal needle and retrieved at the mentum. The spinal needle is then withdrawn and passed from the mouth through the skin incision a second time, this time along the anterior surface of the mandible. The suture tail is threaded through the needle and the needle is withdrawn.

5. The posterior end of the non-absorbable suture is placed through a free or traumatic needle. The needle is buried deep into the tongue musculature adjacent to the foramen cecum and brought back into the oral cavity at the caudal end of the tongue incision. The suture is tied while drawing the 2-0 silk suture anteriorly to take tension off the tongue. The knot is positioned submucosally.

6. The ventral tongue mucosal flaps are then matched to the flaps of the lower lip and approximated using a 4-0 chromic gut suture. Prophylactic placement of a nasopharyngeal airway reduces the risk of postoperative obstruction after extubation and facilitates suctioning of the airway.

Fig. 63.14 Labioglossopexy for glossoptosis. A, After T-shaped incisions are made on the anterior ventral tongue and lower lip exposing the underlying tongue and orbicularis oris muscles, a periosteal elevator is used to tunnel along the anterior aspect of the mandible and to detach the genioglossus muscle. A spinal needle passed through the soft tissue along the anterior and posterior surfaces of the mandible through the skin of the mentum facilitates placement of a 0 or 2-0 Prolene circumandibular stay suture passed through muscle. The two ends of the suture are then placed into the tongue muscles in a location that will allow approximation of the muscle directly to the alveolar ridge incision (B). The ventral tongue mucosal flaps are then matched to the flaps of the lower lip and approximated using a 4-0 chromic gut suture.

Postoperative management includes nasogastric feeding to prevent aggressive sucking. The nasopharyngeal airway can be discontinued several days postoperatively and oral feedings are usually attempted in about 1 week. Oral feeding is usually unaffected by the procedure. The child can be discharged home once the airway is deemed adequate and the patient is able to obtain adequate nutrition by mouth. Dehiscence of the adhesion is the most common complication of labioglossopexy, but fewer than 5% require a return to the operating room for revision. Other reported complications include tongue lacerations, Wharton duct injury, wound infection, and scarring. Some authors feel that syndromic patients tend to be more difficult to manage due to numerous anomalies or conditions contributing to airway obstruction and impaired feeding. Kirschner et al.³⁴ had an 83% rate of conversion to tracheostomy in syndromic children.

The child should be closely monitored as an outpatient to ensure appropriate growth and development. The patient should be evaluated at about 1 month postop-eratively and then at 2–3 month intervals. Tongue mobility should be closely monitored, although the effect on speech development is minimal. Takedown of labioglossopexy is usually performed at 6–9 months of age, often coinciding with repair of the palate. Adequacy of the airway should be confirmed endoscopically prior to takedown.

When symptoms are too severe for labioglossopexy, multiple sites of obstruction are present, or the patient’s medical situation is exceedingly grave, tracheotomy remains the most reliable and least morbid means of airway management. However, patients should show signs of mandibular ‘catch-up’ growth within the first few months; when this is not the case, distraction osteogenesis should be considered.

First described for the treatment of limb length discrepancies, osteotomy with distraction of bone is now widely accepted as the procedure of choice in the early management of airway obstruction due to craniofacial disproportion.35–38 The procedure takes advantage of the rapid healing and capacity for growth in the pediatric skeleton. Distraction osteogenesis has been used for over a decade to advance the mandible in cases of retrognathia and micrognathia, but indications have expanded to include neuromuscular disorders. With modifications to the expansion devices, distraction of the midface is also beginning to replace Le Fort osteotomies with bone grafting.^37,³⁸

Distraction osteogenesis is divided into four phases: surgery, distraction, consolidation, and removal. Prior to surgery, all candidates for mandibular distraction undergo airway endoscopy and craniofacial assessment by three-dimensional CT scanning. The scans are used to determine whether the distraction vector will be horizontal, vertical, or both, and where the osteotomies are made. Airway patency may also be estimated in relaxed and jaw-thrust positions, and precise bony measurements are taken from the scan. The surgeon usually determines preoperatively whether intraoral or external devices are to be used. While external devices permit multivector control of the distraction and are more easily applied to small mandibles, intraoral distractors may leave a more acceptable facial scar. In addition, intraoral devices have some mechanical advantage since their point of application is closer to the point of distraction resistance.

The operative technique is as follows.

1. Intubation for the procedure may be nasotracheal or orotracheal. Candidates for the procedure are often difficult to intubate using traditional direct laryngoscopy, and plans should be made preoperatively for possible intubation using the flexible or Bullard laryngoscopes.

2. After infiltration of 1% lidocaine with 1:100,000 epinephrine into the mucosa overlying the ascending ramus of the mandible, an initial internal incision is made along the oblique line. Alternatively, an external submandibular incision may be made in infants in whom intraoral exposure is limited. Subperiosteal dissection is performed both medially and laterally, exposing approximately 2 cm of bone on either side of the mandibular angle. The buccal surface of the mandible is marked for osteotomy anterior or posterior to the angle as determined by the preoperative CT scan. The lingula is identified and the lingual cortex osteotomy is marked so as to avoid injury to the inferior alveolar nerve (Fig. 63.15A).

3. For internal devices, the device plates are applied to either side of the planned osteotomy using at least four screws in each plate (Fig. 63.15B). A stab incision is made at the exit point of the activation arm through the skin. A reciprocating saw or a fissure burr is used to perform the osteotomy, with care to remain well away from the inferior alveolar nerve bundle. Lateral, superior, and inferior aspects of the mandible are cut, leaving the mid portion of the lingual cortex intact. An osteotome placed into the cuts and gently twisted completes the medial osteotomy.

4. For external devices, one or two pins are placed on either side of the osteotomy, depending on the distractor used (Fig. 63.15A). A 25-gauge needle is used to mark the insertion sites on the skin, which is injected with local anesthetic. A 5–10 mm incision is made for the pin(s) through which blunt dissection is performed parallel to the marginal nerve until the exposed mandible is encountered. A drill guide is placed through the incision until it is in contact with the bone 5–8 mm from the osteotomy. Holes are hand-drilled so that the hole size is carefully controlled. After both cortices have been drilled, the bit is removed and pins are screwed in without moving the guide. The desired depth is obtained by palpating the pin lingually. The external distractor is adjusted to fit onto the pins. A small gap is left between the device and the skin to facilitate pin care and to accommodate swelling. The position of the device is marked on the pins to facilitate replacement. The distractor is again removed. The osteotomy is completed using osteotomes on the inferior and superior borders and on the lingual aspect to avoid injury to the nerve. The distractor is replaced and tightened. At this point, activation is attempted to ensure movement of the proximal and distal segments. The device is then returned to its starting position. The incision is closed except for the pin sites and the pins are cut flush with the device.

5. The procedure is repeated on the opposite side. We prefer to treat the two sides sequentially so as to avoid destabilizing the anterior mandible and subsequent loss of contour.

Fig. 63.15 A, Technique of mandibular distraction. Planned bone cuts and pin placement for mandibular distraction. Bone cuts are designed as in a bilateral sagittal split osteotomy. For external distractor, two pins are placed on either side of the osteotomy. The medial osteotomy is prepared by scoring the bone, and the bone is released with a gentle twist of the osteotome. (From Darrow DH, Weiss DD. Management of sleep-related breathing disorders in children. Oper Tech Otolaryngol Head Neck Surg 2002;13:111–18.) B, Application of an intraoral mandibular distractor. (From Allen GC. Mandibular distraction osteogenesis for neonatal airway obstruction. Oper Tech Otolaryngol Head Neck Surg 2005;16:187–93.)

After a lag phase of 48–72 hours, distraction is started at a rate of 1–2 mm per day, with adjustments every 12 hours. Once the desired length of the mandible has been achieved, adequacy of the airway is verified by flexible or rigid laryngoscopy prior to consolidation. In children who already have a tracheostomy, downsizing and bedside occlusion can be performed. The consolidation phase is usually about 4–10 weeks during which the hardware may be left in place. The final stage is removal of the hardware and minor scar revision.

Avoidance of or decannulation from tracheotomy in appropriately selected patients undergoing distraction of the mandible is >80%.35–38 Complications include pin site infections, injury to the inferior alveolar and marginal nerves, damage to tooth buds, resorption and ankylosis of the temporomandibular joint, and failure to maintain distraction. Complication rates are difficult to quantify since most series are small and follow-up is limited.

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