Management of Micrognathia Brinda Thimmappa, Elena Hopkins and Stephen A. Schendel NeoReviews 2009;10;e488-e493 DOI: 10.1542/neo.

10-10-e488

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NeoReviews is the official journal of the American Academy of Pediatrics. A monthly publication, it has been published continuously since 2000. NeoReviews is owned, published, and trademarked by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007. Copyright 2009 by the American Academy of Pediatrics. All rights reserved. Online ISSN: 1526-9906.

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Article

respiratory disorders

Management of Micrognathia
Brinda Thimmappa, MD,* Elena Hopkins, RN, MS, PNP, Stephen A. Schendel, MD, DDS

Objectives

After completing this article, readers should be able to:

Author Disclosure Drs Thimmappa and Schendel and Ms Hopkins have disclosed no nancial relationships relevant to this article. This commentary does not contain a discussion of an unapproved/ investigative use of a commercial product/ device.

1. Describe the anatomy and causes of respiratory distress in neonates who have micrognathia. 2. Review the diagnostic evaluation for patients who have micrognathia. 3. Review surgical and nonsurgical management options. 4. Explain the treatment algorithm for micrognathia.

Abstract
Micrognathia and the associated retroposition of the tongue into the oropharynx (glossoptosis) can obstruct the upper airway, producing obstructive apnea. The primary management of respiratory compromise in patients who have micrognathia is controversial. Numerous modalities have been used, including prone positioning, nasopharyngeal airways, tongue-lip adhesion, mandibular distraction, and tracheostomy. The goal of any intervention is to relieve airway obstruction, with secondary goals reported in the literature including avoidance or early removal of tracheostomy, improvement in feeding, and accelerated growth. This review examines nonsurgical and surgical therapeutic options and their outcomes. The diagnostic and treatment algorithm employed at the senior authors institution is presented.

Introduction
Respiratory distress in the neonate may be due to impaired oxygenation or ventilation. The latter may be mediated by neurologic dysfunction (central apnea) or may result from anatomic obstruction (obstructive apnea). Patients who have micrognathia develop obstructive apnea due to glossoptosis of the tongue base, which is associated with signicant morbidity and mortality in infants and children. Affected newborns may exhibit frequent episodes of oxygen desaturation, inspiratory stridor, costal retractions, and feeding difculties. In addition, the normal rapid eye movement (REM) sleep patterns are disturbed. Infants and children who have long-term airway obstruction exhibit failure to thrive, daytime somnolence, hemodynamic changes (including cor pulmonale and pulmonary hypertension), developmental disabilities, insufcient weight gain/malnutrition, increased pulmonary morbidity, and death.

Anatomy
Patients who have congenital retrognathia, glossoptosis, and cleft palate receive the diagnosis of Pierre Robin sequence. This group represents most of the children who are born with micrognathia and respiratory distress. Additional ndings of ocular abnormalities and one of several dened mutations in collagen production Abbreviations make the diagnosis of Stickler syndrome. Children who have mandibulofacial dysostosis disorders such as Treacher Collins syndrome, Nager syndrome, and AHI: apnea hypopnea index bilateral hemifacial microsomia also may have obstructive sleep apnea (OSA). CT: computed tomography Common to these conditions is hypoplasia of the mandible. Normally, the OSA: obstructive sleep apnea tongue is held forward by the attachments of the genioglossus to the lingual REM: rapid eye movement surface of the mandible symphysis. Retrognathia, with or without micrognathia,
*Division of Plastic and Maxillofacial Surgery, Southwest Medical Group, Vancouver, Wash. Craniofacial Coordinator, Lucille Packard Childrens Hospital, Palo Alto, Calif. Professor Emeritus, Division of Plastic Surgery, Stanford University, Palo Alto, Calif. e488 NeoReviews Vol.10 No.10 October 2009

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positions the base of the tongue posteriorly, obstructing the airway. On physical examination, mandibularmaxillary discrepancy can be measured from the apex of the mandibular alveolar ridge and the apex of the maxillary alveolar ridge in the midline. This measure is useful to classify the severity of the deformity and is almost always greater than 1 cm. Airway obstruction is especially pronounced in the supine position as the force of gravity causes the tongue base to abut the posterior pharyngeal wall. Nasoendoscopy study of sleeping patients also demonstrates the tongue becoming caught in the cleft of the palate, the lateral pharyngeal walls moving medially, and the pharynx contracting in a circular pattern. The newborn who has micrognathia and obstruction often presents with labored, noisy breathing and rapid development of fatigue, costal retractions, and difculty feeding.

Diagnostic Studies
Newborns who have evidence of retrognathia and documented desaturations while supine or during feedings require further study. Simple oxygen saturation monitoring is not sufcient to recognize the severity of the obstruction. The evaluation for obstructive apnea includes polysomnography, pH probe or modied barium swallow study, laryngoscopy, and maxillofacial computed tomography (CT) scan. Polysomnography records central apneas, obstructive apneas, and hypopneas by measuring oxygen saturation, electrocardiography, end-tidal PCO2, nasal and oral airow through thermistors and chest and abdominal wall excursions, respiratory rate, and limited electroencephalography leads to determine sleep state. Central apneas are characterized by pause in respiratory effort and absence of airow. Obstructive apneas are associated with continued respiratory effort while air ow ceases. Hypopnea refers to a decrease in airow by 30% to 50% from baseline accompanied by oxygen desaturation. It is important to exclude central apnea as a cause of respiratory distress because this condition does not benet from mandibular advancement. If intubation precludes obtaining complete polysomnography, the patient may be observed on pressure-support ventilation or continuous positive airway pressure to demonstrate gross absence of central apneas. Normal values of the calculated apneahypopnea index (AHI) are different in children compared with those of adults. Studies have suggested that an AHI greater than 1 is abnormal, with an AHI of 1 to 4 classied as mild OSA, an AHI of 5 to 10 indicating moderate OSA, and an AHI greater than 10 documenting severe OSA (Wagner and Torrez, 2007). Patients are placed in the supine position to obtain a

baseline study. Studies obtained with the infant in the prone or lateral position represent the outcome of a specic intervention and do not record the true extents of apneic periods or desaturation. Polysomnography also provides data on sleep architecture. Obstructive events may worsen during REM sleep, as muscle tone decreases. The result is frequent awakenings and less time spent in REM sleep. Feeding difculties often accompany respiratory distress in this population, even in the absence of associated cleft palate. A pH probe study may be performed at the time of polysomnography. A modied barium swallow should be considered if there is concern that the child is at risk for direct aspiration with oral feedings. Direct laryngoscopy, bronchoscopy, or nasoendoscopy conrms the absence of underlying laryngomalacia, tracheomalacia, subglottic stenosis, or vocal cord paralysis prior to surgical intervention. The presence of glossoptosis as the source of obstruction also may be conrmed. Maxillofacial CT scan provides anatomic data on maxillary-mandibular discrepancy and allows calculation of the area of the airway. The true maxillary-mandibular discrepancy may be masked by the presence of oral airways or endotracheal tubes that leave the patient in an open mouth position. In the preoperative planning of mandibular distraction, CT scan demonstrates the amount and quality of bone available for placement of devices. Development of the temporal mandibular joint and location of dental follicles also are obtained from CT data.

Nonsurgical Management
Initial management in the delivery room is prone or lateral positioning. Placement of oral or nasopharyngeal airways may stabilize the airway to preclude acute intubation. This method provides space for the passage of air within the tube and prevents the tongue from falling backwards, reducing the buildup of negative pressure in the back of the hypopharynx. Long-term management with these techniques requires vigilance and education of caregivers. Movement of the patient, coughing, or swallowing may dislodge a correctly placed tube and result in recurrent obstruction or stimulate a gag reex. Although positioning alone may prevent desaturation while resting, obstruction and concomitant apnea may result when the patient is repositioned for feeding. Many affected infants require nasogastric tubes or gastrostomy for adequate enteral nutrition. Case series of patients who have micrognathia and respiratory distress have
NeoReviews Vol.10 No.10 October 2009 e489

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reported successful nonoperative management in 33% to 69% (Meyer et al, 2008).

Surgical Management
Surgical management is indicated when respiratory or feeding difculties persist despite positioning maneuvers. Previous studies (Schaefer et al, 2004) have proposed a mandibular-maxillary discrepancy greater than 8 to 10 mm as an indication for surgical management, although all aspects of examination and diagnostic studies should be included in the decision to proceed with surgical procedures. Tracheostomy is the denitive treatment of subglottic obstruction. However, neonatal tracheostomy is associated with signicant morbidity and mortality, including delayed speech, mucus plugging, dislodgement of the tracheostomy tube, or tracheomalacia. Neonates who have tracheostomies require 24-hour monitoring, placing a large burden on caregivers. The incidence of tracheostomy-related complications is reported as 19% to 49% and related mortality as 2% to 8.5%. Tongue-lip adhesion was described initially by Douglas in 1946. A transverse incision is made in the ventral surface of the tongue and lingual surface of the lower lip. Care must be taken to avoid the openings of the sublingual glands. Following suture of these raw surfaces, an additional retention suture is placed from the base of tongue just above the epiglottitis to the anterior surface of the chin, usually through a button to prevent erosion of the tongue and skin surfaces. The retention suture and button may be removed after 7 days. The tongue may be released after sufcient growth of the mandible occurs, usually after 6 to 7 months of age. Success rates for tongue-lip adhesion in relieving airway obstruction has been reported over the range of 33% to 83%. The most commonly reported complication is dehiscence of the adhesion. Postoperative polysomnography has demonstrated relief of obstructive apnea. However, examination of the long-term follow-up of treated patients shows the need for secondary procedures in most for recurrent obstruction or feeding difculties. The underlying jaw deformity also remains uncorrected. Proponents of this measure cite the limited scarring and lack of damage to tooth buds it offers. The effect on feeding after adhesion is poorly studied. Available data largely reect the protocols of specic centers to place gastrostomy tubes for individuals requiring prolonged (30 days) feeding support. Distraction osteogenesis has become increasingly prevalent as more surgeons develop the skills necessary to perform the technique and evidence accumulates regarde490 NeoReviews Vol.10 No.10 October 2009

Figure 1. Curvilinear mandibular distractor and placement.

ing risks and benets. Many distraction devices exist today. Infant mandibular distraction initially was performed with an external device. Advantages of an external device include the ability to adjust the vector during the course of distraction, reduced dissection that may interrupt blood supply to the regenerate, and removal of the device without a return to the operating room. Alternatively, internal devices are less cumbersome to the patient and caregivers, are less prone to external trauma or pin site irritation, and minimize torque, with less chance of device loosening. Resorbable internal devices also offer the benet of a single-stage distraction. Preoperative planning requires knowledge of the desired vector of distraction based on the anatomy, location of osteotomies, and postoperative distraction regimen. It is the preferred technique of the senior author to use an

Figure 2. Two-week-old infant who has Pierre Robin sequence and is undergoing distraction with activator pins in place.

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Figure 5. Preoperative sagittal (A) view demonstrating glos-

soptosis and airway obstruction. View following completion of distraction and consolidation period (B).
Figure 3. Infant who has Treacher-Collins syndrome and underwent mandibular distraction. Preoperative appearance (A). The mandible was advanced 16 mm (B) prior to activator pin removal.

internal curvilinear device (Fig. 1.) Its design produces a vector that models the logarithmic growth pattern of normal human mandibular growth. Coupled with an oblique osteotomy at the angle of the mandible, it avoids an anterior open bite, which may be created after distraction in a single vector. Patients are transferred to the intensive care unit postoperatively and generally remain intubated. Distraction is started the next day at a rate 1 mm twice daily and continued until overcorrection is achieved. Activation rods are removed at bedside and the pin exit wounds are allowed to heal by secondary intention (Fig. 2). Within 5 postoperative days, correlating with 10 mm of mandibular movement, most children are ready for extubation

(Fig. 3). Oral feeding is introduced under the direction of the occupational therapist. Patients are discharged once a stable airway is maintained and a feeding regimen determined. Patients are scheduled for device removal after a minimum consolidation period of 3 months, although this may be delayed for those who have associated cleft palates until the time of palatoplasty (Figs. 4 and 5). Following distraction, a mean improvement of AHI from 25.24 to 1.72 has been demonstrated. Up to 8% of patients require further intervention for airway management, primarily those who have elements of central apnea. Earlier decannulation in tracheostomy-dependent patients also has been observed. Temporary facial nerve palsy, manifesting as an asymmetric cry, if present, resolves in fewer than 3 months in our centers experience. Following distraction, Monasterio and associates (2004) achieved complete resolution of gastroesophageal reux and feeding tube use after 2 to 4 months. This level of success has not been replicated in other studies, although data suggest improved feeding and fewer patients requiring prolonged (1 month) feeding tube assistance.

Treatment Algorithm
The experience of the authors center using an internal curvilinear device began in 2005. The management algorithm is outlined in Figure 6. As discussed previously, any infant referred for evaluation of respiratory distress in the setting of micrognathia undergoes evaluation that includes CT scan, sleep study, pH probe or modied barium swalNeoReviews Vol.10 No.10 October 2009 e491

Figure 4. A. Six-month-old infant who has isolated Pierre Robin sequence. B. Lateral

corticotomy. C. Distractor placement. D. Postoperative lateral photograph. E. Preoperative CT scan. F. Postoperative CT scan with curvilinear regenerate of ramus and body of mandible.

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Figure 6. Management of infants who have micrognathia. GERDgastroesophageal reux disease, MRImagnetic resonance

imaging

low, and bronchoscopy. Infants who have mild abnormality on sleep study (AHI 4) and no evidence of desaturation or apnea with appropriate positioning are managed nonoperatively. The remainder are prepared for distraction, as long as central apnea or infraglottic obstruction is absent. All patients undergo feeding therapy as soon as the airway is stabilized. Repeat sleep and swallow studies are completed after intervention is completed.

American Board of Pediatrics Neonatal-Perinatal Medicine Content Specications

Recognize the clinical features and know how to diagnose and manage craniofacial anomalies. Know the associations and clinical features and management of macroglossia and hypoplastic mandible, including the Pierre-Robin syndrome.

Conclusion
Coordinated care by a multidisciplinary team is critical in the management of respiratory distress for patients who have micrognathia. Surgical intervention is reserved for those who fail nonsurgical management and have completed a full diagnostic evaluation. The use of distraction osteogenesis of the mandible is increasing as more outcome data become available and the number of surgeons trained in the procedures increases. At present, distraction is associated with a decreased need for reintervention.
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Suggested Reading
Denny AD, Talisman R, Hanson PR, et al. Mandibular distraction osteogenesis in very young patients to correct airway obstruction. Plast Reconstr Surg. 2001;108:302 Evans AK, Rahbar R, Rogers GF, Mulliken JB, Volk MS. Robin sequence: a retrospective review of 115 patients. Intl J Pediatr Otorhinolagol. 2006;70:973 Isadi K, Yellon R, Mandell DL, et al. Correction of upper airway obstruction in the newborn with internal mandibular distraction osteogenesis. J Craniofac Surg. 2003;14:493 Kirschner RE, Low DW, Randall P, et al. Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion? Cleft Palate Craniofac J. 2003;40:13

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Meyer AC, Lidsky ME, Sampson DE, Lander TA, Liu ML, Sidman JD. Airway interventions in children with Pierre Robin sequence. Otolaryngol Head Neck Surg. 2008;138:782 Miller JJ, Kahn DK, Lorenz HP, Schendel SA. Infant mandibular distraction with an internal curvilinear device. J Craniofac Surg. 2007;18:1403 Monasterio F, Molina F, Berlanga F, et al. Swallowing disorders in Pierre

Robin sequence: its correction by distraction. J Craniofac Surg. 2004; 15:934 Schaefer RB, Stadler JA, Gosain AK. To distract or not to distract: an algorithm for airway management in isolated Pierre Robin sequence. Plast Recon Surg. 2004;113:1113 Wagner MH, Torrez DM. Interpretation of the polysomnogram in children. Otolaryngol Clin North Am. 2007;40:745

NeoReviews Quiz
1. Hypoplasia of the mandible, a clinical feature of several syndromes, is one of the causes of obstructive apnea and respiratory distress in the newborn. Of the following, the clinical nding of an ocular abnormality, in addition to mandibular hypoplasia, is most consistent with the diagnosis of: A. B. C. D. E. Hemifacial microsomia disorder. Nager syndrome. Pierre Robin sequence. Stickler syndrome. Treacher Collins syndrome.

2. A term newborn has retrognathia and episodes of oxygen desaturation from airway obstruction. Diagnostic studies are planned to determine if the surgical procedure of mandibular distraction is suitable for this infant. Of the following, the most informative diagnostic study in the preoperative planning of mandibular distraction is: A. B. C. D. E. Direct laryngoscopy. Oxygen saturation monitoring. Maxillofacial computed tomography scan. pH probe study. Polysomnography.

3. A term newborn who has retrognathia and cleft palate has episodes of oxygen desaturation from airway obstruction while supine and during feedings. Diagnostic studies reveal subglottic stenosis. Of the following, the most denitive treatment for this infant is: A. B. C. D. E. Gastrostomy. Mandibular distraction. Palatoplasty. Tongue-lip adhesion. Tracheostomy.

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NeoReviews Vol.10 No.10 October 2009 e493

Management of Micrognathia Brinda Thimmappa, Elena Hopkins and Stephen A. Schendel NeoReviews 2009;10;e488-e493 DOI: 10.1542/neo.10-10-e488

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