Treatment of Upper Airway Obstruction and Feeding Problems in Robin-Like Phenotype

Abstract

Robin sequence (RS) was first delineated by Fairbairn in 1846 and Shukowsky in 19111Davies P.A. Management of the Pierre Robin Syndrome.Dev Med Child Neurol. 1973; 15: 359-362Crossref PubMed Scopus (4) Google Scholar, 2Shukowsky W.P. Zur Ätiologie des Stridor inspiratorius congenitus.Jahrb Kinderheilk. 1911; 73: 459-474Google Scholar; it was more formally investigated by the French stomatologist Pierre Robin in 1934, who later became the source of the eponym for this condition.3Robin P. Glossoptosis due to atresia and hypotrophy of the mandible.Am J Dis Child. 1934; 48: 541-547Google Scholar Robin described it as consisting of a hypoplastic or retropositioned mandible and glossoptosis, resulting in respiratory distress, with or without a cleft palate. It fulfills criteria for a sequence, in which one or all anomalies found are secondary to another anomaly.4Shprintzen R.J. The implications of the diagnosis of Robin sequence.Cleft Palate Craniofac J. 1992; 29: 205-209Crossref PubMed Scopus (182) Google Scholar The incidence varies between one in 8500 and one in 14 000 live births.5Bush P.G. Williams A.J. Incidence of the Robin Anomalad (Pierre Robin syndrome).Br J Plast Surg. 1983; 36: 434-437Abstract Full Text PDF PubMed Scopus (159) Google Scholar, 6Printzlau A. Andersen M. Pierre Robin sequence in Denmark: a retrospective population-based epidemiological study.Cleft Palate Craniofac J. 2004; 41: 47-52Crossref PubMed Scopus (158) Google ScholarThe main functional problems associated with RS are upper airway obstruction (UAO) and failure to thrive (the latter resulting from feeding problems, UAO alone, or both problems).6Printzlau A. Andersen M. Pierre Robin sequence in Denmark: a retrospective population-based epidemiological study.Cleft Palate Craniofac J. 2004; 41: 47-52Crossref PubMed Scopus (158) Google Scholar Feeding problems are often long-lasting, resulting in gastrostomy rates of 50% in large series.7Meyer A.C. Lidsky M.E. Sampson D.E. Lander T.A. Liu M. Sidman J.D. Airway interventions in children with Pierre Robin Sequence.Otolaryngol Head Neck Surg. 2008; 138: 782-787Crossref PubMed Scopus (108) Google Scholar Interventions for RS should thus be assessed by their ability to improve polysomnography (PSG) results and weight gain. Although reported as used in infants with RS by 74% of US pediatric otolaryngology fellowship programs in 1994, only 39% believed PSG to be indicated for most patients with this condition, and just one in 5 performed serial PSG to test for treatment effectiveness.8Myer 3rd, C.M. Reed J.M. Cotton R.T. Willging J.P. Shott S.R. Airway management in Pierre Robin sequence.Otolaryngol Head Neck Surg. 1998; 118: 630-635Crossref PubMed Google Scholar However, it is difficult to document the effect of treatments for RS on UAO without performing PSG, especially because anatomy does not correlate well with function.9de Sousa T.V. Marques I.L. Carneiro A.F. Bettiol H. Freitas J.A. Nasopharyngoscopy in Robin sequence: clinical and predictive value.Cleft Palate Craniofac J. 2003; 40: 618-623Crossref PubMed Scopus (29) Google ScholarFor this Medical Progress article, we searched PubMed for studies on the effectiveness of interventions suggested to improve UAO and weight gain in infants with RS (Table). It does not cover surgical techniques for which evidence in the literature is extremely scant or objective documentation of their effectiveness lacking.TableTreatment approaches used in RSApproach1. Widening the pharyngel space Prone positioning Tongue-lip adhesion Mandibular traction Mandibular distraction osteogenesis2. Bridging/stenting the obstructed airway Nasopharyngeal tube Nasal respiratory support Tracheostomy3. Correction of glossoptosis and functional induction of mandibular growth Palatal plate PEBP Open table in a new tab Procedures Aimed at Widening the Pharyngeal SpaceProne PositioningProne positioning is based on the hypothesis originally proposed by Robin3Robin P. Glossoptosis due to atresia and hypotrophy of the mandible.Am J Dis Child. 1934; 48: 541-547Google Scholar that the narrow pharyngeal space in RS can be corrected by the effect of gravity moving the mandible forward in infants sleeping prone. In some case series, 50% to 80% of patients with RS were reported as having been sufficiently treated with positioning only,10Caouette-Laberge L. Bayet B. Larocque Y. The Pierre Robin sequence: review of 125 cases and evolution of treatment modalities.Plast Reconstr Surg. 1994; 93: 934-942Crossref PubMed Scopus (239) Google Scholar, 11Kirschner R.E. Low D.W. Randall P. Bartlett S.P. McDonald-McGinn D.M. Schultz P.J. et al.Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?.Cleft Palate Craniofac J. 2003; 40: 13-18Crossref PubMed Scopus (141) Google Scholar but none of these studies documented its effectiveness objectively. It has been suggested that the main reason why the prone position appears effective is that the visual cues to UAO (paradoxical chest movements or a pectus excavatum) are less visible in this position.4Shprintzen R.J. The implications of the diagnosis of Robin sequence.Cleft Palate Craniofac J. 1992; 29: 205-209Crossref PubMed Scopus (182) Google Scholar Moreover, it is questionable whether prone positioning alone is sufficient to induce mandibular catch-up growth.12Daskalogiannakis J. Ross R.B. Tompson B.D. The mandibular catch-up growth controversy in Pierre Robin sequence.Am J Orthod Dentofacial Orthop. 2001; 120: 280-285Abstract Full Text Full Text PDF PubMed Scopus (71) Google Scholar Most concerning, however, is that the prone sleep position is associated with a more than 10-fold increase in the risk of sudden infant death syndrome, making it questionable whether parents can safely be advised to place their baby with RS prone for sleep.Tongue-Lip Adhesion or GlossopexyThis idea on how to widen the pharyngeal space was first proposed by Shukowsky2Shukowsky W.P. Zur Ätiologie des Stridor inspiratorius congenitus.Jahrb Kinderheilk. 1911; 73: 459-474Google Scholar and subsequently reported to result in better survival rates.13Douglas B. The treatment of micrognathia associated with obstruction by a plastic procedure.Plast Reconstr Surg. 1946; 1: 300-308Crossref Scopus (117) Google Scholar Success rates for this intervention, on the basis of clinical criteria, vary.11Kirschner R.E. Low D.W. Randall P. Bartlett S.P. McDonald-McGinn D.M. Schultz P.J. et al.Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?.Cleft Palate Craniofac J. 2003; 40: 13-18Crossref PubMed Scopus (141) Google Scholar Except for the aforementioned study in 1946,13Douglas B. The treatment of micrognathia associated with obstruction by a plastic procedure.Plast Reconstr Surg. 1946; 1: 300-308Crossref Scopus (117) Google Scholar we found one study of 48 infants with RS that reported a non-significant increase in weight gain in a mean duration of 1.4 years after tongue-lip adhesion (ie, mean weight increased from the 9.7th to the 17.5th percentile).14Cozzi F. Totonelli G. Frediani S. Zani A. Spagnol L. Cozzi D.A. The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome.J Pediatr Surg. 2008; 43: 296-298Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar Another study documented significant UAO in PSG in 6 infants with RS and reported that a follow-up study after tongue-lip adhesion, performed in 5 infants before hospital discharge, demonstrated resolution of significant airway obstruction15Gilhooly J.T. Smith J.D. Howell L.L. Deschaine B.L. Richey S.L. Bedside polysomnography as an adjunct in the management of infants with Robin sequence.Plast Reconstr Surg. 1993; 92: 23-27Crossref PubMed Scopus (45) Google Scholar; more detailed data were not provided. A recent case series reported improved PSG results in 5 of 8 patients studied after tongue-lip adhesion (of a total group of 22 patients) and an unspecified degree of catch-up growth in 10 patients.16Bijnen C.L. Don Griot P.J. Mulder W.J. Haumann T.J. Van Hagen A.J. Tongue-lip adhesion in the treatment of Pierre Robin sequence.J Craniofac Surg. 2009; 20: 315-320Crossref PubMed Scopus (29) Google ScholarIn another follow-up study, however, 10 of 11 patients with RS required additional interventions for airway or feeding problems subsequent to tongue-lip adhesion.17Denny A.D. Amm C.A. Schaefer R.B. Outcomes of tongue-lip adhesion for neonatal respiratory distress caused by Pierre Robin sequence.J Craniofac Surg. 2004; 15: 819-823Crossref PubMed Scopus (88) Google Scholar Moreover, complications such as wound infection, adhesion dehiscence, and scar formation have been reported to occur in approximately one in 5 to one in 4 patients.11Kirschner R.E. Low D.W. Randall P. Bartlett S.P. McDonald-McGinn D.M. Schultz P.J. et al.Surgical airway management in Pierre Robin sequence: is there a role for tongue-lip adhesion?.Cleft Palate Craniofac J. 2003; 40: 13-18Crossref PubMed Scopus (141) Google Scholar, 14Cozzi F. Totonelli G. Frediani S. Zani A. Spagnol L. Cozzi D.A. The effect of glossopexy on weight velocity in infants with Pierre Robin syndrome.J Pediatr Surg. 2008; 43: 296-298Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar We therefore question whether tongue-lip adhesion can be recommended as a “good surgical treatment for most children” with RS.16Bijnen C.L. Don Griot P.J. Mulder W.J. Haumann T.J. Van Hagen A.J. Tongue-lip adhesion in the treatment of Pierre Robin sequence.J Craniofac Surg. 2009; 20: 315-320Crossref PubMed Scopus (29) Google ScholarMandibular Distraction OsteogenesisThis procedure aims to correct the mandibular hypoplasia in RS by performing a bilateral vertical mandibular osteotomy and placing pins for a multi-vector external (or internal) distractor.Beginning a few days after the operation, distraction is usually done at a rate of 1 to 2 mm daily18Sadakah A.A. Elshall M.A. Farhat A.A. Bilateral intra-oral distraction osteogenesis for the management of severe congenital mandibular hypoplasia in early childhood.J Craniomaxillofac Surg. 2009; 37: 216-224Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar until the patient has a class III occlusion. This over-correction is considered necessary to sustain an adequate airway in case a (partial) relapse occurs after distraction.19Tibesar R.J. Scott A.R. McNamara C. Sampson D. Lander T.A. Sidman J.D. Distraction osteogenesis of the mandible for airway obstruction in children: long-term results.Otolaryngol Head Neck Surg. 2010; 143: 90-96Crossref PubMed Scopus (56) Google Scholar The devices are usually removed 4 to 8 weeks after the end of the distraction period. Recently, the successful use of an internal single-stage self-resorbable device also has been reported.20Burstein F.D. Williams J.K. Mandibular distraction osteogenesis in Pierre Robin sequence: application of a new internal single-stage resorbable device.Plast Reconstr Surg. 2005; 115: 61-67PubMed Google ScholarDespite many studies reporting clinical success with this technique, few of them reported changes in weight gain. One study of 10 patients reported a decline in growth rate in 7 patients in the first 12 months after mandibular distraction, despite continued tube feeding in 3 patients.21Spring M.A. Mount D.L. Pediatric feeding disorder and growth decline following mandibular distraction osteogenesis.Plast Reconstr Surg. 2006; 118: 476-482Crossref PubMed Scopus (39) Google Scholar This may be related to dysphagia, often seen in RS, not being corrected by the distraction procedure.22Pinheiro Neto C.D. Alonso N. Sennes L.U. Goldenberg D.C. Santoro Pde P. Polysomnography evaluation and swallowing endoscopy of patients with Pierre Robin sequence.Braz J Otorhinolaryngol. 2009; 75: 852-856Crossref PubMed Google Scholar In contrast, a study in 17 infants reported accomplishment of full oral feeding in all infants by 3.5 months postoperatively, but provided no growth data.23Looby J.F. Schendel S.A. Lorenz H.P. Hopkins E.M. Aizenbud D. Airway analysis: with bilateral distraction of the infant mandible.J Craniofac Surg. 2009; 20: 1341-1346Crossref PubMed Scopus (35) Google ScholarSeveral case series reported PSG results before and after mandibular distraction.18Sadakah A.A. Elshall M.A. Farhat A.A. Bilateral intra-oral distraction osteogenesis for the management of severe congenital mandibular hypoplasia in early childhood.J Craniomaxillofac Surg. 2009; 37: 216-224Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar, 22Pinheiro Neto C.D. Alonso N. Sennes L.U. Goldenberg D.C. Santoro Pde P. Polysomnography evaluation and swallowing endoscopy of patients with Pierre Robin sequence.Braz J Otorhinolaryngol. 2009; 75: 852-856Crossref PubMed Google Scholar, 23Looby J.F. Schendel S.A. Lorenz H.P. Hopkins E.M. Aizenbud D. Airway analysis: with bilateral distraction of the infant mandible.J Craniofac Surg. 2009; 20: 1341-1346Crossref PubMed Scopus (35) Google Scholar, 24Monasterio F.O. Drucker M. Molina F. Ysunza A. Distraction osteogenesis in Pierre Robin sequence and related respiratory problems in children.J Craniofac Surg. 2002; 13 (discussion 4): 79-83Crossref PubMed Scopus (119) Google Scholar, 25Dauria D. Marsh J.L. Mandibular distraction osteogenesis for Pierre Robin sequence: what percentage of neonates need it?.J Craniofac Surg. 2008; 19: 1237-1243Crossref PubMed Scopus (58) Google Scholar, 26Hammoudeh J. Bindingnavele V. Davis B. Davidson Ward S. Sanchez-Lara P. Kleiber G. et al.Neonatal and infant mandibular distraction as an alternative to tracheostomy in severe obstructive sleep apnea.Cleft Palate Craniofac J. 2010; (Epub ahead of print)PubMed Google Scholar All noted an improved or normalized apnea-hypopnea index (AHI) or respiratory disturbance index in most patients studied, but only 3 series provided detailed PSG results. One of the latter studies included 7 patients and reported a decrease in AHI from 60 (SD, 7.3) before distraction to 1.6 (SD, 1.6) at the end of the expander activation.18Sadakah A.A. Elshall M.A. Farhat A.A. Bilateral intra-oral distraction osteogenesis for the management of severe congenital mandibular hypoplasia in early childhood.J Craniomaxillofac Surg. 2009; 37: 216-224Abstract Full Text Full Text PDF PubMed Scopus (28) Google Scholar No long-term follow-up data were reported. Another study reported a decrease in mean AHI from 10.6 (range, 0-43) to 2.2 (range, 0-12.9) in 13 infants undergoing distraction osteogenesis,23Looby J.F. Schendel S.A. Lorenz H.P. Hopkins E.M. Aizenbud D. Airway analysis: with bilateral distraction of the infant mandible.J Craniofac Surg. 2009; 20: 1341-1346Crossref PubMed Scopus (35) Google Scholar and the third and largest study reported a decrease in AHI from a mean of 39.7 (range, 4.5-177.0) to 5.8 (range, 0-34) in a chart review of 28 infants in whom the procedure was performed and its effect documented with PSG.26Hammoudeh J. Bindingnavele V. Davis B. Davidson Ward S. Sanchez-Lara P. Kleiber G. et al.Neonatal and infant mandibular distraction as an alternative to tracheostomy in severe obstructive sleep apnea.Cleft Palate Craniofac J. 2010; (Epub ahead of print)PubMed Google Scholar The authors describing the self-resorbable device aforementioned also reported resolution of UAO (ie, an respiratory disturbance index <2.0) in all 9 of their 14 patients who did not undergo tracheostomy and who were stable enough to tolerate a preoperative sleep study.20Burstein F.D. Williams J.K. Mandibular distraction osteogenesis in Pierre Robin sequence: application of a new internal single-stage resorbable device.Plast Reconstr Surg. 2005; 115: 61-67PubMed Google ScholarComplications include scarring (sometimes hypertrophic), pin site infections (18%), device failure (10%), persistent inferior alveolar nerve lesions (6%) and, somewhat more rarely, damage to tooth buds resulting in long-term tooth loss, dentigerous cyst formation, or relapse of UAO symptoms and temporo-mandibular ankylosis.27Ow A. Cheung L.K. Skeletal stability and complications of bilateral sagittal split osteotomies and mandibular distraction osteogenesis: an evidence-based review.J Oral Maxillofac Surg. 2009; 67: 2344-2353Abstract Full Text Full Text PDF PubMed Scopus (72) Google Scholar Some of these complications should be preventable with the new internal resorbable devices, but more data are necessary.20Burstein F.D. Williams J.K. Mandibular distraction osteogenesis in Pierre Robin sequence: application of a new internal single-stage resorbable device.Plast Reconstr Surg. 2005; 115: 61-67PubMed Google ScholarMandibular TractionThis procedure, first described in 1937,28Callister A.C. Hypoplasia of the mandible (mikrognathy) with cleft palate: Treatment in early infancy by skeletal traction.Am J Dis Child. 1937; 53: 1057-1059Crossref Google Scholar involves fixation of a percutaneous parasymphysial circumferential wire to the mandible, with traction being applied with suspension weights (50-200 g) left in place for 4 to 6 weeks. Its effect on UAO is unknown, and it requires long-term immobilization of the patient during a critical developmental period. Also, a long-term follow-up study showed persistence of retrognathia on cephalometric radiography.29Schettler D. Koch H. [Growing jaw during and after orthopedic-surgical extension in children with congenital microgenia].Fortschr Kiefer Gesichtschir. 1974; 18: 166-169PubMed Google ScholarProcedures that Bridge the Narrow Upper AirwayNasopharyngeal AirwayThis device, first suggested by a British group,30Heaf D.P. Helms P.J. Dinwiddie R. Matthew D.J. Nasopharyngeal airways in Pierre Robin syndrome.J Pediatr. 1982; 100: 698-703Abstract Full Text PDF PubMed Scopus (79) Google Scholar bridges the narrow pharyngeal space by inserting an endotracheal tube in one of the nares so that it ends just superior to the epiglottis (controlled with endoscopy or radiography). Methods of estimating the required length of the tube without endoscopy or radiography and securing it safely to the nose have been described.31Masters I.B. Chang A.B. Harris M.O. Neil M.C. Modified nasopharyngeal tube for upper airway obstruction.Arch Dis Child. 1999; 80: 186-187Crossref PubMed Scopus (28) Google Scholar One study reported on 22 infants with RS (3 syndromic), with 20 being treated with a nasopharyngeal airway and high-calorie nasogastric tube feeding. Infants were gradually weaned from both tubes while being monitored for oxygen saturation. During a mean duration of hospital stay of 60 days (range, 25-162 days), 18 infants were reported to grow along the percentile of their birth weight, but only 3 were fully bottle fed at discharge. Also, only 10 infants maintained their weight percentile until the time of cleft repair. Unfortunately, no oximetry data were provided.32Wagener S. Rayatt S.S. Tatman A.J. Gornall P. Slator R. Management of infants with Pierre Robin sequence.Cleft Palate Craniofac J. 2003; 40: 180-185Crossref PubMed Scopus (100) Google Scholar Another study reported weight gain in 8 infants with a nasopharyngeal airway (median age, 50 days; range, 15-180 days; 4 also had supplemental oxygen administered). Their weekly weight gain increased from 86 g before to 255 g with the nasopharyngeal airway, but no data on weight gain after hospital discharge or any PSG results were reported.33Chang A.B. Masters I.B. Williams G.R. Harris M. O’Neill M.C. A modified nasopharyngeal tube to relieve high upper airway obstruction.Pediatr Pulmonol. 2000; 29: 299-306Crossref PubMed Scopus (35) Google ScholarComplications include blockage of the tube by secretions and aspiration of gastric contents potentially occurring when the airway is too long.32Wagener S. Rayatt S.S. Tatman A.J. Gornall P. Slator R. Management of infants with Pierre Robin sequence.Cleft Palate Craniofac J. 2003; 40: 180-185Crossref PubMed Scopus (100) Google Scholar Moreover, it provides no stimulus for the mandible to grow or the tongue to assume a more horizontal position. Although certainly valuable as a temporary measure, it does not solve the anatomical problems underlying the UAO and poor weight gain.Pneumatic Airway Stenting with Nasal Continuous Positive Airway Pressure or Intermittent Positive Pressure VentilationThere are some case series on the use of nasal continuous positive airway pressure or intermittent positive pressure ventilation in RS.34Essouri S. Nicot F. Clement A. Garabedian E.N. Roger G. Lofaso F. et al.Noninvasive positive pressure ventilation in infants with upper airway obstruction: comparison of continuous and bilevel positive pressure.Intensive Care Med. 2005; 31: 574-580Crossref PubMed Scopus (120) Google Scholar, 35Leboulanger N. Picard A. Soupre V. Aubertin G. Denoyelle F. Galliani E. et al.Physiologic and clinical benefits of noninvasive ventilation in infants with Pierre Robin sequence.Pediatrics. 2010; 126: e1056-e1063Crossref PubMed Scopus (50) Google Scholar In a recent single-center analysis of 81 patients with RS, 7 (9%) had been treated with nasal intermittent positive pressure ventilation, starting at a mean age of 2 months and lasting for a mean of 16.7 months. Reported benefits included a decrease in the proportion of time spent with oxygen saturation <90% from a mean of 14% to 1% (at a mean airway pressure of 8.3 cm H2O) and a decrease in mean transcutaneous carbon dioxide from 57 to 31 mm Hg. All 7 infants were discharged home with the device, which was used at home for an average of >8 hours per day. No facial adverse effects were reported.35Leboulanger N. Picard A. Soupre V. Aubertin G. Denoyelle F. Galliani E. et al.Physiologic and clinical benefits of noninvasive ventilation in infants with Pierre Robin sequence.Pediatrics. 2010; 126: e1056-e1063Crossref PubMed Scopus (50) Google Scholar There is anecdotal evidence, however, that long-term nasal continuous positive airway pressure use in young children may result in mid-face hypoplasia.36Li K.K. Riley R.W. Guilleminault C. An unreported risk in the use of home nasal continuous positive airway pressure and home nasal ventilation in children: mid-face hypoplasia.Chest. 2000; 117: 916-918Crossref PubMed Scopus (126) Google Scholar Although particularly relevant to patients with RS, who may have a hypoplastic maxilla anyway,37Suri S. Ross R.B. Tompson B.D. Craniofacial morphology and adolescent facial growth in Pierre Robin sequence.Am J Orthod Dentofacial Orthop. 2010; 137: 763-774Abstract Full Text Full Text PDF PubMed Scopus (45) Google Scholar this potential adverse effect has not been studied systematically.TracheostomyTracheostomy clearly resolves UAO, but it does not correct the underlying malformation and puts considerable burden on the families, who often require additional nursing staff and expensive supplies.38Evans A.K. Rahbar R. Rogers G.F. Mulliken J.B. Volk M.S. Robin sequence: a retrospective review of 115 patients.Int J Pediatr Otorhinolaryngol. 2006; 70: 973-980Abstract Full Text Full Text PDF PubMed Scopus (189) Google Scholar It is also fraught with numerous complications, including granuloma formation, which occurs in most patients and requires intervention with general anesthesia in 10% of cases,39Mahadevan M. Barber C. Salkeld L. Douglas G. Mills N. Pediatric tracheotomy: 17 year review.Int J Pediatr Otorhinolaryngol. 2007; 71: 1829-1835Abstract Full Text Full Text PDF PubMed Scopus (142) Google Scholar bleeding, pneumothorax, tracheal stenosis, tube displacement, impaired speech development, and even sudden death (the latter in 1%-4% of cases).40Carr M.M. Poje C.P. Kingston L. Kielma D. Heard C. Complications in pediatric tracheostomies.Laryngoscope. 2001; 111: 1925-1928Crossref PubMed Scopus (229) Google Scholar In a survey of parents of 41 pediatric patients with RS who underwent tracheostomy, 60% of children required ≥3 hospitalizations and 23% of children reported airway problems after decannulation.41Demke J. Bassim M. Patel M.R. Dean S. Rahbar R. van Aalst J.A. et al.Parental perceptions and morbidity: tracheostomy and Pierre Robin sequence.Int J Pediatr Otorhinolaryngol. 2008; 72: 1509-1516Abstract Full Text Full Text PDF PubMed Scopus (31) Google ScholarAlthough the procedure should be reserved for severe cases, the proportion of patients undergoing tracheostomy is as high as 50% in some case series.38Evans A.K. Rahbar R. Rogers G.F. Mulliken J.B. Volk M.S. Robin sequence: a retrospective review of 115 patients.Int J Pediatr Otorhinolaryngol. 2006; 70: 973-980Abstract Full Text Full Text PDF PubMed Scopus (189) Google Scholar Corresponding to this, 52% of respondents in the aforementioned survey considered tracheostomy the treatment of choice in patients who failed observation and positioning.8Myer 3rd, C.M. Reed J.M. Cotton R.T. Willging J.P. Shott S.R. Airway management in Pierre Robin sequence.Otolaryngol Head Neck Surg. 1998; 118: 630-635Crossref PubMed Google Scholar This, however, is in contrast to other centers’ experience (see below).Palatal PlatesThese plates have been used in infants with RS since the late 1960s.42Pielou W.D. Non-surgical management of Pierre Robin syndrome.Arch Dis Child. 1967; 42: 20-23Crossref PubMed Scopus (21) Google Scholar Their effect is thought to be mediated via an improved tongue function, with the latter stimulating mandibular growth, although data supporting this concept are sparse.43Zschiesche S. Can a relationship between tongue function and mandibular shape in patients with Pierre-Robin syndrome be demonstrated?.Fortschr Kieferorthop. 1985; 46: 72-78Crossref PubMed Scopus (3) Google Scholar, 44Steinhardt G. Die Bedeutung funktioneller Einflüsse für die Entwicklung und Formung der Kiefergelenke.Dtsch Zahn-, Mund- und Kieferheilk. 1935; 2: 711Google ScholarIn a recent case series involving 188 infants with RS seen in one center, a palatal plate was used in 134, resulting in a resolution of glossoptosis and, thereby, of clinically evident airway obstruction in 122 (91%).45Butow K.W. Hoogendijk C.F. Zwahlen R.A. Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol.J Pediatr Surg. 2009; 44: 2112-2118Abstract Full Text Full Text PDF PubMed Scopus (54) Google Scholar Feeding problems, however, were reported to persist in 26% of patients despite palatal plate therapy, and the extent of airway obstruction was not assessed objectively nor were any data provided on weight gain. Only 9% of the 188 patients, however, received invasive treatments (tracheostomy, 2.1%; glossopexy, 6.9%), which is considerably less than in other large case series.38Evans A.K. Rahbar R. Rogers G.F. Mulliken J.B. Volk M.S. Robin sequence: a retrospective review of 115 patients.Int J Pediatr Otorhinolaryngol. 2006; 70: 973-980Abstract Full Text Full Text PDF PubMed Scopus (189) Google ScholarIn infants with more severe UAO, a modified acrylic palatal plate in which a velar extension resembling a spur or baton shifts the base of the tongue forward may considerably improve the UAO (Figures 1 and 2).46Bacher M. Sautermeister J. Urschitz M. Buchenau W. Arand J. Poets C. An oral appliance with velar extension for treatment of obstructive sleep apnea in infants with the Pierre Robin sequence.Cleft Palate Craniofac J. 2011; 48: 331-336Crossref PubMed Scopus (41) Google Scholar The baton’s stability in this so-called pre-epiglottic baton plate (PEBP) is increased by incorporating a wire into the acrylic (Figure 1). The correct length and angle of the baton are controlled endoscopically, and its effectiveness in relieving UAO can be confirmed with PSG. Treatment is supplemented by stimulation of the oral musculature, on the basis of the Castillo-Morales approach47Limbrock G.J. Castillo-Morales R. Hoyer H. Stover B. Onufer C.N. The Castillo-Morales approach to orofacial pathology in Down syndrome.Int J Orofacial Myology. 1993; 19: 30-37PubMed Google Scholar and feeding training (initially via finger feeding, subsequently by a nurser which allows to control the ease of milk flow during sucking [Playtex Drop-Ins, Playtex Products, Neenah, Wisconsin]). The treatment aforementioned requires an interdisciplinary team consisting of an orthodontist, a pediatric sleep specialist, a speech therapist familiar with orofacial regulation therapy, and a pediatrician trained in nasopharyngeal endoscopy. An experienced nursing team is also of paramount importance, especially to train parents in handling the PEBP. Starting this treatment as soon as possible after birth seems to reduce the duration of PEBP treatment, because the mandible has its largest growth potential early in life.44Steinhardt G. Die Bedeutung funktioneller Einflüsse für die Entwicklung und Formung der Kiefergelenke.Dtsch Zahn-, Mund- und Kieferheilk. 1935; 2: 711Google Scholar, 46Bacher M. Sautermeister J. Urschitz M. Buchenau W. Arand J. Poets C. An oral appliance with velar extension for treatment of obstructive sleep apnea in infants with the Pierre Robin sequence.Cleft Palate Craniofac J. 2011; 48: 331-336Crossref PubMed Scopus (41) Google ScholarFigure 2Lateral view of an infant with syndromal RS requiring a naso-pharyngeal airway for airway obstruction. The patient was successfully transitioned to a PEBP, which was used for 3 years with no complications, and had cleft palate repair at 4 years of age. The same patient is shown on the right at 6 years of age.Left panel reprinted from Bacher M. et al., Laryngo-Rhino-Otologie 2010;89:621-7, with permission. Photographs shown with parental permission.View Large Image Figure ViewerDownload Hi-res image Download (PPT)In a randomized controlled crossover trial, the e