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Obstructive sleep apnea (OSA) and airway compromise are common in infants with Pierre Robin syndrome (PRS) due to tongue-based airway obstruction. Tongue-lip adhesion (TLA) is an effective procedure that can alleviate the symptoms of OSA by preventing posterior prolapse of the tongue. Although OSA consistently improved following TLA, it did not fully resolve. Ongoing management of OSA was required in all patients. These results identify the need for OSA to be reevaluated and managed for several years in PRS patients who have had a TLA.

The purpose of this systematic review and meta-analysis was to determine the most effective and least morbid surgical technique for relieving retroglossal airway obstruction in infants with Robin sequence (RS). The study adhered to PRISMA guidelines and included 25 studies (24 cohorts and one case series) that investigated interventions for airway improvement, including conservative measures, tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and tracheostomy. The primary outcome variable was complication rate, while predictor variable was the use of interventions for airway improvement. Results showed that conservative measures were the preferred initial management strategy in most studies, while TLA was recommended for infants with mild obstruction, and MDO or tracheostomy was reserved for severe cases. Only complications could be analysed via meta-analysis due to data heterogeneity, revealing that tracheostomy had a summary odds ratio of 5.39 in favour of TLA, while MDO had a ratio of 2.8 over TLA, and the complication rates were similar between MDO and tracheostomy. If conservative measures fail, the study recommends mandibular distraction as the preferable technique for stable airway improvement. If the infant is unsuitable for distraction, tongue-lip adhesion may serve as an alternative, while tracheostomy should be reserved for cases of severe multi-level obstruction. The authors propose that large-scale, multicentre trials comparing long-term outcomes are required to establish definitive guidelines. [ABSTRACT FROM AUTHOR]

Obstructive sleep apnea (OSA) and airway compromise are common in infants with Pierre Robin syndrome (PRS) due to tongue-based airway obstruction. Tongue-lip adhesion (TLA) is an effective procedure that can alleviate the symptoms of OSA by preventing posterior prolapse of the tongue. Although OSA consistently improved following TLA, it did not fully resolve. Ongoing management of OSA was required in all patients. These results identify the need for OSA to be reevaluated and managed for several years in PRS patients who have had a TLA. [ABSTRACT FROM AUTHOR]

Background: Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery. Methods: All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate. Results: 67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy. Conclusion: The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon. [ABSTRACT FROM AUTHOR]

The purpose of this study was to assess the effect of tongue−lip adhesion (TLA) on the long-term speech and articulation outcomes of patients with Robin sequence (RS) after cleft palate repair. Outcomes were compared to those in patients with RS who required positioning alone and to patients with isolated cleft palate (ICP). All consecutive patients with RS (with or without TLA) versus isolated cleft palate (ICP) who underwent cleft palate repair were retrospectively reviewed. Speech and articulation included all assessments between the age of 3–6 years. Secondary speech operations, velopharyngeal insufficiency (VPI), hypernasality, and articulation errors by cleft-type characteristics (CTC), including 4 categories (passive), non-oral, anterior-oral, and posterior-oral. A total of 41 RS patients and 61 ICP patients underwent repair with sufficient follow-up. Of them, 23 patients underwent a TLA at median age of 12 days. Rates of hypernasality (p = 0.004), secondary speech operations (p = 0.004), and posterior oral CTC (p = 0.042) were higher in RS compared to ICP. Isolated RS had speech outcomes similar to those of ICP; however, syndromic RS patients needed more secondary speech operations compared to isolated RS (p = 0.043). TLA-RS patients did not demonstrate differences in speech outcomes or any CTCs (all p > 0.05) compared to non−TLA-RS patients, except for the anterior oral CTC (74% TLA-RS vs 28% non−TLA-RS, p = 0.005). Within the limitations of the study, it seem that TLA does not affect long-term velopharyngeal function in patients with RS. However, TLA-RS patients demonstrated higher rates of anterior-oral CTC, which might be related to a different positioning of the tongue after TLA. Every effort should be taken to treat patients with RS conservatively instead of with TLA because of this demonstrated a negative effect on one type of articulation error. However, if conservative therapy fails, a TLA is still a valuable adjunct in the treatment of RS, and cleft speech pathologists who treat such patients should be more aware of this phenomenon in order to improve long-term articulation outcomes. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the timing and safety of cleft palate (CP) repair in patients with Robin sequence (RS) treated with mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA) for airway obstruction. Design: Retrospective cohort study. Setting: Tertiary Pediatric Hospital during 2004-2020. Patients: 148 patients with RS underwent MDO, 66 met inclusion by having MDO and followed by palatoplasty. 26 patients with RS underwent TLA, 14 met inclusion by having TLA and followed by palatoplasty. Main Outcome Measures: Patient characteristics, hospital/operative details, postoperative complications, and polysomnographic (PSG) data were compared. Results: Groups were well-matched except more patients with syndromes underwent MDO (N = 27, 41%, P ≤.002). In the MDO and TLA cohorts, mean CP repair age was 12.8 ± 1.9 months and 14.6 ± 1.6 months, respectively (P ≤.002). Despite the earlier CP repair in the MDO group, there were no differences in peri-operative complication rates after palatoplasty in either group. All sleep respiratory parameters improved after MDO/TLA prior to palatoplasty P ≤.050. All PSG parameters remained significantly improved after palatoplasty compared to preoperative values, P ≤.043. Obstructive apnea hypopnea index and Oxygen saturation nadir further improved after palatoplasty within the MDO group, P ≤.050, while no changes in the TLA group, P ≥.500. Conclusions: MDO was associated with earlier age at palatoplasty than TLA with a similar perioperative risk profile. In those patients with pre- and post-palatoplasty PSG data, palatoplasty was not associated with a deterioration in PSG parameters, and in fact in the MDO group, PSG data improved. [ABSTRACT FROM AUTHOR]

The purpose of this study was to evaluate feeding impairment following non-operative or operative management of airway obstruction in a large series of infants with Robin sequence (RS) by rate of G-tube placement. A retrospective study was conducted at Boston Children's Hospital including 225 patients (47.1% female) with RS treated between 1976 and 2018. Subjects were grouped by intervention required for successful management of airway obstruction: non-operative only (n = 120), tongue–lip adhesion (TLA, n = 75), mandibular distraction osteogenesis (MDO, n = 21), or tracheostomy (n = 9). The operative group had a higher rate of G-tube placement (58.1%) than the non-operative group (28.3%, P < 0.0001). Subjects in the TLA and tracheostomy groups had higher odds of G-tube placement than subjects in the MDO group: odds ratio (OR) 5.5 (95% confidence interval (CI) 1.8–17.3, P = 0.004) and OR 27.0 (95% CI 3.2–293.4, P = 0.007), respectively. Syndromic patients and those with gastrointestinal anomalies also had higher odds of G-tube placement: OR 3.5 (95% CI 1.7–7.2, P = 0.001) and OR 5.9 (95% CI 1.6–21.0, P = 0.007), respectively. Infants with RS who require an airway operation and those with a syndromic diagnosis or gastrointestinal anomalies are more likely to require placement of a G-tube. Of the operative groups, MDO was associated with the lowest G-tube rate, compared to TLA and tracheostomy. [ABSTRACT FROM AUTHOR]

The triad of retrognathia, glossoptosis, and airway obstruction characterizes the Robin sequence along with the detrimental effects of mandibular hypoplasia on feeding, swallowing, and growth, which are very well described. Most of the babies are managed successfully on nonsurgical measures, but selected patients require surgical intervention in the neonatal period for survival. Conventionally, tracheostomy was done, which still remains a first-line surgical procedure for some surgeons. However, presently, most of the craniofacial centers have switched over to mandibular distraction procedures at an early stage and only sometimes tongue–lip adhesion (TLA). The literature is unclear as to which surgical procedure for securing the airway is more effective for these patients, and hence, the choice of procedure depends on the resources and surgical expertise. This article tells the tale of a neonate who survived by just placing a simple U-stitch between the tongue and lip, retracting the tongue outside, which is the basic concept of all TLA procedures. It also reemphasizes the importance of TLA in Robin patients to improve the airway obstruction and helps buy the time in which the mandible and associated structures grow. [ABSTRACT FROM AUTHOR]

Objective Tongue-lip adhesion may be used to relieve obstructive sleep apnoea in infants with Pierre Robin sequence (PRS), but only a few studies have objectively evaluated its efficacy. The purpose of this study was to evaluate the results of tongue-lip adhesion by polysomnography. Materiel and methods A single-centre retrospective study was conducted in infants with PRS treated by tongue-lip adhesion from 2004 to 2015, in whom at least laryngotracheal endoscopy and polysomnography were performed. The variables collected were the syndromic diagnosis, demographic data, respiratory management before tongue-lip adhesion, symptoms, and additional airway interventions. Obstructive sleep apnoea was classified into 3 groups according to severity. Polysomnography was performed one month after tongue-lip adhesion. Statistical analysis was performed with Wilcoxon signed-rank test with a limit of statistical significance of P < 0.005. Results Thirty-seven subjects in whom tongue-lip adhesion was performed at a mean age of 45 days (8 to 210 days) were included. Thirty-one patients had isolated PRS and 6 patients had associated anomalies. All patients had confirmed severe obstructive sleep apnoea. All patients required respiratory support prior to surgery: 8 intubated patients, 15 patients with noninvasive ventilation and 14 patients with nasopharyngeal airways. Eight patients had bradycardia before tongue-lip adhesion. All parameters were improved on postoperative polysomnography: oxygen saturation, hypercapnia, apnoea-hypopnoea index, bradycardia ( P < 0.005). Only 8 patients had persistent severe obstructive sleep apnoea and required tracheostomy ( n = 5) or noninvasive ventilation ( n = 3). No significant correlation was observed between treatment success and any predictive variables. Conclusion Tongue-lip adhesion improved airway obstruction in all infants with PRS and resolved obstructive sleep apnoea in 29 patients. However, 8 patients required additional airway interventions. [ABSTRACT FROM AUTHOR]

The article discusses research which examined the rate of failure of each type of surgical interventions for airway obstruction in infants born with Pierre Robin sequence. Topics discussed include the indication for the need for tracheostomy, the proportion of infants that have signficant airway obstruction postoperatively as determined by polysomnography, and mandibular distraction osteogenesis (MDO) as the most effective technique.

Cerebro-costo-mandibular syndrome (CCMS) consists of severe micrognathia, glossoptosis, posterior rib-gap defects and developmental delay. It may cause upper airway obstruction and flail chest, resulting in neonatal hypoxia, and possibly death. Early airway management or surgical intervention to maintain a patent airway is critical to avoid hypoxia in CCMS patients. We report a newborn with CCMS who was successfully weaned from a laryngeal mask after undergoing a tongue–lip adhesion operation at 164 days of age.

Objectives: Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature. Material and methods: A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS. Results: Forty-four infants (59 %) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25 %, p = .014). A mandibular distraction was conducted in 24 % ( n = 18) of cases, a tracheotomy in 9 % ( n = 7), and a tongue-lip adhesion in 8 % ( n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described. Conclusions: RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended. Clinical Relevance: We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence is not a rare condition and paediatric specialists caring for respiratory related issues are likely to encounter cases in their practice. There have been a few recent reviews on the topic, mostly focusing on the surgical interventions performed for cases with severe airway obstruction. In the present review, we will highlight the different challenges that remain today in the global evaluation of infants afflicted with this condition through a thorough review of the medical literature, giving the clinician a full scope of the disease and of the various management options. The need for an improved objective evaluation of airway obstruction and for a better classification will be emphasized. We are therefore proposing a novel classification scheme that will better account for respiratory and feeding difficulties in these infants. Finally, many knowledge gaps persist regarding this condition, underlining the necessity for further research both in the genetic field and regarding the outcome of therapy. [ABSTRACT FROM AUTHOR]

Objectives/Hypothesis To estimate the current birth prevalence of isolated and syndromic Pierre Robin sequence (iPRS and sPRS), including demographic variations. To assess for regional variations in surgical airway interventions for PRS, and to determine the mean length of stay (LOS), cost of admission, complication rate, and rate of associated procedures related to tongue-lip adhesion (TLA), neonatal mandibular distraction osteogenesis (MDO), and tracheotomy. Study Design Retrospective cross-sectional study. Methods The 2006 and 2009 Kids Inpatient Databases were used to identify newborns and infants with PRS; analysis using cross tabulations and linear regression modeling was performed. Results In 2006 and 2009, the estimated birth prevalence of iPRS was 1.8:10,000 live births and sPRS 1.4:10,000 live births. The highest rate was in whites and the lowest in non-Hispanic blacks. There were 145 TLAs (36%), 176 MDOs (43%), and 85 tracheotomies (21%). The Northeast favored a TLA strategy; the Midwest favored MDO. The mean LOS for TLA was 24.5 days, MDO 36.7 days, tracheotomy (iPRS) 44.9 days, and tracheotomy (sPRS) 53.0 days. Conclusions The birth prevalence of PRS may be higher than previously described, especially in whites. Surgical management strategies vary between regions. The overall cost of a TLA admission is lower than an MDO or tracheotomy admission, owing primarily to shorter LOS. This study was limited by not taking into account outpatient expenses (nursing care, monitoring) or need for further airway/feeding intervention over subsequent admissions. Level of Evidence 2c Laryngoscope, 124:2818-2825, 2014 [ABSTRACT FROM AUTHOR]

Abstract: The objective was to review and compare outcomes after tongue–lip adhesion (TLA) and mandibular distraction osteogenesis (MDO) in infants with severe breathing difficulties related to Pierre Robin sequence (PRS). A single-centre retrospective (2002–2012) study was carried out; 18 infants with severe breathing difficulties related to PRS resistant to conservative treatment, who underwent TLA or MDO to correct airway obstruction, were enrolled. The primary outcome measures were successful weaning from respiratory support and resumption of full oral feeding. Nine underwent TLA and nine MDO. Eight of the nine infants who underwent MDO and all those treated with TLA were successfully weaned from respiratory support. After discharge, residual respiratory distress was diagnosed more commonly after TLA than after MDO (6/9 vs 1/9, P =0.050). Infants resumed oral feeding sooner after MDO than after TLA (mean days after surgery to full oral feeds 44±24 vs 217±134, P <0.003). The length of hospital stay was longer for infants treated with MDO than for those treated with TLA. The rate of complications was similar. Infants with severe airway obstruction related to PRS can benefit safely from either TLA or MDO. Although MDO lengthens the time to discharge, this option stabilizes airway patency of infants with PRS more efficiently and achieves full oral feeding more rapidly than TLA. [Copyright &y& Elsevier]

The article presents a study on the tongue suspension technique for performing tongue-lip adhesion. It notes that tongue-lip adhesion with suspension entails several advantages including the prevention of button aspiration and potential interference in early bottle-feeding and developmental oral motor function. It mentions that the technique expands the armamentarium of craniofacial surgeons in managing difficult airways in Pierre Robin sequence.

The article presents a study which characterizes obstructive sleep apnea before and after tongue-lip adhesion in children with micrognathia utilizing polysomnography. It notes that the findings reveal that tongue-lip adhesion is performed to alleviate airway obstruction in micrognathic infants. It was also found out that polysomnographic evaluation of tongue-lip adhesion before and after surgery reveals that only 38% had complete resolution.

Cerebro-costo-mandibular syndrome (CCMS) consists of severe micrognathia, glossoptosis, posterior rib-gap defects and developmental delay. It may cause upper airway obstruction and flail chest, resulting in neonatal hypoxia, and possibly death. Early airway management or surgical intervention to maintain a patent airway is critical to avoid hypoxia in CCMS patients. We report a newborn with CCMS who was successfully weaned from a laryngeal mask after undergoing a tongue–lip adhesion operation at 164 days of age. [Copyright &y& Elsevier]

Pierre Robin sequence is a congenital malformation. One of its typical sign is micrognathia, which often causes retroposition of the tongue with possible significant airway obstruction and feeding difficulties. If conservative treatment is not sufficient, a surgical procedure can be performed. Labio-glossopexy in patients with PRS should be balanced against the other operations, namely tracheostomy and mandibular distraction. Labio-glossopexy is less invasive method then tracheostomy, simplifies nursing care, shortens hospital stay and makes homecare less demanding. [ABSTRACT FROM AUTHOR]

Objective: There is considerable variation in opinion regarding the optimal management of patients with Pierre Robin sequence (PRS). No single method of airway intervention or feeding strategy is universally appropriate and effective. This study was performed to examine methods used for airway and feeding management and to identify specific problems encountered. Study Design: A retrospective study of 252 patient charts between 1989 and 1997 at Children's Hospital of Wisconsin. Methods: Patient information was collected regarding perinatal history, genetics evaluation, and airway and feeding evaluations and intervention. A group of 47 patients was determined as having PRS. Results: Secondary respiratory difficulties, defined as respiratory abnormalities in addition to the expected PRS obstruction, were identified in 23% of patients. Also, intrinsic feeding abnormalities not associated with airway obstruction were identified in 11% of patients. Analysis by Fisher's Exact Test revealed patients with a syndromic diagnosis to have a significantly higher rate for tracheotomies and gastrostomy tube placement ( P = .041, and P = .0004, respectively). Syndromic patients were also found to have significantly lower Apgar scores and longer hospital stays. Positioning techniques, tongue-lip adhesion, and tracheotomy were also employed effectively with specific indications and specific difficulties that need to be considered. Conclusion: Patients with PRS require thorough airway and feeding evaluation. Those with additional syndromic diagnoses demonstrate higher rates of more invasive interventions. Patients with PRS must undergo individualized approaches with consideration of multiple factors for successful management. [ABSTRACT FROM AUTHOR]

Abstract: Aim: In infants with Pierre Robin syndrome (PRS), mandibular distraction may be more advantageous than glossopexy as it not only relieves oropharyngeal airway obstruction but also reverses body growth retardation. Because no data are available on body weight velocity after glossopexy, we assessed longitudinally the body weight velocity in a cohort of children undergoing glossopexy. Methods: The records of 48 infants with PRS undergoing glossopexy after unsuccessful nonoperative treatment between 1981 and 2005 were reviewed. Weight measurements were analyzed at 4 time-points: at birth, on admission for glossopexy, on admission for lysis of lip-tongue adhesion (TLA), and at follow-up. Weight velocity was assessed using Tanner''s tables. Main Results: Adhesion dehiscence occurred in 9 patients (18.7%). Lip-tongue adhesion resolved airway compromise in 36 infants (75%). Release of TLA was accomplished in 34 patients. Data on weight velocity from birth to follow-up (mean, 5.57 ± 0.59 years) were available for 31 patients. After glossopexy, mean body weight increased from the 9.7 ± 2.6th to the 17.5 ± 4.6th percentile (P > .05), whereas mean weight velocity increased from the 19.1 ± 4.9th to the 74.2 ± 4.7th percentile (P < .001). No temporal correlation was found between glossopexy and oropharyngeal dysphagia. Conclusion: In infants with PRS, glossopexy is a valid alternative to mandibular distraction because it does not cause decline in body growth. [Copyright &y& Elsevier]