Systematic review and meta-analysis of surgical approaches for improving airway stability in infants with Robin sequence: evaluating complications and outcomes.

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.

Tongue lip adhesion (TLA) in the management of airway obstruction and feeding in Pierre Robin sequence, a case report.

INTRODUCTION AND IMPORTANCE: Pierre Robin Sequence (PRS) is a rare and complex condition that often necessitates the collaboration of a full medical team from various disciplines to save the lives of babies with this genetic mutation, characterized by three clinical manifestations: glossoptosis, micrognathia, and cleft palate. Treatment primarily involves freeing upper airway obstructions and enhancing nutrition to allow the babies to lead a normal life. The lip-tongue adhesion procedure has been identified in medical literature as the recommended approach to addressing the issues associated with Pierre Robin sequence, and this method was successfully adopted in this case. CASE PRESENTATION: 2.5 kg, a newborn male baby with an abnormal position of the tongue and the inability to breastfeed and feed normally, without any medical, family, or social history. Following an examination, it was discovered that the baby had a posterior position of the tongue, micrognathia, and a cleft palate, leading to a diagnosis of Pierre Robin Sequence (Figs. 1, 2). Preparations for the baby's surgery have commenced. The baby was solely fed intravenously and provided with an oxygen mask for 25 days until all necessary consultations were completed and the baby's readiness for surgery and general anesthesia was confirmed. The surgical plan involved attaching the tongue to the lower lip to enhance the tongue's muscular strength, addressing the posterior position issue, and delaying the palate repair until the age of 1.5 years. CLINICAL DISCUSSION: PRS is a clinical entity characterized by the triad of mandibular hypoplasia (small jaw), glossoptosis (hypotonic, retracted tongue) and respiratory obstruction that require a multidisciplinary team for initial evaluation and management and maintenance care. TLA is a simple and effective procedure for increasing the cross-sectional area of oropharyngeal port. CONCLUSION: Handling airway obstruction in Pierre Robin Sequence involves various factors, and there is no universal treatment that can address all cases. Appropriate airway management strategies and feeding programs are essential for each individual with PRS. Our review highlights that TLA is a straightforward surgical procedure with minimal or no short-term complications. TLA should be considered as the primary surgical intervention when relief is needed.

Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers. STUDY DESIGN: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge. RESULTS: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both). CONCLUSIONS: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Effects of nasoalveolar molding and lip adhesion in the treatment of complete cleft lip-alveolus-palate

Introduction: Cleft lip and palate rank third among congenital defects, demanding early intervention for correction. Aim: To describe the results obtained with the use of nasoalveolar molding (NAM) and lip adhesion in patients with complete cleft lip-alveolus-palate, treated in the Maxillofacial Surgery service of the Pediatric Hospital of Matanzas between 2010 and 2020. Methods: An observational, descriptive, prospective research was carried out, in a universe of 16 patients with a diagnosis of complete cleft lip-alveolus-palate, treated in the Maxillofacial Surgery Service of Matanzas pediatric hospital during the period from January 2010 to December 2020, determining reference points to perform linear measurements that represented the study variables, both in patients treated only with nasoalveolar molding and in those in whom the nasoalveolar molding-lip adhesion binomial was used. Results: With the exclusive use of nasoalveolar molding, there was a significant reduction of the palatal cleft in all patients under study, with increase in maxillary and hemi-maxillary transverse dimension, as well as a significant reduction of the alveolar cleft. The total reduction of the alveolar cleft was only achieved by combining lip adhesion with nasoalveolar molding. Conclusion: The nasoalveolar molding-lip adhesion combination constitutes an effective binomial in the treatment of complete cleft lip-alveolus-palate.

Introducción: Las fisuras de labio y paladar ocupan el tercer lugar entre los defectos congénitos, demandando una temprana intervención para su corrección. Objetivo: Describir los resultados del empleo del moldeo nasoalveolar y la adhesión labial en pacientes con fisura labio-alveolo-palatina completa, atendidos en el Servicio de Cirugía Maxilofacial del hospital pediátrico de Matanzas, entre 2010 y 2020. Métodos: Se realizó una investigación observacional, descriptiva, prospectiva, en un universo de 16 pacientes con diagnóstico de fisura labio-alveolo-palatina completa, atendidos en el Servicio de Cirugía Maxilofacial del hospital pediátrico de Matanzas, durante el período comprendido de enero de 2010 a diciembre de 2020, determinándose puntos de referencia para realizar mediciones lineales que representaron las variables de estudio, tanto en pacientes atendidos solo con moldeo nasoalveolar como en los que se empleó el binomio moldeo nasoalveolar-adhesión labial. Resultados: Con el empleo exclusivo del moldeo nasoalveolar, existió una significativa reducción de la hendidura palatina en los pacientes estudiados, con incremento de la dimensión transversal maxilar y hemimaxilar, así como una significativa reducción de la hendidura alveolar. La reducción total de la hendidura alveolar solo se logró al combinar la adhesión labial con el moldeo nasoalveolar. Conclusión: La combinación moldeo nasoalveolar-adhesión labial constituye un binomio efectivo en el tratamiento de la fisura labio-alveolo-palatina completa.

Long-term speech outcome in patients with Robin sequence after cleft palate repair and tongue-lip adhesion: A 21-year retrospective analysis.

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.

Cleft Palate Repair in Robin Sequence following Mandibular Distraction Osteogenesis Compared to Tongue-Lip Adhesion.

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.

Airway and Feeding Outcomes in Pierre Robin Sequence: A Comparison of Three Management Strategies.

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.

The management of upper airway obstruction in Pierre Robin Sequence.

Pierre Robin Sequence (PRS) is defined by a constellation of characteristics including micrognathia, glossoptosis and airway obstruction. PRS can occur in isolation or can be associated with syndromes and another anomalies. Airway obstruction and feeding difficulties are the major presenting issues, and the severity of the condition ranges from mild, with minimal to no symptoms, to severe, with overt obstruction resulting in apnoeas, severe respiratory distress and cyanosis. The presence of airway obstruction can result in obstructive sleep apnoea and abnormalities in gas exchange, as well as exacerbation of already present feeding difficulties and failure to thrive, secondary to mismatch of caloric intake to energy usage associated with increased effort of breathing. Management of airway obstruction for infants with PRS varies between centres. This paper explores the surgical and non-surgical management options available, their effectiveness and pitfalls in children with PRS. Despite the pros and cons of each management option, it is evident that resource availability and multidisciplinary clinical support are key factors to successful management.

Long-Term Evaluation of the Lip and Nose in Bilateral Complete Cleft Lip Patients following Lip Adhesion and Secondary Nose Correction.

Background Surgical correction of bilateral cleft lip deformities remains one of the most challenging areas in facial plastic surgery. Many surgical techniques and conservative devices have been offered for the early management of bilateral cleft lip in infants. The purpose of this study was to evaluate the effect of lip adhesion on the lip and nose of patients with bilateral cleft lip. Methods A retrospective review of 13 patients with bilateral cleft lip was performed and compared with age-matched noncleft children. Patients underwent lip adhesion at a mean age of 2.8 months, and cheiloplasty at 6.6 months of age using a modification the Mulliken method. Secondary rhinoplasty was performed at the age of 6 in 13 patients. The surgical results were analyzed using photographic records obtained at the age of 1 and 7 years. Twelve length measurements and one angle measurement were obtained. Results All measurements were not statistically different from those of the noncleft age-matched control group at the age of 1. At 7 years of age, upper lip height and vermilion mucosal height were shorter ( p < 0.05) than in the control group. Nasal tip protrusion and the nasolabial angle were greater ( p < 0.05) than in the control group. Conclusion Lip adhesion followed by secondary rhinoplasty resulted in an acceptable lip and nasal appearance. Although nasoalveolar molding is now widely used, lip adhesion can be an appropriate alternative if an orthodontist is not available due to geographical or economic constraints.

Tongue-lip adhesion in Pierre-Robin sequence: Role redefined.

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.

A comparison of airway interventions and gastrostomy tube placement in infants with Robin sequence.

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.

Lip synechiae: A rare complication of azithromycin-associated Stevens-Johnson syndrome.

Stevens-Johnson syndrome (SJS) is a severe form of erythema multiforme, is a self-limiting acute inflammatory disease of multifactorial origin, but can also present as a chronic recurrent lesion. It causes a whole plethora of lesions, mostly mucocutaneous. It is a dermatologic emergency that occurs with a spectrum of severity and can result in severe morbidity and mortality. Lip adhesion is an unusual complication of healing in the lesions of SJS, for which only a few cases have been reported till date which not only causes esthetic morbidity but also impairs the proper functioning of the patient. The importance of this lesion also lies in its multifactorial and varied origin, this being the first case to report azithromycin as a causative drug, leading to SJS associated with lip adhesion. In this paper, we present a case report of SJS with lip adhesion, azithromycin being the causative drug, which was treated surgically with chalinoplasty. Along with it, the clinical features, its pathogenesis, the preventive measures, and the treatment modalities for the same including conservative as well as surgical have also been extensively discussed with a review of the existing English literature to date.

Lip Adhesion- A Viable Alternative To Pre-Surgical Orthodontics For The Management Of Wide Cleft Lips In Third World Countries.

BACKGROUND: Cleft lip and palate is a relatively common condition presenting a considerable technical challenge, especially the wide cleft (>8 mm), for the surgeons. Pre-surgical orthodontics, which reduces the cleft width and facilitates definitive repair, is expensive and not universally available, especially in the third world. Lip adhesion could be a cheaper alternative to pre-surgical orthodontics. METHODS: This six-year prospective observational study, from 2010 to 2016, was conducted at the paediatric surgical units of PNS Shifa Hospital, Karachi and Military Hospital Rawalpindi. All children with wide (8 mm or more gap in the alveolus) complete ULCP (unilateral cleft lip and palate) were included in the study. Lip adhesion with concomitant vomer flap palatal repair was followed by definitive lip repair once the desired moulding, i.e., alveolar gap <5 mm or adequate narrowing as per surgeon's subjective assessment during the 3 and 6 monthly follow up, had been achieved. RESULTS: A total of 53 children with the mean age 4.5±1.5 months were subjected to surgery, 32 (60.4 %) were males and 21 (39.6%) were females. The mean gap in the cleft alveolus was 11.1±1.7 mm, which was reduced to a mean of 3.2±1.3 mm, after a follow up of 4.3±1.1 months. The outcome of the lip repair, based on parental satisfaction, was excellent in 41 (77.3%), good in 10 (18.9%) and poor in 2 (3.8%) cases. CONCLUSIONS: Lip adhesion is a safe and effective substitute for pre-surgical orthodontics in wide ULCP.

Evaluation of the efficacy of tongue-lip adhesion in Pierre Robin sequence.

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.

Airway Management in Pierre Robin Sequence: The Vancouver Classification.

BACKGROUND: Pierre Robin sequence (PRS) is a triad of micrognathia, glossoptosis, and respiratory distress. There is no standard clinical classification used in the management of neonatal airway in patients with PRS. The goal of our study was to review the presentation and management of patients with PRS and formulate a clinical grading system and treatment algorithm. METHODS: A 10-year retrospective review of all neonates diagnosed with PRS was performed after obtaining institutional ethics approval. Patients were identified using our cleft lip and palate program database. Inclusion criteria were 2 of the following 3 clinical features-glossoptosis, retrognathia, or airway obstruction. We collected demographic data, clinical information (coexisting airway morbidity, maxillary-mandibular discrepancy, type of intervention used, complications, and outcomes (feeding, length of stay, and airway status) during the first year of life. RESULTS: Sixty-three patients met our inclusion criteria. Of these, 55 (87%) had cleft palate and 17 (27%) were syndromic. Forty-eight (76%) patients were managed by prone positioning. Of the 15 surgically managed patients, the initial procedure was floor of mouth release in 7, mandibular distraction osteogenesis (MDO) in 4, and tongue-lip adhesion in 4. Five patients with coexisting airway morbidity needed a second surgery; 2 had MDO and 3 tracheostomies (one patient was later decannulated). Seven (47%) of the surgically managed patients required a gastrostomy tube. CONCLUSION: At present, there is no consensus on neonatal airway management in infants with PRS. From our review of 63 patients with PRS, we hereby propose a simple 4-point classification system and treatment algorithm, based on clinical features.

HISTORIQUE: Le syndrome de Pierre-Robin (SPR) désigne une triade de micrognathie, de glossoptose et de détresse respiratoire. Aucune classification clinique standard n'est utilisée pour assurer l'ouverture des voies respiratoires chez les nouveau-nés présentant un SPR. La présente étude visait à examiner la présentation et la prise en charge des patients ayant un SPR ainsi qu'à formuler un système de classement clinique et un algorithme de traitement. MÉTHODOLOGIE: Après avoir obtenu l'approbation du comité d'éthique de leur établissement, les chercheurs ont procédé à une analyse rétrospective sur dix ans de tous les nouveau-nés ayant reçu un diagnostic de SPR. Ils ont recensé les patients dans leur base de données de fentes labiales et palatines. Deux des trois caractéristiques cliniques suivantes constituaient les critères d'inclusion : glossoptose, rétrognatie ou obstruction des voies respiratoires. Les chercheurs ont recueilli les données démographiques, l'information clinique (morbidité coexistante des voies respiratoires, malocclusion maxillo-mandibulaire, type d'intervention utilisé, complications et résultats cliniques [alimentation, durée du séjour hospitalier et état des voies respiratoires]) jusqu'à l'âge d'un an. RÉSULTATS: Soixante-trois patients respectaient les critères d'inclusion. De ce nombre, 55 (87 %) avaient une fente palatine et 17 (27 %), un SPR. Quarante-huit patients (76 %) ont été traités par positionnement en décubitus ventral. Des 15 patients opérés, sept ont subi une libération du plancher buccal, quatre, une ostéogenèse par distraction de la mandibule (ODM) et quatre, une adhésion de la langue et de la lèvre. Cinq patients ayant une morbidité concomitante des voies respiratoires ont dû être opérés une deuxième fois. Deux avaient une ODM et trois, une trachéostomie (un a été décannulé par la suite). Sept patients opérés (47 %) ont eu besoin d'une sonde de gastrostomie. CONCLUSION: À l'heure actuelle, il n'y a pas de consensus sur la prise en charge des voies respiratoires chez les nourrissons ayant un SPR. D'après l'analyse de 63 patients ayant ce syndrome, les auteurs proposent un système de classification simple en quatre points et un algorithme de traitement reposant sur les caractéristiques cliniques.

Outcome Following Surgical Interventions for Micrognathia in Infants With Pierre Robin Sequence: A Systematic Review of the Literature.

BACKGROUND: Tongue-lip adhesion (TLA), mandibular distraction osteogenesis (MDO), and subperiosteal release of the floor of the mouth (SPRFM) are the most commonly performed surgical procedures to treat severe airway obstruction in infants born with Pierre Robin sequence (PRS). OBJECTIVES: To determine the rate of failure of each type of procedure, in terms of mortality and the need for tracheostomy, and to determine what proportion of infants have significant airway obstruction postoperatively as determined by polysomnography (PSG) and compare the data by procedure type. METHOD: A comprehensive literature search (1981 through June 2015) was done of the National Library of Medicine database using PubMed. Extracted data included diagnosis, type of surgery, and outcome including mortality, need for postoperative tracheostomy and details of PSG. Persistence of significant airway obstruction was defined as an apnea-hypopnea index >15 events/h on PSG. RESULTS: Both mortality rate and need for tracheostomy were low for all procedures. Many studies lacked sufficient detail to identify significant airway obstruction postoperatively. In studies with sufficient data, MDO was associated with the lowest percentage of significant airway obstruction postprocedure (3.6%) compared to 50% for infants who underwent TLA. Insufficient PSG data was available for patients who were treated with SPRFM. CONCLUSIONS: There is a paucity of objective PSG data to definitively assess postoperative airway outcomes for PRS. MDO appears to be the most effective technique based on the available PSG data. Standardized use of PSG may lead to better identification and treatment of patients at risk for suboptimal airway outcomes postoperatively.

Tongue-lip adhesion in Pierre Robin sequence.

Patients with Pierre Robin sequence exhibit varying degrees of airway obstruction and feeding difficulty. In some patients, airway obstruction may be profound, warranting surgical intervention to maintain a patent airway. The purpose of this article is to highlight the advantages of the tongue-lip adhesion procedure for the management of airway obstruction in such patients compared to the currently available options.

Tongue-lip adhesion in Pierre Robin sequence

Patients with Pierre Robin sequence exhibit varying degrees of airway obstruction and feeding difficulty. In some patients, airway obstruction may be profound, warranting surgical intervention to maintain a patent airway. The purpose of this article is to highlight the advantages of the tongue-lip adhesion procedure for the management of airway obstruction in such patients compared to the currently available options.

Pierre Robin sequence: review of diagnostic and treatment challenges.

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.

A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm.

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.