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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. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence poses a great challenge for anesthesiologists during laryngoscopy and intubation, making oxygenation and ventilation difficult. The role of early surgical intervention is recommended for the improvement of the airway and overall survival of the neonate. The situation becomes even more challenging, when the neonate may not be fit for such surgical interventions. The present case posed such a challenge to the team. To the authors' knowledge, the decision to use a face mask as an interim life-saving measure was considered for the first time. This provided a greater window of opportunity for further course of action, only to be later managed by distraction osteogenesis of the mandible. The unconventional use of orthopedic appliances for the management of threatened airways may provide the clinician with time, where further management may be carried out. The present article will explain such a procedure that was carried out as a life-saving measure. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence (PRS) with bilateral temporomandibular joint (TMJ) ankylosis is a rare and challenging case for anaesthesiologists. A 6-year-old girl with PRS along with bilateral progressive TMJ ankylosis was scheduled for gap arthroplasty. Her mouth opening was <1 finger. Securing the airway in a syndromic child with mandibular hypoplasia was challenging. We performed an asleep fibreoptic bronchoscope (FOB)-guided nasotracheal intubation while retaining spontaneous breathing. Managing a difficult paediatric airway needs expertise. We believe that with the use of FOB, difficult airways can be successfully and safely managed. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Pierre Robin sequence (PRS) is characterized by the triad of micrognathia, glossoptosis, and upper airway obstruction. It is commonly associated with the secondary cleft palate. Infants with PRS commonly have sleep-disordered breathing (SDB); including obstructive sleep apnea (OSA) as well as central sleep breathing abnormalities that are present from infancy. AIM OF THE STUDY: Evaluate the prevalence and severity of SDB in infants with PRS using polysomnography (PSG). SETTINGS AND DESIGN: We retrospectively reviewed the sleep laboratory database at The Hospital for Sick Children, Toronto, during the period of May 2007 to March 2016. STATISTICAL ANALYSIS: Comparisons of PSG data were made between the OSA and non-OSA group using the Student's t-test for age and body mass index, Wilcoxon signed ranks test for the continuous PSG data and Chi-squared test for the categorical variables. METHODS: Patients with PRS were identified and their initial PSG was selected for this study. The main indication for referral was ongoing concerns regarding OSA symptoms. RESULTS: A total of 46 patients (28 females) were included with a mean age (±standard deviation) of 0.8 (±0.3) year. Twenty-two out of 46 (47%) had evidence of OSA of which 10 had mild, 3 had moderate, and 9 had severe OSA. The PRS infants with OSA were younger than the non-OSA group. Significant correlations were found between desaturation and arousal indices with obstructive apnea–hypopnea index. CONCLUSION: This retrospective chart review confirms a high prevalence of OSA in this population. Prospective longitudinal studies are needed to evaluate the outcomes of OSA in PRS population. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence (PRS) is characterized by the triad micrognathia, glossoptosis, and airway obstruction, commonly associated with cleft palate. This study reports the case of a patient with characteristics consistent with the diagnosis of PRS in the 1st week of life. To stabilize the airway, oro-tracheal intubation was performed without success, followed by tracheostomy and glossopexy and after a small improvement in symptoms, it was decided to perform the mandibular distraction osteogenesis (MDO). At the age of 6, her maxillofacial development was within expectations, with patent airway, the presence of deciduous teeth, and without neurological impairment. The MDO described in this case allowed patient's respiratory and feeding improvement, thus avoiding episodes of cyanosis, allowing timely removal of the tracheostomy and glossopexy, and execution of palatoplasty at the right time. While it is uncertain whether MDO will replace the possible need for future orthognathic surgery, treatment greatly improved the quality of life of the patient. [ABSTRACT FROM AUTHOR]

Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch‑up growth, midfacial hyperplasia, and early management. Aims of Part 4: To provide a systematic treatment protocol for Fairbairn–Robin triad (FRT) and Siebold Robin sequence (SRS) patients based on clinical findings and experience with 266 PRS cases. Subjects and Methods: A plethora of treatment modalities and their outcome in literature have been compared to those applied in this database and their outcomes. Results: The management of SRS/FRT depends on various factors including compromised airways, feeding difficulties, as well as the sequence of the reconstructive ladder. Conclusion: Based on the novel PRS subdivisions, a stepwise sequential treatment approach is outlined, addressing the particular needs of each disorder systematically. [ABSTRACT FROM AUTHOR]

Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch‑up growth, midfacial hyperplasia, and the early management. Aim: The aims of Part 3 debate the controversial biological theories relating to PRS. Materials and Methods: Oligo‑/poly‑hydramnios, mandibular catch‑up growth, and midfacial hyperplasia, the three in the literature most prevailing theories related to PRS, have been compared and discussed with the findings provided by this large database of 266 Siebold‑Robin sequence (SRS) and Fairbairn‑Robin triad (FRT) cases. Results: History and clinical findings evaluated in this database refute the first two theories. Although manifold midfacial appearances were demonstrated in FRT cases, a third of all SRS cases presented with mid‑facial hyperplasia. Conclusion: The three main biological theories regarding PRS could not be verified after thorough analysis of the database. [ABSTRACT FROM AUTHOR]

Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Therefore, clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in scientific literature related to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch‑up growth, midfacial hyperplasia, and the timing of management. Aims of Part 1: Display disparities of the widely published subject of PRS that exist within the literature. Subjects and Methods: A literature search related to diagnostic criteria was compared to findings of one of the largest PRS databases worldwide. Results: Regarding diagnostic criteria two subdivisions, the Fairbairn–Robin triad (FRT) and the Siebold–Robin sequence (SRS) can be clearly distinguished. Both present with micrognathia and glossoptosis, the former with, the latter, however, without a palatal cleft. Conclusions: According to clear diagnostic criteria, PRS has to be subdivided in the future into FRT and SRS cases, as they may require different treatment approaches. [ABSTRACT FROM AUTHOR]

Pierre Robin Sequence is a congenital condition involving a combination of micrognathia and glossoptosis with or without a cleft palate. Feeding problems are often associated with cleft anamoly which make it difficult for the infant to maintain adequate nutrition. Here we present a case of 58-day-old neonate with Pierre Robin sequence, in whom we contructed a palatal obturtor for feeding. [ABSTRACT FROM AUTHOR]

Pierre Robin sequence (PRS) or anomalad, a well-recognized presentation, is the association of the first brachial arch malformation. It presents with a classic triad of micrognathia, glossoptosis, and cleft palate. In a neonate with a complete cleft palate, problems with feeding are commonly encountered. Presented here are two cases with PRS in whom palatal obturators were constructed. [ABSTRACT FROM AUTHOR]

Context: The disorder currently accepted as Pierre Robin syndrome/anomaly/sequence (PRS) has been plagued by controversy ever since initially being described. Controversy exists not only about the appropriate terminology and etiopathogenesis of the disorder but also about its management. Clinical findings and treatment outcomes of a large database of 266 PRS cases were compared with the current state of knowledge in the scientific literature, relating to history, clinical description, diagnostic criteria, epidemiology, theories of oligohydramnios, mandibular catch‑up growth, midfacial hyperplasia, and the early management. Aims of Part 2: Contribute to the sparse scientific knowledge about pathogenesis and involved genetics. Subjects and Methods: An analysis of this large database was conducted focusing on genetic involvement, family history, and the incidence of additional syndromes. Results: Beside of differences related to clinical signs of dyspnea, feeding problems and mortality rates, various concomitant syndromes, and genetic abnormalities were found in cases of Fairbairn–Robin triad (FRT) and Siebold–Robin sequence (SRS), in addition to differences in relation to clinical signs of dyspnea, feeding problems, and mortality rates. Conclusion: Multiple FRT cases presented with various concomitant syndromes and genetic abnormalities, but only one type occurred in two SRS cases. The latter presented a significantly different mortality rate when compared to the FRT subgroup. [ABSTRACT FROM AUTHOR]

Pierre Robin syndrome or Pierre Robin sequence (PRS) is a congenital etiologically heterogeneous condition presenting with various malformations. Here we are reporting the surgical management of an 18-month-old female baby who was referred from Department of Pediatrics with a complaint of a large cleft palate. She was taken up for palatoplasty with consent for elective tracheostomy. After genetic evaluation, the authors conclude that the presented case was a PRS in isolation with mild cardiac anomalies and an inferiorly placed hypoplastic epiglottis. Patient should be followed up and growth modifications of the jaws should be done. [ABSTRACT FROM AUTHOR]