pierre robin syndrome intubation

Stickler syndrome (SS) is a congenital autosomal dominant condition that affects the formation of collagen. Of primary importance to the anesthesia provider is the propensity for difficulties in managing the airway due to orofacial abnormalities associated with this syndrome. The authors present a two-year-old infant with SS who required anesthetic care for a cleft palate repair. The potential anesthetic implications of this syndrome are discussed and the importance of proper planning and preparation and the usefulness of ultrasound as an airway evaluation tool are highlighted...

BACKGROUND: Tracheostomy is the definitive treatment for airway management in severe cases of craniofacial-associated upper airway obstruction, like the Pierre-Robin sequence, but is associated with significant morbidity. The purpose of this study was to examine tracheostomy-associated morbidities and mortalities in craniofacial patients to identify opportunities to improve clinical care and patient prognosis. METHODS: The study was a retrospective review of pediatric craniofacial patients who were tracheostomized between 2016 and 2022...

KEY CLINICAL MESSAGE: Femoral hypoplasia-unusual facies syndrome is a rare condition of unknown etiology. The phenotype consists of significant femoral hypoplasia with characteristic facial malformations that often overlap with findings seen in patients with Pierre Robin sequence. Anesthesia providers must prepare for difficult intravenous access, difficult airway management, and uncertainties with regional anesthesia. ABSTRACT: Femoral hypoplasia-unusual facies syndrome (FHUFS) or femoral facial syndrome is a rare and sporadic condition of unknown etiology...

Pierre Robin syndrome (PRS) neonates are one of the most difficult cases to intubate even for an experienced paediatric anaesthesiologist. We describe a case of a PRS-related anatomical anomaly that hindered attempts to manage the airway and the final approach that made it possible to insert an endotracheal tube (ETT). We describe the novel use of a video ureteroscope (Olympus URF-V2) as an airway endoscope. A 7-day-old, 2-kg boy was referred to our tertiary care hospital with diagnosed PRS. He was planned for correction of the mandible with mandibular distraction osteogenesis under general anaesthesia...

INTRODUCTION: As part of surgical interventions in pediatric patients, children with craniofacial malformations and syndromes are presented in the field of oral and maxillofacial surgery, anesthesia and also in all other clinical disciplines. In particular, the Pierre Robin sequence in the clinical context leads to a situation albeit a rare one, which should be given high attention in preoperative, intraoperative and postoperative care. MATERIAL AND METHODS: In a retrospective analysis from 1993 to 2020 in the Department of Oral and Maxillofacial Surgery at the University Hospital Halle (Saale), a total of 54 patients were identified with syndromic changes and a need for surgical treatment...

Our objective was to compare video-assisted laryngoscopy (VAL) with direct laryngoscopy (DL) for glottic visualization in a pediatric intensive care unit in terms of the success rate in first attempts. Our study included patients aged from 1 month to 18 years who were admitted to the pediatric intensive care unit. We excluded patients with limited neck extension (C-spine immobilization, congenital abnormality), congenital anomalies (e.g., Pierre Robin syndrome, micrognathia, macroglossia), and recent airway surgery...

OBJECTIVE: To study the role of upper airway endoscopy (UAE) in craniofacial malformations in all different management approaches described in the literature. METHODS: A narrative review was performed, based on a search in pubmed. RESULTS: UAE use was reviewed over five domains: 1) Diagnosis of glossoptosis and endoscopic classification: drug-induced sleep endoscopy is recommended to graduate tongue base obstruction; there is still no consensus on the graduation system to be used...

Pierre Robin Sequence (PRS) is a congenital condition characterized by micrognathia, glossoptosis, and cleft palate that presents with airway obstruction and developmental delay with or without other congenital anomalies. These patients' anesthesia management is challenging because of difficult ventilation and intubation. Regional anesthesia methods should be considered for these patients on a case-by-case basis. This report presents primary use of regional anesthesia for circumcision of a 9-year-old boy with PRS...

BACKGROUND: Video laryngoscopy is an effective tool in the management of difficult pediatric airway. However, evidence to guide the choice of the most appropriate video laryngoscope (VL) for airway management in pediatric patients with Pierre Robin syndrome (PRS) is insufficient. Therefore, the aim of this study was to compare the efficacy of the Glidescope® Core™ with a hyperangulated blade, the C-MAC® with a nonangulated Miller blade (C-MAC® Miller) and a conventional Miller laryngoscope when used by anesthetists with limited and extensive experience in simulated Pierre Robin sequence...

Airway management in children with craniofacial anomalies can be complicated and may require multiple attempts with conventional direct laryngoscopy (DL). Videolaryngoscopes (VLs) have a well-established role in difficult airway management in adults; however, their role remains to be fully elucidated in paediatric age group. There is a relative paucity in the literature regarding the role of VLs in cases of syndromic children, and it is not clear whether they should be used as an initial option or as a rescue device...

BACKGROUND: The severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) outbreak is spreading worldwide. To date, no specific treatment has convincingly demonstrated its efficacy. Hydroxychloroquine and lopinavir/ritonavir have potential interest, but virological and clinical data are scarce, especially in critically ill patients. METHODS: The present report took the opportunity of compassionate use and successive drug shortages to compare the effects of two therapeutic options, lopinavir/ritonavir and hydroxychloroquine, as compared to standard of care only...

OBJECTIVE: To determine weight gain during treatment with the modified palatal plate (MPP) in infants with isolated and syndromic Pierre Robin Sequence (PRS) suffering from micrognathia, upper airway obstruction (UAO), and failure to thrive (FTT), the authors conducted a retrospective study of infants treated with the MPP. METHODS: The main outcome measure was infant weight (g) for up to three months after birth. Demographic and outcome data (associated syndromes, comorbidities, presence of cleft lip or palate, intubation attempts, tracheotomy and cleft repair) were collected...

BACKGROUND: Difficult tracheal intubation is a common problem encountered by anesthesiologists in the clinic. This study was conducted to assess the difficulty of tracheal intubation in infants with Pierre Robin syndrome (PRS) by incorporating computed tomography (CT) to guide airway management for anesthesia. METHODS: In this retrospective study, we analyzed case-level clinical data and CT images of 96 infants with PRS. First, a clinically experienced physician labeled CT images, after which the color space conversion, binarization, contour acquisition, and area calculation processing were performed on the annotated files...

BACKGROUND: The purpose of this study was to evaluate the association between epidemiological, clinical and radiographic factors of patients with tibial shaft fractures and the occurrence of acute compartment syndrome. METHODS: 270 consecutive adult patients sustaining 273 tibial shaft fractures between January 2005 and December 2009 were included in this retrospective cohort study. The outcome measure was acute compartment syndrome. Patient-related (age, sex), fracture-related (high- vs...

Objective: To discuss the diagnosis and evaluation of upper airway obstruction in neonates, classify the possible causes of neonatal upper airway obstruction, establish a standardized diagnosis and treatment procedure to improve the of treatment efficacy. Method: Clinical data of 71 cases with upper airway obstruction history were retrospective analyzed, 38 cases were treated by combined medical and surgical treatment, 17 cases underwent medical treatment, 16 cases abandonment the treatment. The effectiveness of fibrolaryngoscope, CT, neck ultrasound, MRI and other results was evaluated, and to analyze the prognosis of the treatment, the outcomes and risk factors were also analysed, to evaluate risk factors associated with upper airway obstruction...

Pierre Robin sequence is a triad of micrognathia, glossoptosis, and airway obstruction that has reported associations with numerous birth defects and anomalies including craniocervical pathologies. Given the number of airway assessments and neck manipulations these neonates experience, undetected cervical instability can result in detrimental consequences. The authors present a case of a neonate with non-syndromic Pierre Robin sequence planned for mandibular distraction osteogenesis that developed abnormal motor activity following intubation...

Pierre Robin sequence is defined by a triplet of clinical signs in newborns: micrognathia, glossoptosis and tongue-based airway obstruction often accompanied by U-shaped cleft palate. The reported incidence is ranging from 1 to 8.500 to 30.000 newborns. Therapeutic management of Pierre Robin sequence is based on the degree of the airway obstruction. A priori management of such cases can be extremely challenging due to the phenotypic plethora of Pierre Robin Sequence. A ten-day male newborn diagnosed with Pierre Robin was referred to our department for investigation and management of severe airway obstruction...

PURPOSE: High-flow nasal cannula (HFNC) therapy is increasingly proposed as first-line respiratory support for infants with acute viral bronchiolitis (AVB). Most teams use 2 L/kg/min, but no study compared different flow rates in this setting. We hypothesized that 3 L/kg/min would be more efficient for the initial management of these patients. METHODS: A randomized controlled trial was performed in 16 pediatric intensive care units (PICUs) to compare these two flow rates in infants up to 6 months old with moderate to severe AVB and treated with HFNC...

PURPOSE: Mandibular distraction osteogenesis (MDO) has been shown to be successful in treating upper airway obstruction caused by micrognathia in pediatric patients. The purpose of this study was to assess the success rate of MDO and possible predictors of failure. PATIENTS AND METHODS: The records of all neonates and infants who underwent MDO from 2008 to 2015 were retrospectively reviewed. Procedural failure was defined as patient death or the need for tracheostomy postoperatively...

OBJECTIVE: This study aimed to identify risk factors for adverse perioperative events (APEs) after cleft palatoplasty to develop an individualized risk assessment tool. DESIGN: Retrospective cohort. SETTING: Tertiary institutional. PATIENTS: Patients younger than 2 years with cleft palate. INTERVENTIONS: Primary Furlow palatoplasty between 2008 and 2011. MAIN OUTCOME MEASURE(S): Adverse perioperative event, defined as laryngo- or bronchospasm, accidental extubation, reintubation, obstruction, hypoxia, or unplanned intensive care unit admission...