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Effective intubation technique to manage the airway obstruction in A case of Pierre Robin sequence: A case report.

ABSTRACT: The case was a 4-day-old boy referred to the hospital with a diagnosis of Pierre Robin Sequence and with airway Obstruction. Using a laryngoscope in the hyperextended position and putting pressure on the cricoid, the vocal cords were visualized, and with the help of an assistant, the intubation was performed.

SUBMITTER: Mehrpisheh S

PROVIDER: S-EPMC8905138 | biostudies-literature | 2022 Mar

REPOSITORIES: biostudies-literature

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Effective intubation technique to manage the airway obstruction in A case of Pierre Robin sequence: A case report.

Mehrpisheh Shahrokh S Farhadi Roya R

Clinical case reports 20220308 3

The case was a 4-day-old boy referred to the hospital with a diagnosis of Pierre Robin Sequence and with airway Obstruction. Using a laryngoscope in the hyperextended position and putting pressure on the cricoid, the vocal cords were visualized, and with the help of an assistant, the intubation was performed. ...[more]

PMID: 35280079

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Modified mandible traction with wires to treat neonatal Pierre Robin sequence: A case report.

Project description:BackgroundPierre Robin sequence (PRS) is a congenital craniofacial deformity characterized by micrognathia, glossoptosis and airway obstruction. Some affected neonates are born with severe life-threatening upper airway obstruction that requires surgery. If without timely treatment, it is possible to cause not only organ damage and developmental abnormalities but also early newborn mortality.Case presentationIn this report, a 51-hours-old neonate was diagnosed with PRS, who had severe upper airway obstruction and required surgery. We performed the modified mandible traction with wires at four days old and achieved a satisfactory result in improving airway obstruction. No other complications were observed except for mild local infection. No overlap of other more complex syndromes was found, such as ocular abnormalities, hearing loss, other skeletal abnormalities, cardiac abnormalities or other atypical abnormalities. At the present follow-up until 2 years old, there were no significant differences in the maxillofacial appearance, teeth growth, breathing, feeding, growth and development between the patient and normal children.ConclusionThe modified mandible traction with wires can safely and effectively resolve micrognathia, the key to treating PRS, which is minimally invasive, simple and provides immediate relief of airway obstruction with no long term complications compared with other surgical methods. This report aims to provide more evidence of the successful treatment of neonatal PRS micrognathia by modified mandible traction with wires.

| S-EPMC9632957 | biostudies-literature

Capnography guided awake nasal intubation in a 4 month infant with pierre robin syndrome for cleft lip repair-a better technique.

Project description:This four-month-old Pierre Robin child was admitted for cleft lip repair with history of two failed attempts at intubation and subsequent cancellation of surgery. The capnography guided awake nasal intubation was considered as the child's parents were desperate to get the surgery done. A modified cuffless endotracheal tube was used with a capnography sampling tube placed within it. With the capnograph guidance the expiratory gas flow was followed to successfully intubate the child.This technique was found to be very convenient and helpful. The use of this technique in an infant has not been reported so far.

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BMPR1B mutation causes Pierre Robin sequence.

Project description:BackgroundWe investigated a large family with Pierre Robin sequence (PRS).Aim of the studyThis study aims to determine the genetic cause of PRS.ResultsThe reciprocal translocation t(4;6)(q22;p21) was identified to be segregated with PRS in a three-generation family. Whole-genome sequencing and Sanger sequencing successfully detected breakpoints in the intragenic regions of BMRP1B and GRM4. We hypothesized that PRS in this family was caused by (i) haploinsufficiency for BMPR1B or (ii) a gain of function mechanism mediated by the BMPR1B-GRM4 fusion gene. In an unrelated family, we identified another BMPR1B-splicing mutation that co-segregated with PRS.ConclusionWe detected two BMPR1B mutations in two unrelated PRS families, suggesting that BMPR1B disruption is probably a cause of human PRS.MethodsGTG banding, comparative genomic hybridization, whole-genome sequencing, and Sanger sequencing were performed to identify the gene causing PRS.

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A comprehensive scoring system in correlation with perioperative airway management for neonatal Pierre Robin Sequence.

Project description:PurposeTo evaluate a comprehensive scoring system which combines clinical manifestations of Pierre Robin Sequence (PRS) including severity of breathing difficulties, body weight and preoperative Cormack-Lehane grade, for its correlation with perioperative PRS airway management decision.DesignForty PRS children were retrospectively recruited after surgery. Specialists examined all subjects and scored for clinical manifestations (1´ - 4´), weight gain (1´- 4´), dyspnea scores (1´- 4´), and Cormack-Lehane grade (1´- 4´). The correlation of the integrated scores and the necessity of endotracheal intubation or laryngeal mask application were analyzed. In addition, the score correlation with postoperative dyspnea and/or low pulse oxygen saturation (SPO2) levels after extubation was determined.FindingsIn our study every individual patient had a score from 0´ to 16´, while the higher in the numbers represented higher risk of breathing difficulty. All patients with comprehensive scores <10 points underwent endotracheal intubation successfully. Patients scoring 10-12 points had an intubation success rate of 47%, whereas all patients scored >13 points required a laryngeal mask assisted airway management and were considered to have difficult airways. Dyspnea after extubation and postoperative low SPO2 occurred among patients who scored over 10 points.ConclusionIn PRS patients, preoperative weight gaining status and severity of dyspnea in combination with Cormack-Lehane classification provide a scoring system that could help to optimize airway management decisions such as endotracheal intubation or laryngeal mask airway placement and has the potential to predict postoperative dyspnea or low SPO2 levels.

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Pierre robin sequence and the pediatric dentist.

Project description:This article on the dental management of a neonate with Pierre Robin sequence describes the clinical and laboratory procedures for construction of a feeding plate due to the presence of a cleft palate. Emphasis has also been laid on a few literatures to describe medical complications associated with this condition. A 56-day-old neonate had been referred to the outpatient department with the complaint of difficulty in feeding, description, and management of which has been described in the case report.

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Mixed infected laryngocoele presenting as airway obstruction: a case report.

Project description:Laryngocoele is a rare entity, defined as an abnormal cystic dilatation of saccule of the laryngeal ventricle. Three types of laryngocele have been described, based on their relation to the thyrohyoid membrane: internal, external or mixed type. Symptoms are variable, including neck swelling, shortness of breath, dysphonia and fever, if the laryngocoele becomes infected. Patients may also present in extremis with airway obstruction. We present the case of a healthy 34-year-old gentleman with acute airway obstruction due to a mixed infected laryngocoele. Flexible nasoendoscopy showed a large cystic swelling arising from the laryngeal ventricle. Computed tomography of neck confirmed a right paraglottic collection extending into the ventricle and glottis, causing significant airway compromise. The patient was managed with microlaryngoscopy and cystic decompression. At outpatient follow up, he was completely asymptomatic and is currently under surveillance. Endoscopic decompression is a safe and effective initial management for mixed laryngocoele.

| S-EPMC7937032 | biostudies-literature

Tracheal intubation in patients with Pierre Robin sequence: development, application, and clinical value based on a 3-dimensional printed simulator.

Project description:Background: The main clinical manifestations of patients with Pierre Robin sequence (PRS) include micrognathia, the glossoptosis and dyspnoea. The difficulty of tracheal intubation (TI) in such patients is increased. Objective: The purpose of the study was to evaluate the reliability and efficacy of the PRS simulator. Methods: A PRS simulator was developed by using 3-dimensional (3D) printing technology under computer-aided design. A total of 12 anaesthesiologists each trained 5 times for TI on the PRS Training Simulator-1 and recorded the simulation time. After the training, they were randomly divided into three groups with a total of 12 nontrained anaesthesiologists, and the simulation was completed on PRS Simulator-2, 3 and 4. The simulation time was recorded, and the performance was evaluated by three chief anaesthesiologists. Then, all 24 anaesthesiologists completed the questionnaire. Results: A PRS simulator developed by 3D printing was used to simulate the important aspects of TI. The average number of years worked was 6.3 ± 3.1 years, and 66.7% were female. The time for the 12 anaesthesiologists to complete the training gradually decreased (p < 0.01). Compared with the trained anaesthesiologists, the simulation time of TI in the nontrained anaesthesiologists was much longer (all p < 0.01). In addition, the simulation performance of the trained anaesthesiologists was relatively better (all p < 0.01). Conclusion: The reliability and efficacy of the PRS simulator is herein preliminarily validated, and it has potential to become a teaching and training tool for anaesthesiologists.

| S-EPMC10875733 | biostudies-literature

Video-Assisted Intubating Stylet Technique for Difficult Intubation: A Case Series Report.

Project description:Induction of anesthesia can be challenging for patients with difficult airways and head or neck tumors. Factors that could complicate airway management include poor dentition, limited mouth opening, restricted neck motility, narrowing of oral airway space, restricted laryngeal and pharyngeal space, and obstruction of glottic regions from the tumor. Current difficult airway management guidelines include awake tracheal intubation, anesthetized tracheal intubation, or combined awake and anesthetized intubation. Video laryngoscopy is often chosen over direct laryngoscopy in patients with difficult airways because of an improved laryngeal view, higher frequency of successful intubations, higher frequency of first-attempt intubation, and fewer intubation attempts. In this case series report, we describe the video-assisted intubating stylet technique in five patients with difficult airways. We believe that the intubating stylet is a feasible and safe airway technique for anesthetized tracheal intubation in patients with an anticipated difficult airway.

| S-EPMC9027904 | biostudies-literature

Congenital high airway obstruction with tracheoesophageal fistula: A case report.

Project description:RATIONALE:Congenital high airway obstruction syndrome (CHAOS) is defined as complete or partial obstruction of the fetal upper airways. Laryngeal atresia is the most frequent cause. PATIENT CONCERN:A male neonate born with poor reactions, weakly spontaneous breathing and cyanosis of the limbs was referred to our hospital. DIAGNOSIS:CHAOS with tracheoesophageal fistula. INTERVENTION:A tracheostomy was performed and a 3.0-mm internal diameter tracheostomy tube was inserted. OUTCOMES:Neonatal survival depended on our immediate postnatal intervention. LESSONS:In summary, a multidisciplinary team including otolaryngologists, radiologists, obstetricians, and anesthesiologists must be present during the whole diagnosis and treatment process.

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