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Pierre Robin Sequence

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https://www.readbyqxmd.com/read/29447809/difficult-airway-intubation-simulation-using-bonfils-fiberscope-and-rigid-fiberscope-for-surgical-training
#1
Harish Dharmarajan, Yi-Chun Carol Liu, Helena Karlberg Hippard, Binoy Chandy
INTRODUCTION: Pediatric otolaryngologists are frequently called to assist in difficult airway management in newborns with Pierre Robin Sequence (PRS) who have microretrognathia, glossoptosis, and an anterior larynx. The Bonfils fiberscope (BF) is a curved rigid scope designed to provide superior visualization in the anterior larynx. OBJECTIVE: (1) to assess whether BF provides an improvement in intubation success rate, time to intubation, or airway visualization as compared to rigid fiberscope (RF) in a difficult airway simulation setting and (2) to determine whether a training program for BF can improve time to intubation through practice trials...
February 2018: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/29446988/diagnosing-tongue-base-obstruction-in-pierre-robin-sequence-infants-sleep-vs-awake-endoscopy
#2
Jake J Lee, Matthew D Ford, Allison B Tobey, Noel Jabbour
OBJECTIVE: To investigate whether awake endoscopy can diagnose base-of-tongue obstruction as reliably as sleep endoscopy in infants with Pierre Robin sequence (PRS). DESIGN: The study was retrospective with the clinicians blinded to patient identity. Endoscopy findings were assessed and measured by the performing pediatric otolaryngologist. SETTING: Tertiary care children's hospital. PATIENTS: All infants with PRS managed between January 2005 and July 2015 were included...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29317143/predictors-of-speech-outcomes-in-children-with-pierre-robin-sequence
#3
Anne Morice, Francis Renault, Véronique Soupre, Cécile Chapuis, Chantal Trichet Zbinden, Natacha Kadlub, Amerigo Giudice, Marie-Paule Vazquez, Arnaud Picard
BACKGOUND: Pierre Robin sequence (PRS) has worse speech outcomes than isolated cleft palate. We aimed to search for possible associations of phonological outcomes with PRS status (isolated vs syndromic), clinical severity, soft palate muscles deficiency, or surgical procedure. METHODS: We designed a retrospective study of 130 children (male/female ratio: 0.4) with isolated (96) or syndromic (34) PRS with cleft palate. Grading systems were used to classify retrognathia, glossoptosis, and respiratory and feeding disorders...
December 13, 2017: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/29222685/prediction-of-outcome-using-pretreatment-18f-fdg-pet-ct-and-mri-radiomics-in-locally-advanced-cervical-cancer-treated-with-chemoradiotherapy
#4
François Lucia, Dimitris Visvikis, Marie-Charlotte Desseroit, Omar Miranda, Jean-Pierre Malhaire, Philippe Robin, Olivier Pradier, Mathieu Hatt, Ulrike Schick
PURPOSE: The aim of this study is to determine if radiomics features from 18fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) and magnetic resonance imaging (MRI) images could contribute to prognoses in cervical cancer. METHODS: One hundred and two patients (69 for training and 33 for testing) with locally advanced cervical cancer (LACC) receiving chemoradiotherapy (CRT) from 08/2010 to 12/2016 were enrolled in this study. 18F-FDG PET/CT and MRI examination [T1, T2, T1C, diffusion-weighted imaging (DWI)] were performed for each patient before CRT...
December 9, 2017: European Journal of Nuclear Medicine and Molecular Imaging
https://www.readbyqxmd.com/read/29216230/a-comprehensive-scoring-system-in-correlation-with-perioperative-airway-management-for-neonatal-pierre-robin-sequence
#5
Ning Yin, Lei Fang, Xiaohua Shi, Hongqiang Huang, Li Zhang
PURPOSE: To 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. DESIGN: Forty 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´)...
2017: PloS One
https://www.readbyqxmd.com/read/29215441/pierre-robin-sequence-an-evidence-based-treatment-proposal
#6
Oswaldo J Gómez, Oscar I Barón, Martha L Peñarredonda
BACKGROUND: The Pierre Robin sequence (PRS) has been defined as the presence of micrognathia, glossoptosis, and respiratory obstruction in the neonatal period. Since its original description, different therapeutic approaches have been proposed obtaining different success rates, but there is no consensus about its management. METHODS: A literature review was conducted in PubMed, Embase, and Cochrane databases, for the period of January,1985 to November, 2016. A number of 23 articles resulting from clinical studies, discussing diagnostic tests or therapeutic approaches, and directly or indirectly comparing diagnostic or treatment modalities were selected and assessed using the GRADE methodology...
December 6, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29210721/technique-utilizing-a-modified-oral-ring-adair-elwyn-tube-to-provide-continuous-oxygen-and-sevoflurane-delivery-during-nasotracheal-intubation-in-an-infant-with-a-difficult-airway-a-case-report
#7
Janice Y Man, John E Fiadjoe, Grace Hsu
Managing the airway of an infant with Pierre Robin sequence (PRS) is particularly challenging for anesthesiologists. Patients with PRS have the triad of micrognathia, glossoptosis, and airway obstruction that potentially and frequently leads to difficulty with both ventilation and intubation. Thus continuous oxygenation and spontaneous ventilation during intubation are essential. We describe a new method to deliver continuous oxygen and volatile anesthetic during nasotracheal intubation in an infant with PRS...
November 27, 2017: A & A Case Reports
https://www.readbyqxmd.com/read/29195837/evaluation-of-the-efficacy-of-tongue-lip-adhesion-in-pierre-robin-sequence
#8
H Broucqsault, M-D Lamblin, G Hosana, P Fayoux
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...
November 28, 2017: European Annals of Otorhinolaryngology, Head and Neck Diseases
https://www.readbyqxmd.com/read/29159987/med13l-loss-of-function-variants-in-two-patients-with-syndromic-pierre-robin-sequence
#9
Christopher T Gordon, Maya Chopra, Myriam Oufadem, Olivier Alibeu, Marc Bras, Nathalie Boddaert, Christine Bole-Feysot, Patrick Nitschké, Véronique Abadie, Stanislas Lyonnet, Jeanne Amiel
We report two unrelated patients with Pierre Robin sequence (PRS) and a strikingly similar combination of associated features. Whole exome sequencing was performed for both patients. No single gene containing likely pathogenic point mutations in both patients could be identified, but the finding of an essential splice site mutation in mediator complex subunit 13 like (MED13L) in one patient prompted the investigation of copy number variants in MED13L in the other, leading to the identification of an intragenic deletion...
November 21, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29155841/habitat-and-soil-related-drivers-of-the-root-associated-fungal-community-of-quercus-suber-in-the-northern-moroccan-forest
#10
Fatima Zahra Maghnia, Younes Abbas, Frédéric Mahé, Benaissa Kerdouh, Estelle Tournier, Mohamed Ouadji, Pierre Tisseyre, Yves Prin, Naïma El Ghachtouli, Salah Eddine Bakkali Yakhlef, Robin Duponnois, Hervé Sanguin
Soil fungi associated with plant roots, notably ectomycorrhizal (EcM) fungi, are central in above- and below-ground interactions in Mediterranean forests. They are a key component in soil nutrient cycling and plant productivity. Yet, major disturbances of Mediterranean forests, particularly in the Southern Mediterranean basin, are observed due to the greater human pressures and climate changes. These disturbances highly impact forest cover, soil properties and consequently the root-associated fungal communities...
2017: PloS One
https://www.readbyqxmd.com/read/29084117/a-review-of-randomized-controlled-trials-in-cleft-and-craniofacial-surgery
#11
Jonathan M Bekisz, Elise Fryml, Roberto L Flores
This study presents a systematic review of randomized controlled trials (RCTs) in cleft and craniofacial surgery. All studies reporting on RCTs in cleft and craniofacial surgery were identified on PubMed using the search terms "cleft," "velopharyngeal insufficiency," "velopharyngeal dysfunction," "nasoalveolar molding," "gingivoperiosteoplasty," "Pierre Robin sequence," "craniofacial," "craniosynostosis," "craniofacial microsomia," "hemifacial microsomia," "hypertelorism," "Le Fort," "monobloc," "distraction osteogenesis," "Treacher Collins," and "Goldenhar...
October 27, 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29069271/intensive-treatment-of-speech-disorders-in-robin-sequence-a-case-report
#12
Maria Daniela Borro Pinto, Maria Inês Pegoraro-Krook, Laura Katarine Félix de Andrade, Ana Paula Carvalho Correa, Linda Iris Rosa-Lugo, Jeniffer de Cássia Rillo Dutka
PURPOSE: To describe the speech of a patient with Pierre Robin Sequence (PRS) and severe speech disorders before and after participating in an Intensive Speech Therapy Program (ISTP). METHODS: The ISTP consisted of two daily sessions of therapy over a 36-week period, resulting in a total of 360 therapy sessions. The sessions included the phases of establishment, generalization, and maintenance. A combination of strategies, such as modified contrast therapy and speech sound perception training, were used to elicit adequate place of articulation...
October 23, 2017: CoDAS
https://www.readbyqxmd.com/read/29026807/airway-management-in-pierre-robin-sequence-the-vancouver-classification
#13
Wai-Yee Li, Alana Poon, Douglas Courtemanche, Cynthia Verchere, Sandra Robertson, Marija Bucevska, Claudia Malic, Jugpal S Arneja
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...
February 2017: Plastic Surgery
https://www.readbyqxmd.com/read/28950416/efficiency-of-prenatal-diagnosis-in-pierre-robin-sequence
#14
Elvira Di Pasquo, Jeanne Amiel, Philippe Roth, Valérie Malan, Katia Lind, Christel Chalouhi, Véronique Soupre, Christopher T Gordon, Stanislas Lyonnet, Laurent J Salomon, Véronique Abadie
OBJECTIVES: To analyze the efficiency of prenatal diagnosis of Pierre Robin sequence (PRS) regarding the final specific diagnosis and to determine whether infants have more severe respiratory disorders with than without prenatally suspected PRS. METHODS: Review of the outcome of all prenatal cases of suspected PRS managed in our prenatal diagnosis center during the last 15 years; analysis of the consistency between prenatal and postnatal diagnoses in 2 groups of women with and without a family history of PRS; comparison of the grades of disease severity for infants with and without prenatally suspected PRS...
November 2017: Prenatal Diagnosis
https://www.readbyqxmd.com/read/28938331/postoperative-respiratory-complications-after-cleft-palate-closure-in-patients-with-pierre-robin-sequence-operative-considerations
#15
Yasmin Opdenakker, Gwen Swennen, Lies Pottel, Johan Abeloos, Krisztián Nagy
BACKGROUND: In cleft palate surgery, there is currently no consensus on the management of patients with Pierre Robin Sequence (PRS). The authors aimed to evaluate the treatment strategy of cleft palate in our centers, with emphasis on patients with PRS, as the authors noted some patients with severe respiratory distress. Moreover, the authors aimed to investigate the prevalence of postoperative respiratory complications, using a modified-Furlow palatoplasty in combination with intravelar veloplasty in both patients with PRS and patients with non-PRS...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28938321/the-pierre-robin-mandible-is-hypoplastic-and-morphologically-abnormal
#16
Elizabeth G Zellner, Russell R Reid, Derek M Steinbacher
BACKGROUND: For Pierre Robin sequence (PRS) patients, there is incomplete characterization of 3D differences and effects of mandibular distraction osteogenesis (MDO) on the mandible compared to normal controls. METHODS: PRS infants who underwent MDO at 2 craniofacial referral centerals with pre- and postoperative computed tomography (CT) scans were identified. A group of age-matched control patients with CTs were identified in the PACS database. Demographic and perioperative data were recorded...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28938318/neonatal-mandibular-distraction-without-a-consolidation-period-is-it-safe-is-it-effective
#17
Nataliya Biskup, Ashley L Altman, Christopher M Runyan, Craig Gendron, Haithem Elhadi Babiker, Christopher B Gordon, Brian S Pan
PURPOSE: Consolidation is a standard part of most post-distraction protocols. This study aims to determine whether the deliberate omission of a consolidation period following mandibular distraction (MD) in neonates with Pierre Robin sequence (PRS) affected the airway outcomes and complication rates. PATIENTS AND METHODS: A retrospective chart review of 28 neonates with PRS who underwent MD for severe airway obstruction between 2009 and 2014 was performed. Neonates were split into 2 cohorts: those with a very short or no consolidation (no consolidation group, n = 18) and those with a traditional consolidation period (consolidation group, n = 10)...
November 2017: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/28826759/condylar-positions-before-and-after-bilateral-mandibular-distraction-osteogenesis-in-children-with-pierre-robin-sequence
#18
J Liu, Y Chen, F Li, W Wu, J Hao, D Luo, H Wang
This study aimed to analyze the changes in mandibular morphology after bilateral mandibular distraction osteogenesis (BMDO) in children with Pierre Robin sequence. The positions of the condyles were analyzed in reconstructed three-dimensional craniomaxillofacial images obtained for 18 children before and 8-12 weeks after BMDO. Differences between pre- and postoperative parameters were assessed using paired t-tests. After surgery, a significant decrease in superior joint space was detected (P<0.05), but no change in anterior joint space or posterior joint space was observed...
August 18, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28822675/precise-osteotomies-for-mandibular-distraction-in-infants-with-robin-sequence-using-virtual-surgical-planning
#19
C M Resnick
Mandibular distraction osteogenesis (MDO) has become the first-line operation in many centers for the management of obstructive sleep apnea (OSA) in infants with (Pierre) Robin sequence (RS) not relieved by non-surgical approaches. Preoperative virtual surgical planning (VSP) may improve precision and decrease complications for this operation. This article reports a retrospective study of RS infants who underwent MDO for OSA using preoperative VSP and three-dimensionally printed cutting guides performed by one surgeon...
August 16, 2017: International Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28802380/can-telemetry-data-obviate-the-need-for-sleep-studies-in-pierre-robin-sequence
#20
Nicole Leigh Aaronson, Noel Jabbour
OBJECTIVE: This study looks to correlate telemetry data gathered on patients with Pierre Robin Sequence (PRS) with sleep study data. Strong correlation might allow obstructive sleep apnea (OSA) to be reasonably predicted without the need for sleep study. METHODS: Charts from forty-six infants with PRS who presented to our children's hospital between 2005 and 2015 and received a polysomnogram (PSG) prior to surgical intervention were retrospectively reviewed. Correlations and scatterplots were used to compare average daily oxygen nadir, overall oxygen nadir, and average number of daily desaturations from telemetry data with apnea-hypopnea index (AHI) and oxygen nadir on sleep study...
September 2017: International Journal of Pediatric Otorhinolaryngology
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