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Pierre Robin Sequence

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https://www.readbyqxmd.com/read/29905603/treacher-collins-syndrome-and-tracheostomy-decannulation-using-mandibular-distraction-osteogenesis
#1
Safi Ali-Khan, Christopher Runyan, Gil Nardini, Pradip Shetye, David Staffenberg, Joseph G McCarthy, Roberto L Flores
INTRODUCTION: Treacher Collins syndrome (TC) and Pierre Robin sequence (RS) are associated with hypoplastic mandible, glossoptosis, and consequent airway obstruction. Although TC and RS are often grouped together, airway outcomes of bilateral mandibular distraction osteogenesis (MDO) have not been specifically studied in TC. The purpose of this study is to report on the clinical outcomes of MDO in the TC patient population. MATERIALS AND METHODS: A twenty-year single-institution retrospective review of all patients with TC who underwent bilateral MDO was performed...
June 13, 2018: Annals of Plastic Surgery
https://www.readbyqxmd.com/read/29870511/surgical-management-and-outcomes-of-pierre-robin-sequence-a-comparison-of-mandibular-distraction-osteogenesis-and-tongue-lip-adhesion
#2
Rosaline S Zhang, Ian C Hoppe, Jesse A Taylor, Scott P Bartlett
There is a paucity of primary literature directly comparing tongue-lip adhesion (TLA) versus mandibular distraction osteogenesis (MDO) in surgical treatment of patients with Pierre Robin Sequence (PRS). This study comprehensively reviews the literature to evaluate and compare the effectiveness of MDO and TLA in improving airway and feeding outcomes. A search was performed using the MEDLINE (PubMed interface) and Embase databases for publications between 1960 through June 2017. English-language, original studies involving MDO or TLA in treatment of PRS subjects were included...
May 19, 2018: Plastic and Reconstructive Surgery
https://www.readbyqxmd.com/read/29793779/impact-of-early-intravelar-veloplasty-at-six-months-on-mandibular-growth-in-patients-with-pierre-robin-sequence
#3
Aude Caillot, Béatrice Ambroise, Hervé Bénateau, Alexis Veyssiere
Pierre Robin Sequence (PRS) combines mandible microretrognathia, asynchronism of the pharynx and tongue, glossoptosis and, in some cases, cleft palate. Its principal functional consequences are respiratory and feeding problems during the neonatal period. In this study, we focused on the impact of early closure of the cleft at six months on mandibular growth in patients with PRS. We performed a retrospective study of 15 patients followed for PRS and undergoing surgery performed by the same senior surgeon (HB) at our cleft center between 2005 and 2012...
May 21, 2018: Journal of Cranio-maxillo-facial Surgery
https://www.readbyqxmd.com/read/29791187/a-single-lab-test-to-aid-pierre-robin-sequence-severity-diagnosis
#4
Artur Fahradyan, Beina Azadgoli, Michaela Tsuha, Mark M Urata, Stacey H Francis
OBJECTIVE: The workup of patients with Pierre Robin sequence (PRS) consists of a physical examination, O2 saturation, and polysomnography to determine the severity of respiratory obstruction and need for surgery. We suggest that capillary blood gas (CBG) may be a better physiologic representation of airway obstruction and should be routinely used in the management of patients with PRS. DESIGN: This is a multicenter study based on a retrospective review of medical records...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29777780/pierre-robin-sequence-a-comprehensive-narrative-review-of-the-literature-over-time
#5
Amerigo Giudice, Selene Barone, Kahina Belhous, Anne Morice, Véronique Soupre, Francesco Bennardo, Nathalie Boddaert, Marie-Paule Vazquez, Véronique Abadie, Arnaud Picard
Pierre Robin syndrome (PRS) is characterized of a triad of clinical signs: micrognathia, glossoptosis and obstruction of the upper airways frequently associated with palatal cleft. It is a heterogenic pathological entity and it can be found as isolated disease (nsPRS) or in association with other syndromes (sPRS), with more pronounced symptoms and systemic involvement. This review aims to summarize the principal features of PRS, analysing the different aspects of the disease. Epidemiological data highlight incidence, severity and mortality of PRS; pathophysiological mechanism reports the etiology and pathogenesis of the disease distinguishing between isolated and syndromic form...
May 16, 2018: Journal of Stomatology, Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29757793/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
#6
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...
May 15, 2018: A&A practice
https://www.readbyqxmd.com/read/29728421/glossoptosis-in-pierre-robin-sequence
#7
Kevin C Cahill, David J A Orr
No abstract text is available yet for this article.
May 4, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/29727222/mandibular-catch-up-growth-in-pierre-robin-sequence-a-systematic-review
#8
Chad A Purnell, Lindsay E Janes, Julian L Klosowiak, Arun K Gosain
OBJECTIVE: The concept of mandibular catch-up growth is often quoted in the literature regarding Pierre Robin sequence (PRS). We endeavored to perform a systematic review of whether the literature supports this concept. DESIGN: Systematic review. INTERVENTIONS: A PubMed-based systematic review of the English literature was performed of articles objectively measuring mandibular growth or position after nonoperative management of PRS. MAIN OUTCOME MEASURES: Rate and end point of mandibular length, ramus length, gonial angle, and maxillomandibular discrepancy...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29721538/upper-airway-obstruction-in-neonates-does-sleep-exacerbate-symptoms
#9
Tyler Van Heest, Bridget Ebert, Brianne Barnett Roby, James Sidman
Objective: Describe the factors that exacerbate upper airway obstructions (UAOs) in neonates. Study Design: Retrospective chart review. Setting: Pediatric tertiary care hospital. Subjects and Methods: All neonates hospitalized between 1/1/2010 and 12/31/2014 diagnosed with either: 1) laryngomalacia, 2) Pierre Robin sequence, or 3) vocal cord paralysis were included in this study. Patient charts were reviewed to determine factors that exacerbated symptoms of airway obstruction...
April 2018: Laryngoscope Investigative Otolaryngology
https://www.readbyqxmd.com/read/29708799/combined-tongue-palate-fusion-with-alveolar-bands-in-a-patient-with-pierre-robin-sequence-and-van-der-woude-syndrome
#10
Alexa Robbins, Yuri A Zarate, Larry D Hartzell
This report describes the presentation of a newborn male with circumferential tongue-palate fusion associated with cleft palate and alveolar bands. After intraoral adhesions lysis, the patient was diagnosed with Pierre Robin sequence. A family history of cleft lip and palate was noted, and interferon regulatory factor 6 ( IRF6) sequencing revealed a heterozygous variant, confirming the diagnosis of van der Woude syndrome. The disruption of IRF6 resulted in abnormal orofacial development including micrognathia and intraoral adhesions as well as tongue-palate fusion, then resulting in glossoptosis with airway obstruction and cleft palate...
January 1, 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29695406/a-novel-association-of-campomelic-dysplasia-and-hydrocephalus-with-an-unbalanced-chromosomal-translocation-upstream-of-sox9
#11
Prince Antwi, Christopher S Hong, Daniel Duran, Sheng Chih Jin, Weilai Dong, Michael DiLuna, Kristopher T Kahle
Campomelic dysplasia is a rare skeletal dysplasia characterized by Pierre Robin sequence, craniofacial dysmorphism, shortening and angulation of long bones, tracheobronchomalacia, and occasionally sex reversal. The disease is due to mutations in SOX9 or chromosomal rearrangements involving the long arm of Chromosome 17 harboring the SOX9 locus. SOX9, a transcription factor, is indispensible in establishing and maintaining neural stem cells in the central nervous system. We present a patient with angulation of long bones and external female genitalia on prenatal ultrasound who was subsequently found to harbor the chromosomal abnormality 46, XY, t(6;17) (p21...
June 2018: Cold Spring Harbor Molecular Case Studies
https://www.readbyqxmd.com/read/29664868/active-transcutaneous-bone-conduction-implant-middle-fossa-placement-technique-in-children-with-bilateral-microtia-and-external-auditory-canal-atresia
#12
Carolina Der, Sofía Bravo-Torres, Nicolás Pons
AIM: The aim of this study is to present the middle fossa technique (MFT) as an alternative for patients who cannot undergo traditional surgery for active transcutaneous bone conduction implants (ATBCI) due to their altered anatomy or desire for future aesthetic reconstruction. DESIGN: A case series descriptive study was designed. The MFT was developed. Preoperative and postoperative information from 24 patients with external auditory canal atresia (EACA) and implanted with ATBCI was reviewed...
June 2018: Otology & Neurotology
https://www.readbyqxmd.com/read/29643641/pierre-robin-sequence-with-cervicothoracic-kyphoscoliosis-an-anesthetic-challenge
#13
V Abraham, S Grewal, G Bhatia, N Kaur, W Raghav, P Jain, N Gupta, M Singh, C George
No abstract text is available yet for this article.
January 2018: Journal of Anaesthesiology, Clinical Pharmacology
https://www.readbyqxmd.com/read/29642420/ebna1-oncogenic-activity-immune-evasion-and-biochemical-functions-provide-targets-for-novel-therapeutic-strategies-against-epstein-barr-virus-associated-cancers
#14
REVIEW
Joanna B Wilson, Evelyne Manet, Henri Gruffat, Pierre Busson, Marc Blondel, Robin Fahraeus
The presence of the Epstein-Barr virus (EBV)-encoded nuclear antigen-1 (EBNA1) protein in all EBV-carrying tumours constitutes a marker that distinguishes the virus-associated cancer cells from normal cells and thereby offers opportunities for targeted therapeutic intervention. EBNA1 is essential for viral genome maintenance and also for controlling viral gene expression and without EBNA1, the virus cannot persist. EBNA1 itself has been linked to cell transformation but the underlying mechanism of its oncogenic activity has been unclear...
April 6, 2018: Cancers
https://www.readbyqxmd.com/read/29627422/predictors-of-failure-in-infant-mandibular-distraction-osteogenesis
#15
Jeffrey A Hammoudeh, Artur Fahradyan, Colin Brady, Michaela Tsuha, Beina Azadgoli, Sally Ward, Mark M Urata
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...
March 15, 2018: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/29554066/an-algorithm-for-airway-management-in-patients-with-pierre-robin-sequence
#16
Katherine E Hicks, Kathleen R Billings, Chad A Purnell, John M Carter, Bharat Bhushan, Arun K Gosain, Dana M Thompson, Jeffrey C Rastatter
PURPOSE: Airway management in neonates with Pierre Robin sequence (PRS) can be challenging. The goal was to describe the algorithm developed by the authors over the past 8 years. METHODS: A retrospective case series analyzing airway management in neonates with PRS admitted to the neonatal intensive care unit at a tertiary care pediatric hospital was performed. The utility of the proposed algorithm for airway management incorporating more consistent use of polysomnography (PSG), and airway assessment was assessed...
March 16, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29489401/cervical-spine-injury-from-unrecognized-craniocervical-instability-in-severe-pierre-robin-sequence-associated-with-skeletal-dysplasia
#17
Xiao Zhu, Kelly N Evans, Areeg El-Gharbawy, Jonathan Y Lee, Jack E Brooker, Noel Jabbour, Elizabeth C Tyler-Kabara, Suneeta Madan-Khertarpal, Joseph E Losee, Jesse A Goldstein
Pierre Robin Sequence (PRS) can be associated with skeletal dysplasias, presenting with craniocervical instability and devastating spinal injury if unrecognized. The authors present the case of an infant with PRS and a type II collagenopathy who underwent multiple airway-securing procedures requiring spinal manipulation before craniocervical instability was identified. This resulted in severe cervical cord compression due to odontoid fracture and occipitoatlantoaxial instability. This case highlights the importance of early cervical spine imaging and cautious manipulation in infants with PRS and suspected skeletal dysplasia...
May 2018: Cleft Palate-craniofacial Journal
https://www.readbyqxmd.com/read/29485558/robin-sequence-continuing-heterogeneity-in-nomenclature-and-diagnosis
#18
Daan P F van Nunen, Marie-José H van den Boogaard, Corstiaan C Breugem
OBJECTIVE: Heterogeneity in both nomenclature and diagnostic criteria has hindered the interpretation of research into the congenital condition most widely known as (Pierre) Robin syndrome or sequence. In 2009, the discussion regarding its diagnosis and nosology was reopened to converge on a uniform eponym and standard set of diagnostic criteria. The objective of this study was to assess the impact of this debate. MATERIALS AND METHODS: This is a retrospective review of the nomenclature and diagnostic criteria employed in studies about this condition that were indexed in the MEDLINE literature database (PubMed) and published during 2009 to 2016...
February 26, 2018: Journal of Craniofacial Surgery
https://www.readbyqxmd.com/read/29456481/double-interstitial-deletion-of-the-long-arm-of-chromosome-6-in-a-patient-with-pierre-robin-sequence-dysmorphisms-and-severe-developmental-delay
#19
Giulia Parmeggiani, Stefania Bigoni, Barbara Buldrini, Giampaolo Garani, Luigi Clauser, Manilo Galiè, Alessandra Ferlini, Sergio Fini
Reported here is the case of a 1.8-year-old boy with a 9.6- Mb deletion in 6q13q14.1 and an 11.2-Mb deletion in 6q21q22.31, ascertained through array CGH, as the result of a complex de novo chromosome rearrangement. The clinical picture of this patient is characterized by severe psychomotor delay, dysmorphic features, and some congenital defects. Although, as reported in the literature, phenotypes associated with 6q deletions may vary, an attempt was made to associate the patient's symptoms to either deletion, comparing them to previously reported cases...
December 2017: Molecular Syndromology
https://www.readbyqxmd.com/read/29447809/difficult-airway-intubation-simulation-using-bonfils-fiberscope-and-rigid-fiberscope-for-surgical-training
#20
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
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