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81. Pierre Robin Syndrome
pierre robin syndrome. From Bernadette Castro Category Craniofacial General Date 21 May 2004 Time 210441 Remote Name 64.178.86.132
http://www.cleft.org/_discussion/00001445.htm
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Pierre Robin Syndrome
From: Bernadette Castro
Category: Craniofacial General
Date: 21 May 2004
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Last changed: August 10, 2005

82. Pierre Robin Syndrome - SWMC Serving Vancouver Washington Portland Oregon
pierre robin syndrome Online Medical Encyclopedia courtesy of Southwest Washington Medical Center, a Top 100 Hosptital award winner locacted in Vancouver,
http://www.swmedicalcenter.com/118957.cfm
@import url(default.css);
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Back to Health Library Print This Page Email to a Friend Infant hard and soft palates
Pierre Robin syndrome
Definition: Pierre Robin syndrome (also called Pierre Robin complex or sequence) is a condition present at birth that is characterized by a very small lower jaw (micrognathia). The tongue tends to fall back and downward (glossoptosis) and there is cleft soft palate.
Alternative Names: Robin sequence
Causes, incidence, and risk factors: The specific causes of Pierre Robin syndrome are unknown. It can be part of many genetic syndromes. The lower jaw develops slowly over the first few months of fetal life but catches up over the first year after birth. Posterior placement of the tongue may cause choking episodes, feeding difficulty and breathing difficulties, especially while asleep.
Symptoms:
  • very small jaw with marked receding chin tongue appears large (is actually normal size but big relative to jaw) and is placed unusually far back in the oropharynx high arched palate cleft soft palate choking on tongue (a small opening in the roof of the mouth) natal teeth

Signs and tests: A physical examination is usually sufficient for your health care provider to diagnose this condition. A genetics consultation can rule out other associated anomalies and syndromes.

83. Le Syndrome
The Robin anomalad (pierre robin syndrome)-a follow up study.
http://tremplin.free.fr/Syndrome.htm
Tremplin - Syndrome de Pierre Robin
Association Loi 1901 - JO 16 du 17 avril 1999 Accueil Le syndrome Les témoignages Vos droits Evénements Bibliographie ... Plan du site
Responsable du site
Pierre Vandaele Le Syndrome de Pierre Robin par le Dr Véronique Abadi e Extrait de l'ouvrage "112 maladies génétiques, maladies rares" édité par Allo-Gènes, Centre national d'information sur les maladies génétiques, sous la direction du Pr Marie-Louise Briard, grâce au soutien financier de la CNAMTS. Sommaire : Définition clinique Evolution Génétique G ... Glossaire Définition clinique Le syndrome de Pierre Robin se définit par l'association d'une fente palatine postérieure romane médiane, d'un rétrognathisme et d'une glossoptose . Les trois éléments de la triade sont liés entre eux par un lien de cause à effet. Sa dénomination rappelle la description faite par Pierre Robin, dans les années 20, de nourrissons atteints de détresse respiratoire obstructive par glossoptose et rétromandibulie. Au moment de la croissance anatomique de la cavité orale de l'embryon, la fente se constitue en raison de la persistance de la langue en position verticale lors de la fusion antéropostérieure et latéromédiane des processus palatins secondaires, au cours de la 7e semaine de vie intra-utérine. La langue reste en position verticale postérieure du fait de l'insuffisance de tonus de sa base et d'un défaut de croissance de la mandibule. Ce

84. Pierre Robin Syndrome
The specific causes of pierre robin syndrome are unknown; it can be part of many genetic syndromes. The lower jaw develops slowly over the first few months
http://www.shands.org/health/information/article/001607.htm
Disease Injury Nutrition Poison ... Infant hard and soft palates
Pierre Robin syndrome
Definition: A group of abnormalities marked primarily by a very small lower jaw with a tongue that falls back and downward. The syndrome may include a high arched palate or cleft palate.
Alternative Names: Robin sequence
Causes, incidence, and risk factors: The specific causes of Pierre Robin syndrome are unknown; it can be part of many genetic syndromes. The lower jaw develops slowly over the first few months of fetal life but catches up over the first year after birth. Posterior placement of the tongue may cause choking episodes, feeding difficulty and breathing difficulties , especially while asleep.
Symptoms:
  • very small jaw with marked receding chin tongue appears large (is actually normal size but big relative to jaw) and is placed unusually far back in the oropharynx high arched palate cleft soft palate choking on tongue natal teeth

Signs and tests: Physical examination is usually sufficient for your health care provider to diagnose this condition.
Treatment: Infants must be kept prone (face down), which allows gravity to pull the tongue forward and keep the airway open. These problems abate over the first few years as the lower jaw grows and assumes a more normal size. In moderate cases, the patient requires placement of a nasopharyngeal airway (a tube placed through the nose and into the airway) to avoid airway blockage. In severe cases, surgery is indicated for recurrent upper

85. Association Tremplin
Destin© aux parents d'enfants pr©sentant cette maladie, lieu d'information et d'©change.
http://tremplin.free.fr/
Tremplin - Syndrome de Pierre Robin
Association Loi 1901 - JO 16 du 17 avril 1999
Responsable du site
Pierre Vandaele Pourquoi Tremplin ? Actualités ... Information sur le syndrome
Liens
Pour mettre en relation les parents d'enfants présentant le syndrome de Pierre Robin. Parce qu'il nous a paru important de construire un lieu d'échanges de nos expériences. la page internationale The international page la página ... nternacional Vous êtes adhérents à l'association Tremplin !
L' Espace Adhérent est fait pour vous.
T éléchargement de la plaquette de présentation de l'association ...à diffuser !
Notre Assemblée Générale a eu lieu le 05 juin 2004 à Saint Herblain (44).
souvenirs de cette journée

La marche des maladies rares a eu lieu le samedi 4 décembre 2004 à Paris, de Broussais au Trocadéro.
Quelques photos de cette marche
" Diable d'Homme ! ". C'est le titre de la pièce qui sera jouée au profit de notre association par le dynamique T.A.L.C. de Tatinghem. Rendez-vous le 16 janvier 2005 à la salle des fêtes de Tatinghem !

86. Virtual Children's Hospital: Paediapaedia: Pierre-Robin Syndrome
pierrerobin syndrome. Michael P. D Alessandro, MD Also can be associated with multiple rib abnormalities - cerebro-costomandibular syndrome. Pathology
http://www.vh.org/pediatric/provider/radiology/PAP/NeuroDiseases/PierreRobin.htm
Paediapaedia: Neurological Diseases
Pierre-Robin Syndrome
Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed Clinical Presentation:
Has severe, symmetric mandibular hypoplasia (micrognathia) that is present at birth that is coupled with a widely cleft palate that allows for backward displacement of the tongue into the pharynx obstructing the epiglottis and blocking air flow. Etiology/Pathophysiology:
Have associated congenital heart disease, external ear deformities, microphthalmus, bird like faces. Also can be associated with multiple rib abnormalities - cerebro-costomandibular syndrome. Pathology:
Not applicable Imaging Findings:
Micrognathia DDX:
  • Not applicable
References:
See References Chapter. Section Top Title Page Virtual Children's Hospital Home Virtual Hospital Home ... University of Iowa http://www.vh.org/pediatric/provider/radiology/PAP/NeuroDiseases/PierreRobin.html

87. Virtual Children's Hospital: Neonatal Chest: Imaging For Respiratory Distress: A
Atelectasis/pierrerobin syndrome. R lung atelectasis s/p excision of subglottic hemangioma Lateral face showing pierre-robin syndrome
http://www.vh.org/pediatric/provider/radiology/respiratorydistress/07.html
Neonatal Chest: Imaging for Respiratory Distress
Atelectasis/Pierre-Robin syndrome
Simon C. Kao, M.D.
Peer Review Status: Internally Peer Reviewed R lung atelectasis s/p excision of subglottic hemangioma Lateral face showing Pierre-Robin syndrome Next Page Previous Page Title Page This information is written primarily for providers. See related Provider Textbooks about Radiology See related Provider Topics CT Scans Diagnostic Imaging Procedures and Therapies Pulmonary ... Radiology or X-Rays See related Patient Textbooks about Radiology See related Patient Topics Diagnostic Imaging Procedures and Therapies Radiology or X-Rays Virtual Children's Hospital Home Virtual Hospital Home Site Map ... University of Iowa http://www.vh.org/pediatric/provider/radiology/respiratorydistress/07.html

88. Syndrome De Pierre-Robin : Sites Et Documents Francophones
Translate this page Séquence de pierre robin SPR - syndrome de pierre robin pierre robin, syndrome de Par Dr Aymé S (Orphanet - INSERM SC 11).
http://www.chu-rouen.fr/ssf/pathol/pierrerobinsyndrome.html
Syndrome de Pierre-Robin Synonyme(s) CISMeF Pierre Robin, séquence de ; Pierre-Robin ; SPR .
Synonyme(s) MeSH Pierre robin, syndrome
Arborescence(s) syndrome de Pierre-Robin pierre robin syndrome
maladies de l'appareil locomoteur
maladies de l'appareil stomatognathique maladies et malformations congénitales, héréditaires et néonatales Position du mot-clé dans l' (les) arborescence(s) : Vous pouvez consulter Ou consulter ci-dessous une sélection des principales ressources :
Qualificatifs : image patient
  • Association Tremplin
    [informations sur la pathologie (définition clinique, évolution, génétique, géne, bases biochimiques, méthode de daignostic, conseil génétique et diagnostic prénatal, perspectives thérapeutiques), témoignages, bibliographie, forum ; pays : France ; ville : Longuenesse ; langue : français ; format : html ; accès : gratuit ; non parrainé ; visité le : 24/10/2003].

89. Robin's Syndrome (www.whonamedit.com)
with cleft palate, downward displacement of the tongue, and an absent gag reflex. Also known as pierre robin’s syndrome,robin s anomalad,robin’s sequence.
http://www.whonamedit.com/synd.cfm/1291.html

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Robin's syndrome Also known as: Pierre Robin’s syndrome Robin's anomalad Robin’s sequence Synonyms: Micrognathia-glossoptosis syndrome. Associated persons: Pierre Robin Description: A very rare congenital malformation syndrome characterized by unusual smallness of the jaw combined with cleft palate, downward displacement of the tongue, and an absent gag reflex. The affected child is in serious danger because of difficulty in breathing and feeding. The abnormality may occur in isolation or as a variable component of many genetic syndromes. Inheritance is autosomal recessive. Bibliography:
  • Pierre Robin: La glossoptose. Son diagnostic, ses consequences, son traitement.

90. Pierre Robin (www.whonamedit.com)
pierre robin French dental surgeon, 18671950. Associated with robin s syndrome,Stickler s syndrome.
http://www.whonamedit.com/doctor.cfm/1233.html

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Eponyms A-Z

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Whonamedit.com does not give medical advice.
This survey of medical eponyms and the persons behind them is meant as a general interest site only. No information found here must under any circumstances be used for medical purposes, diagnostically, therapeutically or otherwise. If you, or anybody close to you, is affected, or believe to be affected, by any condition mentioned here: see a doctor.
A recommendation:
Hypography
is an open community about science and all things related
Pierre Robin French dental surgeon, 1867-1950. Associated eponyms: Robin's syndrome A very rare congenital malformation syndrome characterized by unusual smallness of the jaw combined with cleft palate, downward displacement of the tongue, and an absent gag reflex. Stickler's syndrome A fairly common progressive disease of connective tissue with onset at birth affecting ears, eyes, and joints. Biography: Pierre Robin, in his time the doyen of French dental surgeons, was best known for his contributions to orthodentistry, but published on many aspects of dentistry and oral hygiene. His main interest was glossoptisis, on which he wrote more than 20 articles and a monograph. He was professor at the French School of Stomatology, and from 1914, with Nogue, he was editor-in-chief of the Revue de Stomatologie. Ole Daniel Enersen

91. You Ll Find Hundreds Of Files On Cleft Lip, Cleft Palate Here On
pierre robin Sequence can often be part of a larger syndrome, the most common of which is Stickler syndrome. The recurrence risk for Sticklers is different
http://www.widesmiles.org/cleftlinks/WS-901.html
You'll find hundreds of files on cleft lip, cleft palate here on widesmiles.org This one is about: Pierre Robin Sequence Overview (c) 1998 Wide Smiles
This Document is from WideSmiles Website - www.widesmiles.org
Reprint in whole or in part, with out written permission from Wide Smiles
is prohibited. Email: widesmiles@aol.com
PIERRE ROBIN SEQUENCE
by Alana Poos A great aid to coping with a birth defect is simply knowing what it is. While there might be a great deal of information on cleft lip and/or palate, there simply isn't an abundance of information readily available to parents of children with cleft-related birth defects, especially those that are considered rare. The following information has been obtained from a number of sources, and will hopefully help to clarify exactly what Pierre-Robin Sequence is. WHAT IS PIERRE ROBIN SEQUENCE? Pierre Robin Sequence is the name given to a birth defect that involves an abnormally small lower jaw, and a tendency for the tongue to "ball up" and fall backward toward the throat. Babies born with this condition may or may not have a cleft palate, but they do not have a cleft lip. The condition of Pierre Robin Sequence was first described in 1822 and was named after the French physician who associated the above symptoms with breathing problems in affected infants. The current complete name is "Pierre Robin Malformation Sequence", but it can also be known as "Cleft Palate, Micrognathia, and Glossoptosis", "Robin Anomalad", "Pierre Robin Complex", and "Pierre Robin Syndrome".

92. Pierre-Robin Syndrome
pierrerobin syndrome. This article submitted by Indiana Parent Information Netwo on 1/31/97. Author s Email ipin@indy.net. looking for parents of children
http://neuro-www.mgh.harvard.edu/neurowebforum/GeneralFeedbackArticles/1.31.9712
Pierre-Robin Syndrome
This article submitted by Indiana Parent Information Netwo on 1/31/97.
Author's Email: ipin@indy.net
looking for parents of children with Pierre-Robin in
Central Indiana area to talk with parent dealing with
issues related to this Syndrome. Next Article
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93. Stickler Syndrome | Craniofacial Center | Children's Hospital And Regional Medic
How are infants with pierre robin sequence due to Stickler syndrome treated? Some babies with Stickler syndrome are born with pierre robin sequence,
http://craniofacial.seattlechildrens.org/conditions/stickler.asp
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What is Stickler syndrome?
Stickler syndrome is a genetic disorder of connective tissue that results in variable problems with vision, hearing and facial and skeletal development. Connective tissue, made up of collagen, acts like glue or an elastic band and allows muscles to stretch and contract. A syndrome is a disease or disorder that has more than one identifying feature or symptom. Stickler syndrome often affects the connective tissue of the eye, especially in the vitreous humor (interior of the eyeball), and the epiphysis (ends of the bones that make up the joints). Dr. G. B. Stickler first described this condition in 1965.

94. Cleft And Craniofacial Anomalies: Pierre Robin Sequence
There are several different syndromes that can have pierre robin sequence. If you or your baby have pierre robin sequence as part of a syndrome,
http://www.ucdmc.ucdavis.edu/children/services/cleft/health/anomalies/pierre.htm
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Pierre Robin Sequence
Pierre Robin sequence is a combination of problems that begins during pregnancy with micrognathia (small jaw). If the jaw is small, there is often not enough room for the tongue to lie flat in the mouth, so it rests at the back of the mouth (this is called glossoptosis). When the tongue rests at the back of the mouth, it may prevent the palate (roof of the mouth) from closing, resulting in a cleft palate (cleft lip is not usually associated with Pierre Robin). If a child has a cleft palate this is usually repaired between the ages of 12 and 18 months. Isolated Pierre Robin sequence is often not a lifelong condition. The jaw of an affected child is often able to grow and catch up to normal size. In fact, many children have a completely normal profile by the time they start school. Isolated Pierre Robin sequence does not affect any other part of the body. A baby with isolated Pierre Robin sequence is at no greater risk than any other child for other problems, including learning difficulties. If a baby just has Pierre Robin sequence, there is only a small chance that the parents, other family members, or indeed the baby later in life will have another baby with Pierre Robin sequence. The chances of it happening again if you are the affected baby or the parents of that baby, is thought to be between 1 and 5%. Conversely, there is a 95-99% chance that it will not happen again. The risk to other family members is smaller still.

95. Microsomia, Goldenhar Syndrom Pierre Robin Sequence Oculo-Auriculo-Vertebral Spe
OculoAuriculo-Vertebral Spectrum, Hemifacial Microsomia, Goldenhar syndrome pierre robin Sequence Stickler syndrome Treacher Collins syndrome
http://www.ucdmc.ucdavis.edu/children/services/cleft/health/anomalies/oculo.html
For Health Consumers For Health Professionals Find a Clinic Find a Doctor ...
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Oculo-Auriculo-Vertebral Spectrum, Hemifacial Microsomia, Goldenhar Syndrom Pierre Robin sequence
These three conditions are believed by many to be related to each other and may be part of the same variable disorder. Hemifacial Microsomia Hemifacial means one side of the face. Microsomia means small. Hemifacial microsomia means that one side of the face is smaller than the other side. Goldenhar Syndrome In addition to the facial findings, those with Goldenhar Syndrome also have other parts of the body affected. Heart defects can occur, as can differences in the way the kidneys are formed. In some cases one kidney may be absent. A person can live a full life with only one kidney, but it would be advisable to be cautious and avoid contact sports to reduce the risk of injuring the remaining kidney. There may be difference in the spine, such as two bones being fused together. This occurs in 20-35% of people with Goldenhar Syndrome. Most people with Goldenhar Syndrome have normal intelligence. Oculo auriculo vertebral spectrum, or OAVS (Oculo refers to the eye, auriculo to the ear, and vertebral to the spine.)

96. Anesthesiology - Fulltext: Volume 83(3) September 1995 P 646-647 Laryngeal Mask
In neonates with mandibular hypoplasia such as pierrerobin syndrome or Treacher-Collins syndrome, the posteriourly protruded tongue may result in airway
http://www.anesthesiology.org/pt/re/anes/fulltext.00000542-199509000-00039.htm
LWWOnline LOGIN eALERTS REGISTER ... ASA Meeting Abstracts Laryngeal Mask Airway for Resuscitation... ARTICLE LINKS:
References (4)
View full size inline images Anesthesiology Volume 83(3) September 1995 pp 646-647
Laryngeal Mask Airway for Resuscitation of a Newborn with Pierre-Robin Syndrome
Baraka, Anis MD, FRCA Professor and Chairman, Department of Anesthesiology, American University of Beirut, Beirut, Lebanon. To the Editor:Paterson et al. have shown that the laryngeal mask airway (LMA) can be used as an effective method of airway management during neonatal resuscitation as an alternative to bag-and-mask ventilation. The investigators suggest caution in the use of the LMA in neonates beyond the population studied, which did not include neonates who were suffering from congenital oropharyngeal pathology. The current report describes the use of LMA for resuscitation of a newborn with Pierre-Robin syndrome after failure of tracheal intubation and face-mask ventilation. The newborn was a 5-day-old, 3.2 kg, full-term male with Pierre-Robin syndrome (micrognathia, cleft palate). The anesthesiology team was paged when serious airway obstruction and cyanosis developed that were not relieved by lateral positioning of the baby, traction of the tongue, and face-mask oxygen. Pulse oximetry showed the hemoglobin oxygen saturation (SpO ) to be 40% and the electrocardiogram showed sinus bradycardia (50 beats/min). An oropharyngeal airway was inserted, and ventilation via face mask with 100% Oxygen

97. Karger Publishers
pierrerobin syndrome; Three-dimensional sonography; Antenatal diagnosis A case of pierre-robin syndrome with polyhydramnios is described.
http://content.karger.com/ProdukteDB/produkte.asp?Doi=52911

98. ORPHANET - Maladies Rares - Médicaments Orphelins
Translate this page MALADIE pierre robin, syndrome de. Synonyme(s) pierre robin, séquence de. CIM Q87.0. Le syndrome de pierre-robin est une anomalie du développement de la
http://www.orpha.net/consor/cgi-bin/OC_Exp.php?Lng=FR&Expert=718

99. Pierre Robin, Syndrome De
Translate this page Base de données sur les maladies rares et les médicaments orphelins.
http://www.orpha.net/static/FR/pierre_robin.html
Accès à la base de données Orphanet
Pierre Robin, syndrome de
Accès direct aux détails Résumé
Le syndrome de Pierre-Robin est une anomalie du développement de la cavité orale, caractérisé par une micrognathie avec cavité orale de taille réduite, une glossoptose avec pseudomacroglossie, et un palais ogival ou fendu. Ce syndrome est lié à une anomalie du développement de la mandibule avant la 9ème semaine d'âge gestationnel. La croissance de la mandibule pendant les premiers mois de vie postnatale supprime la glossoptose et la croissance ultérieure corrige l'hypoplasie mandibulaire en 4 à 6 ans. Ce syndrome a des étiologies multiples. *Auteur : Equipe Editoriale d'Orphanet (septembre 2002)*. Signes de la maladie
  • FENTE PALATINE
  • GLOSSOPTOSE
  • RETROGNATHISME/MICROGNATHISME
  • DETRESSE RESPIRATOIRE
  • PHARYNX ANOMALIE/DEGLUTITION ANOMALIE
Mise à jour : 04/09/2005
Accès à la base de données Orphanet

100. Le Syndrome De Pierre-Robin
Translate this page Le syndrome de pierre-robin (SPR) est une maladie rare qui concerne environ 60 à 80 enfants chaque année en France. Cette maladie orpheline se traduit par
http://www.essentielsante.net/Html/MaladiesRares/cadre/120060400.htm
Accueil Maladies rares Le syndrome de Pierre-Robin Maladies rares
Le syndrome de Pierre-Robin
Chaque année, un enfant sur 8 000 naît porteur d’un syndrome de Pierre-Robin. Cette maladie rare est facilement diagnostiquée car le faciès est caractéristique dès la naissance.
Il n’existe pas de traitement de fond de la maladie, mais la chirurgie permet de remédier aux fentes palatines, opérées en général entre 6 et 19 mois, et à la micrognathie. La glossoptose et la micrognathie régressent un peu avec l’âge.
Ni le chromosome, ni le gène responsable n’ont été encore identifiés.
Association Tremplin-Syndrome de Pierre-Robin
42, bis rue Gaston Brogniart
62219 Longuenesse
Tél. : 03 21 38 10 96 - 03 21 95 11 33
Fax : 03 21 38 19 45
contact@tremplin-spr.org
http://www.tremplin-spr.org Retour à la liste des maladies rares Dr Daniel Gloaguen -15 janvier 2003 redaction@essentielsante.net

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