Pierre Robin syndrome

(redirected from Pierre Robin Sequence)
Also found in: Acronyms.

Pierre Robin syndrome

 [pyār ro-ban´]
an autosomal recessive disorder characterized by smallness of the mandible, cleft palate, and often drooping of the tongue, backward and upward displacement of the larynx, and angulation of the manubrium sterni. Cleft palate makes sucking and swallowing difficult, permitting easy access of fluids into the larynx.

Pierre Rob·in syn·drome

(pē-yār' rō-ban[h]'), [MIM*261800]
retrognathia and glossoptosis, often associated with upper airway obstruction and feeding difficulties; may be accompanied by cleft palate, laryngomalacia, ocular and cervical spine abnormalities; weak evidence of autosomal recessive inheritance.

Pierre Robin syndrome

Pierre Robin sequence Pediatrics A group of oral defects–very small lower jaw with a posterior-placed tongue that causes choking and dyspnea, especially while asleep; other findings include a high arched palate or cleft palate. See Cleft palate.

Pi·erre Rob·in syn·drome

(pē-yār' rō-ban[h]' sin'drōm)
A complex of congenital anomalies including micrognathia and abnormal smallness of the tongue, often with cleft palate, severe myopia, congenital glaucoma, and retinal detachment. Intelligence of those affected is usually normal.
Synonym(s): Robin syndrome.

Robin,

Pierre, French pediatrician, 1867-1950.
Pierre Robin syndrome - micrognathia and abnormal smallness of the tongue, often with cleft palate, severe myopia, congenital glaucoma, and retinal detachment. Synonym(s): Robin syndrome
Robin syndrome - Synonym(s): Pierre Robin syndrome

Pi·erre Rob·in syn·drome

(pē-yār' rō-ban[h]' sin'drōm) [MIM*261800]
Complex of congenital anomalies including micrognathia and abnormal smallness of tongue, often with cleft palate. Intelligence of those affected is usually normal.
References in periodicals archive ?
Pierre Robin sequence (PRS) is a congenital abnormality which is characterised by the presence of a combination of mandibular hypoplasia, glossoptosis and often Labio-palatin clefts which is the most common type of craniofacial malformation encountered in practice and also among most common congenital abnormalities.
A rudimentary epiglottis associated with Pierre Robin sequence. Int J Oral Maxillofac Surg 2006; 7: 668-70.
Pierre Robin sequence: appearances and 25 years of experience with an innovative treatment protocol.
Pierre Robin sequence may be caused by dysregulation of SOX9 and KCNJ2.
Renault (2011) (22) evaluated the relevance of EMG combined techniques in the evaluation and management of children with Pierre Robin sequence (SPR) from the needle electromyography of facial muscles, tongue, pharynx and larynx (Table 1 and 2); They followed 81 infants among them 57 with Pierre Robin sequence alone (SPRI) and 24 with Pierre Robin sequence associated (SPRA) (Table 2).
Infants born with Pierre Robin sequence are born with their tongue positioned posteriorly, often causing blockage of the pharynx and airway, a process called gloosoptosis.
Mandibular distraction osteogenesis for Pierre Robin sequence: what percentage of neonates needs it?
Airway management in two newborns with Pierre Robin Sequence: the use of disposable vs multiple use LMA for fiberoptic intubation.
The term Pierre Robin Sequence is given to a birth defect which involves an abnormally small lower jaw (migrognathia) and a tendency for the tongue to "ball up" and fall backward toward the throat (glossoptosis).
Non-Respiratory Causes Diagnosis Cases Percentage Birth Asphyxia 26 10.4% Congenital Diaphragmatic Hernia 3 1.2% Tracheo-Oesophageal Fistula 2 0.8% Congenital Heart Disease 1 0.4% Pierre Robin Sequence 1 0.4% Table 3.
In this article, we describe the cases of 2 neonates with syndromic Pierre Robin sequence who experienced feeding difficulties and severe upper airway obstruction.
She has been diagnosed with a variant of Pierre Robin sequence (a combination of a small jaw, cleft palate, and the forward displacement of the tongue, producing facial deformities).