Pierre Robin Sequence with Esophageal Atresia and Congenital Radioulnar Synostosis

2006 ◽  
Vol 43 (3) ◽  
pp. 317-320 ◽  
Author(s):  
Keramettin Ugur Ozkan ◽  
Yusuf Kenan Coban ◽  
Murat Uzel ◽  
Mehmet Ergun ◽  
Hafize Oksuz
Keyword(s):  
Cleft Palate ◽  
Esophageal Atresia ◽  
Wide Spectrum ◽  
Pierre Robin Sequence ◽  
Radioulnar Synostosis ◽  
Robin Sequence ◽  
Congenital Radioulnar Synostosis ◽  
Pierre Robin

A wide spectrum of anomalies can be associated with Pierre Robin sequence. This report presents a 3-day-old infant with micrognathia, U-shaped cleft palate, low-set right ear with microtia, glossoptosis, esophageal atresia, and right congenital radioulnar synostosis. The association of congenital radioulnar synostosis and esophageal atresia with Pierre Robin sequence has not been previously described.

Pierre Robin Sequence With Esophageal Atresia and Congenital Radioulnar Synostosis

2006 ◽  
Vol 43 (3) ◽  
pp. 317 ◽  
Author(s):  
Keramettin Ugur Ozkan ◽  
Yusuf Kenan Coban ◽  
Murat Uzel ◽  
Mehmet Ergun ◽  
Hafize Oksuz
Keyword(s):  
Esophageal Atresia ◽  
Pierre Robin Sequence ◽  
Radioulnar Synostosis ◽  
Robin Sequence ◽  
Congenital Radioulnar Synostosis ◽  
Pierre Robin

Pierre Robin Sequence With Esophageal Atresia and Congenital Radioulnar Synostosis

2006 ◽  
Vol 43 (3) ◽  
pp. 317
Author(s):  
Keramettin Ugur Ozkan ◽  
Yusuf Kenan Coban ◽  
Murat Uzel ◽  
Mehmet Ergun ◽  
Hafize Oksuz
Keyword(s):  
Esophageal Atresia ◽  
Pierre Robin Sequence ◽  
Radioulnar Synostosis ◽  
Robin Sequence ◽  
Congenital Radioulnar Synostosis ◽  
Pierre Robin

scholarly journals Methadone, Pierre Robin sequence and other congenital anomalies: case–control study

2019 ◽  
Vol 105 (2) ◽  
pp. 151-157
Author(s):  
Brian Cleary ◽  
Maria Loane ◽  
Marie-Claude Addor ◽  
Ingeborg Barisic ◽  
Hermien E K de Walle ◽  
...  
Keyword(s):  
Cleft Palate ◽  
Congenital Anomalies ◽  
Population Based ◽  
Pierre Robin Sequence ◽  
Opioid Use ◽  
Robin Sequence ◽  
Risk Increase ◽  
Increased Risk ◽  
Pierre Robin ◽  
Control Study

ObjectiveMethadone is a vital treatment for women with opioid use disorder in pregnancy. Previous reports suggested an association between methadone exposure and Pierre Robin sequence (PRS), a rare craniofacial anomaly. We assessed the association between gestational methadone exposure and PRS.Design/settingThis case-malformed control study used European Surveillance of Congenital Anomalies population-based registries in Ireland, the Netherlands, Italy, Switzerland, Croatia, Malta, Portugal, Germany, Wales, Norway and Spain, 1995–2011.PatientsCases included PRS based on International Classification of Disease (ICD), Ninth Edition-British Paediatric Association (BPA) code 75 603 or ICD, Tenth Edition-BPA code Q8708. Malformed controls were all non-PRS anomalies, excluding genetic conditions, among live births, fetal deaths from 20 weeks’ gestation and terminations of pregnancy for fetal anomalies. An exploratory analysis assessed the association between methadone exposure and other congenital anomalies (CAs) excluding PRS. Methadone exposure was ascertained from medical records and maternal interview.ResultsAmong 87 979 CA registrations, there were 127 methadone-exposed pregnancies and 336 PRS cases. There was an association between methadone exposure and PRS (OR adjusted for registry 12.3, 95% CI 5.7 to 26.8). In absolute terms, this association reflects a risk increase from approximately 1–12 cases per 10 000 births. A raised OR was found for cleft palate (adjusted OR 5.0, 95% CI 2.7 to 9.2).ConclusionsThese findings suggest that gestational methadone exposure is associated with PRS. The association may be explained by unmeasured confounding factors. The small increased risk of PRS in itself does not alter the risk–benefit balance for gestational methadone use. The association with cleft palate, a more common CA, should be assessed with independent data.

scholarly journals Craniofacial Morphology in Japanese Patients with the Pierre Robin Sequence and Isolated Cleft Palate

2005 ◽  
Vol 17 (2) ◽  
pp. 105-113
Author(s):  
Yuichi NISHIMOTO ◽  
Yuko TOI ◽  
Tetsutaro YAMAGUCHI ◽  
Ayako NAGASHIMA ◽  
Kan NAKAJIMA ◽  
...  
Keyword(s):  
Cleft Palate ◽  
Japanese Patients ◽  
Craniofacial Morphology ◽  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Isolated Cleft Palate ◽  
Pierre Robin

Pediatric Feeding in Infants With Pierre Robin Sequence Following Mandibular Distraction Osteogenesis

2016 ◽  
Vol 1 (13) ◽  
pp. 5-9
Author(s):  
Kelly Mabry
Keyword(s):  
Cleft Palate ◽  
Airway Obstruction ◽  
Distraction Osteogenesis ◽  
Pierre Robin Sequence ◽  
Mandibular Distraction ◽  
Post Surgery ◽  
Feeding Experience ◽  
Robin Sequence ◽  
Mandibular Distraction Osteogenesis ◽  
Pierre Robin

Pierre Robin sequence (PRS) causes anomalies that can include micrognathia, cleft palate, and glossoptosis. At birth, infants typically present with a small mandible (micrognathia) which displaces the tongue posteriorly and causes breathing difficulties due to airway obstruction. A large, U-shaped cleft palate is often associated with this condition and the combined phenotype can be seen in isolation or in conjunction with a syndrome. In the neonatal period, management of PRS focuses on airway obstruction and feeding through conservative positioning techniques that are often successful. However, infants that do not respond to conservative measures require surgical intervention such as tongue-lip adhesion, tracheostomy, or mandibular distraction osteogenesis (MDO) to manage their airway. While each of these surgical procedures have been used effectively, the process of MDO has become the definitive technique to surgically correct the airway obstruction in PRS by lengthening the mandible, which also brings the tongue forward and out of the airway. Feeding intervention is directly related to the resolution of micrognathia which in turn advances the tongue base anteriorly, resolving the glossoptosis and airway obstruction. The infant's feeding experience, including pre-surgery and post-surgery considerations, are discussed.

Do Patients With Pierre Robin Sequence Have Worse Outcomes After Cleft Palate Surgery?

2013 ◽  
Vol 71 (3) ◽  
pp. 292-296 ◽  
Author(s):  
Carrie Stransky ◽  
Marten Basta ◽  
Cynthia Solot ◽  
Marilyn Cohen ◽  
David W. Low ◽  
...  
Keyword(s):  
Cleft Palate ◽  
Pierre Robin Sequence ◽  
Robin Sequence ◽  
Pierre Robin
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