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Pierre Robin Syndrome
5
Pierre Robin Syndrome
By Katie Powell
History
PRS was named after Pierre Robin (1867-1950) who was a French dental surgeon or stomatologist.
Pierre Robin was a professor at the French School of Stomatology.
For the therapist
Living with PRS
- The therapist should project a good example of proper maintenance.
- It is the therapist's job to ensure proper education.
References
Conclusions
- https://en.wikipedia.org/wiki/Pierre_Robin_(surgeon)
- https://en.wikipedia.org/wiki/Pierre_Robin_syndrome#Management
- Wilcox BK, Tatum SA. Cerebro-costo-mandibular syndrome presenting as Pierre Robin sequence. Am J Otolaryngol. 2004 Jul-Aug;25(4)258-62.
- Tewfik TL, Trinbh N, Teebi AS. Pierre Robin Syndrome. Emedicine Web site. Available at www.emedicine.com. Accessed November 11, 2004.
- K. D. Anderson, A. Cole, C. B. Chuo, R. Slator, Home Management of Upper Airway Obstruction in Pierre Robin Sequence Using a Nasopharyngeal Airway, The Cleft Palate-Craniofacial Journal. 2007
- Pierre Robin syndrome is a combination of one or more abnormalities affecting the face and head of infants.
- This can involve the infants jaw, tongue, or airways.
- PRS can be treated with simple procedures, but can also require a series of surgeries depending on the severity of the abnormality.
- Respiratory therapist play an important role in treatment, recovery and management of PRS.
- Parents must learn to maintain their child’s treatment at home.
- The goal of each of these treatments is to help the infant or child to feed, and breathe normally as any other child would.
Definition
This is accomplished through
Signs and Symptoms
Pierre Robin Syndrome is a set of malformations of the head and face, these malformations include:
Causes
Micrognathia: A condition where the jaw is smaller than average.
Glossoptosis: A condition where the tongue is displaced downward or retracts from the front of the mouth.
- Various positions such as prone and sidelying
- short term intubation, and nasopharyneal airways.
- Distraction osteogenesis or "Mandibular Distraction".
- Primary cleft lip repair.
Dislocation of the jaw bone during fetal development.
Genetic inconsistencies in chromosomes 2, 11, and 17.
PRS is characterized by an abnormally small jaw (micrognathia), displacement or retraction of the tongue (glossoptosis), and upper airway obstruction.
Cleft Palate: Congenital split of the roof of the mouth.
Effect the ability of the infant or child to breathe and eat properly.
Diagnosis
Treatment
Infants are diagnosed shortly after birth due to obvious respiratory distress, especially while lying in supine position.
The ultimate goal in treatment is to optimize growth and nutrition in spite of the respiratory complications the patient has.