- Typically presents as a NEONATAL EMERGENCY
- Due to airway obstruction from abnormally positioned tongue
The triad of cleft palate, micrognathia, and airway obstruction was described by St Hilaire in 1822, by Fairbain in 1846, and by Shukowsky in 1911. Pierre Robin, a French stomatologist, first described glossoptosis and its relationship with micrognathia and airway complications that can occur in the condition that now bears his name. In 1923, Robin described “liberation of the oral pharynx” with a prosthetic device that pulled the jaw and tongue forward. He later reported growth failure and death caused by the respiratory complications that occur with micrognathia and glossoptosis.
- Fairbairn–Robin triad (FRT): Micrognathia + glossoptosis + palatal cleft
- Siebold–Robin sequence (SRS): Micrognathia + glossoptosis
Syndromic PRS (sPRS):
- Strickler syndrome (30-50%, M/C)
- Treacher Collins syndrome
- Velocardiofacial syndrome & DiGeorge syndrome
- CHARGE syndrome
- Edward’s syndrome
- Trisomy 11q syndrome
- Möbius syndrome
- Fetal alcohol syndrome
Non-syndromic PRS (nsPRS):
- SOX9 gene
- Cleft palate (U-shaped)
- Retrognathia: Abnormal positioning of the jaw/mandible
- Glossoptosis (backwards displacement of the tongue base) → Airway obstruction
Prone positioning (50% success rate):Allow the mandible and tongue to fall forward and reduce airway obstruction at the tongue-base level.
Nasopharyngeal airway (NPA):Obstruction may be relieved as the NPA breaks the seal between the tongue and posterior pharynx, and the child can breathe through the tube and contralateral nostril.
Tongue-Lip Adhesion (TLA): GlossopexyAnterior ventral tongue anchored to lower lip (mucosa plus or minus muscle), and posterior tongue is anchored to mandible.
Mandibular distraction osteogenesis (MDO):Procedure includes bilateral osteotomies and placement of distraction devices, which can be internal or external with percutaneous pins.
Tracheotomy: Last resortReserved for patients whose condition fails to respond to other measures, although it is still used as the main surgical option for children with RS and airway obstruction at some institutions.