Title: Early Mandibular Distraction Osteogenesis in Pierre Robin Sequence
1Early Mandibular Distraction Osteogenesisin
Pierre Robin Sequence
2Pierre Robin Sequence
- Pierre Robin case report 1926
- one in 9000 births
- micrognathi, glossoptosis, cleft palate.
- Theories
- fetal head positioning, frequently associated
with oligohydramnios. - a delay in neurological maturation
- rhombencephalic dysneurulation
- rare familial cases reported - localized
intrinsic failure of mandibular growth may be a
factor in some cases. - Catchup mandibular growth in most, but mandibular
dimensions will remain below age-matched norms.
3Early Considerations
- varying degrees of airway obstruction and feeding
difficulties. - mechanism - falling back of the tongue into the
oral pharynx. - Immediate supportive measures required in over 70
percent of affected infants. - Caouette-Laberge ( 1994) clinical classification
of respiratory symptoms - group I, adequate respiration in prone position
and bottle feeding - group II, adequate respiration in prone position
but feeding difficulties requiring NGT - group III, children with respiratory distress
requiring respiratory support and NGT.
4Early Management
- Supportive measures
- Lying prone
- Tongue-lip adhesion
- Kirschner (2003) - gt40 Group III infants
required tracheostomy after tongue-lip adhesion - Denny (2004) - additional 1.9 secondary
procedures - Nasopharyngeal airway
- Tracheostomy (12-42)
- K wire fixation, genioglossus stripping
5Problems with tracheostomy
- Increased morbidity
- Donnelly, Int J Pediatr Otorhinolaryngol. 1996
- n29 41 complication rate (lt1yo- 64)
- 25 months average decannulation
- Midwinter, J Laryngol Otol. 2002
- n-143 46 complication rate
- 25 months mean decannulation
- Mortality 2.7
- Carr, Laryngoscope. 2001
- N142 43 serious complications
- Mortality 0.7
6Problems with tracheostomy
- Poorer Speech Outcomes
- Jiang, Int J Pediatr Otorhinolaryngol. 2003
- Affects speech and language development in those
with and without neurological disorders. - Risk factors age at tracheostomy, and duration.
- Better outcome with early decannulation
- Simon, Int J Pediatr Otorhinolaryngol. 1983
- All children decannulated during the linguistic
stage exhibited specific spoken language delays - phonological impairment proportional to duration
7Problems with tracheostomy
- Prolonged
- Tomaski, Laryngoscope 1995
- Average 3 years decannulation in PRS
- Carer Impact
- Financial Burden
- Developmental Problems
- Singer, Dev Med Child Neurol. 1989
- n130
- Slower growth rate
- Higher risk of behavioural problems
- Most will require special educational
intervention
8Mandibular Distraction Background
- External traction with halo (Callister 1937)
9Mandibular Distraction Background
- External traction with pulley/ weight (Longmire,
Sandford 1940)
10Mandibular Distraction Background
- Mandibular DOG
- McCarthy 1992, Molina/Ortiz-Monasterio 1995
- Use in children with airways obstruction
- Moore, David 1994
- Cohen 1999
- Use in Pierre Robin
- Denny 2001,2002
- Monasterio 2002
- Burstein 2005 (internal resorbable device)
11Mandibular Distraction Background
- External distractor (Denny 2002)
- linear Howmedica distraction device
12Mandibular Distraction Background
13Mandibular Distraction Background
14Mandibular Distraction Background
- Internal resorbable device
15Early Distraction Controversies
- Conservative management alone
- 20-40 will not respond to positioning or
glossopexy - Rapid distraction
- 2mm/day vs 1mm/day
- In goats demyelination noted at 2mm/day (Hu,
J Oral Maxillo Surg 2001) - Effect on dentition
- Screw holes
- Infraalveolar nerve
- Effect on subsequent mandibular growth
- Facial scarring
16Indications for early distraction in Pierre Robin
- Failure of conservative measures to improve
respiration and feeding - Documented tongue base obstruction
- Center with expertise In distraction