Early Mandibular Distraction Osteogenesis in Pierre Robin Sequence - PowerPoint PPT Presentation

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Early Mandibular Distraction Osteogenesis in Pierre Robin Sequence

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Title: Early Mandibular Distraction Osteogenesis in Pierre Robin Sequence


1
Early Mandibular Distraction Osteogenesisin
Pierre Robin Sequence
2
Pierre 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.

3
Early 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.

4
Early 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

5
Problems 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

6
Problems 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

7
Problems 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

8
Mandibular Distraction Background
  • External traction with halo (Callister 1937)

9
Mandibular Distraction Background
  • External traction with pulley/ weight (Longmire,
    Sandford 1940)

10
Mandibular 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)

11
Mandibular Distraction Background
  • External distractor (Denny 2002)
  • linear Howmedica distraction device

12
Mandibular Distraction Background
13
Mandibular Distraction Background
14
Mandibular Distraction Background
  • Internal resorbable device

15
Early 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

16
Indications for early distraction in Pierre Robin
  • Failure of conservative measures to improve
    respiration and feeding
  • Documented tongue base obstruction
  • Center with expertise In distraction
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