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This syndrome was described in 1923 by Pierre Robin in which he described
airway obstruction associated with glossoptosis and hypoplasia of the
mandible. Today this syndrome is characterized by retrognathia or micrognathia,
glossoptosis, and airway obstruction. An incomplete cleft of the palate
is associated with the syndrome in approximately 50% of these patients.
In patients with micrognathia (small jaw) or retrognathia, the chin is
posteriorly displaced causing the tongue to fall backward toward the posterior
pharyngeal wall. This results in obstruction of the airway on inspiration.
Crying or straining by these children can often keep the airway open. However,
when the child relaxes or falls asleep, airway obstruction occurs. Due
to these respiratory problems, feeding may become very difficult. This
can lead to a sequence of events: glossoptosis, airway obstruction, crying
or straining with increased energy expenditure and decreased oral intake.
This vicious cycle of events if untreated can led to exhaustion, cardiac
failure, and ultimately death.
| Treatment of this syndrome can be divided into conservative therapy
versus surgical intervention. The majority of these patients can
be managed by placing the infant in the prone position until adequate
growth of the jaw occurs. This causes the jaw and the tongue to fall
forward opening the airway. If this type of treatment fails the infant
should then be considered for a tongue-lip adhesion (a procedure
to pull the tongue forward) or a tracheostomy. |
Two year old with Pierre Robin and severe airway obstruction requiring
tracheostomy
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 Bilateral
mandibular lengthening devices surgically placed with lengthening
in progress.
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In children with severe underdevelopment of the lower jaw, a new technique
called mandibular bone expansion is now available. This technique also
called distraction osteogenesis involves placement of an expansion device
that is turned daily to slowly lengthen the jaw. An external incision
is required to make a surgical cut through the jaw bone with placement
of pins that are secured to the expansion device. Once the amount of
expansion of the bone has been obtained (4-5 weeks) the device is then
kept in place until the bone gap heals with new bone formation
(8 weeks). This technique can be performed at a very early age which
is a significant advantage over the traditional technique of lower jaw
lengthening.
Results after removal of lengthening devices. Patient now ready for removal/closure of trachiostomy. 
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