TIMING OF TREATMENT
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Craniofacial malformations are very diverse and
optimal timing is different from case to case : the timing varies
from operating in infancy - the first six months - for an isolated
craniosynostosis to operating from 4 to 5 years of age for hypertelorism.
Therefore, it is essential to take advice from
a craniofacial team during the first months of life to :
- establish the diagnosis,
- to evaluate the severity of the situation,
- to determine the plan of treatment.
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Craniosynostosis |
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The ideal time for correction is between 4 months and 12 months
of age, according to the anomaly.
We see an increasing number of positionnel deformities of skull, which
are not craniosynostosis, and need a simple early nursing treatment
(cf « conditions treated »)
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Faciocraniosynostosis
(Crouzon, Apert, etc.) |
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An operation on the frontal and cranial part of the malformation
is usually also performed between 4 and 12 months of age. The
facial retrusion is corrected later : in severe cases, before
school age, around 5 years. In moderate cases, one waits until
after final dentition, around 12 years of age.
This timing can be modified
- in very severe cases with breathing
problems,
- with the use of gradual advancement
by distraction in selected cases.
In most cases, a late advancement of the upper
teeth through an oral approach (Le Fort I) will be necessary
in adolescence, as well as different facial aesthetic touch-ups
(eyelids, chin, forehead) to obtain the best possible final
result.
The principle of simultaneous correction
of frontal and facial retrusion in faciocraniosynostosis is logical,
but the inherent morbidity of a classical monobloc fronto-facial
advancement is significant (50%). This morbidity limits the indications
of the procedure and must be discussed at length prior to it being
undertaken.
The techniques of distraction first used in the mandible have been
progressively applied to the rest of the craniofacial skeleton, which
has led to a simplification of the procedures involved. The addition of
distraction to the numerous other craniofacial procedures has permitted
the progression surgical interventions for morphological modification.,
while reducing their morbidity. This has been confirmed in the case of
the monobloc advancement in various small series, as well as in our own
experience. We initially saw occasional minor complications, but with
increased experience, these allowed a more defined protocol to be
devised, as follows:
- Distractor fixation with metallic screws as the
period of their use has been increased to 6 months. A new type of
resorbable fixation is being studied.
- Re-inforcement of bone with resorbable plates and screws, if required
- The use of Molina caps at the end of the temporo-malar distractor
- Obturation of nasal fossa
- Complete pterygo-maxillary disjunction, division of midline structures and intra-operative postional control
- At least 5mm of on table advancement, in order to augment the intra-cranial volume from the start
- Bone paste application in the coronal region to encourage eventual re-ossification
- Maintenance in ICU with ventilation for at least 24-48 hours.
- Distraction commencement is deferred until between day 7 and day 15 in case of a CSF leak.
- A distraction rate of 0.3 – 0.5 mm/day
- Maintenance of the distractors in place for at least
6 months after the end of the distraction process (which renders
external distractors contra indicated).
The protocol for antibiotic prophylaxis
remains unstandardised as the monobloc procedure, goes beyond the usual
confines of surgery. The combination of the use of distractors with a
complete fronto-facial osteotomy, poses the problem of a transitory
implantation of constantly contaminated prostheses. We currently opt
for preventative antibiotic therapy of short duration (48hrs) begun at
induction, with the option of restarting therapy if signs of clinical
infection are evident. It could be however, that broad spectrum
prophylactic antibiotic therapy is in fact useful for the entire period
of distraction, despite the risk of the development of resistant
bacterial strains.
The absence of ossification at the frontal osteotomy sites seems to
contradict the usual osteogenecity found at most sites of distraction.
It does not however pose any problem, if the procedure is carried out
early as secondary cranial osteogenesis, remains possible until 2 years
of age. This reinforces our conviction of the benefits of early
distraction, before the age of 12 months and allows the question of
whether the one stage strategy should be adopted as routine, if
morbidity is reduced and the stability of results is confirmed.
Since frontofacial monobloc advancement can be applied to the more
severe neonatal deformities as well as the more minor, more slowly
progressive deformities, much more frequent use of distraction for the
technique is justified. This early series has allowed us to define a
one stage strategy, while remaining aware that minor secondary
interventions remain indispensable to correct the ultimate growth
disturbances of faciocraniosynostosis.
In order to reduce the risks of the routine treatment of
faciocraniosynostoses, a two stage strategy has remained widely
practised for twenty years as it has allowed a dissociation of problems
and a separation of risks.
The Monobloc frontofacial advancement allows a combination of these two
stages into one, with diminished risk. It is probably the only way in
which a patient can be weaned from his tracheotomy or an early
tracheotomy can be avoided entirely.
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Hypertelorism,
Medial Facial Clefts, Encephalocele |
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When the eyes are positioned significantly apart, an early evaluation
has to be done because there are cases where an early operation
has to be done on the skull and forehead (associated craniosynostosis
or encephalocele), on the eyelids, or the nose.
In most cases, one will not operate until the
child is 4 or 5 years old. Then the orbits and eyes will be
brought together and other anomalies corrected simultaneously.
Secondary limited operations on the nose and
eyelids are often necessary later.
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Lateral
Clefts Involving Orbits, Eyelids, Ears (Tessier Clefts, Treacher-Collins,
Hemifacial Dysplasia) |
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A deficiency of the eyelids is an emergency situation, and an
eyelid repair has to be done in the immediate post-natal period
to protect the eye.
A remodelling of the skull is sometimes done
in infancy, but usually, one waits a few years before undertaking
reconstruction. Distraction has some indications, especially
for mandibles.
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Neurofibromatosis
: Orbito-palpebral and Facial |
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An evaluation has to be made when the anomaly is detected, but
early operations are rare, with the exception of eyelid hypertrophy
that can be corrected during childhood.
A protruding eye may be corrected by a craniofacial operation,
but the timing varies for each individual case.
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Fibrous
Dysplasia, Osseous Hypertrophia |
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Often diagnosed late and slow growing, each individual case once again has to be evaluated.
The extent of the involvement will dictate the operative possibilities
and the follow-up of visual acuity is very important. A medical
treatment hoping to stabilize fibrous dysplasia is currently on
clinical trial.
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Post-traumatic
Sequelae |
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Usually, one has to wait at least six months after the accident
to allow the oedema disappear and the scars to diminish. An
evaluation can then be made, with a good CT scan, and a plan
of secondary treatment can be proposed if improvements are required.
Some rare situations (bone or devices exposed, instability,
infection, ectropion) may require a rapid intervention. Otherwise,
patience is essential.
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