PRINCIPLES OF TREATMENT OF MAIN CONDITIONS MANAGED
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Visible scars are avoided as far as possible. The
main approach to the craniofacial skeleton is made through the
hairy scalp. A long incision from ear to ear is performed, with
a zigzag in the temporal region. This type of incision enables
good exposure and usually remains practically invisible after
closure.
The hair is not shaved. An additional incision
is sometimes performed at the eyelid level, with no scar if inside
the eyelid, or a nearly invisible scar if in the skin of the eyelid.
Sometimes, an opening is also made in the mucosa of the mouth,
at the inner side of the lip..
In some severe cases of medial cleft and hypertelorism,
an incision on the nose, to remove an excess of skin or repair
a distortion, is necessary. Also, very exceptionally, the forehead,
eyelids or cheeks have to be approached by direct incisions.
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Craniosynostosis
- Frontocranial Remodeling |
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The deformed skull will be corrected by mobilizing the distorted
part or parts and by maintaining them in normal position by adapted
fixation.
The craniosynostosis are operated usually during
the first year of life. At this age, the reossification of bone
defects is fast, and one can leave, after repositioning, open
gaps that will close rapidly.
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A different procedure is performed for each
type of craniosynostosis
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Most common craniosynostosis
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Brachycephaly
(recessed and often vertical forehead).
An advancement of the supraorbital ridge is performed and the
forehead advanced accordingly.
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Correction of a brachycephaly. Supra orbital barr is advanced 10 to 20 mm.
Fixations are made at the facial and frontal level.
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Oxycephaly (backward tilted forehead
with pointed head).
This is usually a late-appearing deformity, between 3 and 5 years
of age. Correction is obtained by rocking and advancement of the
supraorbital ridge and frontal correction.
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Correction of an oxycephaly.Removal of supra orbital
barr A which is then advanced and rocked.Removal of flattened
forehead B which is exchanged the C of good curvature.
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Plagiocephaly (asymmetry of the
forehead, orbits and nose).
A bilateral correction is performed, advancing the recessed side
and reconstructing a one-piece forehead in normal position.
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Correction of a right plagiocephaly.Removal of
supra orbital barr A which is straightened. Removal of distorted
forehead B which is replaced by a suitable piece taken from the
cranial vault.
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Scaphocephaly (elongation and
narrowing of the skull).
When detected early, a simple operation of enlarging the medial
restricted part of the skull is sufficient. When discovered later,
or in some severe cases, a remodelling of the frontal and posterior
part of the skull has to be performed as well.
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Trigonocephaly (triangular forehead
with a midline crest).
The frontal part of the skull is corrected by straightening the
supraorbital ridge and reconstructing a normal frontal bone.
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Facial
Advancement |
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A retruded midfacial mass is observed in faciocraniosynostosis.
The upper teeth are recessed, the nose is small, the eyes are
protruding.
The correction of this anomaly is obtained by an
advancement, after an osteotomy performed to allow to mobilize
the retruded parts.
Most frequently the advancement is a Le Fort III
osteotomy, advancing the nose, the malar bones, the upper maxilla.
Sometimes a Le Fort II is performed, leaving the
malar bones in place.
A Le Fort I advances only the upper maxilla, along
with the upper teeth.
Distraction is used in selected cases to place
the advance skeleton in the desired position. An orthodontic preparation
and follow-up is very important (cf chapter « Distraction »)
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Le
Fort I
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Le
Fort II
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Le
Fort III
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Monobloc
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Hypertelorism
Correction |
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The distance between the orbits must be reduced by resecting part
of the enlarged nose.
The orbits are brought together after circumferential
osteotomies have been performed..
Sometimes, a medial vertical slit is made between
the incisor teeth. It will allow for enlarging the upper part
of the mouth.
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The excess
bone in the middle is removed and the two hemi-faces
are brought together enlarging the upper Dental arch....
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Orbital asymmetry, Orbital dystopia |
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According to the case, one will mobilize one orbit, usually as
a box, "en bloc", to bring it to the desired position
and level. Sometimes one mobilizes differently the two orbits
if an hypertelorism, an enlargement of the interorbital distance,
is associated.
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Distraction Osteogenesis : Faciocraniosynostosis treatment |
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Surgical treatment of faciocraniosynostosis involves complex techniques which must address two issues:
Prevention of cerebral damage secondary to craniosynostosis (most commonly bi-coronal or multiple).
Optimal morphological correction of facial retrusion and resultant
exorbitism (which threatens vision when severe) and of the upper
airways to improve respiratory function.
The conventional approach to the cranium involves augmentation of its
volume with a fronto-orbital advancement, best performed before one
year of age. This cranio-orbital intervention corrects the problems
associated with craniosynostosis in one single procedure in the vast
majority of cases. Cerebral growth is almost complete by three years of
age and the risks to the brain are minimal after this time.
For those in whom there is facial retrusion, correction is performed at
a variable age depending on the severity of the deformity and its
aesthetic and functional consequences. Facial growth is complete at
around 16-18 years and operative treatment is therefore performed at
this age to correct facial malformations definitively in one procedure.
Whereas this strategy is applicable for minimal deformities, it is not
possible to delay facial correction in those children who display more
severe malformation, without the risk of major psychological sequelae.
In such cases it is possible to operate sooner, accepting that since
the genetic growth disturbance will remain, further surgery will be
required in due course. Generally, the magnitude of the requisite
interventions decreases as the years pass.
Two stage strategy:
Distraction osteogenesis (distraction being the reverse of contraction)
is an a surgical option which allows a gradual separation of bone over
time. This innovative approach was initially applied to the mandible
(McCarthy in New York, Molina in Mexico, Diner in Paris), and was
subsequently applied to the bones of the craniofacial skeleton.
Since 1995, we have applied the techniques of distraction osteogenesis
to Le Fort III facial advancements. This has allowed early correction
of facial retrusion in younger children requesting treatment or
requiring treatment for respiratory compromise (snoring, sleep apnoea
or hypoxaemia in more severe cases). This approach therefore requires
two principal interventions (cranial surgery and facial distraction
surgery) followed by minor facial procedures. This two stage approach
is still the classical management, despite the fact that distraction
techniques allow further refinements in the treatment of even younger
children:
A one stage surgical strategy has existed for some time, but was almost
abandoned because of the risks involved; fronto-facial monobloc
advancement (1978, Ortiz-Monasterio) allows simultaneous correction of
the various deformities of the forehead and the face. But this
procedure which is technically complex, results in two inevitable
consequences which are associated with not insignificant danger: These
are, a retro-frontal dead space and a communication between this space
and the upper part of the nasal airways as a result of the anterior
cranial osteotomies. Major complications (meningitis, frontal bone
necrosis) can ensue, particularly when the anterior cerebral
re-expansion is not rapid enough, which is the case in adults or older
children. These risks have resulted in the majority of teams
drastically reducing the indications for classical Monobloc
advancements, despite certain technical modifications having been
proposed in order to attempt to reduce these risks; specifically
ensuring a watertight repair of the anterior fossa floor and filling
the deadspace with a flap or split forehead.
The use of distraction osteogenesis for fronto-facial monobloc
advancement has been performed since the year 2000 in our unit. We have
operated on more than 40 cases with this technique with good results,
especially with regard to the improvement of respiratory function and
the correction of exorbitism. The morbidity in the monobloc procedure
can be significantly reduced as a result of distraction. We have
developed our protocol using two pairs of external distractors which
seem to improve the effectiveness of the procedure, while also
decreasing its morbidity. The progressive experience gained has
resulted in gradual changes in the surgical technique as well as the
distraction protocol.
Surgical technique and distractors:
The surgical technique used, is a fronto-facial monobloc osteotomy,
this is performed without removal of the frontal bone. The bilateral
pterygo-maxillary disjunction aswell as the division of midline
structures are performed systematically allowing operative mobilisation
of the complete fronto-facial assembly.
The distractors used are prototypes manufactured by Martin-Medezin with
cylinders controlled by a percutaneous flexible device. Two types of
distractors are necessary; frontocranial distractors used in the
supra-orbital region and temporo-malar distractors with a rotatory axis
(modifations of the distractors originally produced by
MicroFrance-Xomed) and positioned behind the zygomas. The control
mechanism of the lower distractors are exteriorised posteriorly in all
patients. The superior distractors are also exteriorised posteriorly.
In one of our patients however, the superior distractor was
exteriorised anteriorly through the eyebrow in an approach we no longer
recommend. The screws used to fix the distractors in place are
resorbable (Bionix, diameter 2mm or Champy 2mm). When fronto-zygomatic
support is required, resorbable or metallic miniplates are used. In one
of our patients undergoing secondary surgery a reconstruction of the
bandeau had to be undertaken and a transfacial pin (Staca, 2mm) as
described by Pellerin was connected to the anterior extremity of the
two temporo-malar retractors to re-inforce the assembly and maintain
control of the facial bipartition.
Distraction protocol:
The distraction protocol was classical for the first 15 patients, with
a daily advancement of around 1mm in each of the first four patients.
The start of distraction was deferred to the 7th or 8th day. In the
immediate post-operative period, no major infective episodes were
noted, although a pyrexia of 37.5 – 38 ˚C was present in all
patients over the first few days.
The distraction was achieved with 0.9mm daily advancement of the
frontal distractors (3 turns of 3mm each) and with 1mm daily advancemt
of the temporal distractors (2 turns of 0.5mm). Distraction was
continued to the maximal extent (15mm) in the frontal distrators, which
required some 14 to 17 days, depending on the initial position of the
cylinders. The duration of temporo-zygomatic distraction was longer as
the distractors do not have a limiting abutment, and was around 20
– 28 days, until class 1 occlusion was achieved. By the end of
the distraction period, the control mechanisms of the distractors were
sectioned flush with the skin and an arrangement made to remove the
distractors at least 3 months later, in the first 6 patients. This
consolidation period has progressively increased to 9 months with
subsequent patients, in order to decrease the degree of bony resorbtion
and fronto facial retraction seen in distractors removed too early.
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