Case Series
Dual Midfacial Distraction of Le Fort III minus I and Le Fort I Advancement in Syndromic Craniosynostosis: Extension of application to Younger Ages
Kaneshige Satoh1* and Nobuyuki Mitsukawa2
1Department of Plastic and Aesthetic Surgery, Kawasaki Saiwai Hospital, Japan
2Department of Plastic and Reconstructive Surgery, Chiba University, Japan
*Corresponding author: Kaneshige Satoh, Department of Plastic and Aesthetic Surgery, Kawasaki Saiwai Hospital, 31-27, Ohmiyamachi, Saiwai-ku, Kawasaki, Kanagawa, 212- 0014, Japan
Published: 03 Mar, 2017
Cite this article as: Satoh K, Mitsukawa N. Dual Midfacial
Distraction of Le Fort III minus I and
Le Fort I Advancement in Syndromic
Craniosynostosis: Extension of
application to Younger Ages. Clin Surg.
2017; 2: 1328.
Abstract
Objective: Le Fort III distraction or mono bloc fronto-facial distraction has been widely applied for
the midfacial recession in syndromic craniosynostosis patients. Conversely considering the degree
of upper and lower half of the midfacial recession, dual midfacial distraction of Le Fort III minus I
and Le Fort I advancement in Syndromic craniosynostosisis a useful procedure to reduce morbidity
of adolescence and adult patients. Here the authors describe the application of extension to younger
patients, exhibiting several case reports.
Method: 14 cases of Crouzon disease and 4 Apert syndrome are included in this series. Age ranges
10 to 32 years of age. In three cases with severe maxilla-mandibular discrepancy, mandibular setback
surgery with sagittal splitting osteotomy was associated with. 4 cases of 10-12 years of age, 4
cases of 13-16 years of age, and 6 cases of 17-32 years of age have been distributed. In all cases, after
the Le Fort III osteotomy, Le Fort I osteotomy is added. In the upper portion of Le Fort III minus I,
the internal device was used, and in the lower half of Le Fort I segment, bone borne distraction using
soft wire connected to Halo device is used. Distraction has been completed from 3-5 days after the
surgery to the end of gradual distraction of 1 mm per day.
Result: The amount of distraction was noticed in 15-20 mm in the upper half of the midface and in
12-18 mm in the lower half of midface. No particular complications were noticed including tooth
germ injury at the osteotomy and in the distraction phase. Halo device was able to be removed in
around 3-4 weeks.
Conclusion: Dual midfacial distraction of Le Fort III minus I and Le Fort I is an ideal technique
to apply midfacial recession in syndromic craniosynostosis. This report verified the application to
younger ages of 10 to 12 years of aged patients.
Keywords: Syndromic craniosynostosis; Dual midfacial distraction; Maxilla-mandibular discrepancy
Introduction
Although Monoblocdistraction [1] has currently been the champion in particular for children with the craniofacial deformity in syndromic craniosynostosis, Le Fort III distraction has separately been performed worldwide after the fronto-orbital advancement for the midfacial retrusion in syndromic craniosynostosis [2]. The midface distraction by either Monobloc or Le Fort III advancement induces effectively movement of the midface enbloc. Since the relapse or growth impairment of the midface for children has been noticed after the midfacial distraction, over correction has been postulated for the midfacial distraction even if substantial degree of malocclusion is created. The surgical timing is very important for this procedure, but midfacial distraction can been performed even in any age group. However, Le Fort III distraction alone results malocclusion for elder children to adults. The procedure separating upper and lower portion is good candidates respecting the occlusion for the adolescence and adults. Dual midfacial distraction of separating Le Fort III minus I and Le Fort I portion for the adolescence and adults patients in syndromic craniosynostosis has been an ideal procedure and reported in 2004 [3]. Herein the follow-up series extending much younger patients will be described and the appropriate protocol to reduce the patients’ burden by long maintenance of Halo device has been proposed by dual midfacial distraction described above.
Table 1
Table 2
Materials and Methods
Demographic data of cases
14 cases of 10 Crouzon disease and 4 Apert syndrome are included
in this series. 8 females and 6 males are consisted of. Age ranges 10 to
32 years of age (Table 1). 4 cases of 10-12 years of age, 4 cases of 13-16
years of age, and 6 cases of 17-32 years of age have been distributed.
All the patients consulted to the orthodontists and preoperative
conference between us was routinely performed. Postoperative
continuous manage orthodontic management is definitely required
to obtain more favorable occlusion for long term. Although patients
with syndromic craniosynostosis may have some degree of airway
obstruction, in this series patients complained of no airway problems,
and no particular assessment of airway was conducted. In two adult
cases, no fronto-orbital advancement for craniosynostosis has been
performed previously. In all cases, after the Le Fort III osteotomy, Le
Fort I osteotomy is added simultaneously. In the upper portion of Le
Fort III minus I portion, internal distraction device (zygoma-zygoma
typed device, W. Lorenz, Jacksonville, USA) was attached in the
zygomatic bone. In the lower half of Le Fort I segment, bone borne
distraction using soft wire attached to the maxilla and connected to
the Halo device.
Operative procedure and postoperative protocol
Through coronal incision, subciliary, and buccal incision, the
subperiosteal undermining is completed and Le Fort III osteotomy
was accomplished, and then Le Fort I osteotomy is added. The internal
device is attached to the zygoma bilaterally and soft wire piercing to
the upper part of the maxilla of Le Fort I portion bilaterally. The welldesigned
internal device does not disturb Le Fort I osteotomy and
attachment of soft wires to Halo (Figure 1). After the scalp closure,
Halo device is attached to the temporal area. Soft wires are connected
to the Halo (Figure 1). The concept of this procedure is to obtain
the preferable occlusion controlled by Halo device and to augment
infraorbital area advancing forward using by internal device. The
amount and the direction of the distraction in the upper and lower
portion is changed according to the severity of respective cases. The
distraction of the internal device is ceased after substantial amount
of advancement. Then Halo device is taken off to release the physical
burden to the patient wearing the Halo device as early as possible.
Postoperatively, distraction has started on 3-5th day and 1 mm
(2 turns) advancement per day was conducted. The internal device
applied to the upper portion of the midface is kept in place for
consolidation for around six months.
Figure 1
Figure 1
A schema of dual midfacial distraction of Le Fort III minus I and le
Fort I, in the upper portion of Le Fort III minus I, internal distraction device is
attached and in the lower portion of Le Fort I, Halo external device is attached.
Figure 2
Figure 2
Case 1; A: Preoperative frontal view, Preoperative lateral view, midfacial retreat of Crouzon disease obviously revealed. B: During the retention period.
Left; Halo device is attached to the skull Right; lateral cephalogram reveals midfacial advancement by distraction. C: 7 years postoperative views (frontal and lateral), significant improvement of the facial configuration noticed. D: Above, Preoperative occlusal view, below, 7 years postoperative occlusal view.
Figure 3
Figure 3
Case 2; A: preoperative views (frontal and lateral view), Typical midfacial retreat is noticed. B: left; 3D-CT reveals the midfacial recession is revealed.
Right; in the orthopan-tomogram, low positioned dental radix is noticed at the age of 10 years. Le Fort I osteotomy should be carried out to avoid the root injury.
C: 1 year postoperative views (frontal and lateral), D: left; 1 year postoperative occlusal view, right; 1 year postoperative lateral 3D-CT view, significant skeletal
change is obviously noticed.
Figure 4
Figure 4
Case 3; A: preoperative view (frontal and lateral view), typical change of the facial contour is revealed. B: 2 years-postoperative view (frontal and lateral),
C: left preoperative lateral 3D-CT, right; 2-years postoperative 3D-CT, preoperative and 2 years postoperative change of the midfacial configuration is obviously
noticed.
Results and Complications
In 3 cases with severe maxilla-mandibular discrepancy, mandibular set-back surgery with sagittal splitting osteotomy was associated with. The amount of mandibular set-back was 5 mm in all of them. In the upper half of Le Fort III minus I portion, distraction amount ranged 15-20 mm. In the lower half of Le Fort I, distraction amount ranged 12-18 mm to obtain pre-planned preferable occlusion (Table 2). After the distraction in the upper and lower portion, Halo device has been taken off and fixed the Le Fort I osteotomy site to the upper portion with a mini-plate at the pyriformis area bilaterally under second general anesthesia. Then the patient can be freed from Halo and internal device exposure. In the distraction phase, no particular unfavorable events including tooth germ injury was noticed in any case. The internal distraction devices for the upper portion and miniplates for Le Fort I osteotomy site had been removed at 6-12 months postoperatively. Although postoperative cephalometric assessment wasn’t done in any case, no particular visible relapse was exhibited for long. Comparing with the conventional Le Fort III osteotomy with rigid fixation, distraction technique guarantees much more stability postoperatively. Any other complications were noticed for a postoperative period of 2-16 years.
Representative Cases
Case 1
16 years of aged boy, Crouzon disease (Case 5 in Table 1).
Fronto-orbital advancement had been conducted at the age of 1
year at nearby children’s hospital. The patient was referred to us to
consult the facial deformity with recessed midface at the age of 16
years. Le Fort III advancement was required to repair facial deformity.
He was a high school boy, and more than a month of inconvenience
wearing Halo device was not tolerated. Le Fort III minus I and Le
Fort I portion was planned to advance separately by distraction after
the osteotomy. Internal distraction device for the Le Fort III minus I
portion, and Halo distraction for Le Fort I portion is planned. At the
age of 17 years, the operation was conducted described above and
postoperatively 18 mm advancement in the upper portion and 14
mm advancement was obtained by distraction with 1 mm per day.
Three weeks later, Halo device was taken off completely and Le Fort
I portion was secured to the upper portion with preferable occlusion
by two titanium mini-plates. The internal devices were hidden
subcutaneously. Postoperative clinical course was uneventful. The
midfacial recession was corrected and the Class III malocclusion was
repaired. 10 years have passed with good result (Figure 2A and D).
Case 2
9 years of aged boy, Crouzon disease (Case 7 in Table 1).
The patient was referred to us after fronto-orbital advancement by distraction in a nearby children’s hospital to repair the craniofacial
deformity due to Crouzon disease. The patient was referred to us
to consult. The midface revealed to be retarded much with severe
exophthalmos and class III malocclusion. Improvement of the
midfacial deformity and Class III mal-occlusion was required. At the
age of 10 years, Le Fort III minus I and Le Fort I distraction separately
was planned to perform. The orthopan-tomography revealed the low
setting of the teeth. Le Fort I osteotomy was assumed to be conducted
carefully to avoid the tooth germ injury. The internal device was
attached to the upper portion and Halo device was attached to the
lower portion, and on the 5th day after the surgery, distraction with 1
mm per day was started and finalized to obtain 20 mm in the upper and 15 mm in the lower. On the 28th day after the surgery, Halo device was
taken off completely and the lower part was secured to the upper part
in a preferable occlusion with mini-plates. The midfacial deformity
was corrected considerably and the occlusion was improved very
well. Five years have passed with good result (Figure 3A and D).
Case 3
12 years of aged boy, Crouzon disease (Case 9 in Table 1).
The patient was referred to us to repair the facial recession due to
Crouzon disease at the age of 12 years. At the 1 and half years of age,
fronto-orbital advancement was conducted in a nearby children’s
hospital to repair the syndromic craniosynostosis due to Crouzon
disease. The midface was retarded much with severe exophthalmos
and Class III malocclusion. The patient was required to improve the
midfacial deformity and the occlusion. Le Fort III minus I and Le Fort
I distraction was planned separately to perform. Le Fort I osteotomy
was conducted carefully to avoid the tooth germ injury. The internal
device was attached to the upper portion and Halo device was attached
to the lower portion, and on the 5th day after the surgery, distraction
with 1 mm per day was started and finalized to obtain 18 mm in the
upper and 13 mm in the lower part. On the 28th day after the surgery,
Halo device was taken off completely and the lower part was secured
to the upper part in a preferable occlusion with mini-plates. The
midfacial deformity was corrected very much and the occlusion was
improved very well. Four years have passed with remained operative
result (Figure 4 A,B).
Discussion
In syndromic craniosynostosis, midfacial advancement is
indispensable. Le Fort III or Monobloc distraction is routinely
performed worldwide. However, the surgical timing of these differs
depending on the patients’ severity and the age of consultation.
In children Le Fort III distraction can be planned without strict
consideration of the occlusion. Conversely, in adolescence or
adults, Le Fort III should be conducted with strict consideration
of the occlusion. Hence the first author insisted on the importance
of separate distraction of the upper and lower part of Le Fort III
distraction for adolescence or adults patients and reported the
surgical technique in 2004 [3]. Le Fort III advancement guarantees
improvement of the typical midfacial depression revealing dish face.
However, occlusal function is extremely important for adolescence
and adults. The upper and lower part of Le Fort III portion should
be advanced separately in either one-staged advancement or gradual
distraction. Halo distraction can induce large amount of advancement
by distraction, but long period of consolidation for 2-3 months
results physical and mental burden to patients. As early as possible
detachment of Halo can reduce the mental load to the patients. Halo
device can be limited to Le Fort I lower part, and consolidation of
Halo can be reduced 3 weeks. From this point of view, the upper part
of Le Fort III minus I by distraction is better secured with internal
devices.
Since all our 14 patients visited us to repair the facial unbalance,
they didn’t complain of the airway obstruction. They came to us
rather late in childhood, adolescence, and adults. No particular
evaluation of apnea-hypopnea index was assessed for any of them.
In this series, mandibular set-back was associated with in 2 cases.
Large maxillo-mandibular discrepancy is sometimes encountered
in syndromic craniosynostosis, and mandibular set-back can be
conducted simultaneously to correct the facial unbalance. Although
airway problem is often recognized in syndromic craniosynostosis, it
is basically induced by midfacial retrusion. Mandibular set-back left
no airway problems postoperatively in 2 cases.
Although the precise assessment of occlusion may not be feasible,
post-distraction occlusion is extremely important for 9-10 years
of aged children. Le Fort I distraction to obtain more preferable
occlusion makes sense. Long term orthodontic care is required for
postoperative period to observe better occlusion. Complete exchange
from baby to permanent teeth depends on the patients. Surgical
avoidance to injure teeth germ can be accomplished by careful
osteotomy in early adolescence. In our case series, the surgical timing
of Le Fort I osteotomy can be lowered down to 10 years of age case by
case, although careful follow up is required. No particular impairment
of tooth growth was noticed up to 3-7 years postoperatively.
References
- Witherow H, Dunaway D, Evans R, Nischal KK, Shipster C, Pereira V, et al. Functional outcomes in monobloc advancement by distraction using the rigid external distractor device. Plast Reconstr Surg. 2008; 121: 1311- 1322.
- Fearon JA. Halo distraction of the Le Fort III in syndromic craniosynostosis: A long-term assessment. Plast Reconstr Surg. 2005; 115: 1524-1536.
- Satoh K, Mitsukawa N, Hosaka Y. Dual midfacial distraction osteogenesis: Le Fort III minus I and Le Fort I for syndromic craniosynostosis. Plast Reconstr Surg. 2003; 111: 1019-1028.
- Uwe Klammert, Hartmut Böhm, Tilmann Schweitzer, Kristian Würzler, Uwe Gbureck, Jürgen Reuther, et al. Multi-directional Le Fort III midfacial distraction using an individual prefabricated device. J Cranio-Maxillofac Sur. 2009; 37: 210-215.