Avance Frontofacial Con Distraccion

YIJOM-2364; No of Pages 6

Clinical Paper

Craniofacial Surgery

Int. J. Oral Maxillofac. Surg. 2012; xxx: xxx–xxxdoi:10.1016/j.ijom.2012.03.014, available online at http://www.sciencedirect.com

Frontofacial advancement byinternal distraction devices.A technical modification for themanagement of craniofacialdysostosis in early childhoodN. Adolphs, M. Klein, E.-J. Haberl, H. Menneking, B. Hoffmeister: Frontofacialadvancement by internal distraction devices. A technical modification for themanagement of craniofacial dysostosis in early childhood. Int. J. Oral Maxillofac.Surg. 2012; xxx: xxx–xxx. # 2012 International Association of Oral and MaxillofacialSurgeons. Published by Elsevier Ltd. All rights reserved.

Abstract. Normal craniofacial growth is characterized by a different growth pattern ofneuro- and viscerocranium. In craniofacial dysostosis (CFD) syndromes there iscomplex disturbance of this physiological growth pattern. Modern surgicalmanagement of CFD is staged with respect to the severity of the deformity, the ageand the development of the patient. Early single stage management of anteriorcranial vault deformity and midfacial retrusion in children affected by syndromiccraniosynostosis is possible when anterior cranial vault remodelling is performedtogether with gradual Le Fort III midfacial advancement. One pair of internaldistraction devices, placed in accordance with the midfacial growth vector afterinitial cranial vault remodelling, can be sufficient for this purpose. Technicalaspects of this modified frontofacial advancement procedure are presented anddiscussed based on a case report with a postoperative follow up time of 50 months.

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal dis

for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Su

0901-5027/000001+06 $36.00/0 # 2012 International Association of Oral and Maxillofacial Surge

N. Adolphs1, M. Klein2,E.-J. Haberl3, H. Menneking1,B. Hoffmeister1

1Department of Oral and MaxillofacialSurgery, Surgical Robotics and Navigation,University Hospital Charite, Campus Virchow-Klinikum, Germany; 2Fachklinik Hornheide ander Westfalischen Wilhelms-Universitat,Germany; 3Pediatric Neurosurgery, UniversityHospital Charite, Campus Virchow-Klinikum,Germany

Key words: craniofacial dysostosis; frontofacialadvancement; internal distraction devices.

Accepted for publication 12 March 2012

Normal craniofacial growth is character-ized by a different growth pattern ofneuro- and viscerocranium according toEnlow.1 The term craniosynostosisdescribes the premature fusion of cranio-facial sutures with consecutive distur-bance of craniofacial growth. Prematuresynostosis may be isolated, complex, oreven of syndromal origin leading to theexpression of specific phenotypes depend-ing on the location and extent of the

affected sutures.2 If facial growth is alsoaffected, the terms faciocraniostenosis(French)3 or craniofacial dysostosis(CFD) (Anglo-American)4 may be appliedto describe the inclusion of both neuro-and viscerocranium. Regarding the surgi-cal therapy, nonsyndromic craniosynosto-sis has to be distinguished from CFDsyndromes with the involvement of multi-ple sutures. For single suture synostosis,current surgical management is focused on

the release of the affected suture andregional decompression to enable braingrowth and reconstruction of a normalanatomical shape. The surgical strategydepends mainly on the extent of cranialdeformity and the patient’s age at surgery.According to Ruiz, in most cases ofsingle-suture synostosis one procedurethat simultaneously releases the sutureand reshapes the skull is the definitivetherapy.5

traction devices. A technical modification

rg (2012), doi:10.1016/j.ijom.2012.03.014

ons. Published by Elsevier Ltd. All rights reserved.

http://dx.doi.org/10.1016/j.ijom.2012.03.014



2 Adolphs et al.


Fig. 1. Preoperative situation at 30 months ofage. Severe form of Crouzon’s syndrome withproptosis and upper airway impairment; fron-tal view.

Fig. 3. Three dimensional model of the patient with internal distraction devices. Osteotomydesign and segmentation according to P. Tessier. Frontal cover, bandeau and midfacialmobilization at the Le Fort III level.

In complex forms, in which the mid-facial structures are affected, a progressivecourse of the malformation might requirerepeated corrective procedures. Modernsurgical concepts favour individuallystaged management related to age anddeformity.4,6,7 Early craniofacial correc-tion within the first years of life is relatedto neurosurgical and functional indica-tions (intracerebral hypertension, com-pression of the optic nerve, proptosis/exorbitism with corneal exposure, severeupper airway impairment).8 Depending onthe urgency of intervention surgical tech-niques are adapted.3 During infancy up toschool age, surgery is focused on cranialdecompression and reshaping procedureswith respect to the location of the cranialcompression. The correction of midfacialdeformities due to aesthetic and psycho-social indications can be postponed and isperformed during childhood and adoles-cence (depending on the severity of thedeformity and individual psychological

Please cite this article in press as: Adolphs N

for the management of craniofacial dysostos

Fig. 2. Lateral view of the preoperative situa-tion.

strain). Final reconstruction of craniofa-cial deformities is recommended from 6years onwards and should achieve stableadult dimensions in the cranio-orbito-

, et al. Frontofacial advancement by internal dis

is in early childhood, Int J Oral Maxillofac Sur

Fig. 4. Intraoperative situation after midfacialAnterior cranial vault remodelling and decomprbone cover and reshaping of the ‘bandeau’. Bothmidfacial complex, which was advanced by bilateLe Fort III disimpaction.

zygomatic regions. Correction of occlusalrelations is less important and can beachieved by orthognathic surgery afterskeletal maturity.4,9 Staged management


g (2012), doi:10.1016/j.ijom.2012.03.014

mobilization and mounting of the devices.ession were achieved after removal of frontal

parts were set back and left floating above therally mounted internal distraction devices after


Frontofacial advancement by internal distraction devices 3


Fig. 5. Intraoperative situation after midfacial mobilization and mounting of the devices (rightside view).

is difficult for the patient due to the per-sisting obtrusive appearance and repeatedprocedures with an increased risk of infec-tion. Craniomaxillofacial distractionosteogenesis has become an acceptedtechnique, as severe maxillofacial growthdeficiencies can be compensated for usingthe principle of gradual lengthening ofbone and surrounding soft tissues accord-ing to Ilizarov.10–12 There are still con-troversies about indications, limitationsand technical specifications.

If midfacial retrusion and anterior cra-nial vault malformation are to beaddressed simultaneously in CFD, the sur-gical plan might consist of anterior cranialvault remodelling by a floating techniqueaccording to Marchac, in combinationwith advancement of the midfacial com-plex after Le Fort III disimpaction by onepair of internal distraction devices. The



authors present their experience with thistechnical modification in a 30-month-oldpatient affected by Morbus Crouzon. Thefollow up since the craniofacial procedureis 50 months.

Case report

A male patient from Estonia, the youngestof four children of healthy non consangui-neous parents, affected with a severe formof Crouzon’s syndrome was referred to theauthors’ institution for secondary cranio-facial correction predominantly due to afunctional indication. Early frontaldecompression within his first year of lifewas performed in Estonia due to increasedintracerebral pressure. At 30 months thepatient was referred to the authors’ insti-tution due to increasing functional impair-ment caused by the syndromal growth


is in early childhood, Int J Oral Maxillofac Su

deficiency of the anterior skull base andmidface and the corresponding syndromalappearance. Preoperative photographicdocumentation clearly demonstratedsevere proptosis and mouth breathingcaused by upper airway impairment (Figs1 and 2). A non-invasive preoperativeneurological and ophthalmologicalcheck-up revealed no additional pathol-ogy. Increased cranial pressure could beassumed as indicated by the ‘fingerprints’on the postoperative cephalogram.

Craniofacial correction was intended asa single step procedure at the age of 30months. Decompression and fronto-orbitalremodelling was planned simultaneouslywith gradual midfacial advancement afterLe Fort III disimpaction. Osteotomieswere planned according to Tessier’sdesign for frontofacial advancement andanterior cranial vault remodelling.7

Midfacial advancement should beachieved by gradual distraction usinginternal distraction devices.13,14 For thatpurpose one pair of KLS-Martin temporaldistraction devices for adults according toMarchac (Art. 51-620-35, maximum feedof 35 mm, Gebruder Martin GmbH, 78532Tuttlingen, Germany) with Molina pivots(Art. 51-605-01, Gebruder Martin GmbH,78532 Tuttlingen, Germany) was selectedpreoperatively according to the best fit to athree dimensional model of the patient’sskull, taking into consideration that thedevices could be positioned in a parallelmanner close to the assumed centre ofresistance of the ‘facial mass’. With suffi-cient anchorage of the posterior footplatesat the mastoideal regions the devicesshould be able to push the zygomatico-maxillary complex without twisting it incorrespondence with its physiologicalgrowth pattern to a position more ante-rocaudal with reference to the anteriorskull base. The devices should providesufficient stability during the active dis-traction and consolidation phases, whichwere supposed to be achieved by theselected model (Fig. 3).

After coronal incision, subperiostealdissection and frontal osteotomy thefronto-orbital bandeau was created andmobilized. Individual fronto-orbitalremodelling was performed and Le FortIII midfacial disimpaction was achievedby transcranial, transconjunctival andtransoral approaches. Both internal dis-traction devices were inserted zygoma-tico-temporally in a parallel manner inaccordance with the preoperative model(Figs 3–5). After intraoperative activationof the devices and presetting the threads at3 mm, the frontal bone was attached to thebandeau by non-resorbable transosseous


rg (2012), doi:10.1016/j.ijom.2012.03.014


4 Adolphs et al.


Fig. 6. Cephalogram during active distraction. The midfacial mass is pushed with an ante-rocaudal vector; bandeau and frontal bone are floating above the midface.

Fig. 8. Lateral view at 3 years of age.

sutures, and both bony units were leftfloating above the advancing midface.12

The obvious convergence of the devicesto the midline had no impact on the dis-



Fig. 7. Frontal view at 3 years of age. Followup 6 months postoperatively before removalof the devices. Functional and aestheticimprovement after modified frontofacialadvancement.

traction process or the clinical result (Figs4–6). Subgaleal wound drainage wasplaced and removed on the second post-operative day. Wound closure was carriedout with resorbable sutures. Prophylacticantibiotic treatment consisted of fluclox-acillin. The operation time was about 7 h.

Active distraction was started after 6days of latency with a rate of 1 mm/dayunder intensive care conditions, continuedfor 15 days and terminated after overcor-rection as assessed by the dental overjet.18 mm of total activation, measured afterremoval of the threads, was achieved(Fig. 6). 5 weeks after active distraction,a frontal haematoma required drainageand antibiotic treatment. No other compli-cations occurred. After 6 months ofconsolidation the devices were removed.Care and hygiene of the devices during theconsolidation period were maintained bythe patient’s parents without problems.

Results

Frontal decompression and simultaneousmidfacial advancement at 30 monthsresulted in relevant functional improve-ment and obvious correction of the cra-niofacial appearance at the time ofmaterial removal, 6 months after the cra-niofacial procedure. Clinically, proptosisand upper airway impairment werereduced. Midfacial advancement resulted



in an improved naso-zygomatico-maxil-lary projection with a physiological pro-file, compared to the preoperativesituation (Figs 2, 7 and 8). Slight residualhypertelorism was not addressed by theprocedure and required no surgical correc-tion for the time being. With respect to thedental situation, regular overjet wasachieved, but the pre-existing anterioropen bite persisted postoperatively andwill be managed when permanent denti-tion is complete. The patient’s motherreported a marked increase in psychomo-tor development after the procedureduring the consolidation period. No neu-rological examination was performedpostoperatively as the preoperative statuswas unremarkable. From the psychosocialpoint of view, persistent improvement inthe patient’s social acceptance wasreported by the family. Follow up, 50months after the craniofacial procedure,shows a stable clinical situation withrespect to the midfacial advancementachieved (Fig. 9). In the meantime, man-dibular growth has flattened the initiallymore convex profile.

Discussion

There seems to be an agreement that sur-gical strategies in the treatment of patientsaffected by CFD should follow an indivi-dually adapted, staged, growth and agerelated, tailor-made concept.4,6,7 The sur-gical strategy in this patient consisted of asingle stage correction of the craniofacialdeformity with respect to the different


g (2012), doi:10.1016/j.ijom.2012.03.014


Frontofacial advancement by internal distraction devices 5


Fig. 9. Frontal view at 6 years of age. Stableclinical situation 50 months after modifiedfrontofacial advancement. Mandibular growthseems to catch up with initial midfacialadvancement hinting at residual syndromerelated maxillary deficiency.

Fig. 10. Lateral view at 6 years of age.

growth pattern of neuro- and viscerocra-nium using the principle of gradualadvancement of midfacial structures byinternal distraction devices. Frontaldecompression and fronto-orbital remo-delling was performed according to thepatient’s individual situation. Classicmonobloc osteotomy, in which thefronto-orbito and zygomaticomaxillarycomplex are mobilized and advanced asa unit, was not performed.15 After reshap-ing the ‘bandeau’ and the frontal bone,both parts were adapted by sutures and left‘floating’ above the midfacial complex,which was gradually advanced by one pairof internal distraction devices in order toprovide a correct anterocaudal position forthe midfacial structures in accordancewith the physiological growth pattern.

In the surgical therapy of severe growthdeficiencies of the craniofacial skeleton,the use of distraction osteogenesis hasbeen indicated in order to reduce the risksof classic frontofacial advancement osteo-tomies; it might also provide betterresults.10,16 In 1995, Polley et al. werethe first to describe midfacial advance-ment by an external distraction device ina newborn with extreme midfacial retru-sion.17 Chin and Toth were the first to useinternal distraction devices in maxillofa-cial applications.18 With respect to thedifferent growth pattern of neuro- andviscerocranium, Joos described the



combination of fronto-orbital advance-ment with subsequent Le Fort III osteot-omy followed by traction treatment withDelaire mask.19 In 2004, Kubler et al.published their results after simultaneousfronto-orbital advancement with Le FortIII distraction using external devices.20

The disadvantages of external distractiondevices are well known. Arnaud et al.described their experience with two pairsof internal distractors mounted with par-allel vectors for frontofacial advance-ments.21

According to Shin et al., monoblocdistraction might result in a more turrice-phalic appearance in CFD. A more aes-thetic result should be expected by adistraction design that allows the facialmass to move slightly forward of the fore-head.14

Anderson et al. described the simulta-neous advancement of the forehead with ahorizontal vector and the midface with amore vertical vector by two pairs of inter-nal distraction devices,22 but two pairs ofdistraction devices might double the riskof device related complications. The con-solidation phase was scheduled to takeplace abroad with weekly updates byemail. As is known from experimentalwork, complete remodelling of distractedbones can be expected when sufficientstability during the consolidation phaseis provided23; a long consolidation periodof 6 months was intended from the begin-ning. Owing to this and the arguable com-pliance of the patient at 30 months of age,external devices were not considered fromthe beginning. The internal devicesworked according to the treatment plan.Despite initial overcorrection mandibulargrowth has caught up in the meantime,thus hinting at the syndrome related max-illary growth deficiency, which is demon-strated by the flattened profile of thepatient at 6 years of age (Fig. 10) com-pared to the convex profile 3 years earlier(Fig. 8). Although even more midfacialadvancement would have been possibleusing the devices, active distraction wasterminated after 18 mm of activationbecause of the apprehension that furtheractivation might harm the optic nerves,considering that additional corrective sur-gery might be required in future.

In conclusion, according to this casereport, single stage correction of anteriorcranial vault pathology and midfacialretrusion by internal distraction in patientsaffected by CFD is possible in early child-hood. Appropriate device selection isemphasized in order to achieve an idealvector of midfacial movement in accor-dance with the physiological growth pat-


is in early childhood, Int J Oral Maxillofac Su

tern. If sufficient stability during the activedistraction and subsequent consolidationphase is provided, one pair of internaldistraction devices may be adequate forthat purpose.

This technical modification might con-tribute to surgical strategies for the correc-tion of complex growth deficiencies inpatients affected by CFD.

Funding

None.

Competing interests

None declared.

Ethical approval

Not required.

Acknowledgements. The authors wish toexpress their thanks to the Anaesthesiateam and the Paediatric ICU team forthe perioperative management of thepatient. Special thanks to Franz Hafnerfor his organizational skills in arrangingphotographic documentation for this arti-cle over the last years.

This case report is dedicated to HorstMenneking, MD, DDS in acknowledge-ment of his almost 35-year long lastingmaxillofacial surgical activity atthe Rudolf-Virchow-Klinikum, Berlin,Germany.


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Address:Nicolai AdolphsDepartment of Oral and Maxillofacial

SurgerySurgical Robotics and NavigationUniversity Hospital ChariteCampus Virchow-KlinikumAugustenburger Platz 113353 BerlinGermanyTel: +49 30 450 555022Fax: +49 30 450 555901E-mail: [email protected]


g (2012), doi:10.1016/j.ijom.2012.03.014

http://dx.doi.org/10.1097/01.prs.0000233010.15984.4d



http://dx.doi.org/10.1097/01.scs.0000235110.92988.fb



http://dx.doi.org/10.1016/j.cps.2004.03.009

http://dx.doi.org/10.1016/j.cps.2004.03.009

http://dx.doi.org/10.1097/01.prs.0000 278068.99643.8e



http://dx.doi.org/10.1016/j.jcms.2004.04.009


http://dx.doi.org/10.1016/j.bjps.2004.12.029

http://dx.doi.org/10.1016/j.bjps.2004.12.029



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Avance Frontofacial Con Distraccion