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

Clinical Paper Craniofacial Surgery

Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood
N. Adolphs, M. Klein, E.-J. Haberl, H. Menneking, B. Hoffmeister: Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood. Int. J. Oral Maxillofac. Surg. 2012; xxx: xxxxxx. # 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved. Abstract. Normal craniofacial growth is characterized by a different growth pattern of neuro- and viscerocranium. In craniofacial dysostosis (CFD) syndromes there is complex disturbance of this physiological growth pattern. Modern surgical management of CFD is staged with respect to the severity of the deformity, the age and the development of the patient. Early single stage management of anterior cranial vault deformity and midfacial retrusion in children affected by syndromic craniosynostosis is possible when anterior cranial vault remodelling is performed together with gradual Le Fort III midfacial advancement. One pair of internal distraction devices, placed in accordance with the midfacial growth vector after initial cranial vault remodelling, can be sufcient for this purpose. Technical aspects of this modied frontofacial advancement procedure are presented and discussed based on a case report with a postoperative follow up time of 50 months.

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

Department of Oral and Maxillofacial Surgery, Surgical Robotics and Navigation, , University Hospital Charite Campus VirchowKlinikum, Germany; 2Fachklinik Hornheide an der Westfalischen Wilhelms-Universitat, Germany; 3Pediatric Neurosurgery, University , Hospital Charite Campus Virchow-Klinikum, Germany
1

Key words: craniofacial dysostosis; frontofacial advancement; internal distraction devices. Accepted for publication 12 March 2012

Normal craniofacial growth is characterized by a different growth pattern of neuro- and viscerocranium according to Enlow.1 The term craniosynostosis describes the premature fusion of craniofacial sutures with consecutive disturbance of craniofacial growth. Premature synostosis may be isolated, complex, or even of syndromal origin leading to the expression of specic phenotypes depending on the location and extent of the
0901-5027/000001+06 $36.00/0

affected sutures.2 If facial growth is also affected, the terms faciocraniostenosis (French)3 or craniofacial dysostosis (CFD) (Anglo-American)4 may be applied to describe the inclusion of both neuroand viscerocranium. Regarding the surgical therapy, nonsyndromic craniosynostosis has to be distinguished from CFD syndromes with the involvement of multiple sutures. For single suture synostosis, current surgical management is focused on

the release of the affected suture and regional decompression to enable brain growth and reconstruction of a normal anatomical shape. The surgical strategy depends mainly on the extent of cranial deformity and the patients age at surgery. According to Ruiz, in most cases of single-suture synostosis one procedure that simultaneously releases the suture and reshapes the skull is the denitive therapy.5

# 2012 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014

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Fig. 1. Preoperative situation at 30 months of age. Severe form of Crouzons syndrome with proptosis and upper airway impairment; frontal view.

In complex forms, in which the midfacial structures are affected, a progressive course of the malformation might require repeated corrective procedures. Modern surgical concepts favour individually staged management related to age and deformity.4,6,7 Early craniofacial correction within the rst years of life is related to neurosurgical and functional indications (intracerebral hypertension, compression of the optic nerve, proptosis/ exorbitism with corneal exposure, severe upper airway impairment).8 Depending on the urgency of intervention surgical techniques are adapted.3 During infancy up to school age, surgery is focused on cranial decompression and reshaping procedures with respect to the location of the cranial compression. The correction of midfacial deformities due to aesthetic and psychosocial indications can be postponed and is performed during childhood and adolescence (depending on the severity of the deformity and individual psychological

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

strain). Final reconstruction of craniofacial deformities is recommended from 6 years onwards and should achieve stable adult dimensions in the cranio-orbito-

zygomatic regions. Correction of occlusal relations is less important and can be achieved by orthognathic surgery after skeletal maturity.4,9 Staged management

Fig. 2. Lateral view of the preoperative situation.

Fig. 4. Intraoperative situation after midfacial mobilization and mounting of the devices. Anterior cranial vault remodelling and decompression were achieved after removal of frontal bone cover and reshaping of the bandeau. Both parts were set back and left oating above the midfacial complex, which was advanced by bilaterally mounted internal distraction devices after Le Fort III disimpaction.

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014

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Frontofacial advancement by internal distraction devices

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

is difcult for the patient due to the persisting obtrusive appearance and repeated procedures with an increased risk of infection. Craniomaxillofacial distraction osteogenesis has become an accepted technique, as severe maxillofacial growth deciencies can be compensated for using the principle of gradual lengthening of bone and surrounding soft tissues according to Ilizarov.1012 There are still controversies about indications, limitations and technical specications. If midfacial retrusion and anterior cranial vault malformation are to be addressed simultaneously in CFD, the surgical plan might consist of anterior cranial vault remodelling by a oating technique according to Marchac, in combination with advancement of the midfacial complex after Le Fort III disimpaction by one pair of internal distraction devices. The

authors present their experience with this technical modication in a 30-month-old patient affected by Morbus Crouzon. The follow up since the craniofacial procedure is 50 months.
Case report

A male patient from Estonia, the youngest of four children of healthy non consanguineous parents, affected with a severe form of Crouzons syndrome was referred to the authors institution for secondary craniofacial correction predominantly due to a functional indication. Early frontal decompression within his rst year of life was performed in Estonia due to increased intracerebral pressure. At 30 months the patient was referred to the authors institution due to increasing functional impairment caused by the syndromal growth

deciency of the anterior skull base and midface and the corresponding syndromal appearance. Preoperative photographic documentation clearly demonstrated severe proptosis and mouth breathing caused by upper airway impairment (Figs 1 and 2). A non-invasive preoperative neurological and ophthalmological check-up revealed no additional pathology. Increased cranial pressure could be assumed as indicated by the ngerprints on the postoperative cephalogram. Craniofacial correction was intended as a single step procedure at the age of 30 months. Decompression and fronto-orbital remodelling was planned simultaneously with gradual midfacial advancement after Le Fort III disimpaction. Osteotomies were planned according to Tessiers design for frontofacial advancement and anterior cranial vault remodelling.7 Midfacial advancement should be achieved by gradual distraction using internal distraction devices.13,14 For that purpose one pair of KLS-Martin temporal distraction devices for adults according to Marchac (Art. 51-620-35, maximum feed of 35 mm, Gebruder Martin GmbH, 78532 Tuttlingen, Germany) with Molina pivots (Art. 51-605-01, Gebruder Martin GmbH, 78532 Tuttlingen, Germany) was selected preoperatively according to the best t to a three dimensional model of the patients skull, taking into consideration that the devices could be positioned in a parallel manner close to the assumed centre of resistance of the facial mass. With sufcient anchorage of the posterior footplates at the mastoideal regions the devices should be able to push the zygomaticomaxillary complex without twisting it in correspondence with its physiological growth pattern to a position more anterocaudal with reference to the anterior skull base. The devices should provide sufcient stability during the active distraction and consolidation phases, which were supposed to be achieved by the selected model (Fig. 3). After coronal incision, subperiosteal dissection and frontal osteotomy the fronto-orbital bandeau was created and mobilized. Individual fronto-orbital remodelling was performed and Le Fort III midfacial disimpaction was achieved by transcranial, transconjunctival and transoral approaches. Both internal distraction devices were inserted zygomatico-temporally in a parallel manner in accordance with the preoperative model (Figs 35). After intraoperative activation of the devices and presetting the threads at 3 mm, the frontal bone was attached to the bandeau by non-resorbable transosseous

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014

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

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

sutures, and both bony units were left oating above the advancing midface.12 The obvious convergence of the devices to the midline had no impact on the dis-

traction process or the clinical result (Figs 46). Subgaleal wound drainage was placed and removed on the second postoperative day. Wound closure was carried out with resorbable sutures. Prophylactic antibiotic treatment consisted of ucloxacillin. The operation time was about 7 h. Active distraction was started after 6 days of latency with a rate of 1 mm/day under intensive care conditions, continued for 15 days and terminated after overcorrection as assessed by the dental overjet. 18 mm of total activation, measured after removal of the threads, was achieved (Fig. 6). 5 weeks after active distraction, a frontal haematoma required drainage and antibiotic treatment. No other complications occurred. After 6 months of consolidation the devices were removed. Care and hygiene of the devices during the consolidation period were maintained by the patients parents without problems.
Results

in an improved naso-zygomatico-maxillary projection with a physiological prole, compared to the preoperative situation (Figs 2, 7 and 8). Slight residual hypertelorism was not addressed by the procedure and required no surgical correction for the time being. With respect to the dental situation, regular overjet was achieved, but the pre-existing anterior open bite persisted postoperatively and will be managed when permanent dentition is complete. The patients mother reported a marked increase in psychomotor development after the procedure during the consolidation period. No neurological examination was performed postoperatively as the preoperative status was unremarkable. From the psychosocial point of view, persistent improvement in the patients social acceptance was reported by the family. Follow up, 50 months after the craniofacial procedure, shows a stable clinical situation with respect to the midfacial advancement achieved (Fig. 9). In the meantime, mandibular growth has attened the initially more convex prole.
Discussion

Fig. 7. Frontal view at 3 years of age. Follow up 6 months postoperatively before removal of the devices. Functional and aesthetic improvement after modied frontofacial advancement.

Frontal decompression and simultaneous midfacial advancement at 30 months resulted in relevant functional improvement and obvious correction of the craniofacial appearance at the time of material removal, 6 months after the craniofacial procedure. Clinically, proptosis and upper airway impairment were reduced. Midfacial advancement resulted

There seems to be an agreement that surgical strategies in the treatment of patients affected by CFD should follow an individually adapted, staged, growth and age related, tailor-made concept.4,6,7 The surgical strategy in this patient consisted of a single stage correction of the craniofacial deformity with respect to the different

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014

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Frontofacial advancement by internal distraction devices

combination of fronto-orbital advancement with subsequent Le Fort III osteotomy followed by traction treatment with Delaire mask.19 In 2004, Kubler et al. published their results after simultaneous fronto-orbital advancement with Le Fort III distraction using external devices.20 The disadvantages of external distraction devices are well known. Arnaud et al. described their experience with two pairs of internal distractors mounted with parallel vectors for frontofacial advancements.21 According to Shin et al., monobloc distraction might result in a more turricephalic appearance in CFD. A more aesthetic result should be expected by a distraction design that allows the facial mass to move slightly forward of the forehead.14 Anderson et al. described the simultaneous advancement of the forehead with a horizontal vector and the midface with a more vertical vector by two pairs of internal distraction devices,22 but two pairs of distraction devices might double the risk of device related complications. The consolidation phase was scheduled to take place abroad with weekly updates by email. As is known from experimental work, complete remodelling of distracted bones can be expected when sufcient stability during the consolidation phase is provided23; a long consolidation period of 6 months was intended from the beginning. Owing to this and the arguable compliance of the patient at 30 months of age, external devices were not considered from the beginning. The internal devices worked according to the treatment plan. Despite initial overcorrection mandibular growth has caught up in the meantime, thus hinting at the syndrome related maxillary growth deciency, which is demonstrated by the attened prole of the patient at 6 years of age (Fig. 10) compared to the convex prole 3 years earlier (Fig. 8). Although even more midfacial advancement would have been possible using the devices, active distraction was terminated after 18 mm of activation because of the apprehension that further activation might harm the optic nerves, considering that additional corrective surgery might be required in future. In conclusion, according to this case report, single stage correction of anterior cranial vault pathology and midfacial retrusion by internal distraction in patients affected by CFD is possible in early childhood. Appropriate device selection is emphasized in order to achieve an ideal vector of midfacial movement in accordance with the physiological growth pat-

Fig. 9. Frontal view at 6 years of age. Stable clinical situation 50 months after modied frontofacial advancement. Mandibular growth seems to catch up with initial midfacial advancement hinting at residual syndrome related maxillary deciency.

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

growth pattern of neuro- and viscerocranium using the principle of gradual advancement of midfacial structures by internal distraction devices. Frontal decompression and fronto-orbital remodelling was performed according to the patients individual situation. Classic monobloc osteotomy, in which the fronto-orbito and zygomaticomaxillary complex are mobilized and advanced as a unit, was not performed.15 After reshaping the bandeau and the frontal bone, both parts were adapted by sutures and left oating above the midfacial complex, which was gradually advanced by one pair of internal distraction devices in order to provide a correct anterocaudal position for the midfacial structures in accordance with the physiological growth pattern. In the surgical therapy of severe growth deciencies of the craniofacial skeleton, the use of distraction osteogenesis has been indicated in order to reduce the risks of classic frontofacial advancement osteotomies; it might also provide better results.10,16 In 1995, Polley et al. were the rst to describe midfacial advancement by an external distraction device in a newborn with extreme midfacial retrusion.17 Chin and Toth were the rst to use internal distraction devices in maxillofacial applications.18 With respect to the different growth pattern of neuro- and viscerocranium, Joos described the

tern. If sufcient stability during the active distraction and subsequent consolidation phase is provided, one pair of internal distraction devices may be adequate for that purpose. This technical modication might contribute to surgical strategies for the correction of complex growth deciencies in patients affected by CFD.
Funding

None.
Competing interests

None declared.
Ethical approval

Not required.
Acknowledgements. The authors wish to express their thanks to the Anaesthesia team and the Paediatric ICU team for the perioperative management of the patient. Special thanks to Franz Hafner for his organizational skills in arranging photographic documentation for this article over the last years.

This case report is dedicated to Horst Menneking, MD, DDS in acknowledgement of his almost 35-year long lasting maxillofacial surgical activity at the Rudolf-Virchow-Klinikum, Berlin, Germany.

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014

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hypoplasia. Otolaryngol Clin North Am 2000;33: 125784. Bradley JP, Gabbay JS, Taub PJ, Heller JB, OHara CM, Benhaim P, et al. Monobloc advancement by distraction osteogenesis decreases morbidity and relapse. Plast Reconstr Surg 2006;118:158597. 10.1097/ 01.prs.0000233010.15984.4d. Cohen SR, Burstein FD, Williams JK. The role of distraction osteogenesis in the management of craniofacial disorders. Ann Acad Med Singapore 1999;28:72838. Marchac D, Arnaud E. Midface surgery from Tessier to distraction. Childs Nerv Syst 1999;15:68194. Satoh K, Mitsukawa N, Tosa Y, Kadomatsu K. Le Fort III midfacial distraction using an internal distraction device for syndromic craniosynostosis: device selection, problems, indications, and a proposal for use of a parallel bar for device-setting. J Craniofac Surg 2006;17:10508. 10.1097/ 01.scs.0000235110.92988.fb. Shin JH, Duncan CC, Persing J. Monobloc distraction: technical modication and considerations. J Craniofac Surg 2003;14:7636. Molina F. From midface distraction to the true monoblock. Clin Plast Surg 2004;31:46379. 10.1016/j.cps.2004.03.009. Arnaud E, Marchac D, Renier D. Reduction of morbidity of the frontofacial monobloc advancement in children by the use of internal distraction. Plast Reconstr Surg 2007;120:100926. 10.1097/01.prs.0000 278068.99643.8e. Polley JW, Figueroa AA, Charbel FT, Berkowitz R, Reisberg D, Cohen M. Monobloc craniomaxillofacial distraction osteogenesis in a newborn with severe craniofacial synostosis: a preliminary report. J Craniofac Surg 1995;6:4213. 18. Chin M, Toth BA. Distraction osteogenesis in maxillofacial surgery using internal devices: review of ve cases. J Oral Maxillofac Surg 1996;54:4553. 19. Joos U. Functional treatment of craniosynostoses during childhood. Br J Oral Maxillofac Surg 1998;36:918. 20. Kubler AC, Speder B, Zoller JE. Fronto-orbital advancement with simultaneous Le Fort III-distraction. J Craniomaxillofac Surg 2004;32:2915. 10.1016/j.jcms.2004.04.009. 21. Arnaud E, Marchac D, Renier D. Distraction osteogenesis with double internal devices combined with early frontal facial advancement for the correction of facial craniosynostosis. Report of clinical cases. Ann Chir Plast Esthet 2001;46:26876. S0294126001000 437 [pii]. 22. Anderson PJ, Tan E, David DJ. Simultaneous multiple vector distraction for craniosynostosis syndromes. Br J Plast Surg 2005;58:626 31. 10.1016/j.bjps.2004.12.029. 23. Adolphs N, Kunz C, Pyk P, Hammer B, Rahn B. Callus mineralization following distraction osteogenesis of the mandible monitored by scanning acoustic microscopy (SAM). JCraniomaxillofac Surg 2005;33:3147. 10.1016/j.jcms.2005.03.002.

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Address: Nicolai Adolphs Department of Oral and Maxillofacial Surgery Surgical Robotics and Navigation University Hospital Charite Campus Virchow-Klinikum Augustenburger Platz 1 13353 Berlin Germany Tel: +49 30 450 555022 Fax: +49 30 450 555901 E-mail: nicolai.adolphs@charite.de

Please cite this article in press as: Adolphs N, et al. Frontofacial advancement by internal distraction devices. A technical modication for the management of craniofacial dysostosis in early childhood, Int J Oral Maxillofac Surg (2012), doi:10.1016/j.ijom.2012.03.014