orthodontic and surgical treatment of a patient with hemifacial … · 2017. 11. 5. ·...

CASE REPORT

Orthodontic and surgical treatment of a patientwith hemifacial microsomia

Gustavo Zanardi,a Eduardo Varela Parente,b Lucas Senhorinho Esteves,b Rafael Seabra Louro,c

and Jonas Capelli Juniord

Rio de Janeiro and Niter�oi, Brazil

aPostgJaneirbPostUnivecAssisFlumidProfede JaThe aucts oReprinIpanecom.Subm0889-Copyrdoi:10

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This article describes the surgical and orthodontic treatment of a 12-year-old boy with a significant deformity andfunctional involvement caused by hemifacial microsomia. The left mandibular ramus and condyle were hypo-plastic and abnormal in form and location. The lower third of the face was increased, with mandibular retrusionand significant facial asymmetry. He had difficulties in speaking and chewing and problems related to his facialappearance, which caused severe psychosocial disturbances. The patient received orthodontic treatment andtemporomandibular joint reconstruction with a costochondral graft on the left side while he was still growing.Three-year follow-up records are presented. (Am J Orthod Dentofacial Orthop 2012;141:S130-9)

The treatment of dentofacial deformities ischallenging to orthodontists and maxillofacialsurgeons. Attaining good functional, esthetic,and stable results is even more difficult if the patient isstill growing and has associated pathologies such ashemifacial microsomia.

Hemifacial microsomia is the second most commonfacial birth disorder after cleft lip and palate,1 with anincidence of 1 in 3500 to 6000 live births.2 The conditionis bilateral in about 10% of these subjects.3 The cause isunknown, but the pathogenesis seems to be attributableto damage to the stapedial artery, which can cause he-matoma formation in the first and second branchialarches, resulting in abnormal growth and malformationof the mandible.4 Another theory suggests that the deathof neural crest cells can result in dysmorphology of thebranchial arches that is similar to that found in hemifa-cial microsomia.5

The most important clinical findings in hemifacialmicrosomia are mandibular malformation with facial

raduate student, Department of Orthodontics, State University of Rio deo, Rio de Janeiro, Brazil.graduate student, Department of Oral and Maxillofacial Surgery, Statersity of Rio de Janeiro, Rio de Janeiro, Brazil.tant professor, Department of Oral and Maxillofacial Surgery, Federalnense University, Niter�oi, Brazil.ssor, Department of Orthodontics, State University of Rio de Janeiro, Rioneiro, Brazil.uthors report no commercial, proprietary, or financial interest in the prod-r companies described in this article.t requests to: Gustavo Zanardi, Av Visconde de Piraj�a, 414 Sl. 1313,ma, Rio de Janeiro, RJ, Brazil 22410-002; e-mail, gugazanardi@hotmail.

itted, December 2010; revised and accepted, February 2011.5406/$36.00ight � 2012 by the American Association of Orthodontists..1016/j.ajodo.2011.02.028

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asymmetry and microtia. Hypoplasia of the soft tissues,orbital involvement, nerve disorders, and other affectedanatomic structures are present with a wide range of var-iations.6,7 Therefore, different modalities of treatmentmight be needed depending on the age of the patientand the severity of the problems.

The purpose of this article was to describe the treat-ment of a patient with a severe facial deformity due tohemifacial microsomia. Combined orthodontic treat-ment and orthognathic surgery with a costochondralgraft were performed while the patient was still growing.Three-year follow-up records are shown.

DIAGNOSIS AND ETIOLOGY

The patient came to the Oral andMaxillofacial SurgeryDepartment of the State University of Rio de Janeiro, Riode Janeiro, Brazil, with a severe facial deformity withfunctional and esthetic involvement. He had been seeingan orthodontist since he was 8 years of age, and facialphotographs and dental casts from this time were avail-able (Figs 1 and 2). When he came to our institution, hewas wearing fixed orthodontic appliances, which wereplaced when he was 12 years old. His chief complaintswere related to his facial appearance and functionalproblems that were causing severe psychosocialdisturbances. Clinical evaluation showed verticalmaxillary excess, mandibular retrusion, and significantfacial asymmetry with chin deviation to the left side. Amarked occlusal cant in the frontal plane was presentwith tilting of the corners of the mouth. The leftexternal ear was malformed, with a rudimentary auricle(Fig 3). The most noticeable functional problems weredifficulties in speaking and chewing, lack of lip seal,

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Fig 1. Initial facial photographs.

Fig 2. Initial dental casts.

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mandibular deviation during function, and no reproduc-ible centric occlusion. Intraoral photographs showeda Class I malocclusion with a Class III tendency andamild, lateral open bite on the right side; both dentalmid-lines were deviated (Fig 4). Tomographic and prototypingexaminations showed type II-B hemifacial microsomiawith a hypoplastic left mandibular ramus and condylethat were abnormal in form and location, and malforma-tion of the glenoid fossa (Figs 5 and 6, Table).

TREATMENT OBJECTIVES

Surgical and orthodontic treatment objectives wereidentified. Because the patient had reasonable leveling,alignment, and coordination of the arches, the main

American Journal of Orthodontics and Dentofacial Orthoped

objective was to address his chief complaints and im-prove his self-esteem. The appliances were maintained,and surgery was performed to correct the deformity.The specific objectives of treatment were (1) asymmetrycorrection, (2) maxillary impaction and leveling, (3)mandibular advancement with left temporomandibularjoint reconstruction, (4) overjet and overbite rectifica-tion, and (5) lip seal improvement.

TREATMENT ALTERNATIVES

The main alternatives to correct this deformity were(1) orthopedic and orthodontic camouflage, (2) distrac-tion osteogenesis (3) temporomandibular joint recon-struction, and (4) orthognathic surgery.

ics April 2012 � Vol 141 � Issue 4 � Supplement 1

Fig 4. Preoperative intraoral photographs.

Fig 5. Preoperative radiographic films.

Fig 3. Preoperative facial photographs.

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The first alternative is a more conservative approach,indicated for growing patients with minor deformities.However, in this patient, camouflage of the skeletalproblem would be limited to occlusal correction withoutfacial esthetic improvement.

Distraction osteogenesis can be useful when exten-sive lengthening of the mandible is required. However,this therapy has shown a variable rate of recurrence,

April 2012 � Vol 141 � Issue 4 � Supplement 1 American

problems with intraoral and extraoral devices, and lackof long-term follow-up reports. For these reasons, wedid not choose distraction osteogenesis for this patient.

Orthognathic surgery is indicated to obtain adequatejaw relationships and facial symmetry when the temporo-mandibular joint is functional. In more severe cases, tem-poromandibular joint reconstruction with orthognathicsurgery might be necessary and can be accomplished by

Journal of Orthodontics and Dentofacial Orthopedics

Fig 6. Preoperative tomographic 3-dimensional reconstructions and prototyping models.

Table. Cephalometric measurements

Standard Presurgical Postsurgical PosttreatmentSNA angle (�) 82 82.62 81.24 81.38SNB angle (�) 80 77.19 79.87 80.55ANB angle (�) 2 5.43 1.37 0.83FMA (�) 25 34.23 28.21 28.72SN-GoGn (�) 32 38.66 31.75 29.991/NA (�) 22 27.05 29.08 29.861-NA (mm) 4 5.77 4.27 5.061/NB (�) 25 34.00 27.73 29.291-NB (mm) 4 11.57 5.48 6.221/1 (�) 131 111.20 127.05 125.94IMPA (�) 93 91.01 92.24 92.88

Fig 7. Prototyping models for the surgery.

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placing bone grafts or temporomandibular joint prosthe-ses. A costochondral graft is a common method used toreconstruct the ramus-condyle unit, because it has growthpotential. The disadvantages of thismethod are additionaldonor-site morbidity, risk of graft resorption and infec-tion, and possible asymmetrical mandibular growth.A temporomandibular joint prosthesis is a predictable

American Journal of Orthodontics and Dentofacial Orthopedics April 2012 � Vol 141 � Issue 4 � Supplement 1

Fig 8. Costochondral graft on the left side for temporo-mandibular joint reconstruction.

S134 Zanardi et al

option for temporomandibular joint reconstruction with-out donor-site morbidity. However, the financial costs arehigher for this treatment, and it is preferably indicated inpatients whose growth has been completed.

Considering the severity of the patient’s skeletal de-formity, age, chief complaints, and history of psychoso-cial problems, we chose a treatment plan that entailedorthodontic treatment and orthognathic surgery withtemporomandibular joint reconstruction and a costo-chondral graft.

TREATMENT PROGRESS

After the patient was referred to our institution, thefull 0.022 3 0.028-in Roth appliance was maintained,and the surgical procedure was indicated because ofhis lack of motivation with treatment and his psychoso-cial problems. After complete leveling and alignment ofthe teeth, presurgical 0.019 3 0.025-in arches wereplaced, and the patient underwent surgery. Third molarswere extracted three months before surgery.

The surgical planning for this patient included usinga face-bow in a different way. Because he had anabnormal external left ear, the maxillary models weretransferred to the articulator based on the interpupillaryand Frankfort horizontal planes. During model surgery,1 maxillary model was repositioned as planned at theErickson platform. Subsequently, the mandibular modelwas fixed at the final occlusion point, allowing for con-struction of the final splint. The initial maxillary and finalmandibular models were then used to obtain the inter-mediate splint. The traditional sequence of orthognathicsurgery was not followed, and the mandible was oper-ated on first because of the lack of a reproducible centricocclusion. The prototyping models were helpful in thisphase because they allowed for a preview of the surgicalprocedure, the prebending of the fixation plates, anda better final position of the chin (Fig 7).

Orthognathic surgery was performed when the pa-tient was 12 years 4 months of age. Maxillary surgery


involved an anterior impaction of 2 mm, leveling by in-ferior reposition of 9 mm on the left side, and a 2-mmrotation to the left to correct the midline. A 4-mm coun-terclockwise advancement was made on the right side ofthe mandible with a sagittal split osteotomy. On the leftside, a costochondral graft was performed to allow ade-quate advancement of the mandible and temporoman-dibular joint reconstruction (Fig 8). An 8-mmadvancement, leveling, and a 3-mm lateralization gen-ioplasty were also accomplished.

The postoperative orthodontic phase lasted 6 monthsand was based on the following: improvement of theright side and the intermaxillary relationship; mild, lat-eral open bite; and correction of the mandibular midlinedeviation. Class II and box elastics were used on the rightside for this purpose. When the patient turned 13 yearsold, his braces were removed, and he was instructed touse a maxillary circumferential retainer to maintain theachieved dental results. This decision was made becauseof the unpredictability of growth of the costochondralgraft and the patient’s lack of cooperation and oralhygiene after surgery.

TREATMENT RESULTS

The extraoral photographs show better facial symme-try and proportions, a straight soft-tissue profile, ade-quate lip seal, and a more attractive smile with residualparesis of the lower lip. The mandibular asymmetrywas not entirely corrected with a deficient body contouron the left side, but satisfactory facial harmony anda better social relationship were obtained (Fig 9).

The intraoral and final dental cast photographs showgood intercuspation, bilateral Class I molar relationships,a 1-mm lower midline deviation to the right, a 2-mmpositive overbite, and normal overjet (Figs 10 and 11).Masticatory function had improved, and a reproduciblecentric occlusion was obtained with incisal guidanceand lateral canine disclusion.

Figure 12 shows the posttreatment radiographs, andFigure 13 is an overall superimposition based on the struc-tural method. On the computed tomography images, thecartilage in the costochondral graft immediately after sur-gery and 3 years later is noticeable (Fig 14). This graftshowed similar growth to the mandible, resulting ina slightly lower midline deviation to the opposite sidethat can be seen on the 3-year posttreatment records(Figs 15-17, Table).

DISCUSSION

Hemifacial microsomia is a variable and asymmetriccraniofacial malformation.8 Of all causes of asymmetry,it is the most unpredictable and widely variable in itsexpression and response to growth modification.9,10 In


Fig 10. Posttreatment intraoral photographs.

Fig 9. Posttreatment extraoral photographs.

Fig 11. Posttreatment dental casts.

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American Journal of Orthodontics and Dentofacial Orthopedics April 2012 � Vol 141 � Issue 4 � Supplement 1

Fig 12. Posttreatment radiographic films.

Fig 13. Cephalometric superimposition based on thestructural method. Black lines, pretreatment tracing; redlines, 3-year post-retention tracing; blue lines, posttreat-ment tracing.

S136 Zanardi et al

our patient, treatment involved orthognathic surgery anda costochondral graft in the left temporomandibular jointto correct the asymmetry and correlated deformities.

Children with mild deformities might respond favor-ably to functional appliance therapy, and this more con-servative approach should be tried before surgery,because it can improve the esthetics and the stabilityof the final result. This therapy is indicated in patientsfrom 6 to 10 years old and preferably in the mixed den-tition.8,11 Orthodontic treatment is focused on thecontrol of dental eruptions and the correction ofdentoalveolar adaptations to the asymmetric positionof the jaws.8,12 For this reason, these approaches werenot attempted because our patient had a 12-year-oldpermanent dentition and a significant facial deformity.

In more severe cases, several surgical procedures areindicated, including distraction osteogenesis, costo-chondral grafts, orthognathic surgery, and temporo-mandibular joint prostheses. Some of these proceduresmight be followed by relapse and usually require severaloperations.6,8,9,13-18


The main advantages of distraction osteogenesisinclude lack of donor-site morbidity and induction ofboth bone and soft-tissue generation.6,9,19 Nevertheless,distraction before skeletal maturation has shownvariable recurrence of the original deformities20 andproblems with extraoral devices, which are socially incon-venient and can leave hypertrophic cutaneous scars.19

The temporomandibular joint prosthesis is a predict-able option for total joint replacement. However, thesedevices have a finite lifespan, do not adapt to facialgrowth, and are certainly controversial in growingpatients.21

In contrast, the costochondral graft is considered thegold standard for temporomandibular joint reconstruc-tion in growing patients and is conventionally used for re-construction of the ramus and condyle in adults andchildren.21,22 It can be used on its own or combinedwith orthognathic surgery.22 In patients in themixed den-tition, mandibular lengthening and creation of an openbite by a costochondral graft might minimize any second-ary deformities by the vertical growth potential of themidface.8,22 This does not apply after the permanentteeth have erupted, because vertical midfacial growth isessentially complete. Thus, orthodontically controllederuption of the permanent teeth into the open-bite spacewill not be accompanied by vertical growth, and the teethwill simply be extruded.8 In our patient, we decided to


Fig 14. Tomographic images of the costochondral graft: A, immediately after surgery; B, 3 years later.

Fig 15. Three-year postretention extraoral and intraoral photographs.

Zanardi et al S137

perform orthognathic surgery with a costochondral graftto reconstruct the left temporomandibular joint becauseof his age, stage of dentition, and the severity of thedeformity.

Maintenance after surgery was also a challenge. Thegrowth of the rib appears to be controlled by both


intrinsic (growth centers) and extrinsic (functional ma-trix) factors and is unpredictable23,24; it can beinsufficient, adequate, or excessive.25-27

Long-term follow-up is important in these cases, andoccasionally additional surgical procedures are needed.The obtained occlusion at the end of treatment was


Fig 17. Three-year postretention dental casts.

Fig 16. Three-year postretention tomographic 3-dimensional reconstruction.

S138 Zanardi et al

considered satisfactory with Class I molar and caninerelationships. The surgery improved the appearance,body image, and socialization of this patient. Despitethe unpredictability of the graft’s growth and his lackof cooperation after surgery, the 3-year postretentionrecords showed that the results were stable (Figs 15-17).

CONCLUSIONS

Hemifacial microsomia can be treated before com-pletion of facial growth to correct facial deformities,solve esthetic problems, and reduce functional andpsychological disturbances. Orthognathic surgery andtemporomandibular joint reconstruction with a costo-chondral graft might be successful alternatives for thetreatment of this condition. This conclusion is suggested


despite the low predictability of the results in growingpatients and the possible need for additional surgicalprocedures.

REFERENCES

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2. Poswillo D. Otomandibular deformity: pathogenesis as a guide toreconstruction. J Maxillofac Surg 1974;2:64-72.

3. Converse JM, McCarthy J, Wood-Smith D, Cocarro P. Craniofacialmicrosomia. In: Converse JM, editor. Reconstructive plastic sur-gery. Philadelphia: Saunders; 1977. p. 2361.

4. Poswillo D. The pathogenesis of first and second branchial archsyndrome. Oral Surg Oral Med Oral Pathol 1973;35:301-28.

5. Johnston MC, Bronsky PT. Animal models for human craniofacialmalformations. J Craniofac Genet Dev Biol 1991;11:277-91.


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6. Molina F. Mandibular distraction: surgical refinements andlong-term results. Clin Plastic Surg 2004;31:443-62.

7. Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects1969;1:120-8.

8. Kaban LB, Padwa BL, Mulliken JB. Surgical correction of mandib-ular hypoplasia in hemifacial microsomia: the case for treatment inearly childhood. J Oral Maxillofoc Surg 1998;56:628-38.

9. Proffit WR, Turvey TA. Dentofacial asymmetry. In: Proffit WR,White RP, Sarver DM, editors. Contemporary treatment of dento-facial deformity. St Louis: Mosby; 2000. p. 574-644.

10. Cousley RRJ, Calvert ML. Current concepts in the understandingand management of hemifacial microsomia. Br J Plast Surg1997;50:536-51.

11. Silvestre A, Natali G, Ianetti G. Functional therapy in hemifacialmicrosomia: therapeutic protocol for growing children. J OralMaxillofac Surg 1996;54:271-8.

12. Cohen MM Jr. Variability versus “incidental findings” in the firstand second branchial arch syndrome: unilateral variants withanophthalmia. Birth Defects Orig Artic Ser 1971;7:103-8.

13. Converse JM, Horowitz SL, Coccaro PJ, Wood-Smith D. Thecorrective treatment of the skeletal asymmetry in hemifacialmicrosomia. Plast Reconstr Surg 1973;52:221-34.

14. Kaban LB, MosesMH,Mulliken JB. Surgical correction of hemifacialmicrosomia in the growing child. Plast Reconstr Surg 1988;82:9-19.

15. LauritzenC,Munro IR, RossRB. Classificationand treatment ofhem-ifacial microsomia. Scand J Plast Reconstr Surg 1985;19:33-49.

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17. Munro IR. One-stage reconstructionof the temporomandibular jointin hemifacial microsomia. Plast Reconstr Surg 1980;66:669-710.


18. Ortiz-Monasterio F. Early mandibular and maxillary osteotomiesfor the correction of hemifacial microsomia. Clin Plast Surg1982;3:82-91.

19. MommaertsMY, Nagy K. Is early osteodistraction a solution for theascending ramus compartment in hemifacial microsomia? A liter-ature study. J Craniomaxillofac Surg 2002;30:201-7.

20. Maezzini MC, Mazzoleni F, Canzi G, Bozetti A. Mandibular distrac-tion osteogenesis in hemifacial microsomia: long-term follow-up.J Craniomaxillofac Surg 2005;33:370-6.

21. Mercuri LG, Swift JQ. Considerations for the use of alloplastictemporomandibular joint replacement in the growing patient. JOral Maxillofac Surg 2009;67:1979-90.

22. Padwa BL, Mulliken JB, Maghen A, Kaban LB. Midfacial growthafter costochondral graft construction of the mandibular ramusin hemifacial microsomia. J Oral Maxillofac Surg 1998;56:122-7.

23. Isaksson OGP, Lindahl A, Nilsson A, Isgaard J. Mechanism of thestimulatory effect of growth hormone on longitudinal bonegrowth. Endocr Rev 1987;8:426-38.

24. Mulliken JB, Ferraro NF, Vento AR. A retrospective analysis ofgrowth of the constructed condyle-ramus in children with hemifa-cial microsomia. Cleft Palate J 1989;26:312-7.

25. Motta A, Louro RS, Medeiros PJD, Capelli J Jr. Orthodontic andsurgical treatment of a patient with an ankylosed temporomandib-ular joint. Am J Orthod Dentofacial Orthop 2007;131:785-96.

26. Guyuron B, Lasa CI. Unpredictable growth pattern of costochon-dral graft. Plast Reconstr Surg 1992;90:880-9.

27. Behnia H, Motamedi MH, Tehranchi A. Use of activator appliancesin pediatric patients treated with costochondral grafts fortemporomandibular joint ankylosis: analysis of 13 cases. J OralMaxillofac Surg 1997;55:1408-14.


Orthodontic and surgical treatment of a patient with hemifacial microsomiaDiagnosis and etiologyTreatment objectivesTreatment alternativesTreatment progressTreatment resultsDiscussionConclusionsReferences

orthodontic and surgical treatment of a patient with hemifacial … · 2017. 11. 5. ·...

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