The treatment of dentofacial deformities is

CASE REPORT Orthodontic and surgical treatment of a patient with hemifacial microsomia Gustavo Zanardi, a Eduardo Varela Parente, b Lucas Senhorinho Esteves, b Rafael Seabra Louro, c and Jonas Capelli Junior d Rio de Janeiro and Niteroi, Brazil This article describes the surgical and orthodontic treatment of a 12-year-old boy with a significant deformity and functional involvement caused by hemifacial microsomia. The left mandibular ramus and condyle were hypoplastic and abnormal in form and location. The lower third of the face was increased, with mandibular retrusion and significant facial asymmetry. He had difficulties in speaking and chewing and problems related to his facial appearance, which caused severe psychosocial disturbances. The patient received orthodontic treatment and temporomandibular 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 is challenging to orthodontists and maxillofacial surgeons. Attaining good functional, esthetic, and stable results is even more difficult if the patient is still growing and has associated pathologies such as hemifacial microsomia. Hemifacial microsomia is the second most common facial birth disorder after cleft lip and palate, 1 with an incidence of 1 in 3500 to 6000 live births. 2 The condition is bilateral in about 10% of these subjects. 3 The cause is unknown, but the pathogenesis seems to be attributable to damage to the stapedial artery, which can cause hematoma formation in the first and second branchial arches, resulting in abnormal growth and malformation of the mandible. 4 Another theory suggests that the death of neural crest cells can result in dysmorphology of the branchial arches that is similar to that found in hemifacial microsomia. 5 The most important clinical findings in hemifacial microsomia are mandibular malformation with facial a Postgraduate student, Department of Orthodontics, State University of Rio de Janeiro, Rio de Janeiro, Brazil. b Postgraduate student, Department of Oral and Maxillofacial Surgery, State University of Rio de Janeiro, Rio de Janeiro, Brazil. c Assistant professor, Department of Oral and Maxillofacial Surgery, Federal Fluminense University, Niteroi, Brazil. d Professor, Department of Orthodontics, State University of Rio de Janeiro, Rio de Janeiro, Brazil. The authors report no commercial, proprietary, or financial interest in the products or companies described in this article. Reprint requests to: Gustavo Zanardi, Av Visconde de Piraja, 414 Sl. 1313, Ipanema, Rio de Janeiro, RJ, Brazil 22410-002; e-mail, gugazanardi@hotmail. com. Submitted, December 2010; revised and accepted, February 2011. 0889-5406/$36.00 Copyright Ó 2012 by the American Association of Orthodontists. doi:10.1016/j.ajodo.2011.02.028 asymmetry and microtia. Hypoplasia of the soft tissues, orbital involvement, nerve disorders, and other affected anatomic structures are present with a wide range of variations. 6,7 Therefore, different modalities of treatment might be needed depending on the age of the patient and the severity of the problems. The purpose of this article was to describe the treatment of a patient with a severe facial deformity due to hemifacial microsomia. Combined orthodontic treatment and orthognathic surgery with a costochondral graft were performed while the patient was still growing. Three-year follow-up records are shown. DIAGNOSIS AND ETIOLOGY The patient came to the Oral and Maxillofacial Surgery Department of the State University of Rio de Janeiro, Rio de Janeiro, Brazil, with a severe facial deformity with functional and esthetic involvement. He had been seeing an orthodontist since he was 8 years of age, and facial photographs and dental casts from this time were available (Figs 1 and 2). When he came to our institution, he was wearing fixed orthodontic appliances, which were placed when he was 12 years old. His chief complaints were related to his facial appearance and functional problems that were causing severe psychosocial disturbances. Clinical evaluation showed vertical maxillary excess, mandibular retrusion, and significant facial asymmetry with chin deviation to the left side. A marked occlusal cant in the frontal plane was present with tilting of the corners of the mouth. The left external ear was malformed, with a rudimentary auricle (Fig 3). The most noticeable functional problems were difficulties in speaking and chewing, lack of lip seal, S130

Zanardi et al S131 Fig 1. Initial facial photographs. Fig 2. Initial dental casts. mandibular deviation during function, and no reproducible centric occlusion. Intraoral photographs showed a Class I malocclusion with a Class III tendency and a mild, lateral open bite on the right side; both dental midlines were deviated (Fig 4). Tomographic and prototyping examinations showed type II-B hemifacial microsomia with a hypoplastic left mandibular ramus and condyle that were abnormal in form and location, and malformation of the glenoid fossa (Figs 5 and 6, Table). TREATMENT OBJECTIVES Surgical and orthodontic treatment objectives were identified. Because the patient had reasonable leveling, alignment, and coordination of the arches, the main objective was to address his chief complaints and improve his self-esteem. The appliances were maintained, and surgery was performed to correct the deformity. The specific objectives of treatment were (1) asymmetry correction, (2) maxillary impaction and leveling, (3) mandibular advancement with left temporomandibular joint reconstruction, (4) overjet and overbite rectification, and (5) lip seal improvement. TREATMENT ALTERNATIVES The main alternatives to correct this deformity were (1) orthopedic and orthodontic camouflage, (2) distraction osteogenesis (3) temporomandibular joint reconstruction, and (4) orthognathic surgery. American Journal of Orthodontics and Dentofacial Orthopedics April 2012 Vol 141 Issue 4 Supplement 1

S132 Zanardi et al Fig 3. Preoperative facial photographs. Fig 4. Preoperative intraoral photographs. Fig 5. Preoperative radiographic films. The first alternative is a more conservative approach, indicated for growing patients with minor deformities. However, in this patient, camouflage of the skeletal problem would be limited to occlusal correction without facial esthetic improvement. Distraction osteogenesis can be useful when extensive lengthening of the mandible is required. However, this therapy has shown a variable rate of recurrence, problems with intraoral and extraoral devices, and lack of long-term follow-up reports. For these reasons, we did not choose distraction osteogenesis for this patient. Orthognathic surgery is indicated to obtain adequate jaw relationships and facial symmetry when the temporomandibular joint is functional. In more severe cases, temporomandibular joint reconstruction with orthognathic surgery might be necessary and can be accomplished by April 2012 Vol 141 Issue 4 Supplement 1 American Journal of Orthodontics and Dentofacial Orthopedics

Zanardi et al S133 Fig 6. Preoperative tomographic 3-dimensional reconstructions and prototyping models. Table. Cephalometric measurements Standard Presurgical Postsurgical Posttreatment SNA angle ( ) 82 82.62 81.24 81.38 SNB angle ( ) 80 77.19 79.87 80.55 ANB angle ( ) 2 5.43 1.37 0.83 FMA ( ) 25 34.23 28.21 28.72 SN-GoGn ( ) 32 38.66 31.75 29.99 1/NA ( ) 22 27.05 29.08 29.86 1-NA (mm) 4 5.77 4.27 5.06 1/NB ( ) 25 34.00 27.73 29.29 1-NB (mm) 4 11.57 5.48 6.22 1/1 ( ) 131 111.20 127.05 125.94 IMPA ( ) 93 91.01 92.24 92.88 placing bone grafts or temporomandibular joint prostheses. A costochondral graft is a common method used to reconstruct the ramus-condyle unit, because it has growth potential. The disadvantages of this method are additional donor-site morbidity, risk of graft resorption and infection, and possible asymmetrical mandibular growth. A temporomandibular joint prosthesis is a predictable Fig 7. Prototyping models for the surgery. American Journal of Orthodontics and Dentofacial Orthopedics April 2012 Vol 141 Issue 4 Supplement 1

S134 Zanardi et al Fig 8. Costochondral graft on the left side for temporomandibular joint reconstruction. option for temporomandibular joint reconstruction without donor-site morbidity. However, the financial costs are higher for this treatment, and it is preferably indicated in patients whose growth has been completed. Considering the severity of the patient s skeletal deformity, age, chief complaints, and history of psychosocial problems, we chose a treatment plan that entailed orthodontic treatment and orthognathic surgery with temporomandibular joint reconstruction and a costochondral graft. TREATMENT PROGRESS After the patient was referred to our institution, the full 0.022 3 0.028-in Roth appliance was maintained, and the surgical procedure was indicated because of his lack of motivation with treatment and his psychosocial problems. After complete leveling and alignment of the teeth, presurgical 0.019 3 0.025-in arches were placed, and the patient underwent surgery. Third molars were extracted three months before surgery. The surgical planning for this patient included using a face-bow in a different way. Because he had an abnormal external left ear, the maxillary models were transferred to the articulator based on the interpupillary and Frankfort horizontal planes. During model surgery, 1 maxillary model was repositioned as planned at the Erickson platform. Subsequently, the mandibular model was fixed at the final occlusion point, allowing for construction of the final splint. The initial maxillary and final mandibular models were then used to obtain the intermediate splint. The traditional sequence of orthognathic surgery was not followed, and the mandible was operated on first because of the lack of a reproducible centric occlusion. The prototyping models were helpful in this phase because they allowed for a preview of the surgical procedure, the prebending of the fixation plates, and a better final position of the chin (Fig 7). Orthognathic surgery was performed when the patient was 12 years 4 months of age. Maxillary surgery involved an anterior impaction of 2 mm, leveling by inferior reposition of 9 mm on the left side, and a 2-mm rotation to the left to correct the midline. A 4-mm counterclockwise advancement was made on the right side of the mandible with a sagittal split osteotomy. On the left side, a costochondral graft was performed to allow adequate advancement of the mandible and temporomandibular joint reconstruction (Fig 8). An 8-mm advancement, leveling, and a 3-mm lateralization genioplasty were also accomplished. The postoperative orthodontic phase lasted 6 months and was based on the following: improvement of the right side and the intermaxillary relationship; mild, lateral open bite; and correction of the mandibular midline deviation. Class II and box elastics were used on the right side for this purpose. When the patient turned 13 years old, his braces were removed, and he was instructed to use a maxillary circumferential retainer to maintain the achieved dental results. This decision was made because of the unpredictability of growth of the costochondral graft and the patient s lack of cooperation and oral hygiene after surgery. TREATMENT RESULTS The extraoral photographs show better facial symmetry and proportions, a straight soft-tissue profile, adequate lip seal, and a more attractive smile with residual paresis of the lower lip. The mandibular asymmetry was not entirely corrected with a deficient body contour on the left side, but satisfactory facial harmony and a better social relationship were obtained (Fig 9). The intraoral and final dental cast photographs show good intercuspation, bilateral Class I molar relationships, a 1-mm lower midline deviation to the right, a 2-mm positive overbite, and normal overjet (Figs 10 and 11). Masticatory function had improved, and a reproducible centric occlusion was obtained with incisal guidance and lateral canine disclusion. Figure 12 shows the posttreatment radiographs, and Figure 13 is an overall superimposition based on the structural method. On the computed tomography images, the cartilage in the costochondral graft immediately after surgery and 3 years later is noticeable (Fig 14). This graft showed similar growth to the mandible, resulting in a slightly lower midline deviation to the opposite side that can be seen on the 3-year posttreatment records (Figs 15-17, Table). DISCUSSION Hemifacial microsomia is a variable and asymmetric craniofacial malformation. 8 Of all causes of asymmetry, it is the most unpredictable and widely variable in its expression and response to growth modification. 9,10 In April 2012 Vol 141 Issue 4 Supplement 1 American Journal of Orthodontics and Dentofacial Orthopedics

Zanardi et al S135 Fig 9. Posttreatment extraoral photographs. Fig 10. Posttreatment intraoral photographs. Fig 11. Posttreatment dental casts. American Journal of Orthodontics and Dentofacial Orthopedics April 2012 Vol 141 Issue 4 Supplement 1

S136 Zanardi et al Fig 13. Cephalometric superimposition based on the structural method. Black lines, pretreatment tracing; red lines, 3-year post-retention tracing; blue lines, posttreatment tracing. Fig 12. Posttreatment radiographic films. our patient, treatment involved orthognathic surgery and a costochondral graft in the left temporomandibular joint to correct the asymmetry and correlated deformities. Children with mild deformities might respond favorably to functional appliance therapy, and this more conservative approach should be tried before surgery, because it can improve the esthetics and the stability of the final result. This therapy is indicated in patients from 6 to 10 years old and preferably in the mixed dentition. 8,11 Orthodontic treatment is focused on the control of dental eruptions and the correction of dentoalveolar adaptations to the asymmetric position of the jaws. 8,12 For this reason, these approaches were not attempted because our patient had a 12-year-old permanent dentition and a significant facial deformity. In more severe cases, several surgical procedures are indicated, including distraction osteogenesis, costochondral grafts, orthognathic surgery, and temporomandibular joint prostheses. Some of these procedures might be followed by relapse and usually require several operations. 6,8,9,13-18 The main advantages of distraction osteogenesis include lack of donor-site morbidity and induction of both bone and soft-tissue generation. 6,9,19 Nevertheless, distraction before skeletal maturation has shown variable recurrence of the original deformities 20 and problems with extraoral devices, which are socially inconvenient and can leave hypertrophic cutaneous scars. 19 The temporomandibular joint prosthesis is a predictable option for total joint replacement. However, these devices have a finite lifespan, do not adapt to facial growth, and are certainly controversial in growing patients. 21 In contrast, the costochondral graft is considered the gold standard for temporomandibular joint reconstruction in growing patients and is conventionally used for reconstruction of the ramus and condyle in adults and children. 21,22 It can be used on its own or combined with orthognathic surgery. 22 In patients in the mixed dentition, mandibular lengthening and creation of an open bite by a costochondral graft might minimize any secondary deformities by the vertical growth potential of the midface. 8,22 This does not apply after the permanent teeth have erupted, because vertical midfacial growth is essentially complete. Thus, orthodontically controlled eruption of the permanent teeth into the open-bite space will not be accompanied by vertical growth, and the teeth will simply be extruded. 8 In our patient, we decided to April 2012 Vol 141 Issue 4 Supplement 1 American Journal of Orthodontics and Dentofacial Orthopedics

Zanardi et al S137 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. perform orthognathic surgery with a costochondral graft to reconstruct the left temporomandibular joint because of his age, stage of dentition, and the severity of the deformity. Maintenance after surgery was also a challenge. The growth of the rib appears to be controlled by both intrinsic (growth centers) and extrinsic (functional matrix) factors and is unpredictable 23,24 ; it can be insufficient, adequate, or excessive. 25-27 Long-term follow-up is important in these cases, and occasionally additional surgical procedures are needed. The obtained occlusion at the end of treatment was American Journal of Orthodontics and Dentofacial Orthopedics April 2012 Vol 141 Issue 4 Supplement 1

S138 Zanardi et al Fig 16. Three-year postretention tomographic 3-dimensional reconstruction. Fig 17. Three-year postretention dental casts. considered satisfactory with Class I molar and canine relationships. The surgery improved the appearance, body image, and socialization of this patient. Despite the unpredictability of the graft s growth and his lack of cooperation after surgery, the 3-year postretention records showed that the results were stable (Figs 15-17). CONCLUSIONS Hemifacial microsomia can be treated before completion of facial growth to correct facial deformities, solve esthetic problems, and reduce functional and psychological disturbances. Orthognathic surgery and temporomandibular joint reconstruction with a costochondral graft might be successful alternatives for the treatment of this condition. This conclusion is suggested despite the low predictability of the results in growing patients and the possible need for additional surgical procedures. REFERENCES 1. Murray JE, Kaban LB, Mulliken JB, Evans CA. Analysis and treatment of hemifacial microsomia. In: Caronni EP, editor. Craniofacial surgery. Boston: Little, Brown and Company; 1985. p. 377-90. 2. Poswillo D. Otomandibular deformity: pathogenesis as a guide to reconstruction. J Maxillofac Surg 1974;2:64-72. 3. Converse JM, McCarthy J, Wood-Smith D, Cocarro P. Craniofacial microsomia. In: Converse JM, editor. Reconstructive plastic surgery. Philadelphia: Saunders; 1977. p. 2361. 4. Poswillo D. The pathogenesis of first and second branchial arch syndrome. Oral Surg Oral Med Oral Pathol 1973;35:301-28. 5. Johnston MC, Bronsky PT. Animal models for human craniofacial malformations. J Craniofac Genet Dev Biol 1991;11:277-91. April 2012 Vol 141 Issue 4 Supplement 1 American Journal of Orthodontics and Dentofacial Orthopedics

Zanardi et al S139 6. Molina F. Mandibular distraction: surgical refinements and long-term results. Clin Plastic Surg 2004;31:443-62. 7. Pruzansky S. Not all dwarfed mandibles are alike. Birth Defects 1969;1:120-8. 8. Kaban LB, Padwa BL, Mulliken JB. Surgical correction of mandibular hypoplasia in hemifacial microsomia: the case for treatment in early 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 dentofacial deformity. St Louis: Mosby; 2000. p. 574-644. 10. Cousley RRJ, Calvert ML. Current concepts in the understanding and management of hemifacial microsomia. Br J Plast Surg 1997;50:536-51. 11. Silvestre A, Natali G, Ianetti G. Functional therapy in hemifacial microsomia: therapeutic protocol for growing children. J Oral Maxillofac Surg 1996;54:271-8. 12. Cohen MM Jr. Variability versus incidental findings in the first and second branchial arch syndrome: unilateral variants with anophthalmia. Birth Defects Orig Artic Ser 1971;7:103-8. 13. Converse JM, Horowitz SL, Coccaro PJ, Wood-Smith D. The corrective treatment of the skeletal asymmetry in hemifacial microsomia. Plast Reconstr Surg 1973;52:221-34. 14. Kaban LB, Moses MH, Mulliken JB. Surgical correction of hemifacial microsomia in the growing child. Plast Reconstr Surg 1988;82:9-19. 15. Lauritzen C, Munro IR, Ross RB. Classification and treatment of hemifacial microsomia. Scand J Plast Reconstr Surg 1985;19:33-49. 16. Murray JE, Mulliken JB, Kaban LB, Belfer M. Twenty-year experience in maxillocraniofacial surgery: an evaluation of early surgery on growth, function and body image. Ann Surg 1979;190:320-2. 17. Munro IR. One-stage reconstruction of the temporomandibular joint in hemifacial microsomia. Plast Reconstr Surg 1980;66:669-710. 18. Ortiz-Monasterio F. Early mandibular and maxillary osteotomies for the correction of hemifacial microsomia. Clin Plast Surg 1982;3:82-91. 19. Mommaerts MY, Nagy K. Is early osteodistraction a solution for the ascending ramus compartment in hemifacial microsomia? A literature study. J Craniomaxillofac Surg 2002;30:201-7. 20. Maezzini MC, Mazzoleni F, Canzi G, Bozetti A. Mandibular distraction 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 alloplastic temporomandibular joint replacement in the growing patient. J Oral Maxillofac Surg 2009;67:1979-90. 22. Padwa BL, Mulliken JB, Maghen A, Kaban LB. Midfacial growth after costochondral graft construction of the mandibular ramus in hemifacial microsomia. J Oral Maxillofac Surg 1998;56: 122-7. 23. Isaksson OGP, Lindahl A, Nilsson A, Isgaard J. Mechanism of the stimulatory effect of growth hormone on longitudinal bone growth. Endocr Rev 1987;8:426-38. 24. Mulliken JB, Ferraro NF, Vento AR. A retrospective analysis of growth of the constructed condyle-ramus in children with hemifacial microsomia. Cleft Palate J 1989;26:312-7. 25. Motta A, Louro RS, Medeiros PJD, Capelli J Jr. Orthodontic and surgical treatment of a patient with an ankylosed temporomandibular joint. Am J Orthod Dentofacial Orthop 2007;131:785-96. 26. Guyuron B, Lasa CI. Unpredictable growth pattern of costochondral graft. Plast Reconstr Surg 1992;90:880-9. 27. Behnia H, Motamedi MH, Tehranchi A. Use of activator appliances in pediatric patients treated with costochondral grafts for temporomandibular joint ankylosis: analysis of 13 cases. J Oral Maxillofac Surg 1997;55:1408-14. American Journal of Orthodontics and Dentofacial Orthopedics April 2012 Vol 141 Issue 4 Supplement 1