CLINICAL STUDY
Orthognathic Surgery in Patients With Cleidocranial Dysplasia Maria Fernanda Conceic¸a˜o Madeira, DDS, MSc, Isabela Maria Caetano, DDS,y Eduardo Dias-Ribeiro, DDS, MSc,z Julierme Ferreira Rocha, DDS, MSc,z Celso Koogi Sonoda, DDS, PhD,z Eduardo Sant’Ana, DDS, PhD, and Renato Yassutaka Faria Yaedu, DDS, PhD Abstract: The aim of this study was to report the orthodonticsurgical approach of a 21-year-old female patient diagnosed with cleidocranial dysplasia. An orthognathic surgery was performed in the maxilla and mandible during the same procedure to correct an existing dentofacial deformity (class III malocclusion). In addition, malar prostheses were used to correct midface deficiency. After surgical intervention, orthodontic treatment continued in order to promote stability, function, and aesthetics. Cases of cleidocranial dysplasia treated with the defined criteria can bring aesthetic and functional benefits to the patient. Key Words: Cleidocranial dysplasia, orthodontics, orthognathic surgery, mandibular advancement (J Craniofac Surg 2015;26: 792–795)
C
leidocranial dysplasia or dysostosis (CCD) is a relatively rare autosomal dominant genetic disease (1:1,000,000) that can affect both sexes and all races.1–3 The pathognomonic sign of the syndrome is clavicular hypoplasia or aplasia. Unilateral or bilateral absence of the clavicles is observed in 10% of the cases and can result in hypermobility of the shoulders including ability to touch the shoulders together in front of the chest.4 However, in the intraoral and facial clinical examination, maxillary hypoplasia is observed, leading to pseudoprognathism. Mandibular growth is normal, with a submucosal cleft in some cases due to incomplete fusion of the symphysis region, impacted supernumerary, and also the noneruption or delayed eruption of permanent teeth.1,2 After the CCD diagnosis is established, treatment of the dentofacial deformity involves a combination of orthodontic and orthognathic surgeries.1,5–8 The protocol involves surgical removal of deciduous and supernumerary teeth and making nonerupted permanent teeth visible through orthodontically forced eruption. After the alignment of all permanent teeth, a dentofacial discrepancy is
noticed, (usually skeletal class III malocclusion), which may be corrected through orthognathic surgery after the development phase.5,7,9,10 The present study aims to demonstrate a case of CCD treated by combined orthognathic surgery for the correction of the dentofacial discrepancy.
CASE REPORT Clinical and Radiographic Findings A 21-year-old female patient was sent to the Oral and Maxillofacial Surgery Service, Bauru School of Dentistry, University of Sa˜o Paulo, Bauru, Sa˜o Paulo, Brazil, to have evaluated the possibility of combined surgical-orthodontic treatment. The patient was diagnosed as a CCD carrier when she was 6 months old, regularly observed by an orthopedic and a pediatric dentist. The patient was not satisfied with the facial aesthetics and presented difficulty eating. The initial orthodontic mechanotherapy was carried out to facilitate the eruption of permanent teeth, following the realignment, leveling, and decompensation of the dental arches before orthognathic surgery. In current medical history, there were no local or systemic conditions that prevented the surgical approach. During clinical and radiographic analysis, a middle-third facial collapse was observed with malar hypoplasia, maxillary deficiencies (transverse, sagittal, and vertical), and anterior open bite, besides class III malocclusion and inclined occlusal plane (Table 1). The lateral facial profile was slightly concave, and the face was symmetrical on frontal view, the upper dental midline was coincident, and the lower dental midline was slightly to the right (Fig. 1). The dental tissues and periodontal and temporomandibular articulations were clinically normal, without signs or symptoms of dysfunction. The panoramic radiography showed the upper third molars were not erupted and 2 supernumerary teeth: the left fourth upper molar and lower canine (Fig. 2).
Treatment Goals From the Department of Stomatology, Bauru School of Dentistry, and yDepartment of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, University of Sa˜o Paulo, Bauru; and zDepartment of Oral and Maxillofacial Surgery, Dental School of Arac¸atuba, Universidade Estadual Paulista (UNESP), Arac¸atuba; Sa˜o Paulo, Brazil. Received April 8, 2014. Accepted for publication October 28, 2014. Address correspondence and reprint requests to Eduardo Dias-Ribeiro, DDS, MSc, Department of Oral and Maxillofacial Surgery, Dental School of Arac¸atuba, UNESP–Universidade Estadual Paulista Rua Jose´ Bonifacio, 1193, Arac¸atuba, Sa˜o Paulo 16015-050, Brazil; E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001414
792
The goal of the orthodontic-surgical treatment is to reach an ideal occlusion and adequate facial profile of hard and soft tissues, providing health to the oral and periodontal tissues.11 The surgical procedure for the patient carrier of this syndrome should facilitate further psychological development and social interaction, as many of these patients are on the psychosocial margin.12
Treatment Progress After the conclusive clinical and radiographic diagnosis of maxillary and mandibular retrusion with malar hypoplasia, a bimaxillary orthognathic surgery was performed with concomitant implantation of a malar prosthesis. The surgical plan was completed with the assistance of Dolphin Imaging 11.0 software (Dolphin/3M, San Diego, CA) (licensed to Bauru School of Dentistry, University
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
Orthognathic Surgery in Cleidocranial Dysplasia
TABLE 1. Presurgical, Standard, and Posttreatment Analysis Values According to Arnett and McLaughlin13 Dentoskeletal Factors Maxilla Upper incisor inclination (Mx1 MxOP), degrees Upper incisor tip projection (Mx1 Sn), mm Mandible0 Lower incisor inclination (Md1 MdOP), degrees Lower incisor tip projection (Md1 Sn), mm Overjet (Mx1 Md1), mm Vertical Overbite (Mx1 Md1), mm Mx anterior height (Sn0 Mx1), mm Mx occlusal plane (MxOP TVL), degrees Chin height (Md1 Me0 ), mm Facial height Soft tissue height Interlabial gap (ULI LLS), mm Lower vs upper lip length (LLS Me0 Sn0 ULI), % Lower 13 of face (Sn0 Me0 ), mm Facial height (Na0 Me0 ), mm Hard tissue height Upper incisor exposure (ULI Mx1tip), mm Mx anterior height (Sn0 Mx1), mm Soft tissue thickness Upper lip thickness (Mx1 labial ULA), mm Lower lip thickness (LLinside Lloutside), mm Menton thickness (Me Me0 ), mm Projections Maxillary projection Nasal projection, mm Soft tissue A0 point (A0 ), mm Upper lip anterior (ULA Sn), mm Upper lip angle (ULA Sn0 TVL), degrees Nasolabial angle (Col Sn0 ULA), degrees Upper incisor tip projection (Mx1 Sn), mm Mandibular projection Soft tissue B0 point (B0 ), mm Lower lip anterior (LLA), mm Lower incisor tip projection (Md1 Sn), mm Facial harmony Facial angle (G0 Sn0 Pog0 ), degrees Chin to nasal base (Pog0 Sn0 ), mm Mandibular base to maxillary base (A0 B0 ), mm Lower lip to upper lip (LLA ULA), mm
of Sa˜o Paulo, Sa˜o Paulo, Brazil) pointing out the cephalometric spots described in 2004 by Arnett and McLaughlin.13 Then, surgery models for making the intermediate splint were constructed. The orthognathic surgery consisted of advancing the maxilla by 5 mm, using a Le Fort I osteotomy, and advancing the mandible by 2.4 mm through the bilateral sagittal split osteotomy of the mandible, with counterclockwise occlusion. Stabilization of the maxillary osteotomies was accomplished by a rigid internal fixation system 2.0 mm (To´ride, Sa˜o Paulo, Brazil). The hybrid fixation technique was applied to the mandible,14 through the combination of monocortical plaques and bicortical screws of the rigid 2.0-mm internal fixation system (To´ride). In addition, a malar prosthesis was placed (Medpor; Porex Surgical Inc, Kalamazoo, MI) to correct the third midface, screw fixed in the rigid 2.0-mm internal fixation system (To´ride) (Table 1). The suture was performed with Vicryl 4-0 (Ethicon; Johnson & Johnson, Sa˜o Paulo, Brazil), along with the V-Y alar base suture. The dental engagement (canine and molar— #
2015 Mutaz B. Habal, MD
Presurgical
Standard Values
Posttreatment
54.0 11.5
56.8 9.2
56.2 11.6
85.6 10.6 0.9
64.3 12.4 3.2
76.3 15.4 3.9
6.4 22.2 102.5 42.2
3.2 25.7 95.6 48.6
0.6 22.7 108.2 39.8
1.8 238.0 70.8 125.8
3.3 223.0 71.1 125.0
1.1 204.4 63.2 113.7
1.7 22.2
3.5 25.7
2.4 22.7
10.5 10.9 17.0
12.6 13.6 7.4
8.4 9.0 12.6
17.3 2.6 0.5 4.2 111.3 11.5
16.0 0.9 3.7 12.1 104.0 9.2
13.8 0.6 2.8 3.0 107.5 11.6
0.3 3.6 10.6
5.3 1.9 12.4
6.1 1.0 15.4
176.4 2.3 2.3 3.1
169.0 2.0 5.2 1.8
168.0 4.0 5.5 3.9
class I bilateral) was reached after orthodontic treatment was completed, observing harmony of facial thirds, as well as the health of oral tissue through applied treatment (Figs. 3 and 4). After 3 years of observation, there have not been any signs of malocclusion recurrence, and the patient is satisfied with the treatment and the new facial profile (Fig. 5).
DISCUSSION A CCD, because of its low morbidity, may be diagnosed late.15 The confirmation of the diagnosis may be given through a clinical examination of the skull, face, clavicle, and oral cavity. In the oral examination, it is possible to compare the state of eruption and the chronological age of the patient. In the radiographic examination, especially the panoramic view, we are able to determine the number, size, and position of the supernumerary teeth (Fig. 2). After clinical and radiographic evaluation, the patient must be sent
793
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Madeira et al
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
FIGURE 1. Pretreatment extraoral (A, B) and lateral radiographs (C) and intraoral photographs (D–F).
FIGURE 3. Posttreatment extraoral (A, B) and lateral radiographs (C) and intraoral photographs (D–F).
to genetic services for confirmation of the diagnosis, genetic counseling, and verification of other cases in the family.16 There are several therapeutic options that may be adopted to correct dentoskeletal changes in patients with CCD. The choice will depend on the health and dental condition, facial aspect, and patient expectation, considering that the goal of any treatment must include eliminating disturbance and symptoms, restoring masticatory function, and improving the patient’s appearance.6 Nowadays, the orthodontic or orthopedic treatment usually associated to orthognathic surgery and aesthetic rehabilitation are the elected procedures.1,5– 10 When there is a need for orthognathic surgery, the realignment, leveling, and decompensation of dental arches are of fundamental importance. In this context, the poor dental structure and the delayed closing of the apical portion of the root that occur in patients with CCD may influence the spontaneous eruption of permanent teeth, causing the development of crowns and roots to be delayed between 2 and 4 years, when compared with nonsyndromic patients.16 Depending on the type and gravity of the craniofacial anomaly, the timing of orthognathic surgery depends on functional problems, psychosocial factors, and facial growth pattern and should be tailored to the needs of each individual patient.12 There are few cases in the literature concerning the orthognathic surgical approach in patients with CCD. In 2006, Daskalogiannakis et al5 stated an improvement in the occlusal relationship after maxilla advancement, through Le Fort I osteotomy, carried out after skeletal maturity, which was observed in this case. In 1980, Dann et al17 showed an improvement of facial profile in the middle third, through the Le Fort I osteotomy technique, associated to the use of positional graft. The application of Le Fort I osteotomy correcting maxillary dentofacial deformities has produced predictable and stable results. Maxillary advancement provided good support for the upper lip, filled the soft-tissue midface, and favorably improved the patient’s profile.18
Since Hugo Obwegeser’s description of sagittal split ramus osteotomy in 1955, there have been many modifications of this surgical procedure, often used in orthognathic surgery. After the advent of rigid fixation systems, this procedure became more secure and easier to apply to correct mandible deformities,14 with satisfying results when applied to retreat the mandible.19 The ideal age to begin the surgical treatment in patients with no syndrome is usually from mixed dentition because the presence of supernumerary teeth may impact permanent teeth. In specific cases, orthognathic surgery is performed during the phase of development because of aesthetic and psychosocial reasons, considering the severity of dentofacial deformity secondary to less disparate enhanced growth. However, when surgery is performed prematurely, unpredictable results may occur because of the growth of unaffected tissues, and it is important to inform the patient and the family of the need for a new orthognathic surgery procedure.12,20 In 2001, Wolford et al20 demonstrated that early correction of maxillary hypoplasia in growing patients does not produce predictable results because of continued mandibular and vertical maxillary growth. The surgical procedure was chosen after maturity of the bone tissue, seeking a more predictable and stable aesthetic result, because the patient did not present symptoms and clinical signs of stomatognathic system dysfunction and related structures.
FIGURE 2. Pretreatment panoramic radiograph showing supernumerary tooth (maxillary molars) and mandibular left canine.
FIGURE 4. Superimposed cephalometric tracings (grey: posttreatment, black: pretreatment).
794
#
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
FIGURE 5. Facial aspect postoperative 3 years.
Therapy for the patient carrier of CCD is extensive, and it requires the presence of a specialized and multidisciplinary team, seeking the most predicable and stable treatment. Cases of CCD treated with well-defined criteria can bring aesthetic and functional benefits to the patient.
REFERENCES 1. Becker A, Lustman J, Shteyer A. Cleidocranial dysplasia: part 1—general principles of the orthodontic and surgical treatment modality. Am J Orthod Dentofac Orthop 1997;111:28–33 2. Chen S, Santos L, Wu Y, et al. Altered gene expression in human cleidocranial dysplasia dental pulp cells. Arch Oral Biol 2005;50:227–236 3. Furuuchi T, Kochi S, Sasano T, et al. Morfologic characteristics of masseter muscle in cleidocranial dysplasia: a report of 3 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;99:185–190 4. Butterworth C. Cleidocranial dysplasia: modern concepts of treatment and a report of an orthodontic resistant case requiring a restorative solution. Dent Update 1999;26:458–462 5. Daskalogiannakis J, Piedade L, Lindholm TC, et al. Cleidocranial dysplasia: 2 generations of management. J Can Dent Assoc 2006;72:337–342
#
2015 Mutaz B. Habal, MD
Orthognathic Surgery in Cleidocranial Dysplasia
6. Frohberg U, Tiner B. Surgical correction of facial deformities in a patient with cleidocranial dysplasia. J Craniofac Surg 1995;6:49–53 7. Hall RK, Hyland AL. Combined surgical and orthodontic management of the oral abnormalities in children with cleidocranial dysplasia. Int J Oral Surg 1978;7:267–273 8. Smylski PT, Woodside DG, Harnett BE. Surgical and orthodontic treatment of cleidocranial dysostosis. Int J Oral Surg 1974;3:380–385 9. Farrar EL, van Sickels JE. Early surgical management of cleidocranial dysplasia: a preliminary report. J Oral Maxillofac Surg 1983;41:527–529 10. Trimble LD, West RA, McNeill RW. Cleidocranial dysplasia: comprehensive treatment of the dentofacial abnormalities. J Am Dent Assoc 1982;105:661–666 11. Janson M, Janson G, Sant’ana E, et al. Segmental LeFort I osteotomy for treatment of a class III malocclusion with temporomandibular disorder. J Appl Oral Sci 2008;16:302–309 12. Chigurupati R. Orthognathic surgery for secondary cleft and craniofacial deformities. Oral Maxillofac Surg Clin North Am 2005;17:503–517 13. Arnett GW, McLaughlin RP. Facial and Dental Planning for Orthodontists and Oral Surgeons. Edinburgh, UK: Mosby; 2004 14. Pereira FL, Janson M, Sant’ana E. Hybrid fixation in the bilateral sagittal split osteotomy for lower jaw advancement. J Appl Oral Sci 2010;18:92–99 15. Nayar S, Bishop K. Cleidocranial dysplasia—a late diagnosis. Dent Update 2006;33:221–226 16. Becker A, Shteyer A, Bimstein E, et al. Cleidocranial dysplasia: part 2—treatment protocol for the orthodontic and surgical modality. Am J Orthod Dentofac Orthop 1997;111:173–183 17. Dann JJ III, Crump P, Ringenberg QM. Vertical maxillary deficiency with cleidocranial dysplasia: diagnostic findings and surgicalorthodontic correction. Am J Orthod 1980;78:564–574 18. Janson M, Janson G, Sant’ana E, et al. An orthodontic-surgical approach to class II subdivision malocclusion treatment. J Appl Oral Sci 2009;17:266–273 19. Yoshioka I, Khanal A, Tominaga K, et al. Vertical ramus versus sagittal split osteotomies: comparison of stability after mandibular setback. J Oral Maxillofac Surg 2008;66:1138–1144 20. Wolford LM, Karras SC, Mehra P. Considerations for orthognathic surgery during growth, part 2: maxillary deformities. Am J Orthod Dentofacial Orthop 2001;119:102–105
795
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Orthognathic Surgery in Patients With Cleidocranial Dysplasia Maria Fernanda Conceic¸a˜o Madeira, DDS, MSc, Isabela Maria Caetano, DDS,y Eduardo Dias-Ribeiro, DDS, MSc,z Julierme Ferreira Rocha, DDS, MSc,z Celso Koogi Sonoda, DDS, PhD,z Eduardo Sant’Ana, DDS, PhD, and Renato Yassutaka Faria Yaedu, DDS, PhD Abstract: The aim of this study was to report the orthodonticsurgical approach of a 21-year-old female patient diagnosed with cleidocranial dysplasia. An orthognathic surgery was performed in the maxilla and mandible during the same procedure to correct an existing dentofacial deformity (class III malocclusion). In addition, malar prostheses were used to correct midface deficiency. After surgical intervention, orthodontic treatment continued in order to promote stability, function, and aesthetics. Cases of cleidocranial dysplasia treated with the defined criteria can bring aesthetic and functional benefits to the patient. Key Words: Cleidocranial dysplasia, orthodontics, orthognathic surgery, mandibular advancement (J Craniofac Surg 2015;26: 792–795)
C
leidocranial dysplasia or dysostosis (CCD) is a relatively rare autosomal dominant genetic disease (1:1,000,000) that can affect both sexes and all races.1–3 The pathognomonic sign of the syndrome is clavicular hypoplasia or aplasia. Unilateral or bilateral absence of the clavicles is observed in 10% of the cases and can result in hypermobility of the shoulders including ability to touch the shoulders together in front of the chest.4 However, in the intraoral and facial clinical examination, maxillary hypoplasia is observed, leading to pseudoprognathism. Mandibular growth is normal, with a submucosal cleft in some cases due to incomplete fusion of the symphysis region, impacted supernumerary, and also the noneruption or delayed eruption of permanent teeth.1,2 After the CCD diagnosis is established, treatment of the dentofacial deformity involves a combination of orthodontic and orthognathic surgeries.1,5–8 The protocol involves surgical removal of deciduous and supernumerary teeth and making nonerupted permanent teeth visible through orthodontically forced eruption. After the alignment of all permanent teeth, a dentofacial discrepancy is
noticed, (usually skeletal class III malocclusion), which may be corrected through orthognathic surgery after the development phase.5,7,9,10 The present study aims to demonstrate a case of CCD treated by combined orthognathic surgery for the correction of the dentofacial discrepancy.
CASE REPORT Clinical and Radiographic Findings A 21-year-old female patient was sent to the Oral and Maxillofacial Surgery Service, Bauru School of Dentistry, University of Sa˜o Paulo, Bauru, Sa˜o Paulo, Brazil, to have evaluated the possibility of combined surgical-orthodontic treatment. The patient was diagnosed as a CCD carrier when she was 6 months old, regularly observed by an orthopedic and a pediatric dentist. The patient was not satisfied with the facial aesthetics and presented difficulty eating. The initial orthodontic mechanotherapy was carried out to facilitate the eruption of permanent teeth, following the realignment, leveling, and decompensation of the dental arches before orthognathic surgery. In current medical history, there were no local or systemic conditions that prevented the surgical approach. During clinical and radiographic analysis, a middle-third facial collapse was observed with malar hypoplasia, maxillary deficiencies (transverse, sagittal, and vertical), and anterior open bite, besides class III malocclusion and inclined occlusal plane (Table 1). The lateral facial profile was slightly concave, and the face was symmetrical on frontal view, the upper dental midline was coincident, and the lower dental midline was slightly to the right (Fig. 1). The dental tissues and periodontal and temporomandibular articulations were clinically normal, without signs or symptoms of dysfunction. The panoramic radiography showed the upper third molars were not erupted and 2 supernumerary teeth: the left fourth upper molar and lower canine (Fig. 2).
Treatment Goals From the Department of Stomatology, Bauru School of Dentistry, and yDepartment of Orthodontics, Hospital for Rehabilitation of Craniofacial Anomalies, University of Sa˜o Paulo, Bauru; and zDepartment of Oral and Maxillofacial Surgery, Dental School of Arac¸atuba, Universidade Estadual Paulista (UNESP), Arac¸atuba; Sa˜o Paulo, Brazil. Received April 8, 2014. Accepted for publication October 28, 2014. Address correspondence and reprint requests to Eduardo Dias-Ribeiro, DDS, MSc, Department of Oral and Maxillofacial Surgery, Dental School of Arac¸atuba, UNESP–Universidade Estadual Paulista Rua Jose´ Bonifacio, 1193, Arac¸atuba, Sa˜o Paulo 16015-050, Brazil; E-mail: [email protected] The authors report no conflicts of interest. Copyright # 2015 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000001414
792
The goal of the orthodontic-surgical treatment is to reach an ideal occlusion and adequate facial profile of hard and soft tissues, providing health to the oral and periodontal tissues.11 The surgical procedure for the patient carrier of this syndrome should facilitate further psychological development and social interaction, as many of these patients are on the psychosocial margin.12
Treatment Progress After the conclusive clinical and radiographic diagnosis of maxillary and mandibular retrusion with malar hypoplasia, a bimaxillary orthognathic surgery was performed with concomitant implantation of a malar prosthesis. The surgical plan was completed with the assistance of Dolphin Imaging 11.0 software (Dolphin/3M, San Diego, CA) (licensed to Bauru School of Dentistry, University
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
Orthognathic Surgery in Cleidocranial Dysplasia
TABLE 1. Presurgical, Standard, and Posttreatment Analysis Values According to Arnett and McLaughlin13 Dentoskeletal Factors Maxilla Upper incisor inclination (Mx1 MxOP), degrees Upper incisor tip projection (Mx1 Sn), mm Mandible0 Lower incisor inclination (Md1 MdOP), degrees Lower incisor tip projection (Md1 Sn), mm Overjet (Mx1 Md1), mm Vertical Overbite (Mx1 Md1), mm Mx anterior height (Sn0 Mx1), mm Mx occlusal plane (MxOP TVL), degrees Chin height (Md1 Me0 ), mm Facial height Soft tissue height Interlabial gap (ULI LLS), mm Lower vs upper lip length (LLS Me0 Sn0 ULI), % Lower 13 of face (Sn0 Me0 ), mm Facial height (Na0 Me0 ), mm Hard tissue height Upper incisor exposure (ULI Mx1tip), mm Mx anterior height (Sn0 Mx1), mm Soft tissue thickness Upper lip thickness (Mx1 labial ULA), mm Lower lip thickness (LLinside Lloutside), mm Menton thickness (Me Me0 ), mm Projections Maxillary projection Nasal projection, mm Soft tissue A0 point (A0 ), mm Upper lip anterior (ULA Sn), mm Upper lip angle (ULA Sn0 TVL), degrees Nasolabial angle (Col Sn0 ULA), degrees Upper incisor tip projection (Mx1 Sn), mm Mandibular projection Soft tissue B0 point (B0 ), mm Lower lip anterior (LLA), mm Lower incisor tip projection (Md1 Sn), mm Facial harmony Facial angle (G0 Sn0 Pog0 ), degrees Chin to nasal base (Pog0 Sn0 ), mm Mandibular base to maxillary base (A0 B0 ), mm Lower lip to upper lip (LLA ULA), mm
of Sa˜o Paulo, Sa˜o Paulo, Brazil) pointing out the cephalometric spots described in 2004 by Arnett and McLaughlin.13 Then, surgery models for making the intermediate splint were constructed. The orthognathic surgery consisted of advancing the maxilla by 5 mm, using a Le Fort I osteotomy, and advancing the mandible by 2.4 mm through the bilateral sagittal split osteotomy of the mandible, with counterclockwise occlusion. Stabilization of the maxillary osteotomies was accomplished by a rigid internal fixation system 2.0 mm (To´ride, Sa˜o Paulo, Brazil). The hybrid fixation technique was applied to the mandible,14 through the combination of monocortical plaques and bicortical screws of the rigid 2.0-mm internal fixation system (To´ride). In addition, a malar prosthesis was placed (Medpor; Porex Surgical Inc, Kalamazoo, MI) to correct the third midface, screw fixed in the rigid 2.0-mm internal fixation system (To´ride) (Table 1). The suture was performed with Vicryl 4-0 (Ethicon; Johnson & Johnson, Sa˜o Paulo, Brazil), along with the V-Y alar base suture. The dental engagement (canine and molar— #
2015 Mutaz B. Habal, MD
Presurgical
Standard Values
Posttreatment
54.0 11.5
56.8 9.2
56.2 11.6
85.6 10.6 0.9
64.3 12.4 3.2
76.3 15.4 3.9
6.4 22.2 102.5 42.2
3.2 25.7 95.6 48.6
0.6 22.7 108.2 39.8
1.8 238.0 70.8 125.8
3.3 223.0 71.1 125.0
1.1 204.4 63.2 113.7
1.7 22.2
3.5 25.7
2.4 22.7
10.5 10.9 17.0
12.6 13.6 7.4
8.4 9.0 12.6
17.3 2.6 0.5 4.2 111.3 11.5
16.0 0.9 3.7 12.1 104.0 9.2
13.8 0.6 2.8 3.0 107.5 11.6
0.3 3.6 10.6
5.3 1.9 12.4
6.1 1.0 15.4
176.4 2.3 2.3 3.1
169.0 2.0 5.2 1.8
168.0 4.0 5.5 3.9
class I bilateral) was reached after orthodontic treatment was completed, observing harmony of facial thirds, as well as the health of oral tissue through applied treatment (Figs. 3 and 4). After 3 years of observation, there have not been any signs of malocclusion recurrence, and the patient is satisfied with the treatment and the new facial profile (Fig. 5).
DISCUSSION A CCD, because of its low morbidity, may be diagnosed late.15 The confirmation of the diagnosis may be given through a clinical examination of the skull, face, clavicle, and oral cavity. In the oral examination, it is possible to compare the state of eruption and the chronological age of the patient. In the radiographic examination, especially the panoramic view, we are able to determine the number, size, and position of the supernumerary teeth (Fig. 2). After clinical and radiographic evaluation, the patient must be sent
793
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
Madeira et al
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
FIGURE 1. Pretreatment extraoral (A, B) and lateral radiographs (C) and intraoral photographs (D–F).
FIGURE 3. Posttreatment extraoral (A, B) and lateral radiographs (C) and intraoral photographs (D–F).
to genetic services for confirmation of the diagnosis, genetic counseling, and verification of other cases in the family.16 There are several therapeutic options that may be adopted to correct dentoskeletal changes in patients with CCD. The choice will depend on the health and dental condition, facial aspect, and patient expectation, considering that the goal of any treatment must include eliminating disturbance and symptoms, restoring masticatory function, and improving the patient’s appearance.6 Nowadays, the orthodontic or orthopedic treatment usually associated to orthognathic surgery and aesthetic rehabilitation are the elected procedures.1,5– 10 When there is a need for orthognathic surgery, the realignment, leveling, and decompensation of dental arches are of fundamental importance. In this context, the poor dental structure and the delayed closing of the apical portion of the root that occur in patients with CCD may influence the spontaneous eruption of permanent teeth, causing the development of crowns and roots to be delayed between 2 and 4 years, when compared with nonsyndromic patients.16 Depending on the type and gravity of the craniofacial anomaly, the timing of orthognathic surgery depends on functional problems, psychosocial factors, and facial growth pattern and should be tailored to the needs of each individual patient.12 There are few cases in the literature concerning the orthognathic surgical approach in patients with CCD. In 2006, Daskalogiannakis et al5 stated an improvement in the occlusal relationship after maxilla advancement, through Le Fort I osteotomy, carried out after skeletal maturity, which was observed in this case. In 1980, Dann et al17 showed an improvement of facial profile in the middle third, through the Le Fort I osteotomy technique, associated to the use of positional graft. The application of Le Fort I osteotomy correcting maxillary dentofacial deformities has produced predictable and stable results. Maxillary advancement provided good support for the upper lip, filled the soft-tissue midface, and favorably improved the patient’s profile.18
Since Hugo Obwegeser’s description of sagittal split ramus osteotomy in 1955, there have been many modifications of this surgical procedure, often used in orthognathic surgery. After the advent of rigid fixation systems, this procedure became more secure and easier to apply to correct mandible deformities,14 with satisfying results when applied to retreat the mandible.19 The ideal age to begin the surgical treatment in patients with no syndrome is usually from mixed dentition because the presence of supernumerary teeth may impact permanent teeth. In specific cases, orthognathic surgery is performed during the phase of development because of aesthetic and psychosocial reasons, considering the severity of dentofacial deformity secondary to less disparate enhanced growth. However, when surgery is performed prematurely, unpredictable results may occur because of the growth of unaffected tissues, and it is important to inform the patient and the family of the need for a new orthognathic surgery procedure.12,20 In 2001, Wolford et al20 demonstrated that early correction of maxillary hypoplasia in growing patients does not produce predictable results because of continued mandibular and vertical maxillary growth. The surgical procedure was chosen after maturity of the bone tissue, seeking a more predictable and stable aesthetic result, because the patient did not present symptoms and clinical signs of stomatognathic system dysfunction and related structures.
FIGURE 2. Pretreatment panoramic radiograph showing supernumerary tooth (maxillary molars) and mandibular left canine.
FIGURE 4. Superimposed cephalometric tracings (grey: posttreatment, black: pretreatment).
794
#
2015 Mutaz B. Habal, MD
Copyright © 2015 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.
The Journal of Craniofacial Surgery
Volume 26, Number 3, May 2015
FIGURE 5. Facial aspect postoperative 3 years.
Therapy for the patient carrier of CCD is extensive, and it requires the presence of a specialized and multidisciplinary team, seeking the most predicable and stable treatment. Cases of CCD treated with well-defined criteria can bring aesthetic and functional benefits to the patient.
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Orthognathic Surgery in Cleidocranial Dysplasia
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