CASE REPORT
Long-term orthodontic and surgical treatment and stability of a patient with Beckwith-Wiedemann syndrome Rina Hikita,a Yukiho Kobayashi,b Michiko Tsuji,b Tatsuo Kawamoto,c and Keiji Moriyamad Tokyo, Japan Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment. (Am J Orthod Dentofacial Orthop 2014;145:672-84)
B
eckwith-Wiedemann syndrome (BWS) is caused by mutation or deletion of an imprinted domain in the chromosomal region 11p15.5, which harbors the H19, p57 (KIP2), and LIT1 genes. The prevalence of BWS is estimated to be 1 in 13,700 births.1,2 It is characterized by exomphalos (80%), macroglossia (97%), and somatic gigantism (88%) resulting from overgrowth
From the Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan. a Resident. b Assistant professor. c Junior associate professor. d Professor and chair; professor, Hard Tissue Genome Research Center. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported. This study was supported by Grants-in-Aid for Scientific Research Projects (#25463166) from the Japan Society for the Promotion of Science, and by a grant from the Japanese Ministry of Education. Address correspondence to: Tatsuo Kawamoto, Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8510, Japan; e-mail, t-kawamoto. [email protected]. Submitted, June 2013; revised and accepted, August 2013. 0889-5406/$36.00 Copyright Ó 2014 by the American Association of Orthodontists. http://dx.doi.org/10.1016/j.ajodo.2013.08.019
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during the latter half of gestation and during infancy. Previous studies have shown that patients with BWS have protrusion of the mandible with an abnormally widely dilated gonial angle, increased mandibular length, anterior open bite, and a wide dental arch, primarily as a consequence of the macroglossia.3-7 Some reports have suggested that early diagnosis and treatment of the oral manifestations of BWS could reduce the associated aberrant craniofacial development and growth.4 Although there have been some reports concerning the longitudinal craniofacial changes in BWS patients treated with tongue reduction and orthodontic treatment, the long-term outcome of surgicalorthodontic treatment with tongue reduction remains unclear.7-9 We herein report on a patient with BWS showing a good prognosis and long-term stability after tongue reduction, orthopedic and orthodontic treatment, and orthognathic surgery. DIAGNOSIS AND ETIOLOGY
A Japanese girl, 5 years 10 months of age, was referred to the orthodontic department of the Tokyo Medical and Dental University Dental School Hospital. Her chief complaint was a severe anterior crossbite
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Fig 1. Pretreatment facial and intraoral photographs.
with an open bite. She was born by Caesarean delivery at the normal gestational age; her birth weight was 4.324 g. She had been diagnosed with BWS at birth and had undergone surgery for exomphalos immediately after birth. Furthermore, she had a medical history of stomach volvulus and ileus and had undergone tongue reduction surgery when she was 2 years 7 months of age at another medical school hospital.
At the first medical examination, she had no significant systemic or orthodontic family history. Her facial profile was concave with chin protrusion and long lower facial height (Fig 1). The tongue was long enough to reach the mentum, and abnormal earlobe creases were observed (Fig 1). She had an anterior open bite with an overjet of 9.0 mm and an overbite of 3.0 mm, and a constricted width of the maxillary dental arch with the high arched palate
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Fig 2. Pretreatment dental models.
resulting in a total crossbite (Figs 1 and 2). The terminal plane was a mesial step type on both sides (Figs 1 and 2). From the results of the lateral cephalometric analysis, the patient was proven to have a skeletal Class III jaw relationship with severe mandibular protrusion (ANB, 0.3 ; SNB, 82.3 ), long mandibular body length (GoPog, 75.5 mm), and a high mandibular plane angle (MP-FH, 42.7 ) with an obtuse gonial angle (145.0 ) (Fig 3, Table). These data deviated greatly from the normative values for Japanese girls of corresponding age. This patient received a diagnosis of mandibular prognathism with a skeletal Class III jaw relationship, a high mandibular plane angle, a total crossbite, and an anterior open bite. The etiology of the severe skeletal Class III malocclusion with open bite was believed to be hyperplasia of the tongue, a phenotype associated with BWS. TREATMENT OBJECTIVES
The treatment objectives for this patient included accomplishing the following: (1) correct the severe
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skeletal Class III jaw relationship caused by the excessive mandibular growth; (2) correct the anterior open bite and the total crossbite, and establish ideal overjet and overbite; (3) achieve an acceptable occlusion with a good functional Class I occlusion; (4) achieve a favorable facial profile; and (5) maintain the stability of her occlusion during retention for a long term. TREATMENT ALTERNATIVES
There have been some controversies in active phase 1 orthodontic and orthopedic approaches to patients displaying severe skeletal Class III problems. Some authors reported minimal or no effects.10,11 On the other hand, others have reported that phase 1 orthodontic and orthopedic treatment for these patients might be recommended to resolve skeletal problems in the transverse, vertical, and sagittal dimensions.12,13 These effects sometimes contribute to relieving the difficulty of phase 2 of orthodontic treatment, including orthognathic surgery after pubertal growth.8 On the basis of this information, we performed early phase 1
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Fig 3. Pretreatment radiographs.
Table. Cephalometric measurements Normative value* Measurement Age Angular ( ) ANB SNA SNB MP-FH Gonial angle U1-SN L1-MP Convexity Interincisal angle Occlusal plane Linear (mm) Ar-Go Go-Pog Overjet Overbite
Pretreatment 5 y 10 mo
Before phase 2 20 y 7 mo
Posttreatment 28 y 4 mo
6 years postretention 34 y 4 mo
Mean
SD
0.3 82.7 82.3 43.7 145.0 87.9 77.7 3.8 144.5 30.5
3.4 84.7 88.1 45.0 150.6 104.3 72.8 4.8 131.8 14.6
3.7 87.5 83.9 42.7 143.1 94.8 63.6 7.5 153.0 15.2
3.7 87.5 83.9 42.7 143.1 95.7 63.6 7.5 152.1 15.1
3.4 82.3 78.9 28.8 122.2 104.5 96.3 7.6 124.1 11.4
1.8 3.5 3.5 5.2 4.6 5.6 5.8 5.0 7.6 3.6
33.4 75.5 9.0 3.0
48.1 96.7 12.0 12.0
47.0 87.2 4.0 4.0
48.1 88.0 3.0 3.0
41.3 78.0 1.0 1.0
5.0 4.7 1.5 1.5
*Means and standard deviations of normally developed Japanese women.27-29
orthodontic and orthopedic treatment for our patient in the hope of a better prognosis. Regarding the method of orthognathic surgery for our patient with a severe skeletal Class III condition, the options were isolated mandibular setback or bimaxillary
orthognathic surgery. Because of her extremely large tongue and concave profile with mandibular protrusion, we opted for bimaxillary surgery rather than isolated mandibular setback, LeFort I osteotomy combined with horseshoe osteotomy, and genioplasty to achieve the
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Fig 4. Cephalometric superimpositions. The SN plane at S, the palatal plane at ANS, and the mandibular plane at Me were the bases of the overall, maxillary, and mandibular superimpositions, respectively. Black lines, pretreatment tracing; black dashed lines, deciduous central incisors; red lines, before phase 2 treatment; mandibular molars in black lines, shape of the mandibular first molar tooth buds; mandibular molars in red lines: the first and the second molars.
appropriate volume of the oral cavity and a favorable profile. We chose surgically assisted rapid maxillary expansion (RME) because of her age at the time of phase 2 treatment, the damage of periodontal tissues from the lateral expansion of the maxillary dentition, and the amount of expansion required to establish harmonic maxillary and mandibular dental arch widths. TREATMENT PROGRESS
A vertical chincap was applied at 6 years 1 month of age and continued for approximately 10 years as phase 1 orthodontic treatment. At the beginning of phase 2 treatment, the mandibular first molars on both sides were extracted under local anesthesia. The decision to extract these teeth was made for maximal restoration of her open bite during preoperative orthodontic
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treatment by mesial driving of the mandibular second and third molars. Edgewise appliances were placed on the mandibular dental arch at 20 years 10 months of age. Leveling was performed in the mandibular dental arch for 7 months, and space closure in the mandibular dental arch was performed for 2 years 4 months. At 23 years 10 months of age, the constricted maxillary dental arch was expanded by 8.5 mm (from 40.5 to 49.0 mm) between the maxillary first molars with surgically assisted RME. A lingual arch with a palatal bar was placed for 3 months to retain the width of the maxillary dentition, and then edgewise appliances were placed on the maxillary dental arch. At 1 year 3 months after the surgically assisted RME, a quad-helix appliance was placed for 3 months on the maxillary dentition for lateral reexpansion. The duration of the preoperative
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Fig 5. Facial and intraoral photographs before phase 2 treatment.
orthodontic treatment was 7 years. Then a LeFort I osteotomy combined with a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed under general anesthesia at 26 years 9 months of age. All surgical orthodontic procedures were performed in the oral surgery department of the Tokyo Medical and Dental University Dental School Hospital. The total postoperative orthodontic treatment time was 1 year 5 months, and the overall active orthodontic treatment period was 8 years 4 months. After removal of the edgewise appliances, circumferential and Hawley-type retainers were applied on the maxillary and mandibular dentitions, respectively. The observation term of the retention period was almost 6 years 1 month. TREATMENT RESULTS
A hyperdivergent growth pattern was evident throughout the patient's orthopedic treatment with a vertical chincap. Her profile was exacerbated by marked
forward and downward mandibular growth from 5 years 10 months to 20 years 7 months of age (Fig 4). Skeletal anteroposterior growth was evident with a 5.8 change in the ANB angle (the SNB angle had increased from 82.3 to 88.1 ). During this period, a constricted maxillary dental arch width, exacerbation of the open bite, anterior crossbite, labially inclined maxillary anterior teeth, lingually inclined mandibular anterior teeth, and a Class III molar relationship were evident (Figs 5-7). Harmonious maxillary and mandibular dental arch widths were achieved by surgically assisted RME, and the skeletal Class III jaw relationship was improved by orthognathic surgery (Figs 8-10). These results provided a favorable facial profile (Fig 8). We evaluated the linear changes of the maxillary and mandibular bones by measuring the distance between the 2 feet of the perpendicular lines from Point A or B on the Frankfort horizontal plane before and after surgery. The orthognathic surgery resulted in a maxillary advancement of 3.0 mm and a mandibular setback of 8.0 mm. A skeletal
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Fig 6. Dental models before phase 2 treatment.
Fig 7. Radiographs before phase 2 treatment.
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Fig 8. Posttreatment facial and intraoral photographs.
Class I jaw relationship was also achieved (ANB, 3.7 ). Because the maxillary molars were moved upward, the mandibular plane angle was decreased by 2.3 . The negative overjet was corrected to 14.0 mm, and the anterior open bite was improved to an overbite of 14.0 mm. We accomplished the main treatment goals, which were to establish a favorable facial profile and an acceptable occlusion (data not shown). At the end of active orthodontic treatment, at 1 year 5 months after the surgical orthodontic treatment, an ideal occlusion with Class I molar relationships and an adequate interincisal relationship was achieved (overjet, 13.0 mm; overbite, 13.0 mm; Figs 8 and 9). The cephalometric evaluation showed a skeletal Class I jaw relationship (ANB, 3.7 ); the maxilla and mandible were almost stable, and the facial profile was maintained (Fig 14). After 6 years 1 month of retention, at 7 years 6 months after the osteotomy, both the maxilla and the
mandible were stable relative to the reference line. The skeletal Class I jaw relationship was retained (ANB, 3.7 ), and the facial profile and the occlusion were acceptable (Figs 11-13). Her tongue seemed to work in harmony with the newly constructed oral condition, and no signs of dental relapse after orthodontic treatment, dysfunction of pronunciation, or problem with swallowing were observed. DISCUSSION
BWS is characterized by exomphalos, macroglossia, and somatic gigantism. Of these, macroglossia is suggested to be closely associated with oral function and maxillofacial morphology.3,4,6,7 Macroglossia is defined as a resting tongue that protrudes beyond the teeth or the alveolar ridge.3,4,6,7 Although BWS is highly variable with regard to clinical presentation, macroglossia is the most consistent symptom and is evident in 80% to 99% of patients.1,14-16 A logical approach to preventing or
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Fig 9. Posttreatment dental models.
Fig 10. Posttreatment radiographs.
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Fig 11. Facial and intraoral photographs after 6 years of retention.
correcting the effects of macroglossia is performing a glossectomy, preferably before problems in speech development occur,17 but not before the patient is 6 months old.18 This is because an increase in the size of the tongue during the first few months of life has been reported, as has gradual improvement of the condition after 6 months of age.18 Accordingly, glossectomy is generally conducted at 2 to 3 years of age. Recently, it has become acceptable to perform glossectomy on younger patients because of advancements in operative and anesthetic techniques. Kadouch et al19 performed glossectomies in 23 patients with BWS at an average age of 19 months and reported stable outcomes. Glossectomy has resulted in reductions of mandibular length and width in animal studies.7 Furthermore, a radiographic cephalometric study comparing BWS patients who had undergone glossectomy with those who had not showed that anterior open bite and mandibular protrusion were improved via glossectomy.20 Our patient had a
glossectomy when she was 2 years 7 months of age, but some dentoalveolar and skeletal problems remained even at 5 years 10 months of age. Therefore, the glossectomy might have been performed too late to achieve optimal skeletal growth in this patient, considering the subsequent growth of the maxillofacial region. Both her tongue and predisposition for severe skeletal deformity could have influenced her skeletal growth pattern. There is some debate over whether we should provide phase 1 orthodontic treatment for children who might require orthognathic surgery in the future. Although nonsurgical treatment with mandibular growth modification with a chincap followed by multibracket appliances with or without tooth extraction was considered a potentially effective way to improve the Class III malocclusion, this method was deemed inapplicable because of this patient's severe dentoskeletal and facial disharmony. It seemed appropriate to combine glossectomy with phase 1 orthodontic treatment because if the
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Fig 12. Dental models after 6 years of retention.
Fig 13. Radiographs after 6 years of retention.
treatment plan had been limited to a glossectomy, the vertical problems such as increased gonial angle and open bite might not have been corrected.
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With regard to expansion for the treatment of transverse problems in the maxillary dental arch, we carefully considered the age of the patient; the timing of dental
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Fig 14. Cephalometric superimpositions. The SN plane at S, the palatal plane at ANS, and the mandibular plane at Me were the bases of the overall, maxillary, and mandibular superimpositions, respectively. Black lines, before phase 2 treatment; blue lines, posttreatment; red lines, after 6 years of retention; mandibular molars in black lines, the first and the second molars; mandibular molars in red and blue lines, the second and the third molars.
arch expansion; the condition of the periodontal tissues; the methods used, including a quad-helix, a rapid maxillary expander, and surgically assisted RME; the amount of necessary expansion; and the volume of the oral cavity. It was difficult to decide when to initiate expansion of the constricted maxillary dental arch width because it was possible that the patient would require orthognathic surgery after pubertal growth; thus, we could not accurately determine the required degree of expansion. It was also difficult to select treatment methods and associated orthodontic appliances. RME has been reported to be the most effective treatment to correct transverse maxillary discrepancies in growing adolescents; it functions by opening the midpalatal suture.21-23 According to Korn and Baumrind,24 the median palatine suture closes at approximately 14 of 15 years of age in girls and 15 to 16 years in boys. When RME is not feasible, a variety of surgical procedures including surgically assisted RME have been advocated in the treatment of transverse maxillary deficiency. This procedure allows for splitting of the median palatine suture and widening of the
maxilla.22 With regard to our patient, we thought that surgically assisted RME might increase the volume of the oral cavity and reduce the influence of her tongue volume on the stability after orthodontic treatment. Surgically assisted RME was considered to be an appropriate procedure because of her age and the amount of expansion required. Additionally, previous studies have reported that bimaxillary surgery, particularly advancement of the maxilla, can increase the volume of the oral cavity.25,26 Thus, it is important to consider the relationship between the volume of the tongue and the oral cavity to achieve long-term stability of the occlusion. This is the first report of longitudinal surgicalorthodontic treatment of a patient with BWS who had undergone glossectomy during early preadolescence. The results showed that early orthodontic intervention and surgical-orthodontic treatment were effective in resolving the skeletal disharmonies and in improving the facial profile and the occlusion. Orthodontists must make an accurate determination of macroglossia, which is the most prominent feature of BWS, because it is
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suggested to be closely associated with oral function and maxillofacial morphology. Additionally, the results of this patient’s treatment indicated that with the cooperation of an oral surgeon, an orthodontist can improve both facial appearance and occlusion in patients with BWS. This patient expressed great satisfaction with the improvements in her anterior crossbite and facial appearance. CONCLUSIONS
10.
11.
12. 13.
We reported the longitudinal and successful surgicalorthodontic treatment of a patient with severe mandibular prognathism and an anterior open bite associated with BWS. Over the course of orthodontic treatment lasting approximately 30 years combined with surgical correction of the musculoskeletal components—ie, the maxilla, the mandible, and the tongue—the patient acquired a favorable occlusion with harmonized facial esthetics. On the basis of our case report as well as previous studies by others, we suggest that balancing the structure and function of the dentoalveolar and musculoskeletal tissues of the orofacial complex is the key to a good treatment outcome and the long-term stability of patients with BWS.
14. 15. 16.
17. 18.
19.
ACKNOWLEDGMENTS
We thank the patient and her family for their cooperation and consent to publishing this report, and all the team members who were involved in her treatment. REFERENCES
21.
22.
1. Engstrom W, Lindham S, Schofield P. Wiedemann-Beckwith syndrome. Eur J Pediatr 1988;147:450-7. 2. Thorburn MJ, Wright ES, Miller CG, Smith-Read EH. Exomphalosmacroglossia-gigantism syndrome in Jamaican infants. Am J Dis Child 1970;119:316-21. 3. Friede H, Figueroa AA. The Beckwith-Wiedemann syndrome: a longitudinal study of the macroglossia and dentofacial complex. J Craniofac Genet Dev Biol Suppl 1985;1:179-87. 4. Giancotti A, Romanini G, Di Girolamo R, Arcuri C. A less-invasive approach with orthodontic treatment in Beckwith-Wiedemann patients. Orthod Craniofac Res 2002;5:59-63. 5. Irving IM. Exomphalos with macroglossia: a study of eleven cases. J Pediatr Surg 1967;2:499-507. 6. Kawafuji A, Suda N, Ichikawa N, Kakara S, Suzuki T, Baba Y, et al. Systemic and maxillofacial characteristics of patients with Beckwith-Wiedemann syndrome not treated with glossectomy. Am J Orthod Dentofacial Orthop 2011;139:517-25. 7. Liu ZJ, Shcherbatyy V, Gu G, Perkins JA. Effects of tongue volume reduction on craniofacial growth: a longitudinal study on orofacial skeletons and dental arches. Arch Oral Biol 2008;53:991-1001. 8. Abeleira MT, Seoane-Romero JM, Outumuro M, Caamano F, Suarez D, Carmona IT. A multidisciplinary approach to the treatment of oral manifestations associated with Beckwith-Wiedemann syndrome: a long-term case report. J Am Dent Assoc 2011;142:1357-64. 9. Miyawaki S, Oya S, Noguchi H, Takano-Yamamoto T. Long-term changes in dentoskeletal pattern in a case with Beckwith-
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Wiedemann syndrome following tongue reduction and orthodontic treatment. Angle Orthod 2000;70:326-31. Mitani H, Sato K, Sugawara J. Growth of mandibular prognathism after pubertal growth peak. Am J Orthod Dentofacial Orthop 1993; 104:330-6. Sugawara J, Asano T, Endo N, Mitani H. Long-term effects of chincap therapy on skeletal profile in mandibular prognathism. Am J Orthod Dentofacial Orthop 1990;98:127-33. Kanno Z, Kim Y, Soma K. Early correction of a developing skeletal Class III malocclusion. Angle Orthod 2007;77:549-56. Mandall N, DiBiase A, Littlewood S, Nute S, Stivaros N, McDowall R, et al. Is early Class III protraction facemask treatment effective? A multicentre, randomized, controlled trial: 15-month follow-up. J Orthod 2010;37:149-61. Elliott M, Maher ER. Beckwith-Wiedemann syndrome. J Med Genet 1994;31:560-4. Filippi G, McKusick VA. The Beckwith-Wiedmann syndrome. Medicine (Baltimore) 1970;49:279-98. Sotelo-Avila C, Gonzalez-Crussi F, Fowler JW. Complete and incomplete forms of Beckwith-Wiedemann syndrome: their oncogenic potential. J Pediatr 1980;96:47-50. Laroche C, Testelin S, Devauchelle B. Cleft palate and BeckwithWiedemann syndrome. Cleft Palate Craniofac J 2005;42:212-7. Chitayat D, Rothchild A, Ling E, Friedman JM, Couch RM, Yong SL, et al. Apparent postnatal onset of some manifestations of the Wiedemann-Beckwith syndrome. Am J Med Genet 1990;36:434-9. Kadouch DJ, Maas SM, Dubois L, van der Horst CM. Surgical treatment of macroglossia in patients with Beckwith-Wiedemann syndrome: a 20-year experience and review of the literature. Int J Oral Maxillofac Surg 2012;41:300-8. Menard RM, Delaire J, Schendel SA. Treatment of the craniofacial complications of Beckwith-Wiedemann syndrome. Plast Reconstr Surg 1995;96:27-33. Chung CH, Font B. Skeletal and dental changes in the sagittal, vertical, and transverse dimensions after rapid palatal expansion. Am J Orthod Dentofacial Orthop 2004;126:569-75. Lagravere MO, Major PW, Flores-Mir C. Long-term skeletal changes with rapid maxillary expansion: a systematic review. Angle Orthod 2005;75:1046-52. McNamara JA Jr, Baccetti T, Franchi L, Herberger TA. Rapid maxillary expansion followed by fixed appliances: a long-term evaluation of changes in arch dimensions. Angle Orthod 2003;73:344-53. Korn EL, Baumrind S. Transverse development of the human jaws between the ages of 8.5 and 15.5 years, studied longitudinally with use of implants. J Dent Res 1990;69:1298-306. Jakobsone G, Neimane L, Krumina G. Two- and three-dimensional evaluation of the upper airway after bimaxillary correction of Class III malocclusion. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2010;110:234-42. Jakobsone G, Stenvik A, Espeland L. The effect of maxillary advancement and impaction on the upper airway after bimaxillary surgery to correct Class III malocclusion. Am J Orthod Dentofacial Orthop 2011;139(Suppl):e369-76. Iizuka T, Ishikawa F. Normal standards for various cephalometric analysis in Japanese adults. Nihon Kyosei Shika Gakkai Zasshi 1957;16:4-12. Yamanouchi S, Ishihara K, Shirato Y, Sato K, Mitani H. Facial pattern of the present day Japanese associated with normal occlusion. Nihon Kyosei Shika Gakkai Zasshi 1995;54:93-101. Sakamoto T, Miura F, Iizuka T. Linear analyses on the developmental changes of dentofacial complex of Japanese by means of roentgenographic cephalometry. Kokubyo Gakkai Zasshi 1963; 30:169-82.
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Long-term orthodontic and surgical treatment and stability of a patient with Beckwith-Wiedemann syndrome Rina Hikita,a Yukiho Kobayashi,b Michiko Tsuji,b Tatsuo Kawamoto,c and Keiji Moriyamad Tokyo, Japan Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment. (Am J Orthod Dentofacial Orthop 2014;145:672-84)
B
eckwith-Wiedemann syndrome (BWS) is caused by mutation or deletion of an imprinted domain in the chromosomal region 11p15.5, which harbors the H19, p57 (KIP2), and LIT1 genes. The prevalence of BWS is estimated to be 1 in 13,700 births.1,2 It is characterized by exomphalos (80%), macroglossia (97%), and somatic gigantism (88%) resulting from overgrowth
From the Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, Tokyo, Japan. a Resident. b Assistant professor. c Junior associate professor. d Professor and chair; professor, Hard Tissue Genome Research Center. All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest, and none were reported. This study was supported by Grants-in-Aid for Scientific Research Projects (#25463166) from the Japan Society for the Promotion of Science, and by a grant from the Japanese Ministry of Education. Address correspondence to: Tatsuo Kawamoto, Maxillofacial Orthognathics, Department of Maxillofacial Reconstruction and Function, Division of Maxillofacial/Neck Reconstruction, Graduate School, Tokyo Medical and Dental University, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8510, Japan; e-mail, t-kawamoto. [email protected]. Submitted, June 2013; revised and accepted, August 2013. 0889-5406/$36.00 Copyright Ó 2014 by the American Association of Orthodontists. http://dx.doi.org/10.1016/j.ajodo.2013.08.019
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during the latter half of gestation and during infancy. Previous studies have shown that patients with BWS have protrusion of the mandible with an abnormally widely dilated gonial angle, increased mandibular length, anterior open bite, and a wide dental arch, primarily as a consequence of the macroglossia.3-7 Some reports have suggested that early diagnosis and treatment of the oral manifestations of BWS could reduce the associated aberrant craniofacial development and growth.4 Although there have been some reports concerning the longitudinal craniofacial changes in BWS patients treated with tongue reduction and orthodontic treatment, the long-term outcome of surgicalorthodontic treatment with tongue reduction remains unclear.7-9 We herein report on a patient with BWS showing a good prognosis and long-term stability after tongue reduction, orthopedic and orthodontic treatment, and orthognathic surgery. DIAGNOSIS AND ETIOLOGY
A Japanese girl, 5 years 10 months of age, was referred to the orthodontic department of the Tokyo Medical and Dental University Dental School Hospital. Her chief complaint was a severe anterior crossbite
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Fig 1. Pretreatment facial and intraoral photographs.
with an open bite. She was born by Caesarean delivery at the normal gestational age; her birth weight was 4.324 g. She had been diagnosed with BWS at birth and had undergone surgery for exomphalos immediately after birth. Furthermore, she had a medical history of stomach volvulus and ileus and had undergone tongue reduction surgery when she was 2 years 7 months of age at another medical school hospital.
At the first medical examination, she had no significant systemic or orthodontic family history. Her facial profile was concave with chin protrusion and long lower facial height (Fig 1). The tongue was long enough to reach the mentum, and abnormal earlobe creases were observed (Fig 1). She had an anterior open bite with an overjet of 9.0 mm and an overbite of 3.0 mm, and a constricted width of the maxillary dental arch with the high arched palate
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Fig 2. Pretreatment dental models.
resulting in a total crossbite (Figs 1 and 2). The terminal plane was a mesial step type on both sides (Figs 1 and 2). From the results of the lateral cephalometric analysis, the patient was proven to have a skeletal Class III jaw relationship with severe mandibular protrusion (ANB, 0.3 ; SNB, 82.3 ), long mandibular body length (GoPog, 75.5 mm), and a high mandibular plane angle (MP-FH, 42.7 ) with an obtuse gonial angle (145.0 ) (Fig 3, Table). These data deviated greatly from the normative values for Japanese girls of corresponding age. This patient received a diagnosis of mandibular prognathism with a skeletal Class III jaw relationship, a high mandibular plane angle, a total crossbite, and an anterior open bite. The etiology of the severe skeletal Class III malocclusion with open bite was believed to be hyperplasia of the tongue, a phenotype associated with BWS. TREATMENT OBJECTIVES
The treatment objectives for this patient included accomplishing the following: (1) correct the severe
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skeletal Class III jaw relationship caused by the excessive mandibular growth; (2) correct the anterior open bite and the total crossbite, and establish ideal overjet and overbite; (3) achieve an acceptable occlusion with a good functional Class I occlusion; (4) achieve a favorable facial profile; and (5) maintain the stability of her occlusion during retention for a long term. TREATMENT ALTERNATIVES
There have been some controversies in active phase 1 orthodontic and orthopedic approaches to patients displaying severe skeletal Class III problems. Some authors reported minimal or no effects.10,11 On the other hand, others have reported that phase 1 orthodontic and orthopedic treatment for these patients might be recommended to resolve skeletal problems in the transverse, vertical, and sagittal dimensions.12,13 These effects sometimes contribute to relieving the difficulty of phase 2 of orthodontic treatment, including orthognathic surgery after pubertal growth.8 On the basis of this information, we performed early phase 1
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Fig 3. Pretreatment radiographs.
Table. Cephalometric measurements Normative value* Measurement Age Angular ( ) ANB SNA SNB MP-FH Gonial angle U1-SN L1-MP Convexity Interincisal angle Occlusal plane Linear (mm) Ar-Go Go-Pog Overjet Overbite
Pretreatment 5 y 10 mo
Before phase 2 20 y 7 mo
Posttreatment 28 y 4 mo
6 years postretention 34 y 4 mo
Mean
SD
0.3 82.7 82.3 43.7 145.0 87.9 77.7 3.8 144.5 30.5
3.4 84.7 88.1 45.0 150.6 104.3 72.8 4.8 131.8 14.6
3.7 87.5 83.9 42.7 143.1 94.8 63.6 7.5 153.0 15.2
3.7 87.5 83.9 42.7 143.1 95.7 63.6 7.5 152.1 15.1
3.4 82.3 78.9 28.8 122.2 104.5 96.3 7.6 124.1 11.4
1.8 3.5 3.5 5.2 4.6 5.6 5.8 5.0 7.6 3.6
33.4 75.5 9.0 3.0
48.1 96.7 12.0 12.0
47.0 87.2 4.0 4.0
48.1 88.0 3.0 3.0
41.3 78.0 1.0 1.0
5.0 4.7 1.5 1.5
*Means and standard deviations of normally developed Japanese women.27-29
orthodontic and orthopedic treatment for our patient in the hope of a better prognosis. Regarding the method of orthognathic surgery for our patient with a severe skeletal Class III condition, the options were isolated mandibular setback or bimaxillary
orthognathic surgery. Because of her extremely large tongue and concave profile with mandibular protrusion, we opted for bimaxillary surgery rather than isolated mandibular setback, LeFort I osteotomy combined with horseshoe osteotomy, and genioplasty to achieve the
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Fig 4. Cephalometric superimpositions. The SN plane at S, the palatal plane at ANS, and the mandibular plane at Me were the bases of the overall, maxillary, and mandibular superimpositions, respectively. Black lines, pretreatment tracing; black dashed lines, deciduous central incisors; red lines, before phase 2 treatment; mandibular molars in black lines, shape of the mandibular first molar tooth buds; mandibular molars in red lines: the first and the second molars.
appropriate volume of the oral cavity and a favorable profile. We chose surgically assisted rapid maxillary expansion (RME) because of her age at the time of phase 2 treatment, the damage of periodontal tissues from the lateral expansion of the maxillary dentition, and the amount of expansion required to establish harmonic maxillary and mandibular dental arch widths. TREATMENT PROGRESS
A vertical chincap was applied at 6 years 1 month of age and continued for approximately 10 years as phase 1 orthodontic treatment. At the beginning of phase 2 treatment, the mandibular first molars on both sides were extracted under local anesthesia. The decision to extract these teeth was made for maximal restoration of her open bite during preoperative orthodontic
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treatment by mesial driving of the mandibular second and third molars. Edgewise appliances were placed on the mandibular dental arch at 20 years 10 months of age. Leveling was performed in the mandibular dental arch for 7 months, and space closure in the mandibular dental arch was performed for 2 years 4 months. At 23 years 10 months of age, the constricted maxillary dental arch was expanded by 8.5 mm (from 40.5 to 49.0 mm) between the maxillary first molars with surgically assisted RME. A lingual arch with a palatal bar was placed for 3 months to retain the width of the maxillary dentition, and then edgewise appliances were placed on the maxillary dental arch. At 1 year 3 months after the surgically assisted RME, a quad-helix appliance was placed for 3 months on the maxillary dentition for lateral reexpansion. The duration of the preoperative
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Fig 5. Facial and intraoral photographs before phase 2 treatment.
orthodontic treatment was 7 years. Then a LeFort I osteotomy combined with a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed under general anesthesia at 26 years 9 months of age. All surgical orthodontic procedures were performed in the oral surgery department of the Tokyo Medical and Dental University Dental School Hospital. The total postoperative orthodontic treatment time was 1 year 5 months, and the overall active orthodontic treatment period was 8 years 4 months. After removal of the edgewise appliances, circumferential and Hawley-type retainers were applied on the maxillary and mandibular dentitions, respectively. The observation term of the retention period was almost 6 years 1 month. TREATMENT RESULTS
A hyperdivergent growth pattern was evident throughout the patient's orthopedic treatment with a vertical chincap. Her profile was exacerbated by marked
forward and downward mandibular growth from 5 years 10 months to 20 years 7 months of age (Fig 4). Skeletal anteroposterior growth was evident with a 5.8 change in the ANB angle (the SNB angle had increased from 82.3 to 88.1 ). During this period, a constricted maxillary dental arch width, exacerbation of the open bite, anterior crossbite, labially inclined maxillary anterior teeth, lingually inclined mandibular anterior teeth, and a Class III molar relationship were evident (Figs 5-7). Harmonious maxillary and mandibular dental arch widths were achieved by surgically assisted RME, and the skeletal Class III jaw relationship was improved by orthognathic surgery (Figs 8-10). These results provided a favorable facial profile (Fig 8). We evaluated the linear changes of the maxillary and mandibular bones by measuring the distance between the 2 feet of the perpendicular lines from Point A or B on the Frankfort horizontal plane before and after surgery. The orthognathic surgery resulted in a maxillary advancement of 3.0 mm and a mandibular setback of 8.0 mm. A skeletal
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Fig 6. Dental models before phase 2 treatment.
Fig 7. Radiographs before phase 2 treatment.
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Fig 8. Posttreatment facial and intraoral photographs.
Class I jaw relationship was also achieved (ANB, 3.7 ). Because the maxillary molars were moved upward, the mandibular plane angle was decreased by 2.3 . The negative overjet was corrected to 14.0 mm, and the anterior open bite was improved to an overbite of 14.0 mm. We accomplished the main treatment goals, which were to establish a favorable facial profile and an acceptable occlusion (data not shown). At the end of active orthodontic treatment, at 1 year 5 months after the surgical orthodontic treatment, an ideal occlusion with Class I molar relationships and an adequate interincisal relationship was achieved (overjet, 13.0 mm; overbite, 13.0 mm; Figs 8 and 9). The cephalometric evaluation showed a skeletal Class I jaw relationship (ANB, 3.7 ); the maxilla and mandible were almost stable, and the facial profile was maintained (Fig 14). After 6 years 1 month of retention, at 7 years 6 months after the osteotomy, both the maxilla and the
mandible were stable relative to the reference line. The skeletal Class I jaw relationship was retained (ANB, 3.7 ), and the facial profile and the occlusion were acceptable (Figs 11-13). Her tongue seemed to work in harmony with the newly constructed oral condition, and no signs of dental relapse after orthodontic treatment, dysfunction of pronunciation, or problem with swallowing were observed. DISCUSSION
BWS is characterized by exomphalos, macroglossia, and somatic gigantism. Of these, macroglossia is suggested to be closely associated with oral function and maxillofacial morphology.3,4,6,7 Macroglossia is defined as a resting tongue that protrudes beyond the teeth or the alveolar ridge.3,4,6,7 Although BWS is highly variable with regard to clinical presentation, macroglossia is the most consistent symptom and is evident in 80% to 99% of patients.1,14-16 A logical approach to preventing or
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Fig 9. Posttreatment dental models.
Fig 10. Posttreatment radiographs.
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Fig 11. Facial and intraoral photographs after 6 years of retention.
correcting the effects of macroglossia is performing a glossectomy, preferably before problems in speech development occur,17 but not before the patient is 6 months old.18 This is because an increase in the size of the tongue during the first few months of life has been reported, as has gradual improvement of the condition after 6 months of age.18 Accordingly, glossectomy is generally conducted at 2 to 3 years of age. Recently, it has become acceptable to perform glossectomy on younger patients because of advancements in operative and anesthetic techniques. Kadouch et al19 performed glossectomies in 23 patients with BWS at an average age of 19 months and reported stable outcomes. Glossectomy has resulted in reductions of mandibular length and width in animal studies.7 Furthermore, a radiographic cephalometric study comparing BWS patients who had undergone glossectomy with those who had not showed that anterior open bite and mandibular protrusion were improved via glossectomy.20 Our patient had a
glossectomy when she was 2 years 7 months of age, but some dentoalveolar and skeletal problems remained even at 5 years 10 months of age. Therefore, the glossectomy might have been performed too late to achieve optimal skeletal growth in this patient, considering the subsequent growth of the maxillofacial region. Both her tongue and predisposition for severe skeletal deformity could have influenced her skeletal growth pattern. There is some debate over whether we should provide phase 1 orthodontic treatment for children who might require orthognathic surgery in the future. Although nonsurgical treatment with mandibular growth modification with a chincap followed by multibracket appliances with or without tooth extraction was considered a potentially effective way to improve the Class III malocclusion, this method was deemed inapplicable because of this patient's severe dentoskeletal and facial disharmony. It seemed appropriate to combine glossectomy with phase 1 orthodontic treatment because if the
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Fig 12. Dental models after 6 years of retention.
Fig 13. Radiographs after 6 years of retention.
treatment plan had been limited to a glossectomy, the vertical problems such as increased gonial angle and open bite might not have been corrected.
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With regard to expansion for the treatment of transverse problems in the maxillary dental arch, we carefully considered the age of the patient; the timing of dental
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Fig 14. Cephalometric superimpositions. The SN plane at S, the palatal plane at ANS, and the mandibular plane at Me were the bases of the overall, maxillary, and mandibular superimpositions, respectively. Black lines, before phase 2 treatment; blue lines, posttreatment; red lines, after 6 years of retention; mandibular molars in black lines, the first and the second molars; mandibular molars in red and blue lines, the second and the third molars.
arch expansion; the condition of the periodontal tissues; the methods used, including a quad-helix, a rapid maxillary expander, and surgically assisted RME; the amount of necessary expansion; and the volume of the oral cavity. It was difficult to decide when to initiate expansion of the constricted maxillary dental arch width because it was possible that the patient would require orthognathic surgery after pubertal growth; thus, we could not accurately determine the required degree of expansion. It was also difficult to select treatment methods and associated orthodontic appliances. RME has been reported to be the most effective treatment to correct transverse maxillary discrepancies in growing adolescents; it functions by opening the midpalatal suture.21-23 According to Korn and Baumrind,24 the median palatine suture closes at approximately 14 of 15 years of age in girls and 15 to 16 years in boys. When RME is not feasible, a variety of surgical procedures including surgically assisted RME have been advocated in the treatment of transverse maxillary deficiency. This procedure allows for splitting of the median palatine suture and widening of the
maxilla.22 With regard to our patient, we thought that surgically assisted RME might increase the volume of the oral cavity and reduce the influence of her tongue volume on the stability after orthodontic treatment. Surgically assisted RME was considered to be an appropriate procedure because of her age and the amount of expansion required. Additionally, previous studies have reported that bimaxillary surgery, particularly advancement of the maxilla, can increase the volume of the oral cavity.25,26 Thus, it is important to consider the relationship between the volume of the tongue and the oral cavity to achieve long-term stability of the occlusion. This is the first report of longitudinal surgicalorthodontic treatment of a patient with BWS who had undergone glossectomy during early preadolescence. The results showed that early orthodontic intervention and surgical-orthodontic treatment were effective in resolving the skeletal disharmonies and in improving the facial profile and the occlusion. Orthodontists must make an accurate determination of macroglossia, which is the most prominent feature of BWS, because it is
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suggested to be closely associated with oral function and maxillofacial morphology. Additionally, the results of this patient’s treatment indicated that with the cooperation of an oral surgeon, an orthodontist can improve both facial appearance and occlusion in patients with BWS. This patient expressed great satisfaction with the improvements in her anterior crossbite and facial appearance. CONCLUSIONS
10.
11.
12. 13.
We reported the longitudinal and successful surgicalorthodontic treatment of a patient with severe mandibular prognathism and an anterior open bite associated with BWS. Over the course of orthodontic treatment lasting approximately 30 years combined with surgical correction of the musculoskeletal components—ie, the maxilla, the mandible, and the tongue—the patient acquired a favorable occlusion with harmonized facial esthetics. On the basis of our case report as well as previous studies by others, we suggest that balancing the structure and function of the dentoalveolar and musculoskeletal tissues of the orofacial complex is the key to a good treatment outcome and the long-term stability of patients with BWS.
14. 15. 16.
17. 18.
19.
ACKNOWLEDGMENTS
We thank the patient and her family for their cooperation and consent to publishing this report, and all the team members who were involved in her treatment. REFERENCES
21.
22.
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