Miyawaki  Shouichi

Noguchi  Haruhiro

Oya  Shinji

Takano-Yamamoto  Teruko

The Angle Orthodontist

English

Miyawaki, Shouichi

51806

Oya, Shinji

51807

Noguchi, Haruhiro

51808

Takano-Yamamoto, Teruko

51809

Tohoku University Repository (TOUR) / 東北大学機関リポジトリ

Long-Term Changes in Dentoskeletal Pattern ina Case with Beckwith-Wiedemann SyndromeFollowing Tongue Reduction and OrthodonticTreatment著者 Miyawaki  Shouichi, Oya  Shinji, Noguchi Haruhiro, Takano-Yamamoto  Terukojournal orpublication titleAngle Orthodontistvolume 70number 4page range 326-331year 2000URL http://hdl.handle.net/10097/51513326Angle Orthodontist, Vol 70, No 4, 2000Case ReportLong-Term Changes in Dentoskeletal Pattern in a Case withBeckwith-Wiedemann Syndrome Following Tongue Reductionand Orthodontic TreatmentShouichi Miyawaki, DDS, PhDa; Shinji Oya, DDSb; Haruhiro Noguchi, DDS, PhDc;Teruko Takano-Yamamoto, DDS, PhDdAbstract: Long-term changes in the dentoskeletal pattern in a 6-year-old Japanese girl with Beckwith-Wiedemann syndrome were demonstrated. The patient showed macroglossia, which is the most commonsymptom of the syndrome, protruded lower lip, mandibular protrusion and anterior open bite. The jawbase relationship improved to skeletal Class I and the molar relationship to Angle Class I at the earlypreadolescent period following tongue reduction and phase I orthodontic treatment using a chin cap andtongue crib. Optimum intercuspation of teeth was achieved after edgewise treatment without orthognathicsurgery, and a skeletal Class I apical base relationship and good facial profile were maintained after theretention period of 2 years. This case report suggests that early orthodontic treatment with tongue reductioncan be effective in a case with Beckwith-Wiedemann syndrome to improve an abnormal dentoskeletalpattern. (Angle Orthod 2000;70:326–331.)Key Words: Beckwith-Wiedemann syndrome; Macroglossia; Tongue reduction; Orthodontic treatmentINTRODUCTIONBeckwith in 1963 and Wiedemann in 1964 originally re-ported Beckwith-Wiedemann syndrome. Macroglossia isthe predominent finding in Beckwith-Wiedemann syndrome(97%); however, other findings include postnatal somaticgigantism (88%), abdominal wall defects and hernias(80%), abnormal earlobe creases/pits (76%), hypoglycemia(63%), nevus flammeus of the face (62%), nephromegaly(59%), hemihypertrophy (24%), congenital heart defects(6.5%) and cleft palate (2.5%).1–4 Neurologic problems ormental retardation occurs in only a minority of patients, andis likely the result of hypoglycemic attacks in the neonatalperiod. Childhood neoplasms develop in some of the peopleaffected by this syndrome and most are malignant. Theyinclude nephroblastoma, adrenocortical cancer, and hepa-a Assistant professor, Department of Orthodontics, Okayama Uni-versity Dental School, Okayama, Japan.b Postgraduate student, Department of Orthodontics, Okayama Uni-versity Dental School, Okayama, Japan.c Research Assistant, Department of Oral and Maxillofacial Sur-gery, Nara Medical University, Kashihara city, Japan.d Professor and Chair, Department of Orthodontics, Okayama Uni-versity Dental School, Okayama, Japan.Corresponding author: Teruko Takano-Yamamoto, DDS, PhD, De-partment of Orthodontics, Okayama, University Dental School 2-5-1Shikata-Cho, Okayama 700-8525, JAPAN.(e-mail: tpyamamo@dent.okayama-u.ac.jp).Accepted: March 2000. Submitted: March 2000.q 2000 by The EH Angle Education and Research Foundation, Inc.toblastoma. Some experts feel that all of these patientsshould be screened for cancer with regular abdominal ul-trasound and serum alfa-fetoprotein levels.Previous studies have shown that one major effect ofmacroglossia is a protrusion of dentoalveolar structures,which results in a protruding mandible, anterior open bite,abnormally obtuse gonial angle, and increased mandibularlength.5–7 Therefore, early intervention by tongue reduc-tion,8 early functional treatment of the stomatognathic sys-tem,9 or both, is recommended in order to prevent bothmandibular prognathism and anterior open bite.There have been few case reports or studies concerningthe longitudinal dentoskeletal changes in patients withBeckwith-Wiedemann syndrome treated with tongue reduc-tion and orthodontic treatment. This article demonstratesthe successful treatment and the long-term changes in den-toskeletal pattern in a patient with Beckwith-Wiedemannsyndrome who was treated by tongue reduction and phaseI and II orthodontic treatments.CASE REPORTPreadolescenceA 6-year-old Japanese girl with macroglossia, mandibu-lar protrusion, and anterior open bite was diagnosed withBeckwith-Wiedemann syndrome characterized by macro-glossia, postnatal somatic gigantism, and nephromegaly ata national hospital in Japan. The pediatrician at the hospital327DENTOSKELETAL PATTERN AND BECKWITH-WIEDEMANN SYNDROMEAngle Orthodontist, Vol 70, No 4, 2000FIGURE 1. Facial photographs. (A) Initial stage (6Y4M); (B) Post-active-treatment stage (15Y8M); (C) Post-retention stage (18Y7M);Left: Frontal view; Right: Lateral view.detected dentoskeletal disharmony, and orthognathic treat-ment was initiated at a University Dental Hospital. Familyhistory revealed no notable hereditary diseases.Clinical examination. The patient had a concave profileand a severely protruded lower lip (Figure 1). The tonguewas large and, when extended, was long enough to reachthe mentum (Figure 2). Mandibular protrusion and an an-terior open bite were present. The molar relationship wasAngle Class III, and all teeth showed cross bite relation-ships (Figure 3). Lateral cephalometrics showed a skeletalClass III apical base relationship, normal-sized maxilla, an-terior displacement of the mandible, long mandibularlength, and obtuse gonial angle when compared with thenormative values for Japanese girls of corresponding age(Figure 4; Table 1).10 Such a condition hindered verbal abil-ities and the patient experienced difficulty in pronouncing‘‘s,’’ ‘‘t,’’ and ‘‘l’’ sounds.Treatment plan. Treatment was planned as follows: (1)tongue reduction by the Becker method,11 (2) inhibition ofmandibular growth and tongue protrusion using a chin capand tongue crib, and (3) review the diagnosis after com-pletion of the permanent dentition.Treatment progress. Tongue reduction was performed at8 years of age using the Becker method.11 A nighttime chincap appliance was begun 2 months after the surgery andcontinued for 1 year and 5 months. A tongue crib was usedfrom age 8 years 7 months old for 7 months.Results achieved. The protruded lower lip was corrected(Figure 1) and the tongue volume was reduced (Figure 2).The skeletal Class III jaw base relationship and anterioropen bite improved rapidly after tongue reduction andphase I orthodontic treatment (Figures 4 and 5). Total crossbite was partially corrected, and Angle Class I molar rela-tionship was achieved, but a slight anterior open bite re-mained (Figure 5). The difficulties in pronunciation foundprior to the tongue reduction were improved.AdolescenceThe dentoskeletal disharmony was corrected by tonguereduction and phase I orthodontic treatment and the patientwas observed for 2 years and 4 months. At the end of thisobservation period (11 years 6 months of age) data com-parable to the initial stage were recorded. At this time theprofile was straight and moderate crowding with slight an-terior open bite was observed (Figure 5). Lateral cephalo-metric radiographs showed a skeletal Class I jaw base re-lationship and average mandibular plane angle (Figure 4;Table 1) when compared to the normative values for Jap-anese girls of the corresponding age.10 The molar relation-ship was Angle Class I (Figure 5).Treatment plan. Treatment was planned as follows: (1)alignment of teeth using an edgewise appliance, and (2)retention with a Hawley-type retainer and fixed lingual re-tainer on the upper and lower dentitions, respectively.Treatment progress. Edgewise treatment was initiated atage 11 years 7 months of age. Space closure was performedfor 1 year after leveling. Up and down elastics were usedfor 6 months and detailing was conducted for 1 and a halfyears. The appliance was removed when the patient was 15years 8 months of age. A Hawley type retainer and fixedlingual retainer were then applied to the upper and lowerdentitions, respectively. The removable and fixed retainerswere removed at age 18 years 7 months.Results achieved. Optimum intercuspation of teeth withparallel dental roots was achieved from the active ortho-dontic treatment (Figure 5). The skeletal Class I apical baserelationship (Figures 4 and 6) was maintained. Acceptablygood occlusion and facial profile were also maintained afterthe retention period of 2 years (Figures 1 and 6).328 MIYAWAKI, OYA, NOGUCHI, TAKANO-YAMAMOTOAngle Orthodontist, Vol 70, No 4, 2000FIGURE 2. Photographs of protruded tongue. (A) Initial stage (6Y4M); (B) Just before edgewise treatment (11Y6M).FIGURE 3. Photographs of dental casts at the pretreatment stage (6Y4M). (A) Lateral view on the right side; (B) Frontal view; (C) Lateral viewon the left side; (D) Occlusal view of maxillary dentition; (E) Occlusal view of mandibular dentition.329DENTOSKELETAL PATTERN AND BECKWITH-WIEDEMANN SYNDROMEAngle Orthodontist, Vol 70, No 4, 2000FIGURE 4. Cephalometric radiographs. (A) Initial stage (6Y4M); (B) Just before edgewise treatment (11Y6M); (C) Just before debonding(15Y6M); (D) After retention period (18Y7M).TABLE 1. Cephalometric Analysis at the Initial Stage (6Y4M), Just Before Edgewise Treatment (11Y6M) and After the Retention Period (18Y7M)Measurements6Y4MValue Z-Scorea11Y6MValue Z-Scorea18Y7MValue Z-ScoreaN-S (mm)Ar-Me (mm)N-Me (mm)SNA (degree)SNB (degree)MP-SN (degree)Gonial angle (degree)Overjet (mm)Overbite (mm)U1-SN (degree)L1-MP (degree)64.6105.1107.583.887.734.9137.529.126.0108.088.010.814.910.710.914.321.311.628.924.512.910.167.9106.9120.581.782.137.8132.60.920.9111.184.610.310.920.110.311.110.212.222.322.310.721.571.1114.9133.380.381.138.7130.72.31.3109.188.710.911.411.510.110.710.311.610.711.110.410.7a Z-score was calculated as (value 2 norm)/1 SD using norms and SDs of mean Japanese females according to the corresponding age.10330 MIYAWAKI, OYA, NOGUCHI, TAKANO-YAMAMOTOAngle Orthodontist, Vol 70, No 4, 2000FIGURE 5. Intraoral photographs. (A) Just before edgewise treatment (11Y6M); (B) Just before debonding (15Y6M); (C) Just before removingof retainer (18Y6M); Left: Lateral view on the right side; Center: Frontal view; Right: Lateral view on the left side.FIGURE 6. Changes from initial to post-retention stages on super-imposed tracings of lateral cephalograms. Solid line: Initial stage(6Y4M); Dashed line: Just before edgewise treatment (11Y6M); Dot-ted line: After retention period (18Y7M).DISCUSSIONA genetic diagnosis is now possible for Beckwith-Wie-demann syndrome.12,13 A past radiographic cephalometricstudy, using patients who had and had not undergonetongue reduction surgery, showed that the anterior open biteand mandibular protrusion of patients who had undergonetongue reduction were improved.8 The patients in this studyshowed similar improvements. The changes at the preado-lescent period, following tongue reduction surgery andphase I orthodontic treatment using a chin cap and tonguecrib, were dramatic as shown in Figures 4 and 6. Due tothese effects, the patient’s abnormal dentoskeletal pattern,including skeletal Class III and anterior open bite, becamealmost normal at the early stage of the adolescent period.As a result, phase II orthodontic treatment was successful,with the patient showing few signs of relapse 2 years afteredgewise treatment.Recently, a patient with Beckwith-Wiedemann syndromewho underwent surgical orthodontic treatment and was ob-served over a long period of time was reported in Japan.14Although the patient showed severe anterior open bite dueto macroglossia, dentoskeletal disharmony was adjusted bya sagittal split ramus osteotomy without conducting tonguereduction. By 1 year after edgewise treatment, the patientshowed a severe relapse with a 2 mm open bite despite thefact that acceptable occlusion was present at debonding.Accordingly, tongue reduction conducted at a preadoles-cent period surgical orthodontic treatment may preclude theneed for surgical orthodontic treatment, with virtually norelapse and resulting in stable occlusion. Both a previousstudy8 and this case report suggest the efficacy of thetongue reduction in Beckwith-Wiedemann syndrome pa-tients with macroglossia to improve the skeletal Class IIIand open bite. However, there is also a report showing thatfunctional treatment using a pacifier and stimulation plateto the tongue during infancy resulted in no need for tonguereduction.9331DENTOSKELETAL PATTERN AND BECKWITH-WIEDEMANN SYNDROMEAngle Orthodontist, Vol 70, No 4, 2000Based on previous studies and our case report, we rec-ommend that patients with Beckwith-Wiedemann syndromeundergo the following orthognathic treatment: (1) earlyfunctional treatment of the stomatognathic system; (2) ifthe effect is not adequate, tongue reduction should be car-ried out as early as possible during the early stage of thepreadolescent period; and (3) orthodontic treatment, or-thognathic surgery, or both, should be initiated as neces-sary.ACKNOWLEDGMENTSWe extend our appreciation to Mr Virgil Hawkins of Osaka Uni-versity for checking the English grammar of this paper.REFERENCES1. Elliott M, Bayly R, Cole T, Temple IK, Maher ER. Clinical fea-tures and natural history of Beckwith-Wiedemann syndrome: pre-sentation of 74 new cases. Clin Genet. 1994;46:168–174.2. Filippi G, Mckusick VA. The Beckwith-Wiedemann syndrome:report of two cases and review of the literature. Medicine. 1970;49:279–298.3. McManamny DS, Barnett JS. Macroglossia as a presentation ofthe Beckwith-Wiedemann syndrome. Plast Reconstr Surg. 1985;75:170–176.4. Engstrom W, Lindham S, Schofield P. Wiedemann-Beckwith syn-drome. Eur J Pediatr. 1988;147:450–457.5. Irving I. Exomphalos with macroglossia: a study of eleven cases.J Pediatr Surg. 1967;2:499–507.6. Kamogashira K, Ito T, Nakagawa M, Kawagoe H, Ichikawa K,Matsumoto M. Orthodontic findings of a case of Beckwith-Wie-demann syndrome. J Jpn Orthod Soc. 1984;43:564–572.7. Friede H, Figueroa AA. The Beckwith-Wiedemann syndrome: alongitudinal study of the macroglossia and dentofacial complex.J Craniofac Genet Dev Biol Suppl. 1985;1:179–187.8. Menard RM, Delaire J, Schendel SA. Treatment of the craniofa-cial complications of Beckwith-Wiedemann syndrome. Plast Re-constr Surg. 1995;96:27–33.9. Mussig HD, Zschiesche S. Early orthodontic treatment measuresin infants with the EMG syndrome. Fortschr Kieferorthop. 1989;50:460–464.10. Susami R. A cephalometric study of dentofacial growth in man-dibular prognathism. J Japan Orthod Soc. 1967;26:1–34.11. Becker R. Shortening of the tongue as an aid to orthodontic treat-ment. Dtsch Zahn Mund Kieferheilkd Zentralbl Gesamte. 1966;46:210–219.12. Li M, Squire JA, Weksberg R. Molecular genetics of Wiedemann-Beckwith syndrome. Am J Med Genet. 1998;79:253–259.13. Hatada I, Ohashi H, Fukushima Y, Kaneko Y, Inoue M, KomotoY, Okada A, Nabetani A, Morisaki H, Nakayama M, Niikawa N,Mukai T. An imprinted gene p57KIP2 is mutated in Beckwith-Wiedemann syndrome. Nat Genet. 1996;14:171–173.14. Amino K, Kobayashi K. A case report of openbite malocclusionwith Beckwth-Wiedemann syndrome. Nishinihonn Kyousei Shi-kagaku Zassi. 1995;40:74–82.

Long-Term Changes in Dentoskeletal Pattern in a Case with Beckwith-Wiedemann Syndrome Following Tongue Reduction and Orthodontic Treatment

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Long-Term Changes in Dentoskeletal Pattern in a Case with Beckwith-Wiedemann Syndrome Following Tongue Reduction and Orthodontic Treatment

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