Dentin dysplasia type I (DD-I) is a rare hereditary

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1 CLINICIAN'S CORNER Orthodontic treatment of a patient with dentin dysplasia type I Roberto Bespalez-Filho, a Soraya De Azambuja Berti Couto, b Paulo Henrique Couto Souza, c Fernando Henrique Westphalen, d Reinhilde Jacobs, e Guy Willems, f and Orlando Motohiro Tanaka g Guaıra and Curitiba, Parana, and Porto Alegre, Rio Grande do Sul, Brazil, Leuven, Belgium, and St. Louis, Mo Dentin dysplasia is a genetic disorder of the teeth that affects the dentin and the pulp. Type I is sometimes called rootless teeth, because of the loss of organization of the root dentin, which often leads to a shortened root length. The purpose of this article was to present a rare clinical case of a girl who was diagnosed with dentin dysplasia type I when she was referred for an orthodontic evaluation. Panoramic and periapical radiographs showed defective root formation and areas with periapical radiolucencies in several teeth. Her Angle Class I malocclusion was successfully treated, providing esthetic and functional results, without clinical symptoms or signs of periodontitis or odontogenic infections. (Am J Orthod Dentofacial Orthop 2013;143:421-5) Dentin dysplasia type I (DD-I) is a rare hereditary anomaly. Its etiology might be related to an autosomal dominant inheritance. 1 This anomaly is also known as rootless teeth because of the loss of organization of the root dentin, often leading to shortened roots. 2 Clinically, the teeth have normal morphology. 3,4 Some main clinical symptoms include mobile teeth and pain associated with spontaneous dental abscesses or cysts. 5 In addition, premature exfoliation and delayed eruption have also been reported. 2 Radiographically, the disorder is characterized by partial or complete obliteration of the pulp chambers, defective root formation, and a tendency for periapical radiolucencies with no a Private practice (specialist in oral radiology), Guaıra, Parana, Brazil. b Postgraduate student, postgraduate program in stomatology, Dental College, Pontifical Catholic University of Rio Grande do Sul, Porto Alegre, Rio Grande do Sul, Brazil. c Professor, graduate dentistry program in stomatology, School of Dentistry, Pontifical Catholic University of Parana, Curitiba, Parana, Brazil. d Professor, graduate dentistry program in radiology, School of Dentistry, Pontifical Catholic University of Parana, Curitiba, Parana, Brazil. e Professor, Department of Oral Health Sciences, KU Leuven and Dentistry, University Hospitals Leuven, Leuven, Belgium. f Professor of orthodontics, Department of Oral Health Sciences, KU Leuven and Dentistry, University Hospitals Leuven, Leuven, Belgium. g Professor, graduate dentistry program in orthodontics, School of Dentistry, Pontifical Catholic University of Parana, Curitiba, Parana, Brazil; postdoctoral fellow, The Center for Advanced Dental Education, Saint Louis University, St. Louis, Mo. The authors report no commercial, proprietary, or financial interest in the products or companies described in this article. Reprint requests to: Orlando Motohiro Tanaka, Rua Imaculada Conceiç~ao, 1155, , Curitiba, Parana, Brazil; , tanakaom@gmail.com. Submitted, July 2011; revised and accepted, January /$36.00 Copyright Ó 2013 by the American Association of Orthodontists. obvious cause. In addition, pulpal remnants might be visible in permanent teeth as crescent-shaped horizontal radiolucent lines, whereas deciduous teeth show complete pulp obliteration. 3 Numerous periapical radiolucencies are often seen in noncarious teeth. 4 Based on the radiographic features, 4 types of DD-I have been suggested: DD-Ia shows complete obliteration of the pulp chambers and no root development with many periapical radiolucent areas; DD-Ib shows horizontal, crescent-shaped, radiolucent pulpal remnants at the level of the cementoenamel junction and a few millimeters of root development with many periapical radiolucent lesions; DD-Ic has 2 horizontal crescent-shaped radiolucent lines concave toward each other at the cementoenamel junction and significant but incomplete root development with or without periapical radiolucent lesions; and DD-Id has radiographically visible pulp chambers and oval pulp stones in the coronal third of the root canal with bulging of the root. 6 According to several case reports in the literature, some treatments have been carried out in patients with DD-I, including preventive measures and careful observation 7 ; dietary analysis and advice; oral hygiene instructions; fluoride supplements; extractions; restoration of carious molars 3,5 ; treatment to restore the occlusion, enhance mastication, and improve esthetics 8 ; dental implants 9,10 ; and endodontic therapy. 11 However, in the literature, there are only a few articles about patients with DD-I who were managed by orthodontic treatment. 12 Therefore, the objective of this article was to present the successful orthodontic treatment of 421

2 422 Bespalez-Filho et al Fig 1. Pretreatment intraoral photographs show Class I malocclusion, bilateral posterior crossbite, anterior open bite, severely rotated maxillary incisors, and both labially and ectopically positioned maxillary canines. Fig 2. Pretreatment panoramic and periapical radiographs show that almost all teeth had defective, short, and tapered roots. a patient with DD-I. The patient obtained a good occlusal relationship and better dental and facial profile esthetics with no clinical symptoms or signals of periodontitis or odontogenic infections. Aspects regarding the diagnosis and treatment of DD-I are also discussed. March 2013 Vol 143 Issue 3 American Journal of Orthodontics and Dentofacial Orthopedics

3 Bespalez-Filho et al 423 Fig 3. Posttreatment intraoral photographs show that the Class I molar and canine relationships were maintained, with clinically acceptable overjet and overbite. CASE REPORT A girl, aged 11 years 11 months, was referred to a private orthodontic practice (R.B.F., Guaıra, Parana, Brazil). Her chief concerns were her crowded anterior teeth. The extraoral examination showed a convex profile and incompetent lips. The pretreatment intraoral photographs and dental casts showed an Angle Class I malocclusion, bilateral posterior crossbite, anterior open bite, severely rotated maxillary incisors, and bilateral ectopically positioned maxillary canines. The patient's oral hygiene was good (Fig 1). The panoramic and periapical radiographs showed defective roots in almost all teeth, with short and tapered roots, obliteration of root canals, and reduction of the coronal pulp chambers to horizontal slits, with some areas with periapical radiolucencies on the roots of the permanent molars and the mandibular left lateral incisor (Fig 2). Considering the clinical and radiographic features, the patient was diagnosed with DD-I. According to the cephalometric analysis, she had a skeletal Class I (ANB, 2 ) and excessive lower anterior facial height (FMA, 33 ; SN-GoGn, 41 ). The maxillary and mandibular incisors were buccally tipped and protruded (U1-NA, 42 ; L1-NB, 30 ). She had a convex profile (Z-angle, 60 ) (Table). The treatment objectives included correction of the posterior crossbite with a Haas-type appliance followed by extraction of all 4 first premolars. Straight-wire fixed appliances with a in slot were bonded for leveling and alignment of the maxillary and mandibular arches. The archwire sequence progressed from in nickel-titanium to in stainless steel archwires. Light elastic forces were used at night for 7 Table. Pretreatment and posttreatment cephalometric measurements Norm Author Pretreatment Posttreatment SNA ( ) 82 Steiner SNB ( ) 80 Steiner ANB ( ) 2 Steiner 2 3 Facial convexity ( ) 0 Downs 4 4 Y-axis 59.9 Downs Facial angle ( ) 87.8 Downs SN-GoGn ( ) 32 Steiner FMA ( ) 25 Tweed IMPA ( ) 90 Tweed U1-NA ( ) 22 Steiner U1-NA (mm) 4 Steiner 8 4 L1-NB ( ) 25 Steiner L1-NB (mm) 4 Steiner ( ) 130 Downs L1-APo 1 Ricketts 8 3 LS-S 0 Steiner 0 2 LI-S 0 Steiner 3 0 Z-angle ( ) 75 Merrifield months until appliance removal. Treatment length was 2 years 10 months. After debonding, a maxillary Hawley-type retainer was delivered, and a mandibular canine-to-canine retainer was bonded. The teeth were well aligned and leveled over the basal bone. Class I molar and canine relationships were maintained, with good overjet and overbite. There was improvement in incisor inclination (U1-NA, 18 ; L1-NA, 23 ), and a balanced soft-tissue profile was achieved (Z-angle, 68 ) at the end of treatment (Fig 3, Table). The radiographs showed no further shortening of the roots (Fig 4). American Journal of Orthodontics and Dentofacial Orthopedics March 2013 Vol 143 Issue 3

4 424 Bespalez-Filho et al Fig 4. Posttreatment panoramic, periapical, and cephalometric radiographs show no further shortening of the roots. DISCUSSION Although DD-I is a genetic defect of dentin formation, 3 its etiology is still considered a mystery. 1 This kind of dentin dysplasia is also described as the most rare of dentin disorders. 13 As a hereditary disturbance, some authors have published reports of families affected by DD-I. 3,6,14 Our patient s family was not investigated, but this information is most useful to detect more patients with DD-I and to prevent dental problems in future generations. Some authors recommend that, in hereditary dentin disorders, the family history should be established to identify other members who are affected so that a pedigree diagram can be compiled. Because dentin dysplasia is inherited in an autosomal dominant fashion, there is a 50% chance that a child born from an affected parent will develop the same genetic defect. 4 The diagnosis is established through the history reported by the patient and also by clinical and radiographic examinations. However, the molecular genetic diagnosis might be a useful adjunct to clinical analysis, particularly when the precise diagnosis is in doubt. 4 Usually, clinical characteristics include mobility and periodontal disease, which were not found during the clinical examination of this patient, who had a severe Angle Class I malocclusion. The diagnosis of DD-I (DD-Ic) could be confirmed after radiographic analysis based on features such as shortening of the roots and periapical radiolucencies in the permanent molar areas and the mandibular left lateral incisor area. Detailed anamnesis and clinical examination, followed by a radiographic analysis, allowed establishment of the diagnosis and determination of the clinical management. The management of patients with dentinal dysplasia has for many years caused great difficulties for dentists. 5 The orthodontic treatment presented here demonstrates successful results and raises a remarkable question: is it possible to carry out orthodontic therapy in patients with DD-I? To answer this question, we need to point out that in DD-I, the crowns of the deciduous and permanent teeth are normal in shape, form, and color, but they have short roots that cause high mobility, leading to early exfoliation. In addition, periapical radiolucencies are frequently noted. 13 Thus, orthodontic therapy in these patients is not the standard treatment March 2013 Vol 143 Issue 3 American Journal of Orthodontics and Dentofacial Orthopedics

5 Bespalez-Filho et al 425 recommendation, although tooth malalignment is a common characteristic of this disorder. 3 However, treatment of a patient with DD-I depends on the patient's age, the severity of the malocclusion, and the prevailing complaint. 4 In this context, it is known that periapical inflammation in DD-I appears secondary to caries or spontaneous coronal exposure of microscopic threads of pulpal remnants in the defective dentin. 2 Furthermore, spontaneous exfoliation is probably due to periodontal disease on teeth with unfavorable crown-root ratios. 5 Consequently, both alterations do not facilitate any kind of orthodontic treatment in patients with DD-I. Nevertheless, some authors have stated that a conservative attitude toward any invasive treatment of highly mobile teeth, periapical radiolucencies, and malalignment results in optimal preservation of the dentition. 3 Our patient did not have caries or tooth mobility, and she had a vital pulp response to thermal tests. However, a vertical skeletal pattern and an anterior open bite with some dental component associated with excessive lower facial height were considered important factors for the decision to carry out orthodontic treatment. Undoubtedly, the latter features were fundamental for the success of treatment. Patients with shortened roots require special treatment planning considerations, such as the application of light orthodontic forces trying not to shorten the roots even more; since the root is short, the center of resistance moves coronally. 15 The esthetic and functional improvement often justifies the risks of orthodontic treatment in a patient with shortened roots, but these risks must be clearly explained to the patient and the family before treatment begins. 16 CONCLUSIONS Orthodontic treatment can be considered in a patient with DD-I; however, when evaluated and approved by the dentist and also by the patient, it should be done carefully, and all risks should be clearly explained to the patient and the family. In this patient, the clinical results showed a successful treatment, and the proposed goals were achieved, considering the follow-up performed. REFERENCES 1. Kim JW, Simmer JP. Hereditary dentin defects. J Dent Res 2007; 86: Neville BW, Damm DD, Allen CM, Bouquot JE. Oral and maxillofacial pathology. 3rd ed. Philadelphia: Saunders; p Kalk WW, Batenburg RH, Vissink A. Dentin dysplasia type I: five cases within one family. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1998;86: Barron MJ, Donnell ST, Mackie I, Dixon MJ. Hereditary dentine disorders: dentinogenesis imperfecta and dentine dysplasia. Orphanet J Rare Dis 2008;3: Shankly PE, Mackie IC, Sloan P. Dentinal dysplasia type I: report of a case. Int J Paediatr Dent 1999;9: Carroll MK, Duncan WK, Perkins TM. Dentin dysplasia: review of the literature and a proposed subclassification based on radiographic findings. Oral Surg Oral Med Oral Pathol 1991;72: Comer TL, Gound TG. Hereditary pattern for dentinal dysplasia type Id: a case report. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2002;94: Ozer L, Karasu H, Aras K, Tokman B, Ersoy E. Dentin dysplasia type I: report of atypical cases in the permanent and mixed dentitions. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2004; 98: Depprich RA, Ommerborn MA, Handschel JG, Naujoks CD, Meyer U, K ubler NR. Dentin dysplasia type I: a challenge for treatment with dental implants. Head Face Med 2007;3: Munhoz-Guerra MF, Naval-Gıas L, Escorial V, Sastre-Perez J. Dentin dysplasia type I treated with onlay bone grafting, sinus augmentation, and osseointegrated implants. Implant Dent 2006; 15: Ravanshad S, Khayat A. Endodontic therapy on a dentition exhibiting multiple periapical radiolucencies associated with dentinal dysplasia type 1. Aust Endod J 2006;32: Brenneise CV, Dwornik RM, Brenneise EE. Clinical, radiographic, and histological manifestations of dentin dysplasia type I: report of case. J Am Dent Assoc 1989;119: Hart PS, Hart TC. Disorders of human dentin. Cells Tissues Organs 2007;186: Seow WK, Shusterman S. Spectrum of dentin dysplasia in a family: case report and literature review. Pediatr Dent 1994;16: Choy K, Pae EK, Park Y, Kim KH, Burstone CJ. Effect of root and bone morphology on the stress distribution in the periodontal ligament. Am J Orthod Dentofacial Orthop 2000;117: Tanaka OM, Knop LAH, Shintcovski RL, Hirata TM. Treatment of a patient with severely shortened maxillary central incisor roots. J Clin Orthod 2008;42: American Journal of Orthodontics and Dentofacial Orthopedics March 2013 Vol 143 Issue 3