Use of Dental Implants in the Management of Syndromal Oligodontia

This article was published in an Elsevier journal. The attached copy is furnished to the author for non-commercial research and education use, including for instruction at the author’s institution, sharing with colleagues and providing to institution administration. Other uses, including reproduction and distribution, or selling or licensing copies, or posting to personal, institutional or third party websites are prohibited. In most cases authors are permitted to post their version of the article (e.g. in Word or Tex form) to their personal website or institutional repository. Authors requiring further information regarding Elsevier’s archiving and manuscript policies are encouraged to visit: http://www.elsevier.com/copyright Author's personal copy Atlas Oral Maxillofacial Surg Clin N Am 16 (2008) 33–47 Use of Dental Implants in the Management of Syndromal Oligodontia Robert P. Carmichael, DMD, MSc, FRCDCa,b,c,d,*, George K.B. Sándor, MD, DDS, PhD, Dr Habil, FRCDC, FRCSC, FACSa,b,c,d,e,f a Bloorview Kids Rehab, Suite 2E-285, 150 Kilgour Road, Toronto, Ontario M4G 1R8, Canada The Hospital for Sick Children, S-525, 555 University Avenue, Toronto, Ontario M5G 1X8, Canada c University of Toronto, Toronto, Ontario, Canada d Mount Sinai Hospital, Toronto, Ontario, Canada e Regea Institute for Regenerative Medicine, University of Tampere, Biokatu 12, Tampere, Finland f University of Oulu, Oulu, Finland b Every instance of agenesis of a tooth represents an inborn error in dentogenesis. Just as current knowledge of metabolic and regulatory roles of essential molecules has been shaped partly by how inborn errors of their metabolism cause metabolic diseases, so too can accumulation and diffusion of knowledge of defects in dentogenesis facilitate a better understanding of craniofacial morphogenesis. On a practical level, clinicians should know the extent to which protocols for oral habilitation involving dental implants can be borrowed from our primary knowledge base and used to manage patients born with multiple malformations. This article reviews the literature relevant to the use of dental implants in patients with oligodontia secondary to chromosomal syndromes, such as it is, and illustrates the management of several of them. In Sweden, it was estimated that 15% of children and adolescents missing eight or more permanent teeth were afflicted with a syndrome such as ectodermal dysplasia (ED). Of the 2800 syndromes listed in POSSUM, a computer-based resource that helps clinicians to diagnose syndromes in their patients, 126 syndromes are associated with anodontia or oligodontia (http:// www.possum.net.au/). A synthesis of available literature estimated that the most frequent syndromes associated with agenesis of teeth are the EDs and Down syndrome. ED is a syndrome characterized chiefly by abnormalities of the tissues that originate from ectoderm, namely skin, nails, hair, and teeth (Fig. 1). There are more than 150 variants of ED, with hypohidrotic ED (HED) exhibiting the most severe dental anomalies and a typical craniofacial dysmorphology, which makes it of greatest interest to dentists (Figs. 2 and 3). With an incidence of 1/100,000 births, HED is a relatively common syndrome. Depending on the type of treatment required, dental care for patients with ED has a significant financial impact on patients and their families. Articles in the dental implant literature tend not to distinguish among the many variants of ED (Fig. 4), labeling them all simply as ED when it is likely that manydif not mostdreported cases are HED. Numerous single case reports and small cohort studies in the literature describe the use of implants to support mandibular prostheses in children with ED. Since 1991, reports of single cases and small series of children with ED having been treated with implants have appeared in the literature; follow-up periods range from 0 months to 12 years and report few failure statistics. One retrospective study of 61 implants placed in 14 adolescents and young adults * Corresponding author. Bloorview Kids Rehab, Suite 2E-285, 150 Kilgour Road, Toronto, Ontario, Canada M4G 1R8. E-mail address: [email protected] (R.P. Carmichael). 1061-3315/08/$ - see front matter Ó 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.cxom.2007.10.004 oralmaxsurgeryatlas.theclinics.com Author's personal copy Fig. 1. A 17-year-old girl with mild expression of a hidrotic variant of ED. She expressed a pattern of anomalies, including thin hair and nails and severe oligodontia. (A) Facial view. (B) Right profile view. (C) Frontal view. (D) Preoperative panoramic tomography. (E) Maxillary occlusal view after edentulation and placement of dental implants. (F) Mandibular occlusal view after edentulation and placement of dental implants. (G) Postoperative panoramic tomography. (H) Maxillary master cast. (I) Mandibular master cast. (J) Frontal view of fixed bridges. (K) Occlusal view of maxillary fixed bridge. (L) Occlusal view of mandibular fixed bridge. (M) Postoperative facial view. (N) Postoperative right profile. (O) Postoperative view of smile. Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA Fig. 1 (continued ) 35 Author's personal copy 36 CARMICHAEL & SÁNDOR Fig. 2. A 21-year-old woman with hypohidrotic ED characterized by hypotrichosis (note hairpiece), hypohidrosis, and severe oligodontia. The entire permanent dentition comprised cone-shaped maxillary central incisors and canines and a small maxillary left first molar. (A) Facial view. (B) Right profile. (C–F) Initial definitive treatment included decoronation of all five teeth, construction of cast copings and a complete upper overdenture, and placement of an acrylic/gold fixed mandibular bridge. (G–J) Series of panoramic tomographs demonstrates initial presentation at age 21 years (G), maxillary cast overdenture copings and a first mandibular fixed bridge (H), and maxillary implants (I). (J–N) Current dental status at age 41. (J) Frontal view. (K) Maxillary occlusal view. (L) Mandibular occlusal view. (M) Facial view, age 41 years. (N) Right profile. Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA Fig. 2 (continued ) 37 Author's personal copy 38 CARMICHAEL & SÁNDOR Fig. 3. (A) An 8-year-old boy with hypohidrotic ED in whom a two-piece bridge, split at the mandibular midline, on four anterior mandibular implants, was constructed. (B) Panoramic tomography. (C) Despite ongoing mandibular growth, no separation of the proximal surfaces of the right and left sides of the bridge is visible, which suggests that no parasymphyseal growth has occurred. (D) Right half of bridge removed. who were followed from 1 to 5 years reported a 67% success rate. A prospective trial of 51 patients followed for up to 78 months reported survival rates of 91% in the mandible and 76% in the maxilla. The consensus from these reports seems to be that results support the continued use of implants in young children when appropriate precautions are taken not to interfere with growth of the jaws. Research has shown that treatment with implants in patients with HED does not rescue normal craniofacial growth and development. Isolated case reports in the literature have documented the use of dental implants in patients with some other syndromes or conditions that may be associated with oligodontia: Down syndrome (Fig. 5), which occurs at an incidence of 1 in approximately 660 births and is the most common pattern of malformation in humans, and cleidocranial dysplasia (Fig. 6). In one retrospective study, implant survival was investigated retrospectively over 12 years in patients who exhibited various systemic diseases and congenital defects, including eight implants in three adult patients who had Down syndrome who were followed for 2, 9, and 11 years, respectively. The survival rate of the loaded implants was 100%. Oral hygiene that resulted in gingivitis and mucositis was noted as the only complication with these patients. In a preliminary report of a prospective study of patients with neurologic disabilities, one of the two adults who had Down syndrome lost one implant because of bone loss at 17 months after loading, and another experienced loss of half the bone support because of a sequestration after flap dehiscence attributed partly to the patient’s macroglossia. The author speculated that the immune defects known to exist in patients who have Down syndrome may have played a role in implant bone loss and stressed the importance of informing the patient’s caregiver about maintenance of good oral hygiene. Patients with cleidocranial dysplasia commonly present with significant dental problems, such as aplasia, impaction or delayed eruption of permanent teeth, and the presence of supernumerary teeth. Several approaches have been described for the management of such patients. Although there has been a shift in the management paradigm for cleidocranial dysplasia from edentulation and prosthetic replacement to orthodontically assisted forced eruption and fixed appliance orthodontic treatment combined with orthognathic surgery, dental implant supported restorations have been used in both modes of treatment. Retrospective Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA 39 Fig. 4. An 18-year-old young man with Hallermann-Streiff syndrome (oculomandybulodyscephaly with hypotrichosis syndrome) characterized by small stature, microphthalmia, small pinched nose, hypotrichosis, and oligodontia. (A) Preoperative facial view. (B) Preoperative right profile. (C) Frontal view of teeth demonstrates severe oligodontia and retention of primary molars and skeletal anterior open bite. (D) Facial view after removal of retained primary and misaligned and misshapen permanent teeth and restoration with maxillary and mandibular porcelain/gold fixed bridges. (E) Right profile. (F) Frontal view of restored dentition. (G) Postoperative maxillary occlusal view. (H) Postoperative mandibular occlusal view. Author's personal copy 40 CARMICHAEL & SÁNDOR Fig. 5. A 22-year-old man with Down syndrome (trisomy 21) after orthodontic therapy and replacement of missing teeth with dental implants. (A) Facial view demonstrates hypotonia with tendency to keep mouth open and protrude the tongue. (B) Profile demonstrates prognathic mandible. (C) Panoramic radiograph demonstrates implant replacement of missing teeth. (D) Postoperative frontal view of teeth demonstrates class III occlusion with negative overjet. (E) Postoperative maxillary occlusal view. (F) Postoperative mandibular occlusal view demonstrates macroglossia. (G) View of smile. Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA 41 Fig. 6. A 16-year-old girl with cleidocranial dysplasia who presented with a mostly unerupted permanent dentition. She was treated with partial edentulation, alveolar augmentation using coral granules, reconstruction with an implant-retained complete upper overdenture and mandibular fixed bridge and followed for 10 years. (A) Facial view demonstrates brachycephaly with frontal bossing, midfacial hypoplasia, low nasal bridge, and hypertelorism. (B) Front view of teeth demonstrates partially retained, worn primary dentition. (C) Maxillary occlusal view demonstrates the two sole erupted permanent teethdthe first molars. (D) Mandibular occlusal view demonstrates partial retention of primary dentition. (E) Maxillary occlusal view of acrylic partial upper denture. (F) Panoramic tomograph demonstrates deep impaction of permanent dentition and supernumerary teeth. (G) Posttreatment maxillary occlusal view of implant-supported bar assembly. (H) Intaglio surface of bar-retained complete upper overdenture with a cast titanium framework and horizontal and vertical vinyl attachments. (I) Frontal view of complete upper overdenture and mandibular implant-supported fixed bridge. (J) Occlusal view of mandibular implant-supported fixed bridge. (K) Posttreatment facial view. Author's personal copy 42 CARMICHAEL & SÁNDOR Fig. 6 (continued ) Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA 43 Author's personal copy 44 CARMICHAEL & SÁNDOR : Fig. 8. A 22-year-old woman with hemifacial microsomia (oculo-auriculo-vertebral spectrum, Goldenhar syndrome) after mandibular reconstruction involving free-tissue transfer and dental implant replacement of missing teeth. (A) Facial view demonstrates right side hypoplasia of malar, maxillary, and mandibular regions, especially ramus and condyle. (B) Right profile demonstrates mild microtia. (C) Left profile demonstrates normal appearance. (D) Frontal view demonstrates up-to-right cant of occlusal plane, implant replacement of right maxillary lateral incisor, canine, first premolar, and mandibular incisors, and mandibular second premolars. (E) Maxillary occlusal view. (F) Mandibular occlusal view. Fig. 7. An 18-year-old girl with Treacher Collins syndrome (mandibulofacial dysostosis) after Le Fort I and bilateral sagittal split osteotomies, genioplasty, and replacement of missing maxillary central incisors with dental implant-supported crowns. (A) Postoperative facial view demonstrates malar hypoplasia and residual anterior open bite. (B) Right profile view demonstrates residual mandibular retrognathia, malformation of auricle, absence of lower eyelashes, and projection of scalp hair onto lateral cheek. (C) Panoramic tomography demonstrates orthognathic reconstruction hardware and dental implants at maxillary central incisor sites. (D) Frontal view demonstrates splinted implant-supported porcelain/gold crowns replacing maxillary central incisors. (E) Maxillary occlusal view demonstrates splinted crowns at central incisor sites, cemented to compensate for buccal angulation of implants. (F) Mandibular occlusal view. Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA 45 Fig. 9. A 21-year-old woman with frontonasal dysplasia sequence (median cleft face syndrome). (A) Facial view demonstrates result of complex craniofacial reconstruction to correct hypertelorism, broad nasion with a midline cleft in the bony dorsum, midline defect of the frontal bone, absence of the nasal tip, and deformities in the nasal alar region. (B) Right profile. (C) Frontal view of oral cavity demonstrates near total anodontia. (D) Maxillary occlusal view after reconstruction with implant-supported bar assembly for retention of complete upper overdenture followed for 10 years. (E) Mandibular occlusal view at 10-year follow-up of implants used to support fixed bridge. (F) Mandibular fixed bridge at 10-year follow-up. (G) Facial view at 10-year follow-up. (H) Right profile at 10-year follow-up. (I) Smile at 10-year follow-up. Author's personal copy 46 CARMICHAEL & SÁNDOR Fig. 9 (continued ) studies have reported the successful use of osseointegrated implants to support bone-anchored hearing aids in children who have Treacher Collins syndrome (Fig. 7) and hemifacial microsomia (Fig. 8) but not to support dental prostheses in these patients. Figs. 1 to 8 in this article were included as representatives of the most commonly encountered examples of chromosomal syndromes detailed in the foregoing discussion. Other rarer syndromes (Fig. 9) and nonsyndromal diseases and disorders were not included because of lack of space. From the small collection of cases reported here and despite the poverty of data documenting survival of dental implants in these diverse clinical populations, it can be seen that implant therapy can have a lasting and profoundly positive impact on patients with craniofacial anomalies. By their very nature, these patterns of malformation are rarely encountered, even by teams at large tertiary centers. It behooves those of us charged with their care to report treatment outcomes to facilitate the process of patient selection in accordance with an integration of best research evidence with clinical expertise and patient values. Further readings Alcan T, Basa S, Kargül B. Growth analysis of a patient with ectodermal dysplasia treated with endosseous implants: 6-year follow-up. J Oral Rehabil 2006;33(3):175–82. Author's personal copy MANAGEMENT OF SYNDROMAL OLIGODONTIA 47 Bergendal B. Prosthetic habilitation of a young patient with hypohidrotic ectodermal dysplasia and oligodontia: a case report of 20 years of treatment. Int J Prosthodont 2001;14(5):471–9. Bergendal T, Eckerdal O, Hallonsten AL, et al. Osseointegrated implants in the oral habilitation of a boy with ectodermal dysplasia: a case report. Int Dent J 1991;41(3):149–56. Bergendal B, Olgart K. Congenitally missing teeth. In: Kock G, Bergendal T, Kvint S, et al, editors. Consensus conference on oral implants in young patients: state of the art. Jönköping (Sweden): The Institute for Postgraduate Dental Research; 1996. p. 16–27. Daskalogiannakis J, Piedade L, Lindholm TC, et al. Cleidocranial dysplasia: 2 generations of management. J Can Dent Assoc 2006;72(4):337–42. de Rezende ML, Amado FM. Osseointegrated implants in the oral rehabilitation of a patient with cleft lip and palate and ectodermal dysplasia: a case report. Int J Oral Maxillofac Implants 2004;19(6):896–900. Ekfeldt A. Early experience of implant-supported prostheses in patients with neurologic disabilities. Int J Prosthodont 2005;18(2):132–8. Escobar V, Epker BN. Alveolar bone growth in response to endosteal implants in two patients with ectodermal dysplasia. Int J Oral Maxillofac Surg 1998;27(6):445–7. de Rezende ML, Amado FM. Osseointegrated implants in the oral rehabilitation of a patient with cleft lip and palate and ectodermal dysplasia: a case report. Int J Oral Maxillofac Implants 2004;19(6):896–900. Granström G, Tjellström A. The bone-anchored hearing aid (BAHA) in children with auricular malformations. Ear Nose Throat J 1997;76(4):238–47. Guckes AD, Scurria MS, King TS, et al. Prospective clinical trial of dental implants in persons with ectodermal dysplasia. J Prosthet Dent 2002;88(1):21–5. Güler N, Cildir S, Iseri U, et al. Hypohidrotic ectodermal dysplasia with bilateral impacted teeth at the coronoid process: a case rehabilitated with mini dental implants. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005; 99(5):E34–8. Hallonsten A-L, Koch G. Syndromes including tooth agenesis and conditions involving malformations and lost teeth. In: Kock G, Bergendal T, Kvint S, et al, editors. Consensus conference on oral implants in young patients: state of the art. Jönköping (Sweden): The Institute for Postgraduate Dental Research; 1996. p. 28–39. Itthagarun A, King NM. Oral rehabilitation of a hypohidrotic ectodermal dysplasia patient: a 6-year follow-up. Quintessence Int 2000;31(9):642–8. Johnson EL, Roberts MW, Guckes AD, et al. Analysis of craniofacial development in children with hypohidrotic ectodermal dysplasia. Am J Med Genet 2002;112(4):327–34. Kargül B, Alcan T, Kabalay U, et al. Hypohidrotic ectodermal dysplasia: dental, clinical, genetic and dermatoglyphic findings of three cases. J Clin Pediatr Dent 2001;26(1):5–12. Kearns G, Sharma A, Perrott D, et al. Placement of endosseous implants in children and adolescents with hereditary ectodermal dysplasia. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;88(1):5–10. Kramer FJ, Baethge C, Tschernitschek H. Implants in children with ectodermal dysplasia: a case report and literature review. Clin Oral Implants Res 2007;18(1):140–6. Lustig JP, Yanko R, Zilberman U. Use of dental implants in patients with Down syndrome: a case report. Spec Care Dentist 2002;22(5):201–4. Murphy M, Insoft RM, Pike-Nobile L, et al. A hypothesis to explain the immune defects in Down syndrome. Prog Clin Biol Res 1995;393:147–67. Murdock S, Lee JY, Guckes A, et al. A cost analysis of dental treatment for ectodermal dysplasia. J Am Dent Assoc 2005;136(9):1273–6. Oczakir C, Balmer S, Mericske-Stern R. Implant-prosthodontic treatment for special care patients: a case series study. Int J Prosthodont 2005;18(5):383–9. Petropoulos VC, Balshi TJ, Balshi SF, et al. Treatment of a patient with cleidocranial dysplasia using osseointegrated implants: a case report. Int J Oral Maxillofac Implants 2004;19:282–7. Rad AS, Siadat H, Monzavi A, et al. Full mouth rehabilitation of a hypohidrotic ectodermal dysplasia patient with dental implants: a clinical report. J Prosthodont 2007;16(3):209–13. Sándor GKB, Marchac D, Patat J-L, et al. Experience with the use of coral granules as a bone graft substitute in the human cranio-maxillofacial skeleton. Bulle Instit océanographi Monaco 1995;n spécial 14(3). Sweeney IP, Ferguson JW, Heggie AA, et al. Treatment outcomes for adolescent ectodermal dysplasia patients treated with dental implants. Int J Paediatr Dent 2005;15(4):241–8. Warren SM, Borud LJ, Brecht LE, et al. Microvascular reconstruction of the pediatric mandible. Plast Reconstr Surg 2007;119(2):649–61.