Oral Manifestations of Inherited Disorders

Preface1 Genetic Counseling and Oral Examination 1.1 Genetic Counseling 1.2 Physical Examination of the Oral Cavity 1.3 Radiologic Interpretation of Some Oral and Paraoral Structures2 Some Inherited Conditions Affecting Only Calcified Dental Tissue 2.1 Amelogenesis Imperfecta 2.2 Coronal Dentin Dysplasia 2.3 Hereditary Opalescent Dentin 2.4 Radicular Dentin Dysplasia3 Systemic Inherited Disorders with Dental Involvement 3.1 Acroosteolysis 3.2 Amelocerebrohypohidrosis Syndrome 3.3 Cryptodontic Brachmetacarpalia 3.4 Hypodontia and Nail Dysgenesis 3.5 Hypophosphatasia (Phosphoethanolaminuria) 3.6 Hypophosphatemic Vitamin D-Resistant Rickets 3.7 Hypoplastic-Hypocalcified Enamel, Onycholysis, and Functional Hypohidrosis 3.8 Incontinentia Pigmenti 3.9 Oculodentoosseous Dysplasia 3.10 Otodental Syndrome 3.11 Pseudohypoparathyroidism and Pseudopseudohypoparathyroidism 3.12 Rothmund-Thomson Syndrome4 Systemic Inherited Disorders with Oral or Perioral Soft Tissue Involvement 4.1 Acrodermatitis Enteropathica 4.2 Bloom Syndrome 4.3 Chédiak-Higashi Syndrome 4.4 Congenital Indifference to Pain 4.5 Cutis Laxa 4.6 Cyclic Neutropenia 4.7 Double Lip, Blepharochalasis, and Nontoxic Thyroid Enlargement 4.8 Dyskeratosis Congenita with Pigmentation, Dystrophic Nails, Aplastic Anemia, and Leukoplakia Oris 4.9 Fabry Syndrome 4.10 Familial Dysautonomia 4.11 Gingival Fibromatosis with Ear, Bone, and Nail Defects and Hepatosplenomegaly 4.12 Gingival Fibromatosis with Hypertrichosis, Epilepsy, and Mental Retardation 4.13 Gingival Fibromatòsis with Multiple Hyaline Fibromas 4.14 Hereditary Benign Intraepithelial Dyskeratosis 4.15 Hereditary Hemorrhagic Telangiectasia 4.16 Hermansky-Pudlak Syndrome 4.17 Hyperkeratosis Palmoplantaris and Attached Gingival Hyperkeratosis 4.18 Lesch-Nyhan Syndrome 4.19 Moebius Syndrome 4.20 Multiple Hamartoma and Neoplasia Syndrome 4.21 Multiple Mucosal Neuromas, Medullary Carcinoma of the Thyroid, Pheochromocytoma, and Marfanoid Body Build with Muscle Wasting 4.22 Neurofibromatosis 4.23 Pachydermoperiostosis 4.24 Pachyonychia Congenita, Jadassohn-Lewandowski Type 4.25 Peutz-Jeghers Syndrome 4.26 Pseudoxanthoma Elasticum 4.27 Xerodermie Idiocy 4.28 White Sponge Nevus5 Systemic Inherited Disorders with Involvement of Jaw Bones 5.1 Apert Syndrome 5.2 Carpenter Syndrome 5.3 Cerebrohepatorenal Syndrome 5.4 Cherubism 5.5 Chondrodysplasia Punctata 5.6 Craniofacial Dysostosis 5.7 Craniometaphyseal Dysplasia and Craniodiaphyseal Dysplasia 5.8 Infantile Cortical Hyperotosis 5.9 Mandibulofacial Dysostosis 5.10 Marfan Syndrome 5.11 Melnick-Needles Syndrome 5.12 Mucopolysaccharidosis VII 5.13 Pfeiffer Syndrome 5.14 Smith-Lemli-Optiz Syndrome 5.15 Waardenburg Syndrome6 Systemic Disorders Associated with Facial Clefting 6.1 Cleft Lip-Palate and Congenital Lip Pits 6.2 Cleft Lip-Palate and Tetraphocomelia 6.3 Cleft Lip-Palate, Popliteal Pterygium, Digital and Genital Anomalies Syndrome 6.4 Cleft Palate, Flattened Facies, and Multiple Congenital Dislocations 6.5 Diastrophic Dwarfism 6.6 Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) Syndrome 6.7 Hereditary Progressive Arthroophthalmopathy 6.8 Meckel Syndrome 6.9 Multiple Pterygium Syndrome 6.10 Otopalatodigital Syndrome7 Systemic Inherited Disorders with Involvement of Multiple Oral Structures 7.1 Bird-Headed Dwarfism 7.2 Cleidocranial Dysplasia 7.3 Cockayne Syndrome 7.4 Frontometaphyseal Dysplasia 7.5 Gardner Syndrome 7.6 Hypertelorism-Hypospadias Syndrome 7.7 Mucopolysaccharidosis IV 7.8 Multiple Nevoid Basal Cell Carcinoma Syndrome 7.9 Osteogenesis Imperfecta 7.10 Pyknodysostosis 7.11 Rieger Syndrome 7.12 Saethre-Chotzen Syndrome 7.13 Trichodentoosseous Syndrome 7.14 Chondroectodermal Dysplasia 7.15 Congenital Hypertrophy of the Gingiva, Altered Eruption of Teeth, and Corneal Dystrophy 7.16 Endocrine, Candidosis Syndrome 7.17 Epidermolysis Bullosa 7.18 Hyalinosis Cutis et Mucosa 7.19 Hypohidrotic (Anhidrotic) Ectodermal Dysplasia 7.20 Mucolipidosis II 7.21 Mucopolysaccharidosis III 7.22 Mucopolysaccharidosis VI 7.23 Craniocarpotarsal Dysplasia 7.24 Cryptophthalmos Syndrome 7.25 Generalized Gm1 Gangliosidosis 7.26 Gingival Fibromatosis, Hypopigmentation, Microphthalmia, Oligophrenia, and Athetosis 7.27 Mucopolysaccharidosis II 7.28 Myotonic Dystrophy 7.29 Trichorhinophalangeal Syndrome 7.30 Tuberous Sclerosis 7.31 Coffin-Lowry Syndrome 7.32 Dubowitz Syndrome 7.33 Ehlers-Danlos Syndrome 7.34 Fetal Face Syndrome 7.35 Focal Dermal Hypoplasia Syndrome 7.36 Hyperkeratosis Palmoplantaris and Periodontoclasia in Childhood 7.37 Macroglossia-Omphalocele-Visceromegaly Syndrome 7.38 Mucopolysaccharidosis I-H 7.39 Orofacialdigital Syndrome I 7.40 Orofacialdigital Syndrome II 7.41 Jaw-Winking and Winking-Jaw SyndromesGlossary of Dental TermsPhoto CreditsIndex