Front Cover

Oral manifestations of Inherited Disorders

Copyright Page

Table of Contents

Preface

CHAPTER 1. Genetic Counseling and Oral Examination

1.1 Genetic Counseling

1.2 Physical Examination of the Oral Cavity

1.3 Radiologie Interpretation of Some Oral and Paraoral Structures

CHAPTER 2. 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 Dysplasia

References

CHAPTER 3. Systemic Inherited Disorders with Dental Involvement

3.1 Acroosteolysis

3.2 Amelocerebrohypohi drosis Syndrome

3.3 Cryptodontic Brachymetacarpalia

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 Syndrome

References

CHAPTER 4. 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, Dystrophie Nails, Aplastic Anemia, and Leukoplakia Oris

4.9 Fabry Syndrome

4.10 Familial Dysautonomia

4.11 Gingival Fibromatosis with Ear, Nose, Bone, and Nail Defects and Hepatosplenomegaly

4.12 Gingival Fibromatosis with Hypertrichosis, Epilepsy, and Mental Retardation

4.13 Gingival Fibromatosis 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 Nevus

References

CHAPTER 5. 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 Hyperostosis

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-Opitz Syndrome

5.15 Waardenburg Syndrome

References

CHAPTER 6. Systemic Inherited 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 Faciès, 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 Syndrome

References

CHAPTER 7. Systemic Inherited Disorders with Involvement of Multiple Oral Structures

Jaw Bones and Teeth

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

Soft Tissue and Teeth

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 Mucopolysacchari dosis III

7.22 Mucopolysaccharidosis VI

Soft Tissue and Jaw Bones

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 Myotonie Dystrophy

7.29 Trichorhinophalangeal Syndrome

7.30 Tuberous Sclerosis

Soft Tissue, Jaw Bones, and Teeth

7.31 Coffin-Lowry Syndrome

7.32 Dubowitz Syndrome

7.33 Ehlers-Danlos Syndromes

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

Miscellaneous

7.41 Jaw-Winking and Winking-Jaw Syndromes

References

Glossary of Dental Terms

Photo Credits

INDEX