GAMUT B-62
CONGENITAL SYNDROMES AND BONE DYSPLASIAS WITH DEFECTIVE AND/OR DELAYED DENTITION
(See also B-61-2)

COMMON
1. Cherubism; polyostotic fibrous dysplasia (McCune-Albright S.) (delayed eruption or agenesis; displaced teeth)
2. Chondroectodermal dysplasia (Ellis-van Creveld S.) (neonatal teeth; conical peg-shaped teeth; partial anodontia; delayed eruption)
3. Cleidocranial dysplasia  (delayed eruption; supernumerary teeth; partial anodontia; malformed roots; enamel hypoplasia; early loss)
4. Ehlers-Danlos S. (small, irregular teeth; partial anodontia)
5. [Fluorosis]
6. Gorlin S. (nevoid basal cell carcinoma S.) (odontogenic cyst; irregular placement; caries)
7. Homocystinuria (irregular, crowded teeth)
8. Hypoparathyroidism; pseudohypoparathyroidism (hypodontia; delayed eruption; enamel hypoplasia; caries)
9. Hypophosphatasia (early loss; poor dentin; caries)
10. [Hypophosphatemic rickets (delayed eruption; enamel hypoplasia; gingival and periapical infection)]
11. Hypopituitarism (anterior lobe) (delayed eruption)
12. Hypothyroidism, infantile (cretinism); hypothyroidism (delayed eruption)
13. Mucopolysaccharidoses (eg, Hurler S.; Hunter S.; Morquio S.) (small malaligned teeth; thin enamel) (See J-4-S)
19. Osteogenesis imperfecta (types I, III, and IV) (hypodontia; delayed eruption; poor dentin; short roots; opalescent teeth)
15. Osteopetrosis  (delayed eruption)
16. Progeria (delayed eruption; crowded teeth)
17. Pyknodysostosis (delayed eruption; partial anodontia; irregular placement; persistent deciduous teeth)
18. Pyle dysplasia (familial metaphyseal dysplasia) (poor teeth)
19. [Syphilis, congenital (Hutchinson's teeth)]
20. Trisomy 21 S. (Down S.) (hypodontia; microdontia; delayed eruption)


UNCOMMON
1. Aarskog S. (delayed eruption; hypodontia; malocclusion; enamel hypoplasia)
2. Acrocephalosyndactyly (Apert type) (delayed eruption)
3. Aminopterin fetopathy
4. Bardet-Biedl S.
5. Brachmann-de Lange S. (de Lange S.) (delayed eruption)
6. Cerebro-costo-mandibular S.
7. Cockayne S. (caries)
8. Coffin-Lowry S.
9. Cohen S.
10. Congenital insensitivity to pain (early loss)
11. Cranioectodermal dysplasia (microdontia; hypodontia, fusion; enamel dysplasia)
12. Craniometaphyseal dysplasia
13. Crouzon S. (craniofacial dysostosis) (partial anodontia; wide spacing)
14. DOOR S.
15. Dyskeratosis congenita S. (caries; malalignment)
16. Dysosteosclerosis
17. Ectodermal dysplasias (eg, anhidrotic, hypohidrotic, or Robinson type) (partial anodontia; conical teeth)
18. Endosteal hyperostosis (Worth type)
19. Epidermal nevus S. (linear sebaceous nevus sequence) (odontodysplasia)
20. Frontometaphyseal dysplasia (hypodontia)
21. Gardner S. (delayed eruption)
22. Goltz S. (focal dermal hypoplasia) (hypodontia; enamel hypoplasia; delayed eruption; malformed teeth; irregular placement)
23. Hajdu-Cheney S. (familial or idiopathic acro-osteolysis) (early loss)
24. Hallermann-Streiff S. (oculo-mandibulo-facial S.) (hypodontia; supernumerary teeth; neonatal teeth; delayed eruption)
25. Hanhart S. (hypodontia; delayed eruption)
26. Hyperphosphatasia (early loss)
27. Hypoglossia-hypodactylia S. (aglossia-adactylia S.) (missing incisors; neonatal teeth)
28. Hypomelanosis of Ito (irregular spacing; peglike incisors)
29. Incontinentia pigmenti (hypodontia; delayed eruption; conical teeth)
30. Johanson-Blizzard S. (oligodontia; small teeth)
31. KBG S. (macrodontia;oligodontia; malposition; enamel hypoplasia)
32. Kocher-Debré-Sémélaigne S. (delayed eruption)
33. Lacrimo-auriculo-dento-digital S. (LADD S.) (delayed eruption; anodontia; enamel dysplasia)
34. Lenz microphthalmia S.
35. Lenz-Majewski dysplasia (hyperostotic dwarfism) 
36. Lowe S. (oculocerebrorenal S.) (cysts)
37. Marinesco-Sjögren S. (irregular teeth)
38. Marshall S. (prominent upper incisors)
39. Mesomelic dysplasias
40. Oculo-auriculo-vertebral spectrum (Goldenhar S.) (delayed eruption; missing teeth)
41. Oculo-dento-osseous dysplasia (hypodontia; microdontia; enamel hypoplasia)
42. Oro-facio-digital S. I (Papillon-Leage and Psaume S.) (missing lower central and lateral incisors; supernumerary cuspids and bicuspids; enamel hypoplasia; malocclusion)
43. Oro-facio-digital S. II (Mohr S.) (missing central incisors)
44. Osteodysplasty (Melnick-Needles S.) (malaligned teeth)
45. Osteoglophonic dysplasia (delayed eruption)
46. Osteolysis (familial expansile) (progressive tooth mobility and fracture; pulpitis)
47. Otopalatodigital S., types I and II (hypodontia)
48. Pachyonychia congenita (caries; early loss)
49. Papillon-Lefčvre S. (early loss)
50. Prader-Willi S. (dental caries; enamel hypoplasia)
51. Rieger S. (hypodontia; microdontia)
52. Robinow S. (delayed eruption)
53. Romberg S. (delayed eruption)
54. Rothmund-Thomson S. (microdontia; hypodontia; delayed eruption; supernumerary teeth)
55. Rubinstein-Taybi S.
56. Sclerosteosis
57. Seckel S. (bird-headed dwarfism) (missing teeth)
58. Short rib-polydactyly S. type I (Saldino-Noonan)
59. Singleton-Merten S.
60. Sjögren-Larsson S. (dental dysplasia)
61. Stanescu dysostosis (small, crowded teeth; enamel hypoplasia)
62. Stickler S. (arthro-ophthalmopathy) (malocclusion; dental maleruption)
63. Thalidomide embryopathy
64. Treacher Collins S.(mandibulofacial dysostosis)
65. Tricho-dento-osseous S. (delayed eruption; early loss)
66. Weill-Marchesani S. (malformed teeth)
67. Werner S.
68. Weyers acrodental dysostosis
69. Wildervanck S. (defective teeth)
70. Williams S. (idiopathic hypercalcemia) (delayed eruption; microdontia)
71. Winchester S. (delayed eruption)
72. XXXXY S. (hypodontia)
73. Yunis-Varón S. (hypodontia; impacted teeth)


[    ] This condition does not actually cause the gamuted imaging finding, but can produce imaging changes that simulate it.




References