1. 1. a Good afternoon! !
2. 2. Developmental disturbance of face and jaw Christina shrestha Bds 4th year Roll no:38
3. 3. Developmental defects of the Jaws Contents Introduction Agnathia Micrognathia Macrognathia Facial hemihypertrophy Facial hemiatrophy (pierrie rhomberg syndrome) Condylar aplasia Condylar hypoplasia Condylar hyperplasia bifid/trifid condyle Coronoid hyperplasia Summary References 3
4. 4. Introduction Abnormalities of the face and jaw that occurs during the development during the intrauterine life which may be of various etiology . These include complete absence , diminished or excessive development of jaws and face.
5. 5. AGNATHIA 5
6. 6. Agnathia Rare Entire jaw Part of the jaw Autosomal recessive Failure of migration of NCC into 1st arch at 4th to 5th week of gestation 6
7. 7. Maxilla One maxillary process or premaxilla may be present Mandible Partial absence of the mandible Entire mandible of one side, ramus or condyle may be missing Ear may be deformed in case of unilateral defects 7 Prognosis : bad
8. 8. MICROGNATHIA 8
9. 9. Micrognathia smaller jaw maxilla/mandible 9
10. 10. Micrognathia can be classified as Congenital Congenital heart disease Pierrie Robin Syndrome Unknown etiology Acquired 10
11. 11. Clinical features Micrognathia of the maxilla Micrognathia of the mandible Deficiency of premaxillary area Middle face retracted Mouth breathing - (owing to associated maldevelopment of the nasal and nasopharyngeal structures) Severe retrusion of the chin May also be caused by Posterior positioning of mandible Steep mandibular angle 11
12. 12. Acquired type Postnatal origin Etiology: Disturbance in TMJ area Ankylosis of joint (trauma, or infection of the mastoid, of the middle ear or the joint itself) 12
13. 13. Clinical presentation Severe retrusion of the chin A steep mandibular angle A deficient chin button 13
14. 14. Syndromes associated Cohen syndrome : obesity , mental retardation, craniofacial diamorphism. DeGeorge syndrome:learning disabilities,hypothyroidism ,thrombocytopenia Fetal alcohol syndrome:small eye opening , smooth philtrum ,thin upper lip Pierre Robin syndrome:cleft palate , bird facies, respiratory difficulty. Potter syndrome : hypothyroidism ,thrombocytopenia. Rubinstein-Taybi syndrome: developmental retardation, broad thumb and great toes Treacher Collins syndrome: heaing loss , underdeveloped zygoma, absent or malformed ear
15. 15. treatment Orthognathic surgery Condylar implants
16. 16. MACROGNATHIA 16
17. 17. Macrognathia Abnormally large jaws Pituitary gigantism Both jaws are affected Size - frequently proportional to a generalized increase in size of the entire skeleton Other conditions Pagets disease of bone Acromegaly Fibrous Dysplasia (Leontiasis ossea) 17
18. 18. Caused by Increased height of the ramus (depends on condylar growt Increased mandibular body length Increased gonial angle Decreased maxillary length Posterior positioning of the maxilla in relation to the cranium Prominent chin button Varying soft tissue contours 18
19. 19. Clinical features Protruded jaw (mostly mandible) Class III Malocclusion Crossbite Mostly associated with Gigantism Treatment : orthognathic surgery
20. 20. FACIAL HEMIHYPERTROPHY 20
21. 21. Facial Hemihypertrophy Enlargement of one half of the face Unilateral macroglossia , premature development and eruption, increased size of dentition Disproportion is maintained throughout life Clinical significance Associated with neoplasm's of the Kidney Liver Adrenal cortex in children 21
22. 22. Etiology Hormonal imbalance Chromosomal abnormalities Localized alteration of intrauterine development Lymphatic abnormalities Vascular abnormalities Neurogenic abnormalities 22
23. 23. Clinical features: Females are affected more Equal involvement of both sides Intraoral findings: malocclusion Unilateral macroglossia Bone (enlarged) Dentition affected in three aspects Crown size (50% increase) Root size and shape Rate of development 23
24. 24. Permanent teeth are affected - cuspid, premolars and first molar Teeth on affected side develop rapidly and erupt before Premature shedding of the deciduous teeth Bone of the maxilla and mandible - enlarged more wider and thicker, with altered trabecular pattern 24
25. 25. Radiographic feature Facial skull bones ( maxilla, mandible, zygomatic, temporal and frontal bone affected ) In fig right side affected thus bones enlarged 25
26. 26. Soft tissue Tongue Enlargement of papillae Unilateral enlargement of the affected site Contra lateral displacement Buccal mucosa Frequently appears velvety May seem to hang in soft, pendulous folds on the affected site 26
27. 27. Differential Diagnosis Neurofibromatosis Fibrous dysplasia Can be differentiated based on lack of effect on tooth size and rate of eruption 27
28. 28. Treatment Orthodontic tooth alignment Serial staged cosmetic surgeries Functional and esthetic improvement
29. 29. FACIAL HEMIATROPHY (PARRY ROMBERG SYNDROME) 29
30. 30. Facial Hemiatrophy (Parry Romberg Syndrome) Progressive atrophy of some or all of the tissues on one side of the face Characterised by wasting of subcutaneous fat, sometimes by atrophy of skin, cartilage, bone, muscle Occasionally extend to other parts of the body Accompained by - contralateral Jacksonian epilepsy, -trigeminal neuralgia - changes in eye and hair 30
31. 31. Etiology Unknown Suggested factors Atrophic malfunction of the cervical sympathetic nervous system Trauma Infection Heredity Peripheral trigeminal neuritis 31
32. 32. Clinical features Affects the dermatome of one or more branches of the trigeminal nerve Left side of the face is affected more Noticed in the first or second decade of life as a white line, furrow, or mark on one side of the face 32
33. 33. Clinical features(cont.) Large linear scar, between normal and abnormal skin near the midline of the forehead, Known as coup de sabre, (early sign) (i.e., "strike of the sword") 33
34. 34. Atrophy The skin Subcutaneous tissue, alveolar bone, palate, tongue, ear, salivary glands Muscle and bone resulting in facial deformity of varying degree depending upon severity of atrophy 34
35. 35. Clinical features(cont..) Hollowing of the cheek Eye may appear depressed in the orbit (Enopthalmus Neurologic disorders (15%) Cartilage of the nose, ear, larynx may also be involve loss of hair 35
36. 36. T 36 Progressive facial asymmetry can be confirmed by comparing photographs of patients from childhood through adolescence to adulthood
37. 37. Oral manifestation Hemiatrophy of the lips and tongue. Mouth and nose - deviated to the affected side Growth of the teeth affected deficiency of root development. Reduced growth of the jaws of the affected side. Unilateral atrophy of the tongue Eruption of the affected side teeth is also affected. 37
38. 38. D.D and Treatment D.D: - Post traumatic fat atrophy - Hemifacial microsomia Treatment: - No specific - Progressive for some years, then remain unchanged - Reconstructive or microvascular surgery may be needed to repair wasted tissue - . Other treatment is symptomatic and supportive 38
39. 39. Condylar aplasia
40. 40. Complete failure of development of mandibular condyle either unilaterally or bilaterally Incidence:1 in 5600 births associated with :Hemifacial microsomia :Goldenhaars syndrome :Treacher collin syndrome
41. 41. Clinical features Defective or absent ear Underdeveloped ramus Macrostomia Unilateral Facial asymmetry Altered occlusion and mastication Shift of mandible towards affected side
42. 42. Treatment: oOsteoplasty if needed oOrthodontic treatment malocclusion oCosmetic surgery facial deformity
43. 43. Condylar hypoplasia
44. 44. Condyle smaller than normal, underdeveloped or malformed Types Congenital Idiopathic origin Acquired
45. 45. etiology Trauma children and young, infection forcep injury X ray radiation
46. 46. Clinical features: Unilateral commonly Facial asymmetry Limited lateral movement Exaggerated antegonial notch on affected side Midline shifting during closing and opening Older the patient lesser severe facial deformation
47. 47. treatment Cartilage or bone transplants Osteotomy asymmetry and retrusion
48. 48. Condylar hyperplasia
49. 49. Rare Unilateral enlargement Etiology: Local environment mild chronic inflammation
50. 50. Clinical features Slow progressive elongation of face Deviation away from affected site Enlarged condyle evident clinically, can be palpated Severe malocclusion Open bite on affected side
51. 51. investigations Orthopantomograph Open and closed TMJ views Transcranial, transpharengeal and transorbital view Posterioanterior skull view Submentovertex base of skull view Computed tomography
52. 52. treatment Condition generally self limiting Unusually large condyles causing functional and esthetic disturbances surgical recontouring condylectomy
53. 53. Bifid /trifid condyle
54. 54. bifid/trifid condyle Etiology unknown Early stage of development condylar cartilage divided by well vascularized fibrous septa its persistence Rupture of septal vessels secondary to trauma Exposure to teratogenic substance Endocrinal disturbances Infection Exposure to therapeutic radiation Nutritional deficiencies
55. 55. Clinical features Male=female Left condyle being twice more effected than right side Age > 20years Traumatic origin : one glenoid fossa Developmental origin : separate glenoid fossa for each part
56. 56. Treatment No treatment required as it is asymptomatic
57. 57. Coronoid hyperplasia
58. 58. Hyperplasia of the coronoid process Unilateral Bilateral
59. 59. etiology May be Genetic Trauma Increased activity of temporalis muscle Endocrinal stimulus
60. 60. Clinical features Progressive limitation in mandibular movement(due to impingement of elongated coronoid process on the posterior surface of zygomas .) Male: female (5:1) No apparent facial asymmetry or pain
61. 61. investigation Waters view OPG CT scan
62. 62. Syndromes associated trismus- psedocamptodactyly syndrome Nevoid basal cell carcinoma syndrome Treatment: Surgical contouring of coronoid process Coronoidectomy followed by rigorious regimen of physiotherapy.
63. 63. summary Agnathia-complete absence of jaw either maxilla or mandible with bad prognosis Micrognathia-smaller jaw than normal which may be congenital or acquired showing the featutes llike severe retrusion of the chin steep mandibular angle , deficient chin button . Macrognathia-abnormally larger jaw mostly associated with pituitary gigantism and showing clinical features like protruded jaw, class III malocclusion crossbites etc. Facial hemihypertrophy- Enlargement of one half of the face showing features like Unilateral macroglossia, premature development and eruption,,increased size of dentition Facial hemiatrophy (perrie rhomberg syndrome)- Progressive atrophy of some or all of the tissues on one side of the face accompained by contralateral Jacksonian epilepsy , trigeminal neuralgia, changes in face and hair etc Condylar aplasia- complete failure of development of condyle of mandible leading to gross facial asymmetry. Condylar hypoplasia- inadequate condylar development which may be developmental or congenital. Condylar hyperplasia-enlargement of condyle which is usually on one side and is rare condition. bifid/trifid condyle- two or more than two condyles in which there are separate glenoid fossa for each condyle. Coronoid hyperplasia-hyperplasia of coronoid process which may be
64. 64. conclusion Since developmental disturbances of jaws has significant implication in the morphology and function related to oral and maxillofacial region , it is important for us to know these lesions so that we can provide an early treatment for the betterment of patients both functionally and esthetically .
65. 65. references Burkets oral medicine Oral medicine and radiology- Ravikiran Ongole Neville text book of oral pathology Shafers text book of oral pathology National institute of neurologial disorders and stroke http://www.ninds.nih.gov/disorders/parry _romberg/parry_romberg.htm
66. 66. THANKYO U!!