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. AGNATHIA 5
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. 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. MICROGNATHIA 8
9. Micrognathia smaller jaw maxilla/mandible 9
10. Micrognathia can be classified as Congenital Congenital
heart disease Pierrie Robin Syndrome Unknown etiology Acquired
10
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. 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. Clinical presentation Severe retrusion of the chin A steep
mandibular angle A deficient chin button 13
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. treatment Orthognathic surgery Condylar implants
16. MACROGNATHIA 16
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. 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. Clinical features Protruded jaw (mostly mandible) Class III
Malocclusion Crossbite Mostly associated with Gigantism Treatment :
orthognathic surgery
20. FACIAL HEMIHYPERTROPHY 20
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. Etiology Hormonal imbalance Chromosomal abnormalities
Localized alteration of intrauterine development Lymphatic
abnormalities Vascular abnormalities Neurogenic abnormalities
22
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. 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. Radiographic feature Facial skull bones ( maxilla,
mandible, zygomatic, temporal and frontal bone affected ) In fig
right side affected thus bones enlarged 25
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. Differential Diagnosis Neurofibromatosis Fibrous dysplasia
Can be differentiated based on lack of effect on tooth size and
rate of eruption 27
28. Treatment Orthodontic tooth alignment Serial staged
cosmetic surgeries Functional and esthetic improvement
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. Etiology Unknown Suggested factors Atrophic malfunction of
the cervical sympathetic nervous system Trauma Infection Heredity
Peripheral trigeminal neuritis 31
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. 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. 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. 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. T 36 Progressive facial asymmetry can be confirmed by
comparing photographs of patients from childhood through
adolescence to adulthood
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. 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. Condylar aplasia
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. Clinical features Defective or absent ear Underdeveloped
ramus Macrostomia Unilateral Facial asymmetry Altered occlusion and
mastication Shift of mandible towards affected side
42. Treatment: oOsteoplasty if needed oOrthodontic treatment
malocclusion oCosmetic surgery facial deformity
43. Condylar hypoplasia
44. Condyle smaller than normal, underdeveloped or malformed
Types Congenital Idiopathic origin Acquired
45. etiology Trauma children and young, infection forcep injury
X ray radiation
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. treatment Cartilage or bone transplants Osteotomy asymmetry
and retrusion
48. Condylar hyperplasia
49. Rare Unilateral enlargement Etiology: Local environment
mild chronic inflammation
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. investigations Orthopantomograph Open and closed TMJ views
Transcranial, transpharengeal and transorbital view
Posterioanterior skull view Submentovertex base of skull view
Computed tomography
52. treatment Condition generally self limiting Unusually large
condyles causing functional and esthetic disturbances surgical
recontouring condylectomy
53. Bifid /trifid condyle
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. 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. Treatment No treatment required as it is asymptomatic
57. Coronoid hyperplasia
58. Hyperplasia of the coronoid process Unilateral
Bilateral
59. etiology May be Genetic Trauma Increased activity of
temporalis muscle Endocrinal stimulus
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. investigation Waters view OPG CT scan
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. 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. 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. 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