THE DEVELOPMENTAL

DISTURBENCES OF JAWS

1. AGNATHIA
2. AGENESIS
3. MICROGNATHIA
4. MACROGNATHIA
5. FACIAL HEMIHYPERTROPHY
6. FACIAL HEMIATROPHY
7. CLEIDOCRANIAL DYSPLASIA
8. FOCAL OSTEOPOROSIS BONE
MARROW DEFECT
AGNATHIA
 It is also called as “Hypognathous”. It is extremely
rare congenital defect characterized by absence of
maxilla or mandible.
 If mandible is absent the upper part of face may be
normal and the skin of the lower part will be
continuous with the suprasternal integument.
 The hyoid bone is sometimes absent, despite the
presence of a rudimentary tongue in the place of
buccal orifice, there may be a vertical slit.
 In the case of unilateral absence of mandibular
ramus, it is not unusual for ear to be deformed or
absent.
AGENESIS
 The failure of development of same part of
mandible or maxilla is termed as “AGNESIS”.
 The mandible is most commonly affected
than maxilla.
 In maxilla one maxillary process or even
pre-maxilla are usually imperfect.
 The commonest developmental defect in
mandible is the absence of condyle. In which
there is no articular fossa and the eminentia
articularis is either absent or rudimentary.
MICROGNATHIA

 It means small jaws as compared to

normal size.
 In this case either maxilla or mandible
may be affected.
 There are two types of Micrognathia:
i) Apparent Micrognathia
ii) True Micrognathia
Apparent Micrognathia

 This is not due to abnormality of small

jaw, in terms of size but rather to an
abnormal positioning or abnormal
relation of one jaw to another, which
produces illusion of micrognathia.
True Micrognathia

 It is due to small jaw . It is again

classified as :

i)Congenital: It is present since Birth.

ii)Acquired: It is acquired later in the

life.
ETIOLOGY :

i) Congenital: In congenital type etiology is

usually unknown. In many instances it is
associated with other congenital abnormalities
particularly congenital heart disease.
ii) Acquired: Acquired type is post natal type and
results from disturbances in the area of
T.M.JOINT ( Ankylosis)
CLINICAL FEATURES :
 Micrognathia of maxilla is due to
deficiency of pre-maxillary area and
patient with this deformity appears to
have the middle third of face retracted.
 True mandibular micrognathia is
uncommon and patient appears clinically
to have severe retrusion of chin.

Syndrome associated with Micrognithia:

i) Pierre robin syndrome
ii) Hallerman-streiff syndrome
iii) Trisomy-13
iv) Trisomy-18
v) Turner’s Syndrome
vi) Marfan’s Syndrome
MANAGEMENT:
 Surgery or orthodontic appliances may be

recommended.
 If upper jaw is short, then it can be

corrected with surgical orthodontic

treatment.
 Small mendible can be corrected with

depending on the degree of deformity &

problem.
MACROGNATHIA

 It refers to the condition in which jaw size is

larger than the normal. It is also called as
“MEGAGNITHIA”.

ETIOLOGY: it is caused by Pituitary gigantism,

paget’s disease of bone, acromegaly and in
some form of fibrous dysplasia.
CLINICAL FEATURE: Mendibular protrusion or
prognatism is common occurrence is due to
disparity in the size of maxilla to mandible.
 Mandible is larger than normal which results
in increased mandibular body length.
Size of ramus is large.

MANAGEMENT:
Resection of portion of mandible should be
done to decrease the length followed by
orthodontic treatment.
FACIAL
HEMIHYPERTHROPHY(Hyperplasia)

 It is a rare developmental anomaly

characterized by asymmetric overgrowth
of one or more body parts.
 It actually represents a Hyperplasia of
tissue rather than a hypertrophy.
ETIOLOGY
 Hormonal Imbalance
 Incomplete Twinning
 Chromosomal Abnormalities
 Vascular Abnormalities
 Lymphatic Abnormalities
 Neurogenic Abnormalities
CLINICAL FEATURES

 Females are affected than males

 May occur alone or in generalized
hemihypertrophy
 It involves the eyelids, cheeks, lips,
facial bones, tongue, ears, & tonsils.
 Pigmentation & hemangioma may occur
on skin.
 ORAL MANIFESTATIONS

(1) Crown size may be large.

(2) Root size may enlarge.
(3) Primary teeth shed early.
(4) Rate of development of permanent teeth
on the affected site is more rapid
MANAGEMENT

 There is no specific treatment for this

condition other than attempts at
cosmetic repair.
 Cosmetic surgery is advised after
cessation of growth.
FACIAL HEMIATROPHY

 It is also called as
1. Parry – Romberg syndrome
2. Romberg hemifaial atrophy
3. Hemifacial maicrostomia
4. Progressive facial hamitrophy.
 It is a rare disorder characterized by
slowly progressive, atropy of the soft
tissue of half of the face.
ETIOLOGY

 Atrophic malfunction of cervical

sympathetic nervous system.
 Trauma.
 Infection &
 Herediatary factor.
CLINICAL FEATURES

 Hemifacial atrophy is a syndrome with diverse

presentation. The most commonly early sign is
a painless cleft.
 The affected area extends progressively with
atrophy of the skin, subcutaneous tissue,
muscles, bones, cartilages, alveolar bone & soft
palate on that side of the face.
 Female to male ratio is 3:2
MANAGEMENT

1. Orthodontic treatment
2. Plastic surgery
3. Hearing aids are recommended.
CLEIDOCRANIAL DYSPLASIA

 It is also called as “cleidocranial

dysostosis
 “Marie and sainton d/s’
ETIOLOGY

 Hereditary and when inherited, it appear

as a true dominant mendelian
characteristic.
 Incomplete penetration of genetic trait
may be observed.
CLINICAL FEATURES

 This disease affects men and women with

equal frequency. It primary affects, skull,
clavicle and dentition.
 Nasal bridge is depressed with a broad
base.
 Lacrimal & zygomatic bone are also
under developed. Saggital suture is
characteristically sunken, giving the skull
flat appearance.
MANAGEMENT

 Treatment is not specific.

 Prophylatic dental care should be taken.
FOCAL OSTEOPOROSIS BONE
MARROW DEFECT

 They are derived from bone marrow

hyperplasia of persisting embryonic
marrow remnants.
CLINICAL FEATURES
1. Asymptomatic with of pain in that
region.
2. It is more common in female than
males.
3. It is more common in molar, premolar
region in mandible.
4. Usually it is most evident on the
radiograph.
HISTOPATHOLOGY

 Bone marrow contain cellular

haemopoeitic component which may
show variable fatty components.
MANAGEMENT
 There is no specific treatment for it.
SUBMITTED BY

ABHINAV PATEL