Developmental Disturbances in Oral Cavity

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DEVELOPMENTAL DISTURBANCES OF ORAL

AND PARAORAL STRUCTURES

JAWS
LIPS AND PALATE
ORAL MUCOSA
TONGUE
SALIVARY GLANDS
TEETH

JAWS

MACROGNATHIA
AGNATHIA FACIAL
MICROGNATHIA HEMIHYPERTROPHY
AGNATHIA
(OTOCEPHALY, HOLOPROSENCEPHALY AGNATHIA)
THIS LESION IS CHARACTERIZED BY THE ABSENCE OF MANDIBLE

CHARACTERSTICS FEATURES

 Hypoplasia or absence of mandible


 Abnormally position ears
 Commonly only portion of one jaw is missing
 MAXILLA: maxillary process or premaxilla
 MANDIBLE : PARTIAL ABSENCE OF MANDIBLE IS MORE COMMON
(ONLY CONDYLE OF ENTIRE RAMUS OF MANDIBLE ; it can be bilateral or unilateral)

EITIOLOGY

 FAILURE OF MIGRATION OF NEURAL CREST MESENCHYME INTO MAXILLARY


PROMINENCE (4TH TO 5TH WEEK OF GESTATION)

MICROGNATHIA

(SMALL JAW )

CHARACTERSTIC FEATURES

 BOTH IN MAXILLA AND MANDIBLE


 NOT EXACTLY THE SMALL SIZE BUT COMPARATIVELY ABNORMAL SIZE OF
JAW FROM ONE SIDE TO OTHER
TRUE FALSE

(CONGENTIAL) (ACQUIRED)

EITIOLOGY IS UNKNOWN Post natal


PIERR ROBIN SYNDROME Disturbance in tmj, trauma ,ankylosis
Results in steep mandibular angle

Deficient chin button

Retrusion of the chin

ETIOLOGY

Maldevelopment of the nasal and nasopharyngeal structures mouth


breathing looks retracted

PIERRE ROBIN SYNDROME


CHARACTERISTIC FEATURES

 CONGENITAL
1. Micrognathia or retrognathia
2. Glossoptosis (tongue downward and backward)
3. Cleft palate
ETIOLOGY

 MECHANICAL THEORY : GLOSSOPTOSIS ->failure of fusion of developing


palatine shelves
 NEUROLOGICAL MATURATION THEORY : delays in neurological maturation
that prevents tongue from desending
 DYSREGULATION THEORY :motor and regulatory organization of the
rhombencephalus is related to a major problem of ontogenesis.(problem
during fertilization)

CLINICAL

 Presence of small mandible


 Micrognathia BIRDS FACE
 Jaw-normal but resorption

Tongue tends to fall back


GLOSSOPTOSIS
Airway obstruction

MAY LEADS TO:


1. CYANOSIS
2. CHOKING SPELLS
3. OBSTRUCTIVE SLEEP APONEA
4. HYPOXIA
 Cleft palate involving both soft and hard palate without
involving cleft lip .(usually v or u shape)
 Other findings :
1. Oitis media
2. Hearing loss
3. Nasal deformities
4. Labirinthis with equilibrium disturbances
5. Dental
6. Philitral malformation
7. Airway obstructions : bronchitis and pneumonia
8. Rarely :
CNS DEFORMITIES: language delay , epilepsy , hypotonia
and hydrocephalus.
TREATMENT AND PROGNOSIS

 AIRWAY OBSTRUCTION: OBTURATORS


 RESPIRATORY OBSTRUCTION : SURGICAL INTERVENTION

MACROGNATHIA
(ABNORMALLY LARGE JAWS)
CHARACTERISTIC FEATURES
 PAGET DISEASE :
Commonly :cranium and maxilla are involved.
Rarely : mandible
 ARCOMEGALY: progressive enlargement of mandible -> hyperpituitarism
 LEONTIASIS OSSEA: enlargement of maxilla(type of fibrous dysplasia)

ETIOLOGY

Unknown

GENERAL FACTORS
 Increased Chin angle
 Prominent Chin button
 Increased Height of the ramus
 Increased Mandible body length
 Decreased maxillary length
 Posterior positioning of the maxilla in realtion to cranium

TREATMENT

Surgical
OSTECTOMY : resection of a portion of mandible to decrease to its length.
FACIAL HEMIHYPERTROPHY
HEMI :HALF SIDE

HYPERTROPHY:OVERGROWTH

(more hyperplasia thn hypertrophy)

CHARATERISTIC FEATURES
COMPLEX HEMIHYPERPLASIA

• involvement of half of the body(cn be contralateral or


ipsilateral

simple hemihyperplasia

•involvement of single limb

facial hemihyperplasia

•involvement of one side of face

ETIOLOGY
Unknown

Can be ascribed as vascular or lymphatic abnormalities; CNS disturbances or


chromosomal abnormalities.

CLINICAL FEATURES
 Enlargement of one side of the body
 Unilateral macroglossia
 Premature development
 Eruption as well as increased size of dentition
 F>M
ORAL MANIFESTATIONS

 Crown size
 Root size and shape affected side is abnormal in three
 Rate of development respects

(deciduous are less affected thn permanent ; more in posteriors)


 Bone is also enlarged(wider and thicker)
 TONGUE:enlargement of linual papillae
 BUCCAL MUCOSA:velvety ; hang in soft ; pendulous folds

HISTOLOGICAL FEATURES
 Ture muscular hypertrophy is not found

TREATMENT AND PROGNOSIS


 Cosmetic repair(only after the cessation of growth)
 Periodic abdominal ultrasound/MRI are recommended to rule out of
tumours.

DIFFERENTIAL DIAGNOSIS
 Fibrous dysplasia
 Neurofibroma
LIPS AND PALATE
 Van der woude syndrome
 Cleft lip and cleft palate
 Cheilitis glandularis
 Cheilitis granulomatosa

VAN DER WOUDE SYNDROME


(cleft lip syndrome ;lip pit syndrome ;dimpled papillae of the lip)

CHARATERISTIC FEATURES
 Autosomal dominant syndrome
 Cleft lip or palate
 Can be lower lip pits alone ; missing teeth or isolated cleft lip and palate

ETIOLOGY
 Orofacial anomalies
 Abnormal fusion of lips and palate (30-50 days of postconception)
 Due to deletion of chromosome band 1q32

CLINICAL FEATURES
 Rare case
 Only 1-2% patients with cleft lip have van der wounde syndrome
 M=F
 25% assymptomatic
 DIFFERENTIAL FEATURE```````````````````: cleft lip or palate have
lower lip pits.
 Can be unilateral or bilateral
 CLUES OF DIAGNOSIS: hypernasal voice; cleft or bifid uvula
 Lower lip pits:
1. medial ; on the vermillion border
2. infancy : small elevation
3. adult :simple depressions
4. rarely discharge
 maxillary hypodontia
 missing maxillary incisor or premolar
 syngnathia
 narrow ,high arched palate,
 ankyloglossia(short glossal frenum)

EXTRAORAL MANIFESTATION
 rare
 limb anomalies
 popliteal webs
 brain abnormalities
 accessory nipples and congential heart defects

TREATMENT
For cleft lip and palate : surgical
For lower lip pits: surgicall excision to alleviate discomfortor for cosmetic reasons.

Cleft lip and cleft palate


CHARATERISTIC FEATURES
 Autosomal dominant syndrome
 Cleft lip or palate

Etiology
 UNKNOWN
There are two theories which describe different etiology

FOGH-ANDERSEN
According this theory the lesion can be due to one gene or can be due to
different gene acting together. Although it states that this lesion is
because of heredity factor.

Acc. To bixler
Everyone carries some genes which affect the individual with this
syndrome. Whereas for the expression we need particular threshold
which is described as polygenic type. Second type is monogenic or
syndromic that related with variety of syndromes or anomalies .

OTHER FACTORS:
 A defective vascular supply to the area involved
 A mechanical disturbance in which the size of the tongue may
prevent the union palatine shelves.
 Circulating substances , such as alcohol and certain drugs and
toxins.
 Infections
 Lack of inherent development force.

CLINICAL FEATURES
 M>F
 Two types
NON SYNDROMIC SYNDROMIC
No other physical and Additional birth defects like pits
developmental anomalies.
70% 30%
uncategorized

chromosomal
syndromic
teratogens
 It can occur as unilateral or bilateral
 It starts from lateral part of upper lip and continues through philtrum
to alveolous between the lateral incisor and canine tooth and when it
goes upto the incisive foramen we consider cleft lip along with cleft
palate.->CLP

TREATMENT
SURGICALLY BE CORRECTED

CHEILITIS GLANDULARIS
Uncommon and poorly understood inflammatory disorder of lip.

CHARACTERISTIC FEATURES
Progressive enlargement and eversion of lower lip that results in
obliteration of mucosal –vermillion interface. Due to this chronic
exposure to external environment leads to erosion, ulceration,
crusting that ultimately lead to squamous cell carcinoma.

ETIOLOGY
Unusual clinical manifestation
Lip enlargement -> inflammation->hyperaemia->oedema->fibrosis

Surface keratosis->erosion->crusting development->actinic

Sometimes unusal self biting; compulsive licking ; drying


CLINICAL FEATURES
 Chronic progressive condition
 Pain , enlargement and loss of elasticity of lip
 First 3 months there is asymptomatic swelling with clear
secretion
 Burning discomfort
 Atrophy , speckled leukoplakia , ulceration with crusting
 Major risk factor is squamous cell carcinoma (18-35% cases)
 40-70 decade of life
 M>F

CLASSIFICATION

cheilitis
glandularis

superficial deep
simple
suppurative suppurative

Multiple , BAELZ DISEASE Cheilitis glandularis


painless,popular Painless, swelling of Apostematosa ,
surface lesion with lip , with shallow myxadenitis labialis
central depression ulceration and
crusting
Deep infection with
abscess , sinus tracts
and fistulas
HISTOLOGICAL FINDINGS
 Lip biopsy:incisional biopsy
 Suppuration or sinus tracts may be seen
 Some surface epithelium changes and submucosal chnges
 Other:stromal oedema ; hyperaemia;erosion;ulceration

DIFFERNTIAL DIAGNOSIS

 ACTINIC KERATOSIS
 Atopic dermatitis
 Cheilitis granulomatosa (MIESCHER-MELKERSSON-
ROSENTHAL SYNDROME)
 SARCOIDOSIS
 SQUAMOUS CELL CARCINOMA

TREATMENT
ANTIBIOTIC THERAPY

CHEILITIS GRANULOMATOSA
(miescher-melkersson-rosenthal syndrome)

CHARACTERISTIC FEATURES
 Chronic swelling of lip
 Meischer cheilitis term is used when lesion confined to lips only-
>monosymptomatic form of miescher-melkersson-rosenthal
syndrome
 miescher-melkersson-rosenthal syndrome ->cheilitis
occurs with facial palsy and plicated tongue
 occasionally a manifestation of CROHN DISEASE

EITIOLOGY
 UNKNOWN
 Genetic predisposition may exist

CLINICAL FEATURES
 IT IS EPISODIC WITH NON TENDER SWELLING
 Enlargement of one or both lips
 Occasionally similar swelling on periocular region may also seen
 First edema typically subsides completely in hours or days
 After recurrent attack ,swelling may persist and slowly increase
and eventually becoming permanent
 Attacks sometimes accompanied by fever and mild
constitutional symptoms
 Earliest manifestation is sudden diffuse or occasionally nodular
 Once chronically is established ,. The enlarged lip appears
cracked , fissured with reddish brown discolouration.
 It may becomes painful
 Regional lymph nodes are swelled in 50% cases
 A fissured or plicated tongue is seen in 20-40%patients
 Pt. may loose the sense of taste , Decreased salivary secretion
 Facial palsy of lower motor neuron – 30% of cases
1. Can be unilateral or bilateral
2. Partial or complete
3. Poorly defined associated psychiatric and neurological
features are reported
4. Autonomic disturbances may occur

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