Developmental Anomalies
of Jaw, Lip & Tongue
Introduction

Malformation or defects resulting from disturbance
of growth & development are known as
Developmental Anomalies
Manifestation of defects are evident either at birth
or some times after birth
Types of Anomalies  Hereditary / Familial developmental anomalies
 Acquired developmental anomalies


Idiopathic developmental anomalies
Agnathia



Agnathia is a lethal anomaly characterized by
hypoplasia or absence of the mandible or maxilla, it
could be partial or complete. More commonly, only a
portion of one jaw is missing. In the case of the maxilla,
this may be one maxillary process or even the
premaxilla. Partial absence of the mandible is more
common than mandible .
It is probably due to failure of migration of neural crest
mesenchyme into the maxillary prominence at the
fourth to fifth week of gestation (post-conception)
The prognosis of this condition is very poor and is
considered to be lethal.
Micrognathia


Micrognathia literally means a small jaw, and either the
maxilla or the mandible may be affected.
Many cases of micrognathia are not due to an abnormally
small jaw in terms of absolute size, but rather due to an
abnormal positioning or an abnormal relation of one jaw
to the other or to the skull, which produces the illusion of
micrognathia.
Types
True
 Congenital
 Acquired
Apparent / Relative [Abnormal positioning/ relation of one jaw
to the other or skull]
Congenital Micrognathia
Etiology unknown
 Associated with other congenital abnormalities like
congenital heart disease and pierre robin syndrome
 Micrognathia of maxilla is due to a deficiency of the
premaxillary area
 Micrognathia of mandible may be due to
◦ Small jaw
◦ Steep mandibular angle
◦ Agenesis of condyles

Acquired Micrognathia

Post natal in origin

Causes
◦ Ankylosis of joint as result of trauma/infection
Macrognathia

Condition of abnormally large jaws
◦ Pagets disease of bone- Enlarged maxilla
◦ Acromegaly - Enlarged mandible
◦ Leontiasis ossea - Enlarged maxilla
Cases of mandibular protrusion or prognathism,
uncomplicated by any systemic condition, are a
common clinical occurrence.
Facial Hemihypertrophy


a rare developmental anomaly characterized by
enlargement which is confined to one side of the body,
unilateral macroglossia and premature development and
eruption as well as increased size of dentition.
the permanent teeth on the affected side develop more
rapidly and erupt before their counterparts on the
uninvolved side. Coincident to this phenomenon is
premature shedding of the deciduous teeth. The bone of
the maxilla and mandible is also enlarged, being wider
and thicker, sometimes with an altered trabecular
pattern.
FACIAL HEMIATROPHY






Parry-Romberg syndrome
slowly progressive atrophy of the soft tissues of essentially
half the face.
may be a form of localized scleroderma.
The most common early sign is a painless cleft, the “coup de
sabre”, near the midline of the face or forehead.
The affected area extends progressively with the atrophy of
the skin, subcutaneous tissue, the muscles, bones, cartilages,
alveolar bone and soft palate on that side of the face.
Dental abnormalities include incomplete root formation,
delayed eruption, severe facial asymmetry resulting in facial
deformation and difficulty with mastication. Eruption of teeth
on the affected side may also be retarded.
CLEFT LIP AND PALATE



Cleft lip and palate are common congenital
malformations.
Heredity is one of the most important factors in the
etiology of these malformations. However,
environmental factors are important as well. Hence it is
a disease of multifactorial inheritence.
Clefts can be divided into
◦ Nonsyndromic - has no other physical or developmental
anomalies
◦ Syndromic forms - that have additional birth defects like lip pits
or other malformations

Types
◦ Cleft lip
◦ Cleft lip and palate
◦ Cleft palate

Cleft lip and palate can also be divided into
◦ Unilateral
◦ Bilateral
Eating and drinking are difficult because of regurgitation
of food and liquid through the nose. The speech
problem is also serious and tends to increase the
mental trauma suffered by the patient.
Surgical correction has a good prognosis
Developmental Disturbances of Lip
Cleft lip
 Congenital lip pits
 Double lip
 Chelitis glandularis
 Chelitis Granulomatosa
 Peutz Jeghers syndrome

CONGENITAL LIP PITS &
COMMISSURAL PITS

lip pits are malformations of the lips, often following a
hereditary pattern
Pits may result from notching of the lip at an early stage of
development, with fixation of the tissue at the base of the
notch
 Commissural pits may represent defective development of
embryonic fissure.


The lip pit or fistula is a unilateral or bilateral depression or
pit, usually does not have any symptom but sometimes minor
salivary glands may open into the pits causing oozing out of
saliva.

Van der Woude syndrome - cleft lip or cleft palate and
distinctive pits of the lower lips

Double Lip – is a rare anomaly characterized by an
extra fold of tissue on the mucosal side of lip.
Associated with Ascher syndrome –
◦ Double lip
◦ Blepharochalasis
◦ Non toxic thyroid enlargement





Cheilitis Glandularis - inflammatory disorder of the lip,
progressive enlargement and eversion of the lower
labial mucosa. Potential predisposing factor for the
development of actinic cheilitis and squamous cell
carcinoma.
Types –
Simple type
Superficial suppurative type (also referred to as baelz
disease)
Deep suppurative type
CHEILITIS GRANULOMATOSA




Melkersson-Rosenthal Syndrome
chronic swelling of the lip due to granulomatous
inflammation
nontender swelling and enlargement of one or both lips.
The first episode of edema typically subsides completely
in hours or days. After recurrent attacks, swelling may
persist and slowly increase in degree, eventually
becoming permanent.
Melkersson-Rosenthal syndrome is the term used when
this occurs with facial palsy and fissured tongue
Peutz-Jeghers Syndrome




Hereditary intestinal polyposis syndrome
Characterized by intestinal hamartomatous polyps with
peri oral pigmentation.
repeated bouts of abdominal pain in patients younger
than 25 years, unexplained intestinal bleeding in a young
patient
15 times increased risk of developing cancer exists in
this syndrome over that of the general population;
cancer primarily is of the GI tract.
Developmental Disturbances of
Oral Mucosa
Fordyce’s granules - This is not a disease
 heterotopic collections of sebaceous glands at various
sites in the oral cavity.
 Fordyce’s granules appear as small yellow spots, either
discretely separated or forming relatively large plaques,
often projecting slightly above the surface of the tissue

DEVELOPMENTAL
DISTURBANCES OF THE
GINGIVA
Fibromatosis gingivae
 Retrocuspid papilla
