Developmental disturbances of orofacial region Dept of Oral

Developmental disturbances of orofacial region Dept of Oral Pathology

DEVELOPMENT: l Is progression towards maturity (Todd) l Refers to all the naturally occurring unidirectional changes in the life of an individual from its existence as a single cell to its elaboration as a multifunctional unit terminating in death. Thus it encompasses the normal sequential events between fertilization and death. (Moyer’s)

"It is not birth, marriage, or death, but gastrulation, which is truly the most important period in your life. " Lewis Wolpert (1986)

Craniofacial anamolies: l Are a diverse group of deformities in the growth of the head & facial bones. l Anomaly: Irregularity or different from normal. Causes: l Genetics l Environmental l Folic acid deficiency.

Most common craniofacial anomalies: l l l Cleft lip. Cleft palate. Craniosynostosis. Hemifacial microsomia. Vascular malformations.

l Malformation or defects resulting from disturbance of growth & development are known as Developmental Anomalies l Manifestation of defects are evident either at birth or some times after birth

Types of anomalies: l Congenital developmental anomalies l l l Hereditary developmental anomalies Familial developmental anomalies Acquired developmental anomalies Hamartomatous developmental anomalies Idiopathic developmental anomalies

Congenital Developmental Anomalies: l Defects which are present at birth or before birth during the intra-uterine life as a result of either heredity or environmental influences E. g. - Cleft lip & palate

Hereditary Developmental Anomalies: l Defects are genetically transmitted from the parents to the offspring, where definite genetic location is identified E. g: Downs syndrome –Trisomy 21

Familial Developmental Anomalies: Defects are transmitted from the parents to the offspring, where definite genetic location is not identified E. g: Diabetes l

Acquired Developmental Anomalies: l Defects develop during intra-uterine life due to some pathological environment condition Can be Prenatal / neonatal / Postnatal E. g. : 1. Congenital Syphilis - Notched incisors l - Mulberry molars 2. Fluorides - Enamel hypoplasia

Notched incisors Mulberry molars Fluorosis

Hamartomatous Developmental Anomalies: l Defects occuring due to hamartomatous change in the tissues Hamartoma: Excessive focal proliferation of normal tissues which are native to that particular location E. g. – Odontome

Note: Choristoma: Excessive focal proliferation of normal tissues which are not native to that particular location E. g. – Gingival salivary gland choristoma Teratoma: Tumor arising from all the 3 germ layers E. g. – Ovarian teratomas

Idiopathic developmental anomalies: Indicates the developmental anomalies were exact cause is unknown E. g. – Idiopathic enamel hypoplasia l

Syndrome: l The term syndrome derives from the Greek and means literally "run together “ l A group of symptoms that collectively indicate or characterize a disease, a psychological disorder, or another abnormal condition l Large number of syndromes occur in association with many oral diseases l Early diagnosis of a syndrome is important since severity of a disease can be much more when it is occurring in association with a syndrome

E. g: l Gardner's syndrome Multiple polyposis of large intestine Osteomas of bone Desmoid tumours Multiple epidermoid cysts of skin Multiple impacted supernumerary tooth

Developmental disturbances of the lips & palate: l l l l Commissural pits & fistulas van der Woude syndrome Cleft lip & palate Hereditary intestinal polyposis syndrome Chelitis glandularis Chelitis granulomatosa Labial & oral melanotic macule.

Developmental disturbances of oral mucosa: l l Fordyces granules Focal epithelial hyperplasia Developmental disturbances of gingiva: l Fibromatosis gingivae l Retrocuspid papilla

Developmental disturbances of the tongue: l Aglossia l Microglossia l Macroglossia l Ankyloglossia l Cleft tongue l Fissured tongue l Median rhomboid glossitis l Benign migratory glossitis l Hairy tongue l Lingual varices l Lingual thyroid nodule Developmental disturbances of the oral lymphoid tissue: l Reactive lymphoid aggregates l Lymphoepithelial cyst

Developmental disturbances of the salivary glands: l l l Aplasia Xerostomia Hyperplasia Atresia Developmental lingual mandibular salivary gland Anterior lingual depression.

Fissural cysts of the oral region: l l l l Nasopalatine duct cyst Median palatal cyst Median mandibular cyst Nasoalveolar cyst Palatine & alveolar cysts of newborn Thyroglossal tract cyst Epidermal cyst Dermoid cyst

Developmental disturbances of jaws

Developmental disturbances of jaw: l Agnathia l Micrognathia l Macrognathia l Facial hemihypertrophy l Facial hemiatrophy

Agnathia: Congenital absence of maxilla or mandible l Commonly, only a portion of one jaw is missing l In maxilla Maxillary process l Premaxilla In mandible l Entire mandible l Condyle l Entire ramus l l

l In Agnathia, the ears are very close to each other due to absence or hypoplasia of the mandible. The external ears assume a horizontal position with the lobules located near the midline. l In case of maxilla, one maxillary process or premaxilla may be missing.

Agnathia

Micrognathia: l A smaller jaw than normal l Involve either maxilla or mandible Types Ø Apparent [Abnormal positioning/ relation of one jaw to the other or skull] Ø True § § Congenital Acquired

Congenital micrognathia: Etiology unknown l Associated with other congenital abnormalities like congenital heart disease and Pierre Robin syndrome l Micrognathia of maxilla is due to a deficiency of the premaxillary area l Micrognathia of mandible may be due to l l Small jaw Posterior positioning of mandible in relation to skull Steep mandibular angle Agenesis of condyles

MICROGNATHIA: Congenital: • Pierre Robin syndrome • Crouzon syndrome Acquired: • Ankylosis

Cheekbone & mandible hypoplasia, down-slanting palpebral fissures Treacher-Collins Severe mandibular hypoplasia & cleft palate Pierre-Robin

Macrognathia: 1. May be associated with certain conditions: l Pitutary gigantism l Paget’s disease l Acromegaly l Fibrous dysplasia .

Source: Dr. Wisanu Charoenkul May also be unassociated with any other conditions

Macrostomia: l Inadequate fusion of the mandibular and maxillary process with each other may lead to an abnormally wide mouth

Facial hemihypertrophy: l Asymmetric overgrowth which is confined to one side of the body – unilateral macroglossia, premature eruption & increased size of dentition.

Hemihypertrophy

Facial hemiatrophy: l Slowly progressive atrophy of the soft tissue of essentially half the face, which is characterized by progressive wasting of subcutaneous fat, atrophy of the skin, cartilage, bone, muscle. l May be a form of localized scleroderma.