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Developmental disorders of orofacial structures dental oral pathology
 

Developmental disorders of orofacial structures dental oral pathology

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Developmental disorders of orofacial structures dental oral pathology

Developmental disorders of orofacial structures dental oral pathology

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    Developmental disorders of orofacial structures dental oral pathology Developmental disorders of orofacial structures dental oral pathology Presentation Transcript

    • Developmental disorders of orofacial structures
    • Orofacial Clefts • • • • • • • Cleft Lip (CL) Cleft Palate ( CP ) Cleft Lip with Cleft Palate (CL + CP) Lateral Facial Cleft Oblique Facial Cleft Median Cleft of upper lip Median Maxillary anterior alveolar cleft.
    • Etiology • Genetic abnormalities • Inherited • Spontaneous mutation • Environmental factors • • • • Nutritional Deficiency Cigarette smoking Drugs, radiation. Amniotic bands
    • Cleft Lip and Palate • Most common among the facial clefts. • Cleft is a division or separation of parts of the lip or palate that is formed during the early months of development of the fetus. • Clefts may be unilateral or bilateral. • They can vary in severity. • 3-8% of clefts are associated with syndromes
    • Unilateral incomplete cleft lip
    • Unilateral Complete Cleft lip
    • Bilateral Complete Cleft Lip
    • Frequency of Occurrence • In whites,CL ± CP occur in 1 of every 700-1000 births • In Asians,prevalence is 1.5 times higher. • In African Americans,less than 0.4 in 1000. • in Native Americans fequency is 3.6per 1000birth • Incidence of clefts is highest in Native American population and lowest in African Americans
    • Frequency of occurrence • • • • • • • Isolated Cleft Lip : 25% Isolated Cleft Palate : 30% Cleft Lip + Cleft Palate : 45% CL ± CP : more common in males. For isolated CL , M : F = 1.5 : 1 For CL + CP, M : F = 2 : 1 Isolated CP is more common in females.
    • Location • Cleft Lip is more commonly unilateral (80%) • 70% of cleft lips on left side. • Complete CL extends upward into nostril. • CP may involve hard and soft palate or soft palate alone. • Cleft or Bifid Uvula is more common.
    • • • • • • Bifid Uvula: Whites: 1 in 80 Asian and Native American: 1 in 10 African Americans: 1 in 250 Submucous palatal cleft : surface mucosa is intact but underlying musculature of soft palate is defective.
    • Complete cleft palate
    • Bifid uvula
    • Submucous palatal cleft
    • Pierre Robin Syndrome • Triad of : • Mandibular micrognathia • Glossoptosis • Cleft Palate.
    • Bird facies
    • Problems associated with clefts • • • • • Esthetic disfigurement Difficulty in breathing ,feeding and speech Malocclusion. Psychosocial problems. Recurrent upper respiratory tract infections.
    • Treatment • Multidisciplinary approach • Plastic surgery, Dentistry,Otolaryngology,Audiology,speec h pathology,Genetics and Pediatrics • SURGICAL REPAIR.
    • Cleft lip repair • Objective: Closure of cleft to create a pleasing face that will develop normally with minimum scarring • Performed by plastic surgeon when baby is approx. 3 months of age and weighs atleast 10 lbs.
    • • Surgery under GA( takes approx 2-3 hours) • Special considerations in positioning and feeding. • Takes approx. 3 weeks for the wound to gain enough strength. • The lip scar is initially red and swollen but begins to mature and improve in appearance in 6-12 months. • In Bilateral cleft lip , repair in stages. • In alveolar cleft, bone graft required ( 5-10 yrs)
    • Cleft palate repair • Objective: To close the palate to restore normal function of eating and drinking and to enhance the development of normal speech. • Best accomplished in one surgical procedure before the child reaches 12-14 months of age.
    • Pierre Robin syndrome • Infant placed in prone position until adequate growth of jaws occur. • Tongue- Lip adhesion/Tracheostomy • Mandibular bone expansion- Distraction Osteogenesis with an expansion device.
    • Distraction osteogenesis
    • After removal of the expansion device
    • Late cleft treatment • • • • Cleft palate surgery Intensive speech therapy regimen Orthognathic surgery. Orthodontic correction.
    • Genetic counseling • In Non syndromic cases, risk of Cleft development in a sibling or offspring is 3-5% if no other first degree relative is affected. It increases to 10 – 20 % if another first degree relative is affected
    • Developmental disorders of Lips • Lip Pits: • Para median Lip Pits • Commissural Lip Pits • Double Lip
    • Paramedian Lip Pits • Rare • Autosomal dominant inheritance • Persistence of lateral sulci on embryonic mandibular arch • Bilateral symmetric fistulas on either side of the midline
    • Paramedian Lip Pits • Appearance varies from subtle depressions to prominent humps. • Pits can extend to a depth of 1.5 cm and may express salivary secretions • Seen in Van der Woude syndrome with CL±CP • Surgical excision for cosmetic reasons
    • Commissural Lip Pit • Small mucosal invaginations at corners of the mouth on vermilion border. • Failure in the normal fusion of maxillary and mandibular processes during development
    • Commissural Lip Pit • Seen in 12-20 % of adult population. • Males > Females • Unilateral / Bilateral • May be associated with preauricular pits • No treatment required
    • Double Lip • Rare • Redundant fold of tissue on the mucosal side of the lip. • May be Congenital or acquired( from trauma or oral habits such as lip sucking)
    • Double lip • More common in upper lip • Sometimes both lips affected • Seen on smiling. • Feature of Ascher’s syndrome
    • Ascher’s syndrome • Triad of : • Double lip • Blepharochalasis • Nontoxic enlargement of thyroid gland
    • Developmental disorders of tongue • • • • • • • • • • Macroglossia Microglossia Ankyloglossia Lingual Thyroid Median Rhomboid glossitis Benign Migratory glossitis Hairy Tongue Bifid tongue Fissured tongue Lingual Varices
    • Macroglossia
    • Causes • Congenital: • Acquired: • • • • • • • • • • Haemangioma Lymphangioma Cretinism Down syndrome Beckwith Weidemann Syndrome • Neurofibromatosis • Hemi hyperplasia Amyloidosis Myxoedema Acromegaly Angioedema Carcinoma and other tumours • Edentulous state
    • Differentiating features • • • • Cretinism and Beckwith Weidemann Syn: Smooth, diffuse generalized enlargement Lymphangioma: Pebbly appearance with multiple vesicle like blebs • Amyloidosis, Neurofibromatosis: • Multinodular appearance
    • Differentiating features • Down Syndrome: • Papillary fissured surface • Hemifacial Hyperplasia, Neurofibromatosis: • Unilateral enlargement • Edentulous: • Lateral spreading of tongue.
    • Microglossia • Oromandibular- limb hypogenisis syndrome • Frequently Seen along with hypoplasia of mandible
    • Ankyloglossia Anomaly characterized by short thick lingual frenum resulting in restriction in tongue movement. Sever case tongue fused to floor of mouth Frenum some time attached to tip of the tongue
    • Ankyloglossia • Seen in 2-5 % of the population • Males > Females • May be partial or complete • May cause speech defects. • Surgery if the anomaly is severe
    • Lingual Thyroid-origin • Failure of primitive thyroid gland to descend into the neck during development. • About 10 % of both men and women may show thyroid tissue if biopsy is taken from posterior part of tongue.
    • Lingual Thyroid-Clinical features • Symptomatic and clinically evident lingual thyroid seen less commonly. • 4 - 7 times more common in females • May cause dyspnoea, dysphonia, dysphagia
    • Lingual Thyroid-Diagnosis • In 70% of cases this ectopic gland is the patient’s only thyroid tissue. • Thyroid scans using Iodine isotopes or technetium 99m • CT and MRI to know the size and extent
    • Lingual Thyroid-Treatment • • • • • Asymptomatic patients: No treatment, Periodic follow up Symptomatic patients: Suppressive therapy with thyroid supplements In case of airway obstruction,surgery with auto transplantation to another body site. • In males over 30 years ,prophylactic surgical excision as there is a risk of malignancy.
    • Median Rhomboid glossitis
    • Benign Migratory glossitis
    • Hairy Tongue • Accumulation of keratin on the filiform papillae may be due to increased production of keratin or decrease in the normal keratin desquamation
    • • • • • • • • • Etiology Antibiotic therapy Poor oral hygiene General debilitation Radiation therapy Use of oxidizing mouth wash or antacid Overgrowth of fungus or bacterial organism Histopathology- it is characterized by marked elongation and hyper keratosis of filiform papillae
    • Fissured Tongue • Multiple grooves and furrows on the surface of the tongue • Strong association noted between geographic tongue • Seen some time in Melkersson Rosenthal syndrome
    • Lingual Varices • Abnormally dilated and tortuous veins • Seen in 2/3rds of people older than 60 years of age(indicating age related degeneration ) • Asymptomatic
    • Developmental disorders of the jaws • • • • • • Exostoses and Tori Hemifacial atrophy Hemifacial hypertrophy Condylar hypertrophy Coronoid hypertrophy Mandibular aplasia
    • Mandibular Torus
    • Palatine Torus
    • Hemifacial Atrophy
    • Hemifacial hypertrophy
    • Condylar hypertrophy
    • Coronoid hypertrophy
    • Mandibular aplasia
    • Thank You