Oral Patho part 2 Developmental Disturbances OF ORAL Lymphoid Tissue PDF

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developemntal distrubances part 2...

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DEVELOPMENTAL DISTURBANCES OF ORAL LYMPHOID TISSUE Chief -

sites being the posterior portion of the dorsolateral aspect of the tongue Ventral tongue Buccal mucosa Floor of the mouth Soft palate

1. REACTIVE LYPHOID AGGREGATE ( REACTIVE LYMPHOID HYPERPLASIA)  Lingual tonsil – one of the largest oral lymphoid aggregate Located on the posterior portion of the tongue on dorsolateral aspect Surround by crypt lined by stratified squamous epithelial Becomes inflamed and enlarged Bilateral enlargement; *but if unilateral it can be an early carcinoma Clinical Feature -Firm nodular submucosal mass Treatment plan - Conservative local surgical excision 2. LYMPHOID HAMARTOMA ( ANGIOFOLLICULAR LYMPH NODE MALFORMATION) Benign proliferative lesion of lymphoid tissue Localized (mass) hyperplasia or a hamartoma Second common site is on the neck area at the mediastinum 3. ANGIOLYMPHOID HYPERPLASIA WITH EOSINOPHILS (KIMURA’S DISEASE, EOSINOPHILIC LYMPHOID GRANULOMA; EOSINOPHILIC FOLLICULARIS) - Reactive angioproliferative lesion which is usually found in subcutaneous tissue of head and neck - Often in young or middle-aged females - Lesion is characterized by a atypical endothelial proliferation with a chronic inflammatory infiltrates.  Etiology – may be related to some benign vasculitis; localized form of vasculitis 4. LYMPHOEPITHELIAL CYST (BRACHIAL CYST) Clinical feature - Cyst appears small asymptomatic - Well circumscribed - Yellowish elevated nodule

- Usually on the floor of the mouth or ventral tongue - Soft palate - Mandibular vestibule. Anterior pillar and retromolar pad - 1.5 -2.0 cm in diameter Histologic feature - Lined by stratified squamous epithelial - Embedded in, and circumscribed mass of lymphoid tissue - Showing discrete follicles - Lining is quite thin - Lacking rete pegs and parakeratotic Treatment - Conservative local surgical excision DEVELOPMENTAL DISTURBANCES OF SALIVARY GLANDS 1. AGENESIS - Congenital absence of major salivary glands is an unknown occurrence Clinical feature - Chief complaint of patient is xerostomia (dry mouth) - Mucosa appears dry, smooth or pebbly sometimes and shows tendency of accumulation of debris. - Cracking lips - Fissuring corners of the mouth Treatment - Scrupulous oral hygiene - Preserve the teeth 2. XEROSTOMIA - Dryness of mouth - Salivary glands dysfunction Clinical feature - Patient experience severe/extreme discomfort - Mucosa is dry & atrophic sometimes inflamed or often plate and translucent Etiology - Emotional reaction with blockage of the duct by calculus - Drug administrations (i.e anti-histamine drugs) Treatment - Depend upon the nature of disease

DISEASES OF THE BONE

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Bone is a dense calcifies tissue specifically affected by a variety of disease Group, may arise at all ages Congenital and present at birth Others develop in early childhood, young adulthood or even later in life.

1. OSTEOGENESIS IMPERFECTA (“brittle bones’ Fragilitas Ossium; Osteopsathyrosis; Lobstein’s Disease) Etiology - Related to dentinogenesis imperfect  affecting mesodermal tissues - Hereditary autosomal dominant characteristic, autosomal recessive and hereditary type. - Present at birth  Vrolik’s type - Later in life  Lobstein type *infants afflicted with osteogenesis imperfect are still born or die shortly after birth Clinical feature 1. Extreme fragility and porosity of bone  fracture heal readily but the new bone is of a similar imperfect quality 2. Pale blue sclera  generally found in older patients with more sever disease and earlier onset of fractures 3. Deafness  due to otosclerosis 4. Abnormalities of the tooth  hereditary opalscent dentin 5. Laxity of the ligaments 6. Peculiar shape of the skull 7. Abnormal electrical reaction of the muscles 8. Tendency for capilliary bleeding Oral manifestations - Disturbance of mesodermal tissue - Bowing of extremities Histologic features Thin cortices - Compromised of immature spongy bone - Trabeculae of the cancellous bone are delicate and show micro fractures - Osteoblastic activity appears retarded and imperfect Treatment - No known treatment 2. INFANTILE CORTICAL HYPEROSTOSIS

(caffey’s disease) Etiology - Cortical thickening in certain bones of infants

Clinical feature 1. Swelling of cortical thickening or hyperostosis 2. Occurs during first 3 months of life 3. Most affected bones are mandible and clavicle 4. Fever 5. Dysphagia 6. Pseudo paralysis 7. Hyper irritability 8. Pleurisy 9. Anemia 10.Leukocytes 11.Monocytosis 12.Increase rate and increase serum alkaline phosphatase Oral manifestations - Asymmetric deformity of the mandible usually in the angle and ramus area - Malocclusion Radiographic feature - Infantile cortical hyperostosis - Unilateral or bilateral as a gross thickening and sclerosis of the cortex due to proliferation Treatment - No treatment 3. CLEIDOCRANIAL DYSPLASIA - Unknown etiology - When inherited, it appears as a dominant medelian characteristic and may be transmitted by either sex. Clinical feature 1. Abnormalities of the skull, teeth, jaws, and shoulder, girdle as well by stunting of the long bones 2. Fontanels remain open or delayed closing 3. Suture’s remain open 4. Wormian bones are common 5. Sagittal suture are sunken giving the skull a flat appearance 6. Frontal, parietal and occipital bones are prominent

7. Paranasal sinuses are under developed and narrow 8. Brachycephalic (wide and short) with transverse diameter of the skull being increased 9. Unusual mobility of the shoulders. Oral manifestations - High narrow, arched palate - Cleft palate - Underdeveloped maxilla - Prolonged retention of deciduous teeth - Delayed in eruption and permanent teeth - Shorter and thinner roots - Partial anodontia, sometimes supernumerary teeth Treatment - No treatment 4. CRANIOFACIAL DYSOSTOSIS (Crouzon disease or syndrome) - Genetic disease characterized by a variety of cranial deformaties, facial malformatios, eye changes and occasional other associated abnormalities Clinical feature 1. Patients show protuberant frontal region with an anteroposterior ridge overhanging the frontal eminence and passing to the roof of the nose (triangular frontal defect) 2. Malformation consist of hypoplasia of the maxillae with mandibular prognathism 3. High arched palate 4. Facial angle is exaggerated 5. Patient’s nose is described is resembled as parrot’s beak 6. Eyes are hypertension exopthalmus with divergent strabismus and optic neuritis 7. Spina bifida occulta Treatment - Craniectomy 5. MANDIBULAR DYSOSTOSIS “brachial arch syndrome” – heterogenous group of malformations of the head and neck characterized by anatomic alterations - Defects of the head and face often hereditary or familial in pattern Clinical feature 1. Anti-mongoloid palpebral fissures  (outer the lower lids), deficiency of the eyelashes and sometimes upper lid 2. Hypoplasia of the facial bones  malar bone and mandible

3. Malformation of the external ear occasionally of the middle and internal ears 4. Macrostomia, high palate  sometimes cleft and abnormal position and malocclusion of the teeth 5. Blind fistulas between the angle of the ear, and angle of the mouth 6. A typical hair growth in the form of tongue-shaped process. 7. Facial clefts and skeletal deformities Radiographic feature - Agenesis of the malar bones with non-fusion of zygomatic arches - Paranasal sinuses are grossly underdeveloped and mastoids appear infantile and sclerotic - Auditory ossicles are absent and cochlea and vestibular apparatus may be deficient Treatment - No treatment 6. PIERRE ROBIN SYNDROME - Consists of cleft palate, micrognathia and glossoprosis - “bird facies” - Prevents the normal descent of the tongue between the palatal shelves - Important result of jaw malformation is respiratory difficulty - Skeletal anomalies 7. MARFAN SYNDROME - Hereditary disease transmitted as an autosomal dominant trait - A disease of connective tissue related to defective organization of collagen Clinical features a. Excessive length of tubular bones or long thin extremities b. Arachnodactyly or spidery fingers c. Shape of the skull and face is long and narrow d. Hyperextensibilty of joints e. Habitual discolorations f. Kyphosis or scoliosis g. Bilateral ectopia lentis  caused by weakening or rupture of suspensory ligaments h. Cardiovascular complications  aortic aneurysm, regurgitation, vulvar defects, enlargement of the heart. Oral manifestations - High arched palatal vault - Bifid uvula - Malocclusion - Multiple odontogenic cysts of the maxilla and mandible - Temporomandibular dysarthosis

Treatment - No specific treatment 8. DOWN SYNDROME (Trisomy 21; Mongolism) Clinical a. b. c. d. e. f. g. Oral -

features Large anterior fontanels Open sutures Flat face Small slanting eyes with epicanthal folds Cardiac abnormalities Sexual underdervelopment Hypermobility of joint

manifestations Prognathism Open mouth Macroglossia Protruism of the tongue Fissured or pebbly tongue from enlarged papilla High arched palate Malformed teeth Enamel hyperplasia Microdontia

9. OSTEOPETROSIS (Marble bone disease; alber’s schanberg disease) Types: - Clinically benign dominantly inherited form -  develops in life -  almost all are asymptomatic - Clinically malignant recessively inherited form -  present at birth -  more severe Malignant recessive osteopetrosis - Optic atrophy - Hepatosphenomegaly - Poor growth - Frontal bossing - Pathologic feature - Loss of hearing

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Facial palsy Genu valgum Death from anemia or secondary infection Death by age 20

Benign Dominant osteopetrosis - Multiple pathologic fractures - Bone pain - Cranial nerve palsy (including optic and facial nerves) - Osteomyelitis Oral manifestations - Mark predelition for osteomyelitis - Prone to fracture of the jaw - Enamel hypoplasia - Microscopic dentinal defects - Arrested root development - Prone o dental caries Radiographic feature - Diffuse, homogenuous symmetrically sclerotic appearance of bones - Chubbing and transverse striations at the end of long bones - Medullary cavities are replaced by bone - Thickened cortex - Teeth may not be visible due to thickening of the jaws Laboratory findings Myelosphthisic anemia due to displacement of hemopoietic marrow tissue by bone - RBC count : less than 1 minute cells per cubic mm - Elevated serum acid phosphatase in benign dominant osteopetrosis Histologic Features - Endosteal bone production with lack of physiologic bone resorption prominent osteoblast - Persistence of cartilaginous cores of bone trabeculae long after their replacement should have occurred - Fibrous bone marrow - Bone is deficient in collagen matrix fibrils 10. ACHONDROPLASIA Clinical Feature : - Disturbances in endochondrial bone formation which results in a characteristic form of dwarfism - Hereditary; autosomal dominant

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Begins in utero Quite short (under 1.4 meters high) Short, thickened muscular extremities Brachycephalic skull bowed legs small hands, stubby fingers lumbar lordosis; prominent buttocks and protruding abdomen limitation of movement in several joints

Oral manifestations - retruded maxilla due to restriction of growth at the base of the skull - relative mandibular prognathism - malocclusion - congenitally missing teeth - malformed shape of existing teeth Radiographic feature - long bones are shorter than normal - thickening or clubbing of the ends of long bones - epiphysis close early or late - bone at the base of the skull fuse prematurely short and narrow foramen magnum Histologic Features - retardation or aplasia of the zone of provisional calcification of endochondrial growth - cartilage lacks orderly arrangement, fail to calcify properly and are not restored and replaced by bone in usual way. 11. PAGET’S DISEASE (osteitis deformans)  Theories in Etiology – circulatory disturbance; inflammatory process  Incidence - Patient over age 40 - Male - Common in England, France and Germany Clinical Feature : - Bone pain - Severe headache - Deafness; blindness; facial paralysis - Dizziness - Weakness - Mental disturbances - Progressive enlargement of the skull - Deformities of the spine femur and tibia (bowing of legs) - Broadening and flattening of the chest and spinal curvature

Oral -

manifestations Predilection for maxilla Progressive enlargement of maxilla Widening of alveolar ridge Palate is flattened Teeth become loose and migrate With increased jaw size, open mouth appearance as lips do not enlarge with jaws - hypercementosis

Radiographic feature - linitial phase of dessification and softening, followed by bizarre, dysplastic reossification. Histologic Features - “mosaic bone” which is partially resorbed and repaired, leaving reversal lines - Jigsaw puzzle appearance Laboratory findings - Normal serum calcium - Normal serum phosphorous - Elevated serum alkaline phosphtase Differential diagnosis - Fibrous dysplasia - Hyperparathyroidism - Subacute osteomyelitis - Chronic sclerosing osteomyelitis - Fibro osteoma - Osteosarcoma 12. GENERLAIZED CORTICAL HYPEROCTOSIS (Endosteal hyperostosis) Clinical Feature: - Discoloured until adult life - Face is swollen with widening at the angles of the mandible and at the bridge of the nose - Loss of visual acuity - Loss of facial sensation - Facial paralysis and deafness due to cranial nerve involvement Radiographic feature: - Skeletal will reveal increased density of bone of the body - Skull also exhibits diffuse sclerosis

Histologic feature: - Normal dense bone but without evidence of remodelling. Differential diagnosis - Osteopetrosis (marble bone disease) - Osteitis deformans (paget’s disease) - Progressive diaphyseal dysplasia ( Camurati – engel mann disease) Treatment - No Treatment

13.

MASSIVE OSTEOLYSIS - “vanishing bone” - Unusual and uncommon disease characterized by spontaneous progressive resorption bone - Appears to be related to an active hypermia of bone It must be differentiated from: a. Osteolysis associated with an infection such osteomyelitis or with rheumatoid arthritis b. Osteolysis associated with disease of  CNS, Tabes dorsalis, syringomyelia, leprosy or myelodysplasia Clinical Feature: - Common in older children and younf and middle-aged adults - Affecting both sexes equally - Only one bone is affected in a given patient - Most commonly affected bones are the clavicle, scapula, humerus, ribs, ilium, ischium and sacrum - May or may not be painful begins suddenly and advances rapidly until replaced by a thin layer of fibrous tissue surrounding a cavity. Oral manifestations - Involves the mandible and other facial bones - Patient may present with pain or facial asymmetry or both Histologic feature: - Replacement of bone by connective tissue containing many thin-walled blood vessels or anastomosing vascular spaces lined by endothelial cells Treatment - No treatment 14. FIBROUS DYSPLASIA OF BONE - Jaffe’s type  it involves a variable no. of bones; accompanied by “café au ait” skin pigmentation - Albright’s syndrome  involves nearly all bones with skin pigmentations; endocrine disturbances.

Oral manifestations - Expansion and deformity of mandible - Disturbed eruption of teeth due to loss of normal support of developing teeth; endocrine disturbance Radiographic feature: - Thin cortical bones - Irregular medullary bone - Irregular trabeculation looks multicystic - In early stage, fibers appear radioluscent - Smoke screen appearance radiolucencies and radiopacities - “ground glass” appearance - Mature stage – “crange peel” Laboratory findings - Normal serum calcium - Elevated alkaline phosphtase - Premature secretion of FSH - Elevated basal metabolic rate Histologic feature: - Bony trabeculae vary in shape C-shape or Chinese character appearance - Predominance of fibroblast 15.CHERUBISM – uncommon disease involves the jaw Clinical Feature: - Premature shedding of deciduous teeth - Defective permanent teeth - Limited jaw movement - Difficulty in speech, deglutition and mastication Radiographic feature: - Extensive bilateral destruction and thinning of cortical plate of bone giving a multiocular radioluscent appearance Laboratory findings - Normal blood elements - Normal serum elements - Normal calcium - Normal alkaline phosphatase Histologic feature: - Presence of multinucleated giant cells with loose fibrillar connective tissue containing fibroblast and blood vessels - Absence of bony trabeculae...