Developmental Disturbances PDF

# Developmental Disturbances of the Face, Jaws, Lips, Palate, and Tongue ## Developmental Disturbances of the Face - **Congenital:** born with - **Hereditary:** Parental through genes - **Genetic:** Problem in gene of person - **Autosomal:** Any problem in chromosomes other than X or Y...

# Developmental Disturbances of the Face, Jaws, Lips, Palate, and Tongue ## Developmental Disturbances of the Face - **Congenital:** born with - **Hereditary:** Parental through genes - **Genetic:** Problem in gene of person - **Autosomal:** Any problem in chromosomes other than X or Y. - **Sex-linked:** Problem in X or Y chromosomes - **Developmental:** During development - **Acquired:** Teratogens, mutations of embryo - **Developmental Disturbances of the Face:** - **Oblique Facial Cleft (lateral facial cleft):** - A congenital developmental cleft that runs from the inner canthus of the eye to the ala of the nose or upper lip. - **Pathogenesis:** Incomplete fusion of maxillary and lateral nasal processes. - **Transverse Facial Cleft:** - A congenital developmental cleft that runs from the angle of the mouth to the tragus of the ear. - **Pathogenesis:** Incomplete fusion between maxillary and mandibular processes. - May be unilateral or bilateral. - **Macrostomia:** - Is a congenital developmental abnormal large mouth. - **Pathogenesis:** Premature arrest of fusion between maxillary and mandibular processes. - **Microstomia:** - Is a congenital developmental small mouth. - **Pathogenesis:** Excessive fusion between maxillary and mandibular processes. ## Developmental Disturbances of the Jaws - **Agnathia:** - A rare, congenital, developmental, complete, or partial absence of the jaw. - **Macrognathia:** - Large jaw - May be: - **True:** - Congenital - Hereditary - Acquired - Paget's disease (bone) - Protrusion of jaw due to abnormal relation of jaw to the base of skull. - Acromegaly (hormone) - **Relative:** - Protrusion of jaw due to abnormal relation of jaw to the base of skull. - **Micrognathia:** - Small jaw - May be: - **True:** - Congenital - Hereditary - Acquired - Trauma - Infection - **Relative:** - Retrusion of the jaw. ## Developmental Disturbances of the Lip: - **Cleft Lip:** congenital developmental - **Etiology:** - Hereditary - Environmental factors - **Upper Lip:** - **Unilateral Complete:** Extending to the nose. - **Unilateral Incomplete:** Notch in the lip. - **Bilateral Complete:** Extending to the nose. - **Bilateral Incomplete:** Notch in the lip. - **Bilateral Mixed:** Complete and incomplete. - **Pathogenesis** Incomplete fusion between maxillary and medial nasal processes - **Median Cleft:** very rare - **Pathogenesis:** Incomplete fusion of medial nasal processes. - **Lower Lip:** - **Median Cleft:** - **Pathogenesis:** Incomplete fusion between mandibular processes. ## Significance of Cleft Lip - **Poor feeding** - **Poor tooth development** - **Poor speech** - **Poor appearance** - **Surgical treatment from 3-6 months:** - To act as a guide for teeth alignment. ## Congenital Lip Pits - **Congenital developmental blind epithelial lined depression.** - **Etiology:** Hereditary - **Clinical Picture:** - **Vermillion border of lower lip.** - **Oral Commissures (Commissural lip pits).** - **May be unilateral or bilateral.** - **Fistula opens at minor salivary gland duct.** - **Pathogenesis:** Failure of fusion between maxillary and mandibular processes. ## Cheilitis Glandularis Apostematosa - **Inflammatory condition affecting minor SG of lower lip.** - **Etiology:** - Unknown - Sun exposure - Allergy - Tobacco ## Melkerson-Rosenthal Syndrome - **Cheilitis granulomatosa** - **Facial paralysis** - **Fissured tongue** ## Peutz-Jeghers Syndrome (Hereditary) - **Small intestinal polyps (abdominal pain)** - **Spots of melanin pigmentation** ## Gardner Syndrome (Hereditary) - **Multiple large intestine polyps (pre-cancerous).** - **Osteoma of jaws and skull.** - **Fibroma in oral cavity.** - **Supernumerary teeth.** - **Epidermal cysts** ## Developmental Disturbances of the Palate - **Cleft Palate:** - **Pre-alveolar:** Cleft lip - **Alveolar:** - **Post-alveolar:** Cleft palate - **Etiology:** - Hereditary - Environmental factors - **Pathogenesis:** Incomplete fusion between palatine processes. - **May include:** - Uvula - Uvular soft palate - Uvular soft palate and hard palate + incisive - **Complete and/or unilateral.** - **Complete and/or bilateral.** - **Alveolar cleft:** - **Unilateral** - **Bilateral:** - **Results in absence of lateral or supplemental.** - **Least site of cleft palate (incisive foramen)** ## Torus Palatinus: - **Congenital** - **Hereditary developmental hamartoma:** Normal tissue, normal site, but excessive amount. - **Single or multiple.** - **Lobulated or nodular.** - **Covered with intact mucosa but thin.** - **Asymptomatic.** - **Histopathology:** Composed of compact bone, or core of cancellous bone covered by layer of compact bone. - **Significance:** Removed in edentulous patients. ## Developmental Disturbances of the Tongue: - **Aglossia:** - Congenital absence of tongue. - **Pathogenesis:** Failure of development of two lateral lingual swellings - **Significance:** Speech, swallowing, mastication - **Macroglossia:** - Abnormal large tongue. - **Congenital Macroglossia:** - **Cretinism:** Thyroid hormone before puberty. - **Mongolism:** - **Lymphangioma or haemangioma.** - **Fibrous and muscular hypertrophy (large cell size).** - **Acquired Macroglossia:** - **Myxedema:** Thyroid hormone after puberty. - **Acromegaly:** Thyroid hormone after puberty. - **Tumors:** Papilloma, fibroma, carcinoma. - **Chronic infection:** TB or syphilis - **Differentiate between congenital and acquired macroglossia:** - **Congenital:** Smooth borders. - **Acquired:** Scalloped borders because they are compressed against the teeth and between interdental spaces. - **Microglossia:** - Congenital defect in which tongue is small and is associated with hypoplasia of mandible. - **Ankyloglossia (Tongue Tie):** - Developmental defect resulting in limiting tongue movement. - **Complete ankyloglossia:** - Failure of separation of floor of mouth. - **Partial ankyloglossia:** - Short lingual frenum for frenum is attached at tip of tongue. - **Superior ankyloglossia:** - Dorsal surface of tongue is connected to palate (cleft palate). - **Acquired ankyloglossia:** - Due to infection, malignancy, or radiation. - Floor of mouth or ventral surface. - **Rare.** - **Sign:** Speech - **Common:** - **Rare:** - **Hypermobility:** - Rare condition due to abnormal long frenum that can lead to suffocation and death. - **Cleft Tongue (Bifid Tongue):** - Developmental congenital defect, complete or partial cleft. - **Pathogenesis:** Incomplete fusion between two lateral lingual swellings. - **Fissured Tongue:** - Acquired, symmetrical, or semi-symmetrical grooves on the dorsal surface of tongue. - **Etiology:** - Chronic trauma - Vitamin deficiency - Smoking - Syphilis - Stress - Increase with age. - **Clinical Appearance:** - Depapillated - Burning sensation (citric or salty food) - Fissures may be transverse, cribriform, foliacoius or irregular. - **Histology:** Hyperplastic epithelium, chronic inflammatory cells. - **Significance:** Burning sensation with salt and citric food. - **Median Rhomboid Glossitis:** - **Pathogenesis:** - Theory 1: Failure of two lateral swelling lingual swellings to complete submerge the tuberculum impar before fusing with each other (congenital). - Theory 2: Atrophic Candidiasis (acquired). - Treated with antifungal medications - **Clinical Picture:** - Depapillated - Red - Raised - Round, oval or rhomboid - Anterior to foramen cecum - Painless - Glossitis is a misnomer because it's rarely rhomboid in shape and it's not inflammatory condition. - **Patient must be assured it's not malignancy.** - **Geographic Tongue (erythema migrants / benign migratory glossitis):** - **Etiology:** Unknown - **Common in women with skin eruption** - **Clinical Picture:** - Desquamation of filliform papilla. - Fungiform papilla retained as red dots. - Appear as red patches surrounded by elevated irregular yellowish border. - May coalesce with each other. - Persist for one week then disappear, then repair in another area. - **Significance:** Burning sensation - **Hairy Tongue:** - Condition of excessive proliferation of filiform papilla. - **Etiology:** - Unknown - Gastric upset - Poor oral hygiene - Smoking - Fungi - **Clinical Picture:** - Hyperplastic papilla - Poor desquamation of papilla - Yellowish / brown / black - **Significance:** Food impaction / fungal infection / foul odor - **Lingual Thyroid Nodule (Ectopic)** - Ectopic thyroid tissue in tongue. - **Pathogenesis:** Deficient migration a foramen cecum. - **N. B.** It may arise normally at the midline of the neck and also another thyroid tissue differentiates at the tongue. - **Clinical Picture:** Yellowish white elevation near the midline of foramen cecum. - **Significance:** May turn to adenocarcinoma. - **Management:** Gage iellex / dysphagea. Inject radioactive iodine to verify the presence of normal thyroid tissue. - If thyroid gland is present in normal site, we remove the extra thyroid tissue. - If thyroid gland is absent, we transplant the thyroid tissue in normal site. ## Summary of Developmental Disturbances: | Condition | Type | Etiology | |----------------------|-----------|-----------------------------------------------------------| | **Face:** | | | | Oblique Facial Cleft | Congenital| Hereditary, developmental, teratogens, mutations of embryo | | Transverse Facial Cleft | Congenital | Hereditary, developmental, teratogens, mutations of embryo | | Macrostomia | Congenital | Hereditary, developmental, teratogens, mutations of embryo | | Microstomia | Congenital | Hereditary, developmental, teratogens, mutations of embryo | | **Jaws:** | | | | Agnathia | Congenital | Hereditary | | Macrognathia | Congenital | Hereditary, Paget's disease, acromegaly | | Micrognathia | Congenital | Hereditary, Developmental | | **Lip:** | | | | Cleft Lip | Congenital | Hereditary, environmental factors | | Median Cleft | Congenital | Hereditary | | **Palate:** | | | | Cleft Palate | Congenital | Hereditary, environmental factors | | Torus Palatinus | Congenital | Hereditary | | **Tongue:** | | | | Aglossia | Congenital | Hereditary | | Macroglossia | Congenital | Hereditary, cretinism, mongolism, lymphangioma, haemangioma, fibrous & muscular hypertrophy| | Microglossia | Congenital | Hereditary | | Ankyloglossia | Congenital | Hereditary | | Cleft Tongue | Congenital | Hereditary | | Fissured Tongue | Acquired | Chronic trauma, vitamin deficiency, smoking, syphilis, stress, increase with age | | Median Rhomboid Glossitis | Acquired | Atrophic Candidiasis | | Geographic Tongue | Acquired | Unknown | | Hairy Tongue | Acquired | Unknown, gastric upset, poor oral hygiene, smoking, fungi | | Lingual Thyroid Nodule | Ectopic | Deficient migration at foramen cecum | - **Acquired:** - Acquired macroglossia - Acquired ankyloglossia - Fissured tongue - Median Rhomboid Glossitis - Hairy tongue - **Hereditary Hamartomas:** - Torus palatinus - White spongy nevus - **Ectopic:** - Latent bone cyst - Fordyce's granules - Lingual thyroid nodule - Ectopic Thyroid tissue - Facial paralysis - Fissured tongue - Cheilitis granulomatosa - White spongy nevus