Jones 2 Flashcards
What is PROGRESSIVE HEMIFACIAL ATROPHY (ROMBERG or PARRY-ROMBERG SYNDROME)?
Degenerative condition associated with atrophic changes on one side of the face
Cause of Progressive Hemifacial Atrophy
-Cause is unknown: malfunction of the cervical sympathetic nervous system, trauma, Lyme disease
Progressive Hemifacial Atrophy shares similar features to _______.
Scleroderma
When does Progressive Hemifacial Atrophy begin and who is it more prevalent in?
- Begins during the 1st and 2nd decades
- Females > males
Features of Progressive Hemifacial Atrophy
- Atrophy of the skin and subcutaneous structures
- Hypoplasia of the underlying bone
- Pigmented skin in affected area
- “Coup de sabre” (strike of the sword); occurs on the midline of the forehead
- Enophthalmos: due to loss of periorbital fat
- Alopecia
Oral manifestations of Progressive Hemifacial Atrophy
- Mouth and nose are deviated to the affected side
- Unilateral atrophy of the tongue
- Unilateral posterior open bite
- Delayed eruption of teeth
Treatment of Progressive Hemifacial Atrophy
- Atrophy progresses slowly for years and then becomes stable
- Plastic surgery
- Orthodontic therapy
Characterizations of CROUZON SYNDROME (CRANIOFACIAL DYSOSTOSIS)
- Characterized by premature closure of the cranial sutures
- Autosomal dominant trait
- New mutations are due to an increase in paternal age
Cranial malformations of Crouzon Syndrome
Cranial malformations:
- Brachycephaly (short head)
- Scaphocephaly (long and narrow head)
- Trigonocephaly (triangular shaped head)
Ocular proptosis, visual impairment, blindness, headaches, hearing deficits
Oral manifestations of Crouzon Syndrome
- Midface hypoplasia due to an underdeveloped maxilla
- Crowded maxillary teeth
- Occlusal abnormalities
Treatment of Crouzon Syndrome
- Early craniectomy to relieve increased intracranial pressure
- Frontoorbital advancement
- Midface advancement
Characterizations of MANDIBULOFACIAL DYSOSTOSIS(TREACHER-COLLINS SYNDROME)
- Characterized by defects in structures derived from the 1st and 2nd branchial arches
- Autosomal dominant trait
- 60% of new mutations are due to increased paternal age
Features of Mandibulofacial Dysostosis
-Hypoplastic zygomas
-Narrow face
-Depressed cheeks
-Downward slanting palpebral fissures
-75% have a coloboma (notch in the outer portion of the lower eyelid)
-Missing lower lid lashes
Deformed or misplaced pinnae
-Absence of the external auditory canal: Leads to hearing loss
Oral manifestations of Mandibulofacial Dysostosis
- Hypoplastic mandible
- Retruded chin
- Hypoplastic coronoid and condylar processes
- Cleft palate (1/3 of cases)
- Hypoplastic or missing parotid glands
Treatment of Mandibulofacial Dysostosis
-Mild cases: no treatment
-Severe cases:
Cosmetic surgery, Orthodontic therapy, Orthognathic surgery
Characteristics of CLEIDOCRANIAL DYSPLASIA
- Characterized by defects that primarily involve the skull and clavicles
- May affect other bones
- Autosomal dominant trait
- 40% of cases are spontaneous mutations
Features of Cleidocranial Dysplasia
- Short stature, enlarged skull, frontal and parietal bossing
- Ocular hypertelorism, broad nose base
- Midface hypoplasia leading to a relative prognathism
- Long neck, narrow shoulders with marked drooping
- Mobile shoulders due the absence or hypoplasia of the clavicles
- Clavicles are absent in 10% of the cases
Oral manifestations of Cleidocranial Dysplasia
- Narrow high-arched palate, cleft palate
- Retained primary teeth
- Unerupted secondary teeth
- Supernumerary teeth
Treatment of Cleidocranial Dysplasia
- Extraction of primary and supernumerary teeth
- Exposure and orthodontic extrusion of permanent teeth
- Orthognathic surgery
Characteristics of Double Lip
- Redundant mucosal fold
- Incidence: rare
- Congenital or acquired: trauma, parafunctional habits
- Site: upper lip
- Visible with smiling or tensing of lip
Treatment of Double Lip
- Treatment: cosmetic surgical reduction
- Prognosis: excellent
Association of Double Lip with ________.
Association with Ascher syndrome:
- Double lip
- Blepharochalasis
- Non-toxic thyroid enlargement
- Autosomal dominant inheritance
Characteristics of Fordyce Granules
- Ectopic sebaceous glands
- Incidence: common (80% of population)
- Multiple yellow, white papular lesions
- Site: buccal mucosa, upper vermilion
- Age: adults
- Asymptomatic
Histology and Treatment of Fordyce Granules
- Histology: Submucosal sebaceous lobules
- Treatment: none
- Prognosis: excellent
Characteristics of Leukoedema
- Incidence: common
- Etiology: unknown - edematous mucosa
- Normal anatomic variation
- Race: Blacks (90% - adults, 50% - children)
- Site: buccal mucosa
- Milky opalescent gray white folded mucosa
- Diminishes on stretching, does not rub off
- Smokers - more common/severe
Histology and Treatment of Leukoedema
-Histology: Acanthosis Intracellular edema Hyperparakeratosis -Treatment: none required -Prognosis: excellent
Characteristics of Linea Alba
- Common alteration of the buccal mucosa
- Associated with pressure, frictional irritation, or sucking trauma
- Bilateral white lines at the level of the occlusal plane of the adjacent teeth
Histology and Treatment of Linea Alba
Histopathologic features:
- Hyperorthokeratosis
- Intracellular edema
Treatment and prognosis:
- None
- Spontaneous regression may occur
Characteristics of Macroglossia
- Enlarged tongue
- Incidence: common
- Wide variety of causes: Hereditary/congenital, acquired
- Common in children
- Crenated lateral border
Complications of Macroglossia
- Open bite and malocclusion
- Airway obstruction
- Speech difficulties
- Drooling
- Feeding problems
- Ulceration and infection
Heredity/congenital associations of Macroglossia
- Vascular malformations
- Down syndrome
- Neurofibromatosis
- Multiple endocrine neoplasia syndrome
- Cretinism
- Mucopolysaccharidoses
- Beckwith-Wiedemann syndrome**
- Facial hemihypertrophy
Features of Beckwith-Wiedemann Syndrom (associated with Macroglossia)
- Omphalocele
- Visceromegaly
- Gigantism
- Neonatal hypoglycemia
- Visceral malignancies
- Nevus flammeus
- Maxillary hypoplasia
Acquired associations of Macroglossia
- Edentulous
- Amyloidosis
- Myxedema
- Acromegaly
- Angioedema
- Neoplasms: carcinoma
Histopathology and Treatment of Macroglossia
Histopathology: - Related to specific cause Treatment: - Reduction glossectomy Prognosis: - Good - Related to specific cause
Characteristics of Ankyloglossia
- Short thick lingual frenum
- Limitation of tongue movement
- Incidence: common
- Range of severity
- Complications: open bite, speech and swallowing problems
Treatment of Ankyloglossia
- Treatment: frenectomy (defer until after age 4-5)
- Prognosis: good
Characteristics of Lingual Thyroid
- Ectopic thyroid tissue
- Incidence: uncommon
- Failure of thyroid migration
- Tongue base mass
- Gender: female > male (4:1)
- Becomes evident at puberty or during pregnancy and menopause
- 70% - represents only thyroid tissue
Symptoms and Diagnosis of Lingual Thyroid
Symptoms: - Mass sensation - Dysphagia, dysphonia, and dyspnea
Hypothyroidism (15-33%)
Diagnosis: - Thyroid scan (iodine isotopes, technetium) - Biopsy with caution (highly vascular)
Treatment of Lingual Thyroid
Treatment: - Suppression (thyroid hormone therapy) - Ablation (radioactive iodine) - Surgical resection
Prognosis: good
Malignancy - Rare (1% incidence)
Characterizations of Fissured Tongue
- Grooves or fissures on dorsal tongue
- Incidence: common; 2-5% of population
- Local factors, aging
- Gender: male > female
- Increasing prevalence and severity with age
- Asymptomatic; mild burning or soreness
Treatment of Fissured Tongue
Treatment:
- No specific therapy
- Hygiene measures: brushing
Prognosis: good - benign condition
Associations of Fissured Tongue
Geographic tongue Melkersson-Rosenthal syndrome - Orofacial granulomatosis - Facial paralysis - Fissured tongue Down syndrome
Characterizations of Hairy Tongue
Dorsal tongue surface Incidence: 0.5% of adult population Associated conditions: - Smoking - Antibiotic therapy - Debilitation, poor oral hygiene - Oxidizing mouthrinses - Antacids
Features and Symptoms of Hairy Tongue
-Elongated filiform papillae and/or hyperparakeratosis
-Microbial colonization
-Pigmentation/staining (microbial/exogenous)
-Symptoms:
Gagging
Dysgeusia
Halitosis
Esthetic concerns
Treatment of Hairy Tongue
Treatment:
- Eliminate predisposing factors
- Oral hygiene measures: Brushing/scraping tongue
Prognosis: good, benign condition
Characterizations of Varicosities
- Dilated tortuous veins
- Etiology: aging
- No association with hypertension or cardiovascular disease
- Asymptomatic
- Sublingual: 2/3 adults > 60 years of age
- Solitary: lips, buccal mucosa
Histology and Treatment of Varicosities
- Histology: dilated thin walled vein
- Thrombosis: leads to phlebolith
- Treatment: None required or Surgical removal
- Prognosis: good, benign condition
