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Exam 2: DHHD 5002 > Jones 2 > Flashcards

Jones 2 Flashcards

1
Q

What is PROGRESSIVE HEMIFACIAL ATROPHY (ROMBERG or PARRY-ROMBERG SYNDROME)?

A

Degenerative condition associated with atrophic changes on one side of the face

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2
Q

Cause of Progressive Hemifacial Atrophy

A

-Cause is unknown: malfunction of the cervical sympathetic nervous system, trauma, Lyme disease

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3
Q

Progressive Hemifacial Atrophy shares similar features to _______.

A

Scleroderma

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4
Q

When does Progressive Hemifacial Atrophy begin and who is it more prevalent in?

A
  • Begins during the 1st and 2nd decades

- Females > males

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5
Q

Features of Progressive Hemifacial Atrophy

A
  • 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
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6
Q

Oral manifestations of Progressive Hemifacial Atrophy

A
  • Mouth and nose are deviated to the affected side
  • Unilateral atrophy of the tongue
  • Unilateral posterior open bite
  • Delayed eruption of teeth
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7
Q

Treatment of Progressive Hemifacial Atrophy

A
  • Atrophy progresses slowly for years and then becomes stable
  • Plastic surgery
  • Orthodontic therapy
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8
Q

Characterizations of CROUZON SYNDROME (CRANIOFACIAL DYSOSTOSIS)

A
  • Characterized by premature closure of the cranial sutures
  • Autosomal dominant trait
  • New mutations are due to an increase in paternal age
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9
Q

Cranial malformations of Crouzon Syndrome

A

Cranial malformations:

  • Brachycephaly (short head)
  • Scaphocephaly (long and narrow head)
  • Trigonocephaly (triangular shaped head)

Ocular proptosis, visual impairment, blindness, headaches, hearing deficits

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10
Q

Oral manifestations of Crouzon Syndrome

A
  • Midface hypoplasia due to an underdeveloped maxilla
  • Crowded maxillary teeth
  • Occlusal abnormalities
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11
Q

Treatment of Crouzon Syndrome

A
  • Early craniectomy to relieve increased intracranial pressure
  • Frontoorbital advancement
  • Midface advancement
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12
Q

Characterizations of MANDIBULOFACIAL DYSOSTOSIS(TREACHER-COLLINS SYNDROME)

A
  • 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
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13
Q

Features of Mandibulofacial Dysostosis

A

-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

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14
Q

Oral manifestations of Mandibulofacial Dysostosis

A
  • Hypoplastic mandible
  • Retruded chin
  • Hypoplastic coronoid and condylar processes
  • Cleft palate (1/3 of cases)
  • Hypoplastic or missing parotid glands
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15
Q

Treatment of Mandibulofacial Dysostosis

A

-Mild cases: no treatment
-Severe cases:
Cosmetic surgery, Orthodontic therapy, Orthognathic surgery

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16
Q

Characteristics of CLEIDOCRANIAL DYSPLASIA

A
  • Characterized by defects that primarily involve the skull and clavicles
  • May affect other bones
  • Autosomal dominant trait
  • 40% of cases are spontaneous mutations
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17
Q

Features of Cleidocranial Dysplasia

A
  • 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
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18
Q

Oral manifestations of Cleidocranial Dysplasia

A
  • Narrow high-arched palate, cleft palate
  • Retained primary teeth
  • Unerupted secondary teeth
  • Supernumerary teeth
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19
Q

Treatment of Cleidocranial Dysplasia

A
  • Extraction of primary and supernumerary teeth
  • Exposure and orthodontic extrusion of permanent teeth
  • Orthognathic surgery
20
Q

Characteristics of Double Lip

A
  • Redundant mucosal fold
  • Incidence: rare
  • Congenital or acquired: trauma, parafunctional habits
  • Site: upper lip
  • Visible with smiling or tensing of lip
21
Q

Treatment of Double Lip

A
  • Treatment: cosmetic surgical reduction

- Prognosis: excellent

22
Q

Association of Double Lip with ________.

A

Association with Ascher syndrome:

  • Double lip
  • Blepharochalasis
  • Non-toxic thyroid enlargement
  • Autosomal dominant inheritance
23
Q

Characteristics of Fordyce Granules

A
  • Ectopic sebaceous glands
  • Incidence: common (80% of population)
  • Multiple yellow, white papular lesions
  • Site: buccal mucosa, upper vermilion
  • Age: adults
  • Asymptomatic
24
Q

Histology and Treatment of Fordyce Granules

A
  • Histology: Submucosal sebaceous lobules
  • Treatment: none
  • Prognosis: excellent
25
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
26
Histology and Treatment of Leukoedema
``` -Histology: Acanthosis Intracellular edema Hyperparakeratosis -Treatment: none required -Prognosis: excellent ```
27
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
28
Histology and Treatment of Linea Alba
Histopathologic features: - Hyperorthokeratosis - Intracellular edema Treatment and prognosis: - None - Spontaneous regression may occur
29
Characteristics of Macroglossia
- Enlarged tongue - Incidence: common - Wide variety of causes: Hereditary/congenital, acquired - Common in children - Crenated lateral border
30
Complications of Macroglossia
- Open bite and malocclusion - Airway obstruction - Speech difficulties - Drooling - Feeding problems - Ulceration and infection
31
Heredity/congenital associations of Macroglossia
- Vascular malformations - Down syndrome - Neurofibromatosis - Multiple endocrine neoplasia syndrome - Cretinism - Mucopolysaccharidoses - Beckwith-Wiedemann syndrome**** - Facial hemihypertrophy
32
Features of Beckwith-Wiedemann Syndrom (associated with Macroglossia)
- Omphalocele - Visceromegaly - Gigantism - Neonatal hypoglycemia - Visceral malignancies - Nevus flammeus - Maxillary hypoplasia
33
Acquired associations of Macroglossia
- Edentulous - Amyloidosis - Myxedema - Acromegaly - Angioedema - Neoplasms: carcinoma
34
Histopathology and Treatment of Macroglossia
``` Histopathology: - Related to specific cause Treatment: - Reduction glossectomy Prognosis: - Good - Related to specific cause ```
35
Characteristics of Ankyloglossia
- Short thick lingual frenum - Limitation of tongue movement - Incidence: common - Range of severity - Complications: open bite, speech and swallowing problems
36
Treatment of Ankyloglossia
- Treatment: frenectomy (defer until after age 4-5) | - Prognosis: good
37
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
38
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)
39
Treatment of Lingual Thyroid
Treatment: - Suppression (thyroid hormone therapy) - Ablation (radioactive iodine) - Surgical resection Prognosis: good Malignancy - Rare (1% incidence)
40
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
41
Treatment of Fissured Tongue
Treatment: - No specific therapy - Hygiene measures: brushing Prognosis: good - benign condition
42
Associations of Fissured Tongue
``` Geographic tongue Melkersson-Rosenthal syndrome - Orofacial granulomatosis - Facial paralysis - Fissured tongue Down syndrome ```
43
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 ```
44
Features and Symptoms of Hairy Tongue
-Elongated filiform papillae and/or hyperparakeratosis -Microbial colonization -Pigmentation/staining (microbial/exogenous) -Symptoms: Gagging Dysgeusia Halitosis Esthetic concerns
45
Treatment of Hairy Tongue
Treatment: - Eliminate predisposing factors - Oral hygiene measures: Brushing/scraping tongue Prognosis: good, benign condition
46
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
47
Histology and Treatment of Varicosities
- Histology: dilated thin walled vein - Thrombosis: leads to phlebolith - Treatment: None required or Surgical removal - Prognosis: good, benign condition

Exam 2: DHHD 5002 (10 decks)

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