Rubin's 29: Head and Neck (gave up on this one ignore it)

Question 1

Crouzon Syndrome, Apert Syndrome

Answer 1

• autosomal dominant disorders
• associated with craniosynostosis (premature fusion of the cranial sutures)
• can lead to brachycephaly (flat head), scaphocephaly or dolichocephaly (the head is disproportionately long and narrow or “boat” shaped) or trigonocephaly (triangular shaped)
• FGFR2 mutation on chromosome 10

Question 2

Kleeblattschädel deformity

Answer 2

• severe craniosynostosis leads to “cloverleaf” skull

Question 3

FGFR2 gene

Answer 3

encodes a transmembrane protein that, upon binding its ligands, signals to induce bone maturation

Question 4

Fordyce granules

Answer 4

aggregates of sebaceous glands in the oral cavity (choristoma)

Question 5

ectopic thyroid

Answer 5

• Abnormal descent of the thyroid during development may create submucosal foci of ectopic thyroid between the tongue and suprasternal notch
• MC site: The base of the tongue between the foramen cecum and epiglottis–> lingual thyroid
• removal –> hypothyroid, cretinism
• malignancy: papillary thyroid carcinomas.
• may affect parathyroid gland development and localization.

Question 6

Thyroglossal duct cysts

Answer 6

Result from persistence and cystic dilatation of the thyroglossal duct midline in the neck

• occurs above the thyroid isthmus but below the hyoid bone
• under age 40, nodule moves up and down upon swallowing.
• Surgery is the treatment of choice.
• papillary thyroid carcinomas in up to 1% of thyroglossal duct cysts.

Question 7

Branchial cleft cyst

Answer 7

• originate from branchial arch remnants
• in the lateral anterior neck or parotid gland in young adults
• contain thin, watery fluid and mucoid or gelatinous material
• lined by squamous epithelium, with occasional foci of ciliated respiratory or pseudostratified columnar epithelium.

Question 8

Scarlet fever

Answer 8

• caused by several strains of β-hemolytic streptococci (Streptococcus pyogenes)
• Damage to vascular endothelium by the erythrogenic toxin results in a rash on the skin and oral mucosa (strawberry tongue)
• Untreated scarlet fever can lead to glomerulonephritis and heart disease

Question 9

Apthous stomatitis (canker sores)

Answer 9

• painful, recurrent, solitary or multiple, small ulcers of oral mucosa, unknown cause
• shallow ulcer covered by a fibrinopurulent exudate, with underlying mononuclear and polymorphonuclear inflammation
• heal without scarring

Question 10

Acute necrotizing ulcerative gingivitis (Vincent angina)

Answer 10

• caused by infection with two symbiotic organisms, a fusiform bacillus and a spirochete (Borrelia vincentii)
• caused by inadequate nutrition, immunodeficiency or poor oral hygiene
• characterized by punched-out erosions of the interdental papillae. Tends to spread and eventually involves all gingival margins, which become covered by a necrotic pseudomembrane

Question 11

Noma

Answer 11

• severe fusospirochetal infection in people who are malnourished, debilitated from infections or weakened by blood dyscrasias
• rapidly spreading gangrene of oral and facial tissues
• Large masses of tissue slough and leave the bones exposed, especially in children

Question 12

Ludwig angina

Answer 12

• rapidly spreading cellulitis originating in the submaxillary or sublingual space but extending to involve both.
• potentially life-threatening inflammatory process
• uncommon in developed countries except in patients with chronic illnesses associated with immunosuppression.
• related to dental extraction or trauma to the floor of the mouth.

After extraction of a tooth, hairline fractures may occur in the lingual cortex of the mandible, providing microorganisms ready access to the submaxillary space.
- may dissect into the parapharyngeal space along fascial planes and from there into the carotid sheath.
- mycotic internal carotid artery aneurysm may result, erosion of which may cause massive hemorrhage.
- inflammation may also dissect into the superior mediastinum to involve the pleural space and pericardium.


Question 13

diptheria

Answer 13

Corynebacterium diphtheriae
- patchy pseudomembrane, often begins on the tonsils and pharynx but may also involve the soft palate, gingiva or buccal mucosa

Question 14

tuberculosis

Answer 14

• Primary tuberculosis of the oral mucosa is rare.
• lesions spread from the lung, with bacilli carried in sputum and entering small breaks in the mucosa. There, they produce irregular, painful ulcers, mostly on the tongue.
• Caseating granulomatous inflammation

Question 15

syphilis

Answer 15

• Primary syphilitic chancres may form on the lips, tongue or oropharyngeal mucosa after contact with an infectious lesion
• Regional lymphadenitis follows and heals by itself in a few weeks
• diffuse mucocutaneous eruption of the secondary stage follows. Syphilitic lesions in the oral mucosa are multiple gray-white patches overlying ulcerated surfaces. May remit and also recur spontaneously.
• Gummas may appear years after initial infection as firm nodular masses that ulcerate and may cause palatal perforation

Question 16

actinomycosis

Answer 16

• Actinomyces bovis or Actinomyces israelii
• produces chronic granulomatous inflammation and abscesses that drain by fistula formation, with suppurative infection containing characteristic yellow “sulfur granules.” - In cervicofacial actinomycosis, soft tissue infection may extend to adjacent bones, most often to the mandible.

Question 17

Candidiasis

Answer 17

C. albicans

• To cause disease, the fungus must penetrate tissues, albeit superficially
• Oral candidiasis mostly seen in diabetics and people with compromised immune systems.
• Lesions are white, slightly elevated, soft patches that consist mainly of fungal hyphae.

Question 18

HSV1

Answer 18

• Aerosol transmission
• painful inflammation of affected mucosa, followed shortly by formation of vesicles, resulting from “ballooning degeneration” of epithelial cells, some of which show intranuclear inclusions
• vesicles rupture to form shallow, painful, which heal spontaneously without scarring.
• it remains dormant in the trigeminal ganglion until stresses such as trauma, allergy, menstruation, pregnancy, UV light, and other viral infections reactivate it.

Question 19

HPV

Answer 19

causes papillomas

“High-risk” HPV (16, 18, 31, 33, 35) strongly associated with oropharyngeal squamous cell carcinoma