Head & Neck Developmental disorders Flashcards
One of the most common major congenital defects:
Orofacial clefts
Results from disturbances in growth of face & oral cavity:
Orfacial clefts
Defective fusion with the medial nasal process and maxillary process:
Cleft lip
Cleft lip occurs due to defect fusion of:
Medial nasal process & maxillary process
Failure of the palatal shelves to fuse:
Cleft palate
A cleft palate occurs due to failure of fusion of the:
palatal shelves
- cleft lip + cleft palate:
- cleft palate only:
- cleft lip only:
- cleft lip + cleft palate: 45%
- cleft palate only: 30%
- cleft lip only: 35%
Orofacial clefts are most common in what population?
Native Americans and asians
Causes and risk factors for orofacial clefts include:
- genetic factors (syndromic)
- environmental factors
What environmental factors are associated with orofacial cleft risk?
- maternal alcohol & tobacco
- anticonvulsant therapy (Phenytoin: 10x risk!)
What supplement may be a prevention to orofacial clefts?
Folic acid
Orofacial cleft that extends upward into the nostril:
Complete CL
Orofacial cleft that does not involves the nose:
incomplete CL
Orofacial cleft that may involve hard & soft palate:
Cleft palate
A minimal manifestation of orofacial clefts:
Bifid uvula
Orofacial clefts may interfere with teeth development such as:
- hypodontia
- malformed teeth
- bony defects
- malocclusion
Treatment of orofacial clefts includes:
- multidisciplinary approach
- surgical treatment
- prosthetic appliances
Congenital invaginations of the lower lip:
Paramedian lip pits
Paramedian lip pits inheritance pattern:
autosomal dominant
Clinical features:
-B/L & symmetrical fistulas on either side of the midline of lower lip
-subtle depression or prominent bulge
-Van der Woude syndrome
Paramedian lip pits
Van der Woude syndrome (seen in Paramedian lip pits) is characterized by:
lip pits with cleft lip & or cleft palate
Most common form of syndromic clefting:
Van der Woude syndrome
Treatment of Paramedian lip pits includes:
None (except for cosmetic reasons)
Ectopic sebaceous glands in oral mucosa:
Fordyce granules
Clinical features:
-multiple yellow or yellow-white papulate
-common on the buccal mucosa, vermillion of upper lip, retromolar pad, tonsillar area
Fordyce granules
Treatment of Fordyce granules:
none
Leukoedema is a ____ condition with a ____ cause
common; unknown
Clinical features:
-diffuse, gray-white color
-folded, wrinkled mucosa
-B/L buccal mucosa
-White appearance that disappears when mucosa is stretched
Leukoedema
Treatment for Leukoedema:
None
T/F: Microglossia is a common developmental condition
False- rare
Clinical features:
-abnormally small tongue
-may be associated with a syndrome
Microglossia
Treatment of microglossia:
- depends on nature & severity
- surgery and orthodontics
Enlargement of tongue:
Macroglossia
T/F: Macroglossia is primarily associated with one condition
False- may be caused by a variety of conditions
Form of macroglossia characterized by vascular malformations, lymphangioma, hemihyperplasia, & down syndrome:
Congenital macroglossia
Form of macroglossia characterized by edentulous patients, amyloidosis, & myxedema:
Acquired macroglossia
What form of macroglossia is most common in patients with Down syndrome:
Congenital
AKA tongue-tie:
Ankyloglossia
Developmental anomaly of the tongue:
Ankyloglossia
Clinical features:
-short, thick frenum
-may result in speech defects
-breastfeeding difficulties
Ankyloglossia (tongue-tie)
Treatment for Ankyloglossia (tongue-tie):
Frenectomy for functional problems
Typically the thyroid bud descends into:
neck- anterior to trachea
When the primitive thyroid gland does not descend normally & ectopic thyroid tissue is found between foramen cecum & epiglottis:
lingual thyroid
Where is ectopic thyroid tissue found in the case of lingual thyroid:
between foramen cecum & epiglottis
Clinical features:
-most common in females (4x) due to hormonal influences
-ectopic gland (70%) is often only thyroid tissue
-ranges in size: small= asymptomatic but large may = airway blockage, dysphagia, dyspnea
-hypothyroidism (33%)
Lingual thyroid
Small amounts of ectopic thyroid tissue (lingual thyroid) may be ______ however large amounts of ectopic thyroid tissue may ______ causing ____ or ____
asymptomatic; block airway; dysphagia; dyspnea
How would you diagnose a lingual thyroid?
thyroid scan
What would you want to avoid with diagnosis of a lingual thyroid?
excisional biopsy- as this may be the patients only thyroid tissue
Treatment of lingual thyroid:
- periodic follow-up
- thyroid hormone replacement
A fissured tongue is often _____ but may also be a _____
hereditary; degenerative process
Clinical features:
-dorsal surface grooves, furrows
-2-6mm in depth
-usually asymptomatic
-strong association with geographic tongue
Fissured tongue
Treatment for fissured tongue include:
none, encourage good oral hygeine
Marked elongation of filiform papillae due to keratin accumulation:
Hairy tongue
Hairy tongue is due to ____ accumulation
Keratin
Risk factors for hairy tongue include: (5)
- smoking
- poor oral hygiene
- general debilitation
- radiation therapy
- medications
Clinical features:
-dorsal tongue
-elongated papillae (usually brown, yellow, or black)
-patient may complain of bad taste
Hairy tongue
Treatment of hairy tongue:
- eliminate predisposing factors (i.e. tobacco)
- oral hygiene