Head and Neck Developmental Disorders: Flashcards
One of the most common major congenital defects:
Orofacial clefts
results from disturbances in growth of face and oral cavity:
orofacial clefts
Defective fusion with the medial nasal process & maxillary process:
Cleft Lip
Cleft Lip occurs due to defective fusion of:
Medial nasal process and 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: 25%
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
- anti-convulsent therapy (phenytoin): 10x risk!!
What supplement may be prevention to orofacial clefts?
Folic acid
orofacial cleft that extends up into the nostril:
Complete CL
Orofacial cleft that does not involve the nose:
Incomplete CL
Orofacial cleft that may involve hard and soft palate:
CP
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 include:
- multidisciplinary approach
- surgical treatment
- prosthetic appliances
Congenital invaginations of the lower lip:
Paramedian lip pits
Paramedian lip pits inheritance pattern:
autosomal dominant
Clinical features:
- bilateral and symmetrical fistulas on either side of midline of lower lip
- subtle depression or prominent bulge
- Van der Woude syndrome
Paramedian lip pits
Van De Woude Syndrome seen in Paramedian lip pits is characterized by:
lip pits with cleft lip and/or cleft palate
Most common form of syndromic cleftng:
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 papules
- 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 an ____ cause
common; unknown
Clinical features:
- diffuse gray-white color
- folded, wrinkled mucosa
- bilateral 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 and 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, hemihyerplasia, and Down syndrome:
Congenital macroglossia
Form of macroglossia characterized by edentulous patients, amyloidosis, and myxedema:
Acquired macroglossia
What form of macroglossia is most common in Down syndrome individuals?
Congenital
AKA: tongue tie
Ankyglossia
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 decent into:
Neck anterior to trachea
When the primitive thyroid gland does not descend normally, and ectopic thyroid tissue is found between foramen cecum and 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) (large may = airway blockage, dysphagia, and dyspnea)
- Hypothyroidism (33%)
Lingual thyroid
Small amounts of ectopic thyroid tissue (lingual thyroid) may be ____.
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 lingual thyroid?
excisionial 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
- 2-6 mm in depth
- usually asymptomatic
- strong association with geographic tongue
Fissured tongue
Treatment for fissured tongue includes:
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 include: (5)
- smoking
- poor oral hygiene
- general debilitation
- radiation therapy
- medications
Clinical features:
- dorsal tongue
- elongated papillae (usually brown, yellow, black)
- patients may complain of bad taste
Hairy tongue
Treatment for hairy tongue:
- eliminate predisposing factors (i.e. tobacco)
- oral hygiene
Abnormally dilated tortuous veins:
varicosities
Varicosities can be described as a ___ process
Age-related degenerative
Clinical features:
- sublingual varicosities (2/3 of ppl above age 60)
- Blue-purple, elevated papular blebs (ventral + lateral tongue)
- may also occur on the lips and buccal mucosa
- usually asymptomatic
Varicosites
Treatment of varicosities:
sublingual varicosities: none
Solitary varicosities (other locations) may need excision to confirm diagnosis
Localized bony protuberances arising from cortical plate:
Exostosis
Exostosis may be related to:
stresses from teeth
Clinical features
- observed in adults
- occur in the buccal and palatal regions
- may be tooth-stress related
exostosis
Treatment of exostosis:
- distinctive clinically decide whether it needs treatment or not
If a patient with a denture has exostosis what needs to occur?
surgical removal
Bony hard nodule of midline suture of hard palate:
torus palatinus
What sex and population is torus palatines most common in?
Females (2:1)
Asian + Inuit
Treatment of torus palitinus:
Deciding clinically where surgical excision is necessary
Clinical/ radiographie features:
- body hard nodule on lingual aspect of mandible
- 90% bilateral involvement
- most common in premolar region
- most common in asian and Inuit people
- RG: bony nodule superimposed on teeth
Torus mandibularis
Torus mandibularus is most common in what location of the oral cavity? Most in what population?
premolar region- asians and inuit people
How does torus mandibularis appear radiographically?
bony nodule superimposed on teeth
Developmental defect in mandibular alveolar bone:
stafne defect
The developmental defect seen in stafne defect may contain:
normal salivary gland tissue
Stafne affect most commonly occurs in:
males (80-90%)
Radiographic features:
- radiolucency below the mandibular canal
- lingual corticol defect
- occasionally may occur anteriorly
- well circumscribed with sclerotic border
- usually remains static over time
- asymptomatic
Stafne defect
Treatment for stafne defect includes:
none
Small developmental cysts on palate of newborn
Palatal cyst of the newborn
Palatal cyst of the newborn may result from:
trapped epithelium during palatal fusion in embryogenesis
Clinical feature:
- common (may occur in 55-85% of newborns)
- most along the midline of hard and soft palate
- appears as small white or yellow-white papule on palate
- 1-3 mm keratin filled cysts
Palatal cysts of the newborn
Treatment of palatal cyst of the newborn:
None
Most common developmental non-odontogenic cyst:
Nasopalatine duct cyst
The nasopalatine duct cysts arises from:
remnants of nasopalatine duct
Nasopalatine duct cysts are most common in the:
4th-6th decade of life (esp. in males)
Clinical features:
- may exhibit swelling of anterior palate
- occasionally painful
- drainage
- 1-2.5cm in diameter
Nasopalatine duct cyst
Radiographic features:
- well-circumscribed
- radiolucency near midline of anterior maxillary apical to central incisors
- may be difficult to distinguish small cyst from large incisive foramen
Nasopalatine duct cyst
What may be difficult to distinguish from large incisive foramen?
Nasopalatine duct cyst
Treatment of nasopalatine duct cyst:
surgical enucleation
Common cyst of the skin that arises from the hair follicle:
epidermoid cyst of the skin
Account for 80% of follicular cyst of the skin:
Epidermoid cyst of the skin
clinical features:
- common in acne prone regions
- common areas: scalp, face, back
- Nodular fluctuant subcutaneous swelling
- Unusual before puberty unless associated with Gardner syndrome
Epidermoid cyst of the skin
Epidermoid cyst of the skin is unusual before puberty unless:
associated with Gardner syndrome
Treatment of Epidermoid cyst of the skin:
Conservative enucleation
Developmental cystic malformation:
dermoid cyst
Lined by epidermis with dermal adnexal structure within cyst wall:
Dermoid cyst
Benign cystic form of a teratoma:
Dermoid cyst
Developmental tumor composed of more than one germ layer (ectoderm, mesoderm, endoderm)
Teratoma
Teratomas are most common in the:
Ovaries and testes
Head and neck dermoid cysts are most common in ___ @ ____
children and young adults; midline FOM
Describe a dermoid cyst in children and young adults:
soft bony mass - may produce submental swelling
Treatment of dermoid cyst:
surgical excision
Arises from thyroglossal duct remnants that normally undergo atrophy:
Thyroglossal duct cyst
Clinical features:
- develop from foramen cecum to suprasternal notch
- 60-80% develop adjacent to hyoid bone
- 50% of these are diagnosed by age 20
- fluctuant, moveable swelling
- usually asymptomatic
thyroglossal duct cyst
Treatment for a thyroglossal duct cyst:
surgical excision
Developmental cyst that develops from branchial arch remnants:
Branchial cleft cyst
Clinical features:
- located in the upper lateral neck anterior or deep SCM
- Develop in young children and adults
- soft, fluctuant mass, 1-10 cm in diameter
Branchial cleft cyst
Treatment of branchial cleft cyst:
Surgical excision
Lesion that develops from oral lymphoid tissue:
Lymphoepithelial cyst
Clinical features:
- small submucosal mass (usually less than 1cm)
- common on posterior lateral tongue, and anterior tonsillar pillar
- firm or soft to palpation
- often white or yellow: keratin in lumen
- Usually asymptomatic
Lymphoepithelial cyst
Treatment of lymphoepithelial cyst:
- Surgical excision should NOT occur
- If distinctive a biopsy is NOT necessary
Cleft palate
Cleft lip + Cleft Palate
Bifid Uvula
paramedian lip pits
fordyce granules
Leukoedema
Leukoedema
Microglossia
Macroglossia
macroglossia
Ankyloglossia (Tongue tie)
Ankyloglossia (Tongue tie)
lingual thyroid
fissured tongue
Hairy tongue
Hairy tongue
What papillae are involved in hairy tongue:
filiform
varicosities
varicosities
exostoses
torus mandibularis
torus mandibularis
stafne defect
palatal cyst of newborn
nasopalatine duct cyst
nasopalatine duct cyst
epidermoid cyst of the skin
dermoid cyst
dermoid cyst
dermoid cyst
branchial duct cyst
lymphoepithelial cyst
lymphoepithelial cyst
lymphoepithelial cyst