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craniofacial/peds Flashcards

1
Q

First (mandibular) arch

A

Think MMATT: Muscles of mastication, Mylohyoid, Anterior digastric, Tensor tympanic, Tensor veli palitini

a. Nerve: trigeminal nerve (ophthalmic, maxillary, mandibular branches)
b. Muscles: Muscles of mastication (temporalis, masseter, medial and lateral pterygoids), mylohyoid, anterior digastric, tensor tympanic, tensor veli palatini
c. Cartilage: premaxilla, maxilla, zygomatic, part of temporal, incus, malleus, anterior malleolar ligament, sphenomandibular ligament
d. Pharyngeal pouch and groove: tubotympanic recess (tympatic cavity, mastoid antrum, pharyngotympanic tube, internal acoustic meatus, tympanic membrane, adenoids)
e. Vascular: maxillary and external carotid arteries

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2
Q

Second (hyoid) arch responsible for 95% branchial anomalies

A

Think PS: Posterior digastric, Platysma, Stylohoid, Stapedius

a. Nerve: facial
b. Muscles: facial expression, buccinators, stapedius, stylohyoid, posterior digastric, auricular, platysma
c. Cartilage: Reicherts cartilage (stapes, styloid process, stylohyoid ligament, lesser cornue and upper part of body of hyoid)
d. Pharyngeal pouch and groove: palatine tonsil and cervical sinus
e. Vascular: stapedial and hyoid arteries

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3
Q

Third arch

A

a. Nerve: Glossopharyngeal
b. Muscles: stylopharyngeus
c. Cartilage: greater cornu and lower body of hyoid
d. Pharyngeal pouch and groove: inferior parathroids, thymus, cervical sinus
e. Vascular: internal carotid and common carotid arteries

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4
Q

Fourth arch

A

a. Nerve: vagus (superior laryngeal, inferior laryngeal)
b. Muscle: cricothyroid, intrinsic muscles of soft palate (levator veli palatini)
c. Cartilage: thyroid, epiglottic
d. Pharyngeal pouch and groove: superior parathyroids, thyroid parafollicular cells
e. Vascular: subclavian artery, aortic arch

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5
Q

Sixth arch

A

a. Nerve: recurrent laryngeal nerve from vagus
b. Muscles: intrinsic muscles of larynx such as thyroarytenoid (except cricothyroid-4th arch)
c. Cartilage: cricoid, arytenoid, corniculate, cuneiform
d. Vascular: right and left pulmonary arteries and ductus arteriosus

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6
Q

Branchial clefts

A
  1. Becomes external auditory canal
  2. Failure of cervical sinus from cleft 2 to disappear results in branchial cleft cyst, sinus tract or fistula on anterior boarder of the SCM. Runs over the glossopharyngeal nerve, between the external carotid artery and internal carotid artery
  3. Note that thyroglossal duct cysts are midline and result from failure of obliteration of the foramen cecum at the base of the tongue.
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7
Q

Branchial pouches:

A
  1. Internal auditory canal
  2. Palatine tonsil
  3. Inferior parathyroid and thymus
  4. Superior parathyroid
  5. Thyroid C-cells
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8
Q

Germ layers

A

A. Ectoderm: skin (epidermis and appendages), mammary glands, CNS/PNS and neural crest cells
B. Mesoderm: bone, cartilage, muscle, connective tissue and dermis, heart, blood vessels, blood, reproductive organs, GU system
C. GI and respiratory tracts, digestive organs

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9
Q

FGFR gene syndromes

A

a. Pfeiffer syndrome: brachycephaly, mild syndactyly, broad toes and thumbs, normal mental status, chiari, hypertelorism, down slanting palpebral fissures
b. Apert syndrome: brachycephaly, syndactyly and cognitive delay, autosomal dominant, acne, enlarged fontanel
c. kallman syndrome: hypogonadotrophic hypogonadism with anosmia, cleft lip/palate, renal aplasia/agenesis, dental defects
d. Crouzon syndrome: autosomal dominant, shallow orbits (exposure keratitis), craniosynostosis, maxillary hypoplasia, chiari
e. Antley-Bixter: can be associated with FGFR. Craniosynostosis, choanal atresia, radiohumeral synostosis
f. Muenke: coronal synostosis, sensorineural hearing loss, abnormal middle phalanges

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10
Q

van der Woude syndrome

A 
lower lip pits and cleft lip/palate,
IRF6 mutation (also mutation for Popliteal pterygium syndrome: webbing behind knee, lower lip pits, cleft lip/palate)
 autosomal dominant
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11
Q

Saethre-Chotzen syndrome

A

TWIST autosomal dominant, normal mental status, asymmetric brachycephaly, eyelid ptosis, narrow palate, low hairline, prominent helical crus

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12
Q

Carpenters syndrome

A

autosomal recessive, mental impairment, brachychephaly, ventricular septal/atrial septal defects, possible venous and bony abnormalities, low set ears.

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13
Q

Gorlin syndrome/basal cell nevus syndrome

A 
AD: PTCH1 on chromosome 9q22.3-q31
basal cell carcinomas
odontogenic keratocysts
palmar and plantar pits
calcification of falx cerebri
bifid ribs
hypertelorism
broad nasal root
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14
Q

22q11.2 deletion/DiGeorge/velocardiofacial

A

CATCH (cardiac anomalies, abnormal facies, thymic aplasia, cleft palate, hypocalcemia/hypoparathyroidism)

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15
Q

Trisomy 13/Patau syndrome

A

cleft palate, CNS disorders, microcephaly, polydactyly, rocker bottom feet, urogenital defects, cardiac anomalies

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16
Q

branchio-oto-renal syndrome

A

EYA1 and SIX1 autosomal dominant. Auricular malformations, preauricular skin pits, hearing loss, branchial fistulae, external auditory canal stenosis, renal anomalies

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17
Q

VACTERL

A

vertebral defects, anal atresia, cardiac defects, trachea-esophageal fistula, renal anomalies, limb anomalies.

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18
Q

CHARGE

A

coloboma of the eye, heart defects, atresia of the nasal choanae, retardation of growth/development, genital/urinary abnormalities, ear abnormalities/deafness, also cleft lip/palate in up to 20% of patients. Mutation in CHD7 (chromodomain helicase DNA-binding protein 7)

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19
Q

Beckwith-Wiedemann syndrome

A

macrostomia, omphalocele, macroglossia, auricular abnormalities (ear folds). chromosome 11 mutation

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20
Q

Neurofibromatosis 1

A

Autosomal dominant. café-au-lait spots, axillary freckling, skeletal dysplasias (absence of greater wing sphenoid), nervous system tumors (neurofibromas), tumor on optic nerve, high blood pressure, scoliosis, optic nerve tumors, Lisch nodules, learning disabilities, ADHD, Macrocephaly, short stature. Three types of neurofibromas (localized, plexiform, diffuse) pathologically spindle cells and mast cells in collagenous myxoid stroma.

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21
Q

Neurofibromatosis 2

A

central nervous system tumors. Grow in prepubescent ages.

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22
Q

Osler-Weber-Rendu/Hereditary hemorrhagic telangiectasia

A

autosomal dominant ENG mutation. malformed ecstatic vessels in skin, mucosae of naso and oropharynx, pulmonary, GI/hepatic and CNS -> bleeding, anemia, stroke
appear later in life

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23
Q

Bannayan-Riley-Ruvalcava syndrome

A

PTEN autosomal dominant, macrocephaly, genital lentiginosis (speckled penis), GI polyps

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24
Q

Stickler syndrome

A 
COL2A1 autosomal dominant
mutation in gene for type II collagen
ocular problems (retinal detachement, myopia, blindness)
facial abnormalities (flat nose, small mandible, cleft palate)
hearing loss
degenerative joint disease
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25
CLOVES syndrome
(congenital lipomatous overgrowth, vascular malformations, epidermal nevi/skeletal anomalies/scoliosis syndrome). PIK3CA mutation: overgrowth syndrome with vascular malformations. Get MRI to rule out CNS AVM.
26
Goldenhar syndrome
``` sporatic hemifacial microsomia epibulbar dermoids vertebral anomalies anterior accessory auricular appendages low hairline ```
27
Nager syndrome
autosomal recessive, hypoplasia of orbits, zygoma, maxilla, mandible, soft palate, auricular defects, hypoplasia of radius, thumbs and metacarpals, radioulnar synostosis, elbow joint deformities.
28
Treacher Collins
hypoplasia of zygoma, maxilla, mandible, downward slanting palpebral fissures, colobomas, absence of eyelashes, auricular defects.
29
Fibrous dysplasia
overgrowth of bones due to abnormal proliferation of bone-forming mesenchyme
30
McCune-Albright
fibrous dysplasia, precocious puberty, café-au-lait spots
31
Klippel-Feil syndrome
congenital fusion of any two cervical vertebrae. Short neck, low occipital hairline, restricted mobility of upper spine.
32
Paget disease
enlarged, deformed bone. Diagnosed 3-4th decade with blood test for alkaline phosphatase.
33
Kabuki syndorme
long palpebral fissures, everted lower lids, broad nasal tip, arched brows, protruding ears, intellectual disability, microcephaly
34
Periapical cysts
most common, usually for from necrotic pulp after tooth infection. Radiologic lucency at the apex of the tooth with sclerotic rim. Painless. Fibrous shell lined with nonkeratinizing stratified squamous epithelium infiltrated with chronic inflammatory cells.
35
Dentigerous cyst
second most common, develop in dental follicle of unerrupted tooth. Lucency attached at an acute angle to the tooth. Mandibular and maxillary third molars most commonly affected.
36
Keratocystic odontogenic tumors
epithelial lined, associated with Gorlin syndrome, enucleate and chemoablation with Carnoy’s solution (alcohol, chloroform, glacial acetic acid, ferric chloride)
37
Gingival cyst
superficial cyst in the gingiva, develops instead of a tooth, appear on alveolar ridge of infants. Contain keratin. Very rare
38
Ameleoblastoma
palisading basaloid cells with large nuclei, 4th-5th decade, arise odontogenic epithelium
39
Cherubism
AD. mandible and maxilla replaced with fibrous tissue and cysts
40
Timing of maxillary permanent tooth eruption
1st molar > central incisor > lateral incisor > 1st premolar > 2nd premolar > canine > 2nd molar > 3rd molar
41
Pleomorphic adenoma
most common benign parotid tumor. epithelial and connective tissue elements with stellate and spindle cells interspersed with a myxoid background
42
Warthin tumor
second most common benign parotid tumor. males 50-70 years old, smokers 10% bilateral epithelial and lymphatic components and cystic spaces with mucoid fluid
43
Mucoepidermoid carcinoma
most common malignant parotid tumor contain mucus-secreting cells (more in lower grade tumors) and epithelial cells (in nests). Treat low grade with superficial parotidectomy but high grade need total parotidectomy, LND and radiation.
44
Adenoid cystic carcinoma
second most common malignant tumor in parotid and most common in minor salivary glands perineural invasion. Three subtypes: Cribiform has swiss cheese appearance; tubular has glandular architecture; solid/basaloid has sheets of cells
45
risk factors for hand and neck cancer
a. Epstein-Barr virus: nasopharyngeal cancer endemic in Africa and East Asia. Usually treated with chemotherapy and radiation. b. Alcohol and tobacco: laryngeal cancer c. Human papillomavirus: oropharyngeal cancer (tongue, tonsil) d. Cigarette smoking and wood dust: maxillary sinus cancer
46
Nasopharyngeal angiofibroma
benign locally invasive vascular tumor in male adolescents. Present with nasal obstruction and possibly eye displacement and diplopia.
47
branches of the external carotid arterh
``` superior thyroid and superior laryngeal branch ascending pharyngeal artery lingual artery facial artery occipital artery and sternocleidomastoid branch and descending branch posterior auricular artery maxillary artery transverse facial artery superficial temporal artery ```
48
neck dissection levels
Level IA (submittal) and IB (submandibular) separated by anterior digastric muscle. Lips, gums, teeth, tongue and anterior hard palate drain. Group II (upper jugular group). noso/oro/hypopharynx and parotid drain. Group III (middle jugular group). superior boarder: hyoid bone/carotid bifurcation inferior boarder: cricothyroid notch/omohyoid muscle naso/oro/hypopharyx and larynx drain Group IV (lower jugular group) larynx, cervical esophagus, hypo pharynx drain Group V (posterior triangle) naso/oropharynx drain
49
Superior orbital fissure syndrome
1. Pupillary dilation via alteration in cranial nerve III function in it's innervation of the pupillary constrictors. 2. Ptosis - Cranial nerve III involvement causes paresis of the levator palpebrae superiorus muscle 3. Pain 4. Proptosis from engorgement of the ophthalmic vein and lymphatics. 5. Extraocular Paresis of cranial nerves III, IV, and VI causing ophthalmoplegia. 6. Parasthesia injury to the first division of cranial nerve V with hypesthesia of the supraorbital and supratrochlear nerves 7. Loss of the corneal Reflex.
50
orbital apex syndrome
superior orbital fissure syndrome plus optic nerve involvement, leading to changes in visual acuity.
51
Skull base foramina
1. Jugular-glossopharyngeal (IX), vagus (X), spinal accessory (XI) nerves 2. Lacerum-internal carotid artery 3. Ovale-mandibular V3 nerve 4. Rotundum-maxillary V2 nerve 5. Stylomastoid-facial nerve
52
Ear embryology
Branchial arch I: three anterior hillocks (tragus, root of the helix, superior helix) Branchial cleft I: external auditory canal Branchial arch II: three posterior hillocks (antihelix, antitragus, lobule)
53
blood supply to ear
posterior auricular and superficial temporal arteries
54
sensation to ear
``` auriculotemporal (V): tragus great auricular (C2,C3): majority of pinna lesser occipital (C2,C3): superior pinna vagus (CNX): external acoustic meatus and concha ```
55
normal ear measurements
Normal helical rim-to-head measurements for each third of the ear are 10 to 12 mm at the helical apex, 16 to 18 mm at the midpoint, and 20 to 22 mm at the lobule.
56
Facial nerve anatomy
1. Muscles innervated from superficial surface: mentalis, levator anguli oris, buccinators 2. Facial nerve trunk just inferior and medial to tragal pointer 3. Frontal branch 0.5cm below tragus to 1.5cm above lateral brow 4. Innervates lacrymation pump; salivation (parasympathetic) and taste to anterior 2/3 tongue
57
Tear film
1. Inner layer mucin secreting goblet cells in conjunctiva promotes dispersion 2. Middle aqueous layer secreted by lacrimal glands promotes osmotic regulation and antimicrobial 3. Outer lipid layer secreted by Meibomian glands prevent evaporation
58
Maffucci syndrome
venous malformations and multiple enchondromas. Associated with malignant chondrosarcomas and intracranial tumors in 20%
59
Kasabach-Merritt
Hemangioma or diffuse hemangiomatosis. Profound thrombocytomenia. Present with petechiae, ecchymosis and bleeding.
60
Sturge-Weber syndrome
facial capillary malformations in trigeminal nerve pattern, vascular malformations on ipsilateral side of leptomeninges and choroid plexus, seizures. May have hypertrophy of cheek, lip, maxilla or mandible.
61
Klippel-Trenaunay syndrome
patchy port-wine stains of lower extremities with lymphatic/venous malformations and hypertrophy. Embryonal lateral vein of Servelle in lower extremity.
62
Parks-Weber syndrome
Klippel-Trenaunay with AVF
63
Cobb syndrome
capillary malformation in midline scalp overlying an encephalocele or posterior to dysraphism in cervical or lumbosacral spine.
64
Cutler beard flap
anterior and posterior lamella of lower lid used to reconstruct up to 100% upper lid. Will need cartilage graft for support. Two stage procedure.
65
Tenzel semicircular advancement flap
laterally based myocutaneous flap can close up to 60% lateral upper or lower but still need posterior lamella. Requires lateral canthotomy. Similar to Mustarde cheek rotation flap can address anterior lamella of 100% lower lid but the flap is thicker than eyelid skin and risk of malposition.
66
Temporal forehead (Fricke) flap
repair anterior lamella defects of up to 100% eyelid. Skin thicker than eyelid skin.
67
Tripier flap
myocutaneous for anterior lamella defects of lower lid. Can be uni or bipedicled
68
Hughs tarsoconjuctival flap
Up to 100% posterior lamella reconstruction of lower lid (contains tarsus and conjunctiva from upper lid). Two stage procedure. Require skin graft or local flap for anterior lamella.
69
margins for skin cancer
BCC: 4mm low risk, 10mm high risk (depth >2mm, >20mm trunk/ext, morpheaform SCC: 4-6mm
70
TNM staging melanoma
``` Tis insitu -> 0.5cm margin T1 <1.0 mm -> 1 cm margin T2 1.01-2.0 mm -> 2cm margin T3 2.01-4.0 mm -> 2cm margin T4 >4 mm -> 2cm margin N1 one node N2 2-3 nodes N3 >4 nodes M1a mets to skin, subcutaneous, distant M1b mets to lung M1c Mets other viscera, elevated LDH ```
71
propranolol
nonselective beta blocker blocks noradrenaline increase of VEGF production effect on renin-angiotensin: angiotensi-converting enzyme expressed on immature capillaries proliferating side effects: bradycardia, hypotension, hypoglycemia, diarrhea, somnolence
72
von Hippel-Lindau
hemangiomas of the retina hemangioblastomas of the cerebellum cysts of pancreas, liver, adrenal glands and kidneys seizures and mental retardation
73
PHACE syndrome
``` Posterior fossa malformations Hemangiomas (on face) Arterial anomalies Coarctation of aorta Eye abnormalities ```
74
Epithelioid hemangioma
rare tumor of adulthood with borderline malignant potential
75
Bannayan-Zonana syndrome
microcephaly, mulitple lipomas, multiple vascular malformations
76
Riley-Smith syndrome
pseudopapilledema, microcephaly, vascular malformations
77
tongue
1. anterior 2/3: originate pharyngeal arch I and innervated by lingual nerve (CN V3) 2. posterior 2/3: originate from arches III and IV and innervated by CN IX and X 3. muscles from occipital myotomes (CN XII) except palatoglossus (CN X)
78
Triptans
abortive therapy for migranes Tryptamine-based. act on serotonin receptors in cranial blodd vessels and nerve endings inhibit release of CGRP and substance P
79
Normal fusion of sutures
metopic: 6-8 months saggital: 22 years coronal: 24 years lambdoidal: 26 years
80
cleft lip repair flaps
C (columellar) flap: close nasal sill or lengthen columella M (mucosa/medial) flap: mucosa off medial lip element used to line gingivobuccal sulcus L (lateral) flap: superiorly based mucosal flap lateral lip element used to line lateral nasal vestibule
81
blood supply and innervation palate
hard palate: 1. greater palatine artery and nerve pass through greater palatine foramen (dominant supply) 2. nasopalatine artery and nerve communicate with the greater palatine at the incisive foramen to supply the premaxilla 3. anterior and posterior alveolar arteries supply the anterior and posterior alveoli soft palate: 1. ascending pharyngeal and ascending palatine arteries are principle blood supply 2. lesser palatine artery and nerve pass through the lesser palatine foramen 3. all muscles of velum innervated by pharyngeal plexus (CN IX, X, XI) except tensor veli palitini which is innervated by CN V
82
temporal bone trauma
Battle's sign is bruising over the mastoid process 1. longitudinal fractures 80-90% - fascial nerve injury 20% - hearing loss 67% 2. transverse fractures - facial nerve injury 40% - hearing loss 100%
83
modified radical neck dissection
type I: spares CN XI type II: spares internal jugular vein and CN XI type III: spares SCM, internal jugular vein and CN XI
84
supraorbital nerve anatomy
superficial division: forehead and anterior hairline | deep division: frontoparietal scalp
85
orbicularis oculi anatomy
1. pretarsal fibers: over tarsal plate. Involuntary blink, lower lid tone, lacrimal pump 2. preseptal fibers: over septum, assist with blink 3. orbital fibers: over orbital rims, forceful closure, animated eyelid movements.
86
Whitnalls ligament
superior to the levator. Fulcrum to redirect vector of pull from horizontal to superior direction for lid retraction.
87
capsulopalpebral fasica
lower lid retractor

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