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ENT Secrets > Chapter 50 - Congenital Malformations H/N > Flashcards

Chapter 50 - Congenital Malformations H/N Flashcards

1
Q

Lymphatic malformations: tumors? do they expand?

A

not true tumors - grow with patient

can rapidly expand with infection, bleeding, trauma

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2
Q

branchial cleft: deep to which arch? superficial to which?

A

anomalies track deep to structures of own arch, superficial to structures of next one

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3
Q

most common congenital neck mass

A

thyroglossal duct cyst

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4
Q

how does thyroglossal duct cyst form?

A

3 wk gestation thyroid gland forms from diverticulum at tongue (foramen cecum)
descends to fuse with 4th-5th pouch components
5-8wk: tract formed by descent obliterates, forming foramen cecum proximally and pyramidal lobe thyroid distally
Incomplete obliteration –> TDC

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5
Q

Classic TDC PE sign

A

elevates with tongue protrusion

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6
Q

Critical preop imaging prior to TDC removal

A

U/S to r/o ectopic thyroid

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7
Q

DDx and defining characteristics of midline CONGENITAL neck masses

A

TDC - elevate with swallow
Dermoid - submental, move with skin
Teratoma - firm, CT with calcification, rare, larger/more sx than dermoid
Plunging ranula - midline, cystic, extends to FOM

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8
Q

DDx and defining characteristics of lateral CONGENITAL neck masses

A

branchial cleft cyst - deep to ant border SCM, fluctuant
lymphangioma - soft, compressible, transilluminate
external laryngocele - air filled, protrude through thyrohyoid, hoarse, cough
SCM tumor - firm, painless, discrete, fusiform mass

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9
Q

Recurrence rate of TDC after Sistrunk

A

2.6-5%

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10
Q

Can TDC’s harbor malignancy?

A

Yes - well differentiated thyroid CA

Sistrunk removal offers excellent cure rate

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11
Q

Describe sistrunk procedure objectives

A

remove TDC tract connecting it to the foramen cecum

remove central portion of hyoid

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12
Q

Dermoid vs teratoma

A

Dermoid- ectoderm/mesoderm only, from entrapped epithelium along lines of embryonic fusion, enlarge over time, cheesy contents upon excision, fill with sebaceous material

Teratoma- all 3 germ layers, larger/typically more Sx, dx by prenatal u/s often, 30% associated with polyhydramnios (esophageal obstruction)

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13
Q

Branchial cleft vs arch vs pouch

A

At 4wk geswtation, there are 4 pairs of arches, and two rudimentary ones
External lining ectoderm, internal endoderm, meso between
arches separated by pouches internally and clefts externally
clefts and pouches gradually replaced with mesenchyme
when clefts and pouches persist –> branchial anomaly

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14
Q

3 types of branchial anomaly

A

remember this is when clefts/pouches persist
Cyst- remnant with no openings, squamous lining
Sinus- iexternal opening (often drain to skin), often lined with ciliated columnar
Fistula- internal (aerodigestive tract) and external (skin) opening

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15
Q

Type 1 BA

A 
1% (rare)
between EAC and submandibular area
lateral to VII
duplication of EAC
can terminate in EAC or middle ear
Do not contain mesoderm
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16
Q

Type 2 BA

A 
95%
MEDIAL to VII and has mesoderm
Anterior border SCM
Between ICA and ECA
Terminate in tonsillar fossa (but can terminate in EAC/middle ear)
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17
Q

Type 3/4 BA

A

present lower than type 2 but also along anterior SCM border
terminate ipsilateral pyriform sinus
tract of type 4 loops under subclavian (R) or under aortic arch (L)

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18
Q

Arch 1 artery, nerve

A

terminal br maxillary

V

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19
Q

Arch 1 skeletal elements

A

incus, malleus, Meckel’s cartilage, upper portion auricle, maxilla, zygomatic, squamous portion of T bone, mandible

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20
Q

Arch 1 mm

A

mastication, mylo, anterior dig, TT, TVP

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21
Q

Arch 2 arteries, n

A

stapedius (embryologic), corticotympanic (adult)

VII

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22
Q

Arch 2 skeletal elements

A

stapes, styloid, stylohyoid ligament, lesser horns and upper rim hyoid, lower auricle

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23
Q

Arch 2 mm

A

facial expression, posterior dig, stylohyoid, stapedius

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24
Q

Arch 3 a, n

A

common carotid, most of internal carotid

IX

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25
Q

Arch 3 skeletal

A

lower rim, greater horn hyoid

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26
Q

Arch 3 mm

A

stylopharyngeus

27
Q

Arch 4 a/n

A

aortic arch, R subclavian
original sprouts pulm aa
SLN

28
Q

Arch 4 skeletal

A

laryngeal cartilages

29
Q

Arch 4 mm

A

pharynx constrictors, cricothyroid, levator veli palatini

30
Q

Arch 6 a/n

A

ductus arteriosus, roots of definitive pulmonary aa

RLN

31
Q

Arch 6 skeletal

A

laryngeal cartilage

32
Q

Arch 6 mm

A

intrinsic mm larynx

33
Q

Pseudotumor of infancy (fibromatosis coli)

A

firm rounded mass in SCM
presents 2-3 wk after birth
dense fibrous tissue, no striated muscle
birth trauma, muscle ischemia, intrauterine position
Tx: conservative, PT, >80% resolve by 1y/o

34
Q

Midline nasal mass: dermoid vs glioma vs encephalocele

A

Dermoid: most common, noncompressible, glabella to columella, midline pit with hair. Surgery

Glioma: portion of brain tissue isolated after suture closes, may have fibrous stalk maintaining CNS connection, but NO HERNIATED DURA

Encephalocele: meninges and brain matter through SB defect, illuminates, enlarge with strain/cry (furstenberg sign)

35
Q

Choanal atresia: incidence, syndromes, dx

A

1:8000 live births
Usually unilateral right, F>M
Choana is more often membranous than bony
CHARGE, Crouzon, Treacher-Collins
5-6 French catheter cannot pass into nasopharynx at birth

36
Q

Presentation of laryngomalacia

A

few to no sx at birth

gradually develop high pitch inspiratory stridor first few wk of life, usually gone by 12mo

37
Q

Classic endoscopic findings of laryngomalacia

A

omega shaped epiglottis

foreshortened aryepiglottic folds

38
Q

Which other condition is significantly associated with laryngomalacia

A

LPR

So acid-suppression is first line in non-surgical tx

39
Q

Presentation of u/l VC paralysis vs b/l

A

weak cry, breathy cry, poor feeding, aspiration

b/l: biphasic stridor at birth

40
Q

Causes of VC immobility

A 
birth trauma
hydrocephalus
spina bifida
CP
Chiari
41
Q

Congenital subglottic stenosis: definition, two types, presentation

A

subglottis <4mm term infant, <3mm premie
only congenital if prior to any intubation
membranous (more common, less severe) or cartilagenous
first few mo biphasic/inspiratory stridor, recurrent croup

42
Q

Tx of congenital subglottic stenosis

A

manage croup, anterior cricoid split, trach

43
Q

Hemangioma subglottis: presentation, course

A

3-4 wk of life, inspiratory/biphasic stridor, harsh cough, abnormal cry, FTT
50% have associated congenital hemangiomas
Spontaneous regression by 5 yo

44
Q

Tx of subglottic hemangioma

A

BB, cryotherapy, sclerotherapy, steroids, open resection, laser ablation, trach

45
Q

Presentation of laryngotracheal cleft

A

cyanosis, hoarse cry, cough, choke, stridor, aspirate, pneumonia

46
Q

4 types of laryngeal cleft

A

1- absence of interarytenoid muscle, cleft above VC
2- into upper cricoid
3- involves entire cricoid, poss to cervical trachea
4- extend to thoracic trachea

47
Q

5 types of tracheoesophageal anomalies

A 
EA w/ distal TEF (85%)
EA only (8%)
TEF w/o atresia - H type (5%)
EA w/ proximal TEF (1%)
EA w/ prox &amp; dist TEF (0.5-1%)
48
Q

Presentation of TE anomalies

A

EA: copious oral secretions, cough/choke/respiratory distress with feeds first few d life, cannot pass NG tube
H-type: may present later, chronic feeding diff, recurrent respiratory distress/pneumonia, Dx barium esophagram/endoscopy

49
Q

Causes of intrinsic tracheal malformations

A

tracheal agenesis (die in utero), congenital tracheal stenosis (may have complete tracheal rings), tracheomalacia

50
Q

Causes of extrinsic tracheal malformations

A

vascular (innominate, double arch, right arch w/ persistent ductus or ligamentum arteriosum), neck/mediastinal mass, thyromegaly

51
Q

Lop (cup)ear

A

most frequent ext auricular deformity
upper helix folded, wrinkled or tight, middle helix large and 90 deg from skin
Tx mold fold of superior crus, early neonatal

52
Q

Stahl’s ear

A

abnormal fold in scapha

Tx press out this area

53
Q

Protrudring ear

A

third most common ext auricular deformity
tx fix posterior helical rim to posterior retruauricular region with surgical tape; or use dental wax to mold ear in addition to taping

54
Q

Syndromes involving microtia

A

Treacher Collins

Goldenhar

55
Q

Types of microtia

A

I- mild deformities, no need for additional skin/cartilage to reconstruct
II- some structures recognizable
III- no recognizable landmarks

56
Q

Management of type III microtia

A 
2-4 stages of surgery
Stage 1- cartilage implant (rib)
2- 2-3 mo later, transfer lobule
3- elevate cartilage graft, postauricular skin grafting
4- tragus
57
Q

Optimal timing of microtia repair

58
Q

When should you place a BAHA when they have bilateral HL

A

Prior to 12 mo old

59
Q

Protruding ear measurement

A

mastoid to ear angle of 40 degrees or distance of 20mm

60
Q

Hemangioma vs vascular malformation

A

Hem: rarely present at birth, rapid postnatal proliferation then slow involution
10% incidence, most common neonatal tumor
increased capillary tubule formation

VM: always present at birth, abnormal morphogenesis of vascular channels, do not proliferate or involute, may grow larger with existing cell hypertrophy/fill cystic space with trauma/infection/hormones

61
Q

Low flow vs high flow VMs

A

Low: capillary, venous, lymphatic, combined

High: arterial, AVM

62
Q

Klippel Trenaunay syndrome

A

cutaneous capillary malformation
underlying venous/lymphatic malformation
associated with skeletal overgrowth of limb

63
Q

Sturge Weber

A

facial capillary malformation (port wine stain) in CN V, think about leptomeningeal VM on that side

ENT Secrets (79 decks)

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