27 Congenital - Voice, Neck, Nose Flashcards

1
Q

What are 3 mcc of stridor in kids

A

Laryngomalacia, VF paralysis, and congenital subglottic stenosis

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2
Q

What are the 3 modes of supraglottic obstruction causing laryngomalacia

A
  1. prolapse of mucosa overlying the arytenoids
  2. foreshortened AE folds
  3. posterior displacement of epiglottis
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3
Q

What is incidence of synchronous airway lesion in kids with laryngomalacia

A

18-20%

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4
Q

What percent of infants w/ laryngomalacia require surgical tx

A

10%

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5
Q

Indications for surgery for laryngomalacia

A
  • Dyspnea at rest or during effort
  • Feeding difficulties
  • FTT
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6
Q

What is relationship b/w laryngomalacia and GERD

A

Essentially they all have GERD

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7
Q

What percent of kids with choanal atresia have other congenital anomalies

A

50%

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8
Q

What % of cases of choanal atresia are b/l

A

40%

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9
Q

Persistence of what membrane results in choanal atresia

A

buccopharyngeal

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10
Q

What % of cases of chonal atresia involve only a mucosal diaphragm or membrane

A

10%

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11
Q

What percent of cases of congenital VF paralysis are b/l and what percent of these require trach

A

20%; 90%

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12
Q

What is m/c neuro condition causing VF paralysis in kids

A

Arnold-Chiari malformation (get MRI)

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13
Q

What effect does timing of tx for Arnold-Chiari have on outcome of VF paralysis

A

-If ICP is normalized w/in 24 hrs, VF fnc will recover w/in 2 wks in most pts

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14
Q

What is recovery rate for idiopathic VF paralysis in kids

A

20%

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15
Q

Birth trauma accounts for what percent of VF paralysis in kids

A

20% (a/w forceps use and c/s)

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16
Q

Where do most laryngeal webs occur?

A

At the anterior glottis (75%)

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17
Q

When is typical onset of sx in pts with subglottic hemangioma

A

Usu asx at birth and sx by 6 months of age

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18
Q

What is most common vascular ring

A

Innominate artery compression

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19
Q

What is m/c anomaly a/w congenital tracheal stenosis

A

Aberrant left pulmonary artery (pulmonary artery sling complex)

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20
Q

What syndrome is characterized by hypernasal speech, cardiac malformations, cleft palate, and medial displacement of carotids

A

Velocardialfacial syndrome (VCFS)

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21
Q

What syndrome is characterized by deletion of band 11 on long arm of chrom 22 and is a CI to adenoidectomy

A

VCFS

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22
Q

What is platybasia

A

Phenotypic characteristic of VCFS where the cranial base in angled obtusely, resulting in expanded velopharyngeal volume and incomplete velopharyngeal closure

23
Q

DDx midline neck mass in child

A
  • TGDC (m/c)
  • Dermoid cyst
  • Ectopic thyroid
  • LAD
  • Lipoma
  • Hemangioma
  • Fibroma
24
Q

Etiology of TGDC

A

Persistence of connection b/w base of tongue (foramen cecum) and descending thyroid gland

25
Q

Why do some ppl recommend radioisotope scanning or u/s of the thyroid gland prior to removal of TGDC

A

To prevent inadvertent removal of the only functioning thyroid tissue

26
Q

What percent of TGDC contains thyroid tissue

A

20%

27
Q

What is the incidence of carcinoma arising in a TGDC

A

<1%

28
Q

Etiology of preauricular pit

A

Failure of fusion of Hillocks of His 1 and 2

29
Q

What % of preauricular pits are b/l

A

20%

30
Q

What is the etiology of type 1 1st branchial cleft cyst

A

Duplication error of ectodermal elements of EAC

31
Q

Etiology of type 2 1st branchial cleft cyst

A

Duplication error of ectodermal and mesodermal elements of EAC

32
Q

Where are most type 1 cysts located

A

In preauricular region, lateral to VII, connecting skin to EAC

33
Q

Where are most type 2 cysts located

A

just inferior or posterior to angle of mandible w/ variable relationship to VII

34
Q

Where are most 2nd branchial cleft cysts located

A

below angle of mandible and anterior to SCM

35
Q

What is typical course of tract of 2nd branchial cleft cyst

A

“Pass superiorly and laterally to IX, XII; turn medially to pass b/w ICA and ECA; terminate close to the middle constrictor muscle or may open into the tonsillar fossa.

36
Q

What is typical course of tract of 3rd branchial cleft cyst

A

“Ascend lateral to the common carotid artery, pass posterior to the ICA, superior to XII, and inferior to IX; course medially to pierce the thyrohyoid membrane superior to the internal branch of the superior laryngeal nerve. End in piriform

37
Q

What is typical course of tract of 4th branchial cleft cyst

A

Begin at the apex of the pyriform sinus, descend lateral to the recurrent laryngeal nerve into the thorax, loop around the aortic arch (subclavian on right), ascend to the neck posterior to the common carotid artery, cross XII, descend to open into the skin at the anterior-inferior aspect of the sternocleidomastoid muscle.

38
Q

What inflammatory dz is a/w 3rd and 4th branchial anomalies in kids

A

Recurrent acute suppurative thyroiditis (E. coli, klebsiella, proteus, clostridium)

39
Q

What are the 4 types of germ cell tumors

A

dermoid cyst, teratoid cyst, teratoma, and epignathus

40
Q

What is a dermoid cyst composed of and where is m/c found in H&N

A

mesoderm and ectoderm; submental

41
Q

How can one differentiate dermoid cyst from TGDC

A

dermoid cyst won’t elevate with tongue protrusion

42
Q

What is characteristic feature of a teratoid cyst

A

very poor differentiation of all 3 germ layers

43
Q

What differentiates a teratoid cyst from a teratoma

A

the germ layers are well differentiated in teratomas

44
Q

What % of teratomas become malig

A

20%

45
Q

What prenatal condition is a/w higher incidence of cervical teratomas

A

maternal polyhydramnios

46
Q

What is term for connection of 3rd branchial pouch to the thymus gland as the gland descends into thorax

A

thymopharyngeal duct

47
Q

What is the etiology of cervical thymic cysts

A

persistence of thymopharyngeal duct

48
Q

What is a thornwaldt’s cyst

A

cyst in NP bursa 2/2 persistent embryonic communication b/w the anterior tip of the notochord and the NP epithelium

49
Q

What are the 3 m/c congenital nasal masses

A

encephaloceles, gliomas, and dermoid cysts

50
Q

What % of gliomas have a fibrous tract connecting to the subarachnoid space

A

15%

51
Q

What chromosome carries gene for CF

A

Long arm of chrom 7

52
Q

T/F: Frontal sinus hypoplasia is common in pts with CF

A

T

53
Q

What % Caucasians carry gene defect for CF

A

5%