Pediatrics

Question 1

Border between the primary/secondary palate?

Answer 1

incisive foramen

Question 2

Arterial supply of the hard palate?

Answer 2

greater palatine artery (basically everything else is soft palate)

Question 3

Lack of fusion between which structures creates clefting of the primary palate? Secondary palate?

Answer 3

anterior nasal prominences + medial maxillary prominences; maxillary prominence/contralateral maxillary prominence

Question 4

Is cleft palate or cleft lip more likely to be associated with a syndrome? Name 2-3 syndromes a/w CP.

Answer 4

CP more likely to be associated with a syndrome. CP - CHARGE, van der woede, Down

Question 5

Name 2-3 syndromes a/w CL.

Answer 5

DiGeorge, Stickler, OAV

Question 6

2/3 cleft lips are (right/left) and (male/female). 2/3 cleft palates are (male/female).

Answer 6

2/3 CL are left and male. 2/3 CP are female

Question 7

Involvement of what structure(s) differentiates incomplete from complete cleft lip? cleft palate?

Answer 7

Complete CL involves the nasal floor/sill, incomplete does not. Complete CP involves primary palate, incomplete CP does not.

Question 8

Label the following as infancy (<1 year), childhood (1-11 years), adolescence (12-18 years) with respect to timing for multidisciplinary cleft care surgical planning: cleft lip repair, orthognathic surgery, ortho-palatal expanders, palatoplasty, primary rhinoplasty, intermediate rhinoplasty, final rhinoplasty, VPI surgery

Answer 8

infancy: CL repair, primary rhinoplasty, palatoplasty
childhood: ortho-palatal expanders, intermediate rhinoplasty, VPI surgery
adolescence: orthognathic surgery, final rhinoplasty

Question 9

What is the rule of 10s and what is it in reference to?

Answer 9

timing for CL repair - 10 weeks, >10 lbs, >10hgb

Question 10

Name two palatoplasty techniques that lengthen the soft palate

Answer 10

Furlow palatoplasty, V-Y advancement

Question 11

Initial treatment for VPI? When should surgery be considered? Nonsurgical option?

Answer 11

Speech therapy. Failure for 6-12 month trial of speech therapy. Non surgical option - dental obturator

Question 12

Choanal atresia: more common in females or males? right or left?

Answer 12

females, right

Question 13

Name 2-3 syndromes associated with choanal atresia

Answer 13

CHARGE. Also Apert, Crouzon, Treacher Collins.

Question 14

At what age does unilateral choanal atresia commonly present? bilateral?

Answer 14

5-6 months for unilateral; at birth for bilateral.

Question 15

What is paradoxical cyanosis and what diagnosis does it imply?

Answer 15

paradoxical cyanosis is cyanosis that improves with tachypneic breathing, suggests bilateral choanal atresia.

Question 16

Nasopharyngeal or cervical mass with internal calcifications on plain films - pathognomonic for what diagnosis?

Answer 16

Teratoma

Question 17

Rathke’s Pouch Cyst vs Thornwald’s Cyst: a) location b) embryological origin

Answer 17

a) rathke in the sella turcica; thornwald’s in the nasopharynx (but submucosal. b) rathke from rathke’s pouch which becomes anterior pituitary. Thornwald’s is remnant of notochord

Question 18

What is a tumor of rathke’s pouch called?

Answer 18

craniopharyngioma

Question 19

In which type of pediatric CRS is systemic steroids 1mg/kg starting dose supported by level Ib evidence?

Answer 19

CRS with nasal polyps.

Question 20

Name 3-5 indications for sinus surgery in the pediatric population.

Answer 20

orbital or intracranial complications, mucocele, fungus ball/fungal sinusitis

Question 21

Congenital vs infantile hemangioma: a) time of presentation? b) GLUT1 positive/negative?

Answer 21

congenital presents at birth and is GLUT1 negative. infantile presents after birth and is GLUT1+.

Question 22

Infantile hemangioma: a) clinical exam characteristics? b) 3 phases of growth? c) 3 distributions?

Answer 22

a) firm — compressible suggests vascular malformation. b) proliferative - involuting - involuted (50% by 5 years, 70% by 7 years, 90% by 9 years). c) unifocal, multifocal, segmental (often trigeminal).

Question 23

infantile hemangioma: significance of beard/V3 distribution?

Answer 23

needs an endoscopy to rule out subglottic hemangioma

Question 24

what is PHACES syndrome?

Answer 24

posterior cranial fossa, hemangioma, intracranial arteries, cardiac, eye, sternum

Question 25

stridor in a patient with cutaneous hemangioma?

Answer 25

needs a DL/endoscopy to eval for subglottic hemangioma

Question 26

Management of hemangioma: 2 examples of when intervention is indicated?

Answer 26

symptomatic or cosmetically sensitive areas.

Question 27

What is kasabach merritt phenomenon?

Answer 27

consumptive coagulopathy

Question 28

2-3 potential indications for laser in management of hemangioma? what type of laser?

Answer 28

resurfacing, telangiectasias after regression, ulcerated lesions. Nd-YAG for deeper tissue. pulsed dye laser for superficial.

Question 29

when is laser contraindicated in subglottic hemangioma?

Answer 29

contraindicated in circumferential lesions.

Question 30

Name 3 medical treatments for hemangioma.

Answer 30

propranolol, steroid, chemo

Question 31

Slow Flow Vascular Malformations: a) 3 types? b) Which type is Sturge Weber? Which type is a telangiectasia?

Answer 31

a) capillary, venous, lymphatic. b) both are capillary.

Question 32

most common site in the body for lymphatic malformations?

Answer 32

head and neck

Question 33

2 types of lymphatic malformations, what study used to tell the difference, and optimal management strategy for each?

Answer 33

macrocystic vs microcystic; ultrasound; macrocystic=sclerotherapy, microcystic = surgery.

Question 34

What is OK-432?

Answer 34

OK-432 is inactivated strep pyogenes

Question 35

what is first-line therapy for skin & mucosal venous malformations? when should pulsed dye laser be considered?

Answer 35

Nd-YAG or KTP first line for skin & mucosal VENOUS. Pulsed dye laser considered in capillary

Question 36

Fast Flow Vascular Malformations: a) 2 types? b) etiology of each and differences in vascular structure?

Answer 36

a) AVM vs AVF b) AVM is nidus of abnormal capillary bed; AVF is posttraumatic and involves a single arteriovenous connection.

Question 37

Options for management in AVM?

Answer 37

multidisciplinary surgery and may involve preop embolization and a flap, high recurrence rate. Usually operate if very small and resectable or so large as to be life threatening.

Question 38

Options for management in AVF?

Answer 38

AVF surgery also with embo followed by resection if highly visible; can do embo alone if deep lesion