PEDIATRICS Section 3: Head and Neck (MIDFACE MALFORMATIONS)

Question 1

What are the two primary malformations in the Nasal cavity?

Answer 1

Pyriform sinus stenosis
Choanal Atresia

Question 2

What anatomical structures should you check at the NAsal cavity malformations?

Answer 2

This is an axial slice through the nasal cavity, at the level of the maxillary spines (the pointy things “MS”). The vomer (“V”) is in the middle.

Question 3

What are the two anatomical landmarks when assessing nasal cavity malformations?

Answer 3

The two key things to know here are the pyriform aperture (“PA”) is the area in the front (between the maxillary spines), and the posterior choanae (“PC”) is the area in the back.

Question 4

What is the classic look of Pyriform Aperture Stenosis on CT?

Answer 4

The classic look is an axial CT though the maxillary spines demonstrating soft tissue extension across the nostrils and a narrowed / inward bow to the maxillary spines (aperture < 8 mm).

Question 5

Diagnosis?

Answer 5

Pyriform aperture stenosis

soft tissue extension across the nostrils and a narrowed / inward bow to the maxillary spines (aperture < 8 mm).

Question 6

What are the associated conditions of Pyriform Aperture Stenosis

Answer 6

Midline developmental problem - Hypothalamic-pituitary-adrenal axis dysfunction

Holoprosencephaly
Pituitarv dysfunction
Central MEGAincisor (seen in 75% of cases).

Question 7

Results from a membrane that separates the nasal cavity from its normal communication with the oral cavity.

Itis usually unilateral but can be bilateral.

Answer 7

Choanal atresia

Question 8

Diagnosis? Describe

Answer 8

Choanal atresia

The appearance is a unilateral or bilateral posterior nasal narrowing, with thickening of the vomer.

Question 9

Cyclical cyanosis that improves with crying

Answer 9

Bilateral Choanal atresia

Question 10

“Can’t pass the NG tube”

“Respiratory distress while feeding”

Answer 10

Unilateral Choanal Atresia

Choanal Atresia

Question 11

What are the two types of Choanal Atresia?

Answer 11

Boney (90%) and membranous (10%)

Question 12

What are the associated syndromes of Choanal Atresia?

Answer 12

CHARGE!
Coloboma
Heart Defect
Atresia (choanal)
Retarded Growth
Genitourinary Abdnomralities
Ear anomalies

Crouzons
DiGeorge
Treacher Collins, and
Fetal Alcohol Syndrome.

Question 13

What happens in the Nasofrontal region malformations?

Answer 13

These are embryology problems where the dural membrane in the prenasal space don’t regress.

This fuck up in regression leaves a PATENT TRACT which things can protrude or grow into.

Question 14

Diagnosis and findings?

Answer 14

Pyriform Aperture Stenosis

Congenital piriform aperture stenosis. (a) The nasal processes of the maxillae approximate each other closely to narrow the piriform aperture. CPAS is considered to be present if the width of each PA is less than 3 mm or if the total width is less than 8 mm. The presence of a single unerupted maxillary megaincisor (b) should prompt evaluation for intracranial anomalies (holoprosencephaly – note the forebrain fusion in (c)

Question 15

During normal development there is a dural tract in the prenasal region that is supposed to regress and close. If it doesn’t you can have these conditions:

Answer 15

Dermoid or epidermoid cysts.

Question 16

COngenital dermal sinus requires communication throught what structure?

Answer 16

Foremen Cecum

Question 17

Congenital Dermal Sinus with Dermoid / Epidermoid Cysts location

Answer 17

Midline - NOT change in size with crying or jugular vein compression

Question 18

These things aren’t actually gliomas - they have no neoplastic features. They are more like “brain in the wrong place.”

Answer 18

Nasal Glioma

Question 19

Other name for NAsal Glioma

Answer 19

Nasa Cerebral Heterotopia

Question 20

Features of NAsal glioma

Answer 20

growth rate tends to parallel brain growth rate.
these things don’t change size if you cry like a baby or compress the
jugular vein.
They don’t enhance. They just look like a soft tissue mass in the nose (of a baby).

Question 21

if the dural diverticulum that extends through the foramen cecum in early development doesn’t involute you end up with what?

Answer 21

A Tract

Question 22

Describe Encephaloceles

Answer 22

if the dural diverticulum that extends through the foramen cecum in early development doesn’t involute you end up with a tract. If intracranial contents herniate into this tract you get yourself an encephalocele.

Question 23

Conditions associated with Encephaloceles

Answer 23

facial clefts, callosal issues, interhemispheric lipomas

mostly midline stuff, which fits with the theme.

Question 24

IDentify

Answer 24

Midline nasal anomalies.
(a) Normal development. FNF fonticulus nasofrontalis – a transient opening that closes during normal development. FC foramen cecum. (b) Nasal dermal sinus. (c) Encephalocele. (d) Nasal cerebral heterotopia (nasal glioma). (e) Dermoid cyst

Question 25

nasal catheter will be displaced laterally by what condition?

Answer 25

Encephalocele

Question 26

They CAN change size with crying (like a baby) or compression of the jugular vein.

Answer 26

Encephalocele

Question 27

Why don’t you biopsy Encephaloceles?

Answer 27

DON’T BIOPSY this motherfucker!

CSF leaking and MEningitis will happen.

Question 28

the result of an obstructed nasolacrimal duct (the duct that drains your eye into your nose).

Answer 28

Dacryocystocele

Question 29

What is the most common cause of neonatal nasal obstruction?

Answer 29

Choanal Atresia (1st) and dacryocystocele (2nd)

Question 30

Diagnosis?

Answer 30

Dacryocystocele

Question 31

What are the 3 most common Craniofacial Syndromes?

Answer 31

Crouzans
Aperts
Treacher Collins (Manibulofacia Dyostosis)

Question 32

These are the kids with the tiny jaws that are super dangerous to intubate.

Answer 32

Treacher Collins Syndrome

Question 33

Describe Treacher Collins Syndrome

Answer 33

They have small absent zygomatic arches, narrow but over projected maxilla, and mandible hypoplasia with a retruded weak / unmanly chin.

Skinny Protruded Maxilla
Tiny Retruded Chin (destined to drink soy milk) Tiny Absent Zygomas / Malar Bones

Question 34

Describe The Treacher Collins Mandible

Answer 34