PEDIATRICS Section 3: Head and Neck (MIDFACE MALFORMATIONS) Flashcards
What are the two primary malformations in the Nasal cavity?
Pyriform sinus stenosis
Choanal Atresia
What anatomical structures should you check at the NAsal cavity malformations?
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.
What are the two anatomical landmarks when assessing nasal cavity malformations?
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.
What is the classic look of Pyriform Aperture Stenosis on CT?
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).
Diagnosis?
Pyriform aperture stenosis
soft tissue extension across the nostrils and a narrowed / inward bow to the maxillary spines (aperture < 8 mm).
What are the associated conditions of Pyriform Aperture Stenosis
Midline developmental problem - Hypothalamic-pituitary-adrenal axis dysfunction
Holoprosencephaly
Pituitarv dysfunction
Central MEGAincisor (seen in 75% of cases).
Results from a membrane that separates the nasal cavity from its normal communication with the oral cavity.
Itis usually unilateral but can be bilateral.
Choanal atresia
Diagnosis? Describe
Choanal atresia
The appearance is a unilateral or bilateral posterior nasal narrowing, with thickening of the vomer.
Cyclical cyanosis that improves with crying
Bilateral Choanal atresia
“Can’t pass the NG tube”
“Respiratory distress while feeding”
Unilateral Choanal Atresia
Choanal Atresia
What are the two types of Choanal Atresia?
Boney (90%) and membranous (10%)
What are the associated syndromes of Choanal Atresia?
CHARGE!
Coloboma
Heart Defect
Atresia (choanal)
Retarded Growth
Genitourinary Abdnomralities
Ear anomalies
Crouzons
DiGeorge
Treacher Collins, and
Fetal Alcohol Syndrome.
What happens in the Nasofrontal region malformations?
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.
Diagnosis and findings?
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)
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:
Dermoid or epidermoid cysts.
COngenital dermal sinus requires communication throught what structure?
Foremen Cecum
Congenital Dermal Sinus with Dermoid / Epidermoid Cysts location
Midline - NOT change in size with crying or jugular vein compression
These things aren’t actually gliomas - they have no neoplastic features. They are more like “brain in the wrong place.”
Nasal Glioma
Other name for NAsal Glioma
Nasa Cerebral Heterotopia
Features of NAsal glioma
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).
if the dural diverticulum that extends through the foramen cecum in early development doesn’t involute you end up with what?
A Tract
Describe Encephaloceles
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.
Conditions associated with Encephaloceles
facial clefts, callosal issues, interhemispheric lipomas
mostly midline stuff, which fits with the theme.
IDentify
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
nasal catheter will be displaced laterally by what condition?
Encephalocele
They CAN change size with crying (like a baby) or compression of the jugular vein.
Encephalocele
Why don’t you biopsy Encephaloceles?
DON’T BIOPSY this motherfucker!
CSF leaking and MEningitis will happen.
the result of an obstructed nasolacrimal duct (the duct that drains your eye into your nose).
Dacryocystocele
What is the most common cause of neonatal nasal obstruction?
Choanal Atresia (1st) and dacryocystocele (2nd)
Diagnosis?
Dacryocystocele
What are the 3 most common Craniofacial Syndromes?
Crouzans
Aperts
Treacher Collins (Manibulofacia Dyostosis)
These are the kids with the tiny jaws that are super dangerous to intubate.
Treacher Collins Syndrome
Describe Treacher Collins Syndrome
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
Describe The Treacher Collins Mandible
