Congenital Nasal Anomalies

Question 1

How do you classify congenital Nasal anomalies?

Answer 1

Bartlett/Whitaker

Type 1- Agenesis, Hypoplasia

Type 2- Duplication, Hyperplasia

Type 3 - Clefts

Type 4 - Neoplasms and Vascular anomalies

Question 2

Which syndromes are associated with congenital nasal anomalies of ypoplastic/agenesis type?

Answer 2

1. Aperts (bilat narrowed bony vault, chonal atresia/stenosis)
2. Binder (midface retusion, absent ANS, short nose, flat bridge)
3. Fraser (crypopthalmia and nasal midline cleft, choanal atresia)

Question 3

Describe the congenital anomalies of agenesis/hypoplasia

Answer 3

AGENESIS of parts

• Arhinia (total no olfactory system)

HYPOPLASIA of parts

• Nasal cavity atresia
  
  • choanal atresia = posterior outlet= insufficient excavation of nasal pits
  • pyriform aperture stenosis
• Heminose

Question 4

Describe your nasal reconstruction plan for Binder syndrome

Answer 4

Delay until bone maturation (adolescence)

1- orthodontics

2- orthognathic surgery: Lefort 1 or 2 with BG (given mdiface retusion and class 3)

3- nasal augmentation and tip projection

Question 5

Describe congenital nasal anomalies of duplication/hyperplasia

Answer 5

DUPLICATION

• Polyrhinia
• Supernumerary nostrils

HYPERPLASIA

• Proboscis lateralis (tubular fleshy apepndage attached with MCT) 2’ to anomaly of LNP

Question 6

Which Tessier facial clefts are associated with nasal congenital anomalies?

Answer 6

0-14

1-13

2-12

3-11

Question 7

Which tessier cleft with nasal anomaly is most common

Answer 7

TEssier 0-14

= FrontoNasal Dysplasia

cleft of central midface

bifid nasal tip, orbital hypertelorism

frontonasal encephalocele

Question 8

Define an encephalocele

Answer 8

Herniation of crnial contents through bony defect in skull

Question 9

How do you classify Fontonasal Encephaloceles?

Answer 9

1. Naso frontal : encephalocele between frontal and nasal bones
2. Nasoehtmoidal: encephalocele between nasal bones and cartilages
3. Naso-orbital: encephalocele between medial orbital walls
4. Combined

Question 10

Where are encaphaloceles located

Answer 10

• occipital - most common
• frontal
• parietal

Question 11

What are clinical signs of an encephalocele

Answer 11

Location of mass (occipital, parietal, frontonasal)

trasnilluminates

pulsatile

blueish

compressible, expands with valsalva

Question 12

What is the prognosis of encephalocele

Answer 12

high mortality (50% survive w occipital lesion)

hihgh mornity (50% with neurodeficit)

Best prognosis if anterior lesion, pure meningocele

Question 13

What is the management of frontoehtmoidal encephalocele

Answer 13

Multidisciplinary with NSx

• need tight dural seal
• goals to remove mass, repair bone defect after dural repair, reconstruct medial orbital walls
• frontal craniotomy, rotate segments downward medially and posteriorly to correct trigonocephaly, hypertelorism
• Nasal recon with costochondral graft

Question 14

What is your DDX for a congenital nasal mass?

Answer 14

BENIGN

• Dermoid
• Glioma
• Hairy nevus
• Encephalocele
• Neurofibroma
• Pilomatrixoma
• Mesenchymal tumor (lymphagioma, angiofibroma, lipoma, hemangioma)
• polyp
• sinus tract (nasolacrimal duct cyst)

MALIGNANT

Question 15

What is a nasal glioma

Answer 15

Heterotopic growth of neural tissue 2/ deficient regression of neural tissue +/- connection with dura

= sequestrated encephalocele

Most extranasal (60%), some intranasal (30%) or both (10%)

Question 16

What are clinical signs of a nasal glioma

Answer 16

Firm non compressible,reddish

no transillumination

no enlargement with compresion of IJV (Furstenberg sign)

Question 17

How do you investigate a nasal mass you suspect is a nasal glioma

Answer 17

CT and MRI to check for intracranial extension

Question 18

How do you manage a nasal glioma

Answer 18

Multidisciplinary

Extranasal glioma: with no intracraial extesnion, lateral rhinotomy, external rhiploasty, midline nasal, coronal

If intrancranial component - need both intracranial and extracrnail approaches

Recurrence 5%

Question 19

What is a nasal dermoid

Answer 19

Hamartoma; abnormal growth of skin apepndages in epithelial lined cavity or sinus

Most common congenital nasal anomaly

Question 20

What are clinical signs

Answer 20

non compresisble, no trasnillumination, negative furstenberg

MIdline mass along columella, dorsum, nasoglabellar region

*sinus opening or punctum pathognomonic

Question 21

WHat is the etiology of nsal dermoid

Answer 21

Failure of separation of neruoectodermal and ectoderm tissue

• failure of dura to regress and forms sinus cavity
• persistence of dura wihtin cartilage layers

entraped epithelium within fused MNP

Question 22

How do you investigate a nasal mass that you suspect is a nasal dermoid

Answer 22

1. First CT
2. MRI if CT suggest intracrnial extension

Question 23

WHat do you treat nasal dermoid

Answer 23

no INTRACRANIAL EXTENSION

• extracranial appraoch (open rhino approach, lateral rhinotomy

INTRACRANIAL

• Nsx consult
• frontal craniotomy

Question 24

Define nasal Hemangioma

Answer 24

vascular tumor of proliferating endothelial cells with no dysplastic vessels

Question 25

What is the clinical presentation of a nasal hemangioma?

Answer 25

• appears in 1st wk of life
• red/purple
• rapid proliferation til 5-6yrs then involution over years