Congenital Nasal Anomalies Flashcards
How do you classify congenital Nasal anomalies?
Bartlett/Whitaker
Type 1- Agenesis, Hypoplasia
Type 2- Duplication, Hyperplasia
Type 3 - Clefts
Type 4 - Neoplasms and Vascular anomalies
Which syndromes are associated with congenital nasal anomalies of ypoplastic/agenesis type?
- Aperts (bilat narrowed bony vault, chonal atresia/stenosis)
- Binder (midface retusion, absent ANS, short nose, flat bridge)
- Fraser (crypopthalmia and nasal midline cleft, choanal atresia)
Describe the congenital anomalies of agenesis/hypoplasia
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
Describe your nasal reconstruction plan for Binder syndrome
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
Describe congenital nasal anomalies of duplication/hyperplasia
DUPLICATION
- Polyrhinia
- Supernumerary nostrils
HYPERPLASIA
- Proboscis lateralis (tubular fleshy apepndage attached with MCT) 2’ to anomaly of LNP
Which Tessier facial clefts are associated with nasal congenital anomalies?
0-14
1-13
2-12
3-11
Which tessier cleft with nasal anomaly is most common
TEssier 0-14
= FrontoNasal Dysplasia
cleft of central midface
bifid nasal tip, orbital hypertelorism
frontonasal encephalocele
Define an encephalocele
Herniation of crnial contents through bony defect in skull
How do you classify Fontonasal Encephaloceles?
- Naso frontal : encephalocele between frontal and nasal bones
- Nasoehtmoidal: encephalocele between nasal bones and cartilages
- Naso-orbital: encephalocele between medial orbital walls
- Combined
Where are encaphaloceles located
- occipital - most common
- frontal
- parietal
What are clinical signs of an encephalocele
Location of mass (occipital, parietal, frontonasal)
trasnilluminates
pulsatile
blueish
compressible, expands with valsalva
What is the prognosis of encephalocele
high mortality (50% survive w occipital lesion)
hihgh mornity (50% with neurodeficit)
Best prognosis if anterior lesion, pure meningocele
What is the management of frontoehtmoidal encephalocele
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
What is your DDX for a congenital nasal mass?
BENIGN
- Dermoid
- Glioma
- Hairy nevus
- Encephalocele
- Neurofibroma
- Pilomatrixoma
- Mesenchymal tumor (lymphagioma, angiofibroma, lipoma, hemangioma)
- polyp
- sinus tract (nasolacrimal duct cyst)
MALIGNANT
What is a nasal glioma
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%)
What are clinical signs of a nasal glioma
Firm non compressible,reddish
no transillumination
no enlargement with compresion of IJV (Furstenberg sign)
How do you investigate a nasal mass you suspect is a nasal glioma
CT and MRI to check for intracranial extension
How do you manage a nasal glioma
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%
What is a nasal dermoid
Hamartoma; abnormal growth of skin apepndages in epithelial lined cavity or sinus
Most common congenital nasal anomaly
What are clinical signs
non compresisble, no trasnillumination, negative furstenberg
MIdline mass along columella, dorsum, nasoglabellar region
*sinus opening or punctum pathognomonic
WHat is the etiology of nsal dermoid
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
How do you investigate a nasal mass that you suspect is a nasal dermoid
- First CT
- MRI if CT suggest intracrnial extension
WHat do you treat nasal dermoid
no INTRACRANIAL EXTENSION
- extracranial appraoch (open rhino approach, lateral rhinotomy
INTRACRANIAL
- Nsx consult
- frontal craniotomy
Define nasal Hemangioma
vascular tumor of proliferating endothelial cells with no dysplastic vessels
What is the clinical presentation of a nasal hemangioma?
- appears in 1st wk of life
- red/purple
- rapid proliferation til 5-6yrs then involution over years
