Evaluation of Congenital

Midline Nasal Masses

Camysha H. Wright, MD, Resident
Matthew Ryan, MD, Faculty Advisor
The University of Texas Medical Branch
Department of Otolaryngology
Grand Rounds Presentation
June 7, 2006
 Outline
Embryology of the nose
Dermoid cysts
Glioma
Encephalocele/Meningocele
Evaluation of Nasal Mass
Imaging Studies
Surgical Intervention
Conclusion
 Embryology

The critical period in nasal development is in

first twelve weeks of fetal development
Abnormalities of development are believed to
cause gliomas, dermoids, and encephaloceles
 Embryology
Neural tube develops
between the third and
fourth week of gestation
Closure of the neural
tube occurs from the
midline and extends
cranially and caudally
Neural tube then gives
rise to neural crest cells
 Embryology

As the neural tube closes neural

crest cells migrate anteriorly and
laterally around the eyes to the
frontonasal process
Nose formed from the medial and
lateral prominence and
invagination of the nasal pit
 Embryology
In most of the body neural crest cells are
involved in ectodermal components, in the face
the primary role is in the formation of
mesenchymal cells
Bone, cartilage, and muscles of the face are all
derivatives of neural crest cells
 Embryology
Nose develops from
frontonasal processes
and 2 nasal placodes
Medial processes fuse
Nasomaxillary groove
becomes the
nasolacrimal duct
 Embryology
Scanning electron
micrograph
 Embryology
During formation of skull base and nose,
mesenchymal structures are formed from several
centers which will eventually fuse and ossify.
Before their fusion, there are recognized spaces
which are important in the development of
congenital midline nasal masses
Fonticulus frontalis
Prenasal space
Foramen cecum
 Embyrology
Fonticulus frontalis space
between the frontal and nasal
bones
Eventually fuses with foramen
cecum to create a separation
between intracranial and
extracranial structures
Prenasal space is between the
nasal bones and the nasal
capsule (precursor of the
septum and nasal cartilages)
 Pediatric Anatomic Considerations
Neonates can suffer from respiratory distress with nasal
obstruction
Pediatric airway differs from adults in that neonates are
nasal breathers
Epiglottis abuts nasal surface of the soft palate forming
anatomic divide between the airway and digestive tracts
Food from oral cavity is shunted laterally into
esophagus via the pyriform sinuses
Neonates can functionally eat and breathe concurrently
Pediatric Airway
 Nasal Masses
Differential Diagnoses
Inflammatory lesions (abscess)
Traumatic deformity

Benign neoplasms (polyps, JNA)

Malignant neoplasms (rhabdomyosarcoma)

Congenital masses (teratomas, hemangiomas)

 Nasal Dermoids
Can occur as cyst or sinus
Most common congenital midline nasal mass
1-3% of all dermoids
10% dermoids of head and neck
 Nasal Dermoid
Has ectodermal and mesodermal components
(ectodermal components only epidermal cyst, ectoderm,
mesoderm and endoderm - teratoma)
May present as midline nasal pit, fistula, or infected
mass anywhere from glabella to columella
Sometimes presents as single cutaneous tract with hair
at opening
May secrete pus or sebaceous material
 Nasal Dermoid
Superonasal dermoid
 Nasal Dermoid

CNS connection variably reported (4-45%)

Associated congenital anomalies (5-41%), however not
found to be associated with syndromes
Aural Mental Spinal hydrocephalus Hypertelorism Hemifacial microsomia
atresia retardatio column abn
n
albinism Corpus Cerebral Lumbar lipoma Dermal cyst of Coronary artery
callosum atrophy the frontal lobe anomaly
agenesis

Cleft Tracheo- Cardiac Genital Cerebral

lip/palate esophagea anomalies anomalies anomalies
l fistulas
 Nasal Dermoid
Complications:
Intermittent inflammation
Abscess

Osteomyelitis

Broaden nasal root

Meningitis

Cerebral abscess
 Nasal Dermoid abscess
Abscess formation
 Dermoid Cyst
Development
During development a projection of dura projects
through the foramen cecum and attaches to skin
Dura normally separates from nasal skin and retracts
through foramen cecum losing connection
If skin maintains attachment to underlying fibrous
tissue, nasal capsule, or dura ,epithelial elements may
be pulled into the prenasal space with or without
dural connection
Dermoid Cyst Development
 Glioma
Glial cells in a connective tissue matrix
Firm, noncompressible
Red or bluish lump
Can be found at glabella, at nasomaxillary suture,
intranasally
No connection with subarachnoid space
Do not enlarge with crying
Do not transilluminate
May have telangiectasias
 Glioma
Intranasal glioma
 Glioma
Extranasal - 60%
Intranasal - 30%
Both - 10%
Dural connection - 35% intranasal, 9%
extranasal
Overall 15% dural connection
CSF rhinorrhea, meningitis possible, if dural
connection exists
Glioma Formation Hypotheses

Similar to that of nasal dermoids

Develop from extracranial rests of glial tissue
Abnormal closure of fonticulus nasofrontalis
Another theory is that they are possibly
encephaloceles which have lost CSF connection
Glioma Development
 Encephaloceles
Extracranial herniation of meninges and/or
brain
Connection with subarachnoid space
Rare at 1:35,000 births
30-40% associated anomalies:
microcephaly, hydrocephalus, microopthalmia,
anopthalmia, agenesis of the corpus callosum,
porencephaly, cortical atrophy, ventricular dilations
 Encephaloceles
Bluish, soft, compressible, transilluminate,
pulsatile
Enlarge with crying
Positive Furstenberg test (enlarge with bilateral
compression of internal jugular veins)
Originate medially in the nose (cannot pass
probe medially to this mass, versus with glioma
generally can as they originate laterally often)
May have associated CSF leak
 Encephaloceles
Large nasal encephalocele
 Encephaloceles
Divided into three categories:
Occipital 75%
Sincipital 15%
Basal 10%
Sincipital-anterior or frontonasal (dorsum of nose,
orbits, forehead)
Basal-intranasal mass, nasopharynx, posterior orbit
because they herniate through the cribiform plate or
posterior to it (potential for airway obstruction in
neonate)
 Encephaloceles

Basal Encephaloceles
Transethmoidal-through cribiform plate into middle
meatus
Sphenoethmoidal-extends through cranial defect
between posterior ethmoids and sphenoid to
nasopharynx
Transsphenoidal-presents in nasopharynx
Sphenomaxillary-through superior and inferior
orbital fissures to sphenomaxillary fossa
 Encephalocele
Development
Dural projection through fonticulus nasofrontalis
Abnormal closure results in herniated
meninges/brain
May be closely related to glioma
Encephalocele Development
 Nasal Masses
Evaluation
Most often infants and children
Dermoids-fistula tract, hair, pus or sebum, midline
Gliomas-firm, noncompressible, does not
transilluminate, telangiectasias
Encephaloceles-soft, compressible, bluish or red,
enlarge with crying, positive Furstenburg test
Do not biopsy extra or intranasal mass in a child before
imaging (Risk of meningitis or CSF leak if there is an
intracranial connection)
 Imaging
CT and MRI most used
CT findings include: fluid filled cyst, soft tissue
mass, intracranial mass, enlargement of foramen
cecum, distortion of crista galli
CT findings suggestive of intracranial extension
are enlarged foramen cecum and bifidity of
crista galli
Findings valuable if absent, (when present may
be false positive)
 CT Imaging
Nasal Dermoid axial ct with dermoid
anterior to nasal and maxillary bones,
no bony dehiscence or abnormalities
noted
 Nasal Dermoid with
Intracranial Extension
 CT Imaging
Encephalocele with defect noted in cribiform
plate and herniation of brain tissue
 MRI

Better delineates soft tissue

Ability to visualize in the sagittal plane
Denoyelle 36 children with dermoids, 2 patients
had CT suggestive of intracranial involvement
not found at surgery.
Recommended CT followed by MRI to confirm
intracranial connection
 MRI Imaging
Nasal dermoid cyst
 MRI-Glioma
Saggital T1
 MRI-Glioma
Sagittal T2
 MRI-Glioma
Coronal T2
 MRI-Encephalocele
Coronal T2 weighted and sagittal T1 weighted
image of sphenoid encephalocele
 Workup
History/Physical Examination
Nasal obstruction, polypoid intranasal mass, CSF
leak, presence of hair or fistulous tract, compressible
or firm, presence of pulsations, enlargement with
crying or internal jugular compression
Radiologic Evaluation (CT and/or MRI)
No Intracranial Extension (Dermoid/Glioma)
Intracranial Extension
 Treatment

Surgical Treatment
Complete excision (open-transcranial vs extracranial,
vs endoscopic approaches described)
Perform early to avoid nasal distortion, bony
atrophy, osteomyelitis, meningitis
dermoids-must excise entire tract to prevent
recurrence
 Dermoid
Can be removed endoscopically or via open
approach
Transverse rhinotomy has been described
Small to moderately sized lesions
Avoids vertical scar and splaying
 Nasal Dermoid with intracranial extension.
Meher R, Singh I, et al. J Postgrad Med 2005;51:39-40.

Dermoid cyst with intracranial extension without

craniotomy
Nasal bones removed along with anterior part of the frontal
bone
Followed sac through cribiform plate, incised wall of sac and
evacuated contents, and removed all except for its base where
it was attached to dura
Destroyed secretory epithelial surface of remnant of sac with
bipolar, replaced bone and closed wound in layers
Pt did well postoperative and no recurrence noted during 2
year follow up.
 Glioma
Can be removed via open or endoscopic
approach
Lateral rhinotomy or alar incisions may be used for
intranasal gliomas or combined intra-extranasal gliomas
Several authors have reported isolated cases of
endoscopic excision of small gliomas with and without
evidence of intracranial extension.
 Encephaloceles

In the past required combined approach with

neurosurgery
Frontal craniotomy is performed, intracranial
mass excised, bone-dura defect is repaired
Extracranial mass is then removed
More reports describing endoscopic removal
 Conclusion
Midline nasal masses are rare but must be
remembered in the differential
Furstenbergs test
Dont biopsy without imaging
Surgical intervention necessity
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