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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
atresia
Mental
retardatio
n
Spinal
column abn
hydrocephalus Hypertelorism
Hemifacial microsomia
albinism Corpus
callosum
agenesis
Cerebral
atrophy
Lumbar lipoma Dermal cyst of
the frontal lobe
Coronary artery
anomaly
Cleft
lip/palate
Tracheo-
esophagea
l fistulas
Cardiac
anomalies
Genital
anomalies
Cerebral
anomalies
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
Furstenberg’s test
Don’t biopsy without imaging
Surgical intervention necessity
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