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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