Full text of DOI:10.1007/s12009-001-0060-7

A B S T R A C T
Nasal gliomas are rare, benign, congenital, midline tumors. They are
seen most commonly in neonates and children but rarely in adults.
We encountered in a 28-year-old woman a rare case of nasal glioma
presenting as bilateral total blindness. The tumor was present in
both nasal fossae. It extended into the parasellar region, with ero-
sion of the cribriform plate and medial wall of the right orbit.
C A S E R E P O R T
Nasal Glioma Presenting
as Blindness in an Adult
Rajiv K. Jain, MS, Pramod K. Shukla, MD,
asal glioma was first described in the literature in
Nakul C. Aryya, MD, Arvind Srivastava, MD, &
Sanjay Kumar
1
N
1890. However, the first comprehensive descrip-
tion of the tumor and the first complete case reports
2
were published only in 1950. The authors described
that these tumors originated from an encephalocele
and also suggested the possibility of teratomatous ori-
2
gin. Since then, these tumors have been described in
the literature under various names: fibroglioma, gan-
glioblastoma, ganglio-neuro-schwanno-spongioblas-
toma, glioma, nasal glioma, encephalocele, and
3
choristoma. Now the term nasal cerebral heterotopia
is gradually gaining universal acceptance. The term
nasal glioma is probably retained due to its wide-
spread clinical use.
These rare tumors are most commonly seen in
neonates and children and are extremely rare in
adults. Although orbital involvement has been report-
ed, to our knowledge, complete blindness associated
with nasal glioma has not been reported in the litera-
ture so far. We herein report a case of nasal glioma pre-
senting as bilateral total blindness.
Case Report
A 28-year-old woman reported to the ophthalmology
services of our hospital for evaluation of complete loss
of vision in both eyes. Apparently she was asympto-
matic 6 months earlier. Then she noticed a gradual
decrease of vision in both eyes. For the last 2 months
she was unable to see at all. On ophthalmic examina-
tion, in both eyes there was no perception of light. The
Reprints:
Rajiv K. Jain, MS, B-5, New Medical Enclave, Banaras Hindu University, Varanasi 221
005, India.
Drs. Jain and Kumar are from the Department of Otolaryngology, Drs. Shukla and
Aryya are from the Department of Pathology, and Dr. Srivastava is from the Depart-
ment of Radio-Diagnosis, Institute of Medical Sciences, Banaras Hindu University,
Varanasi, India.
ANN OPHTHALMOL. 2001;33(1):67–69
67

remaining findings of the ophthalmologic examina-
tion were within normal limits. A computed tomo-
graphic (CT) scan of the orbit and paranasal sinuses
was advised, which revealed a uniformly enhanced
mass in both nasal fossae in the superior meatus area.
There was erosion of the cribriform plate along with
extension of the mass into the parasellar region. The
medial wall of the right orbit was also eroded (Fig 1).
The patient was referred to otolaryngology services.
On nasal endoscopy, a reddish, globular, shining mass
with capillary prominence was seen on both sides in
the superior meatus. The remaining endoscopic find-
ings were normal. An endoscopic biopsy specimen
was taken from the right side, and the procedure pro-
duced substantial bleeding. The bleeding stopped
with anterior nasal packing.
Fig 1.—CT scan of paranasal sinuses and orbit in coronal plane reveals uniformly
enhanced soft-tissue mass occupying both nasal fossae, eroding cribriform plate and
medial wall of right orbit, and extending into parasellar region.
The histopathologic report established the diagno-
sis of nasal glioma. The patient was informed about
the disease and choices for further management. She
refused to undergo any further treatment and had no
subsequent follow-up.
Histopathologic Findings
Histologic analysis of the biopsy specimens revealed
brain tissue with small areas of capillaries and hemor-
rhage covered by nasal mucosa (Figs 2 to 5). The
tumor mass was unevenly cellular, composed of small
fibrillary astrocytes with pleomorphic and hyperchro-
matic nuclei. Microcystic spaces and mild endothelial
proliferation were also present. There were also some
areas of necrosis.
Discussion
Nasal gliomas are rare tumors usually seen in children.
They are extremely rare in adults and more predomi-
Fig 2.—Nasal epithelium covering brain tissue on biopsy specimen (hematoxylin-
eosin, ×60).
2
nantly found in males. They can be extranasal (60% of
cases), intranasal (30%), or a combination of extranasal
4
and intranasal components (10%). On physical exami-
nation, an extranasal tumor presents most frequently
as a round, firm, red or bluish swelling on either side
of the bridge of the nose. Intranasal tumors resemble
nasal polyps, but they are slightly firmer in consisten-
cy and show increased bleeding tendency on biopsy. In
both types there is no pulsation or alteration of size on
the Valsalva maneuver.
Nasal glioma are considered to be formed in utero
as a herniation of ectodermal neural tissue of the
frontal lobe through a bony cleft into the nose
5
(
“encephalocele” pathogenesis. Other theories for the
formation of nasal glioma are as follows: the amputa-
tion of portions of the olfactory bulb during closure of
Fig 3.—Another histologic view of tumor showing nasal epithelium underneath,
which is gliomatous tumor with unevenly distributed tumor cells, microcystic spaces,
and an area of necrosis (hematoxylin-eosin, ×125).
6
frontal bone sutures ; the migration of glial cells along
7
olfactory nerves during embryonic development ; the
formation of neuroectodermal rests isolated in the
nasal cavity early in embryonic development, forming
Nasal gliomas are described as being occipital (75%
of cases), sincipital (15%), or basal (10%) in location.
The sincipital gliomas are located above the dorsum of
the nose, orbit, and forehead. These are further divid-
ed into 3 subgroups: nasofrontal, nasoethmoidal, and
naso-orbital. The nasofrontal type is formed by pro-
8
9
11
glial ectopia ; and teratomatous formation. Of all
these theories, the “encephalocele” pathogenesis
seems most acceptable due to close association
between encephaloceles and nasal gliomas and the
1
0
frequent occurrence of a stalk to the brain.
68
ANN OPHTHALMOL. 2001;33(1)

Fig 4.—Area of tumor with typical fibrillary background and astrocytes distributed
more evenly. A large number of microcystic spaces are also seen along with mild
degree of vascular endothelial proliferation (hematoxylin-eosin, ×125).
Fig 5.—Another histologic view of tumor showing fibrillary astrocytes with pleomor-
phic and hyperchromatic nuclei (hematoxylin-eosin, ×500).
trusion through the frontal and nasal bones; naso-
orbital protrudes through the medial wall of the orbit,
involving frontal, ethmoidal, and lacrimal bones.
The basal encephaloceles present as a mass pro-
truding into the superior meatus of the nasal cavity,
nasopharynx, or sphenomaxillary fossa. These are fur-
ther grouped into transethmoidal, sphenoethmoidal,
transsphenoidal, and sphenomaxillary. The transeth-
moidal type protrudes through a defect in the cribri-
form plate into the superior meatus. The
sphenoethmoidal protrudes into the nasopharynx
through a defect between the posterior ethmoidal cells
and the sphenoid. The transethmoidal protrudes
through a patent craniopharyngeal canal into the
nasopharynx. The sphenomaxillary protrudes through
the supraorbital tissue, through the infraorbital tissue,
and then into the sphenomaxillary fossa, presenting as
a mass on the medial side of the mandibular ramus.
Histologically, nasal gliomas are composed of brain
tissue in which there is usually a significant and often
dominating gliosis. The nest of glial cells is interlaced
with a vascular fibrous tissue network or septa. Sec-
ondary changes of fibrogliosis or gemistocytic alter-
ation of glial cells are often seen. Ganglion cells and
other neural elements may also be present. The gliosis
is probably secondary to circulatory impairment,
death of cerebral tissue, and obliteration of the sub-
arachnoid and perivascular spaces. Any “bizarre”
astrocytes that may be present in the lesion are due to
hypoxia and compression and should not be confused
with neoplastic cells.
skull, orbit, and the surrounding area are a must. All
patients also should be subjected to nasal endoscopic
examination before any surgery.
This tumor, although not completely radioresistant,
is not radiosensitive; thus, the treatment of choice is
complete surgical excision. Surgery should be done by
a team composed of the otolaryngologist, neurosur-
geon, plastic surgeon, and ophthalmologist. Occasion-
ally the intranasal tumor may be surgically resected
by an intranasal approach. A lateral rhinotomy
approach provides very good visualization of the crib-
riform area for evaluation of the presence of any glial
stalk. Usually the combination approach of intranasal
excision and lateral rhinotomy and craniotomy is pre-
ferred. The operating microscope is of immense help
in the dissection of all types of nasal gliomas. Inoper-
able cases or recurrences after surgical excision
should be treated by radiotherapy.
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In terms of management, nasal gliomas should be
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