Case report/Kazuistyka

Isolated nasal septal neuroglial heterotopia –

A case report

Santosh Kumar Swain 1,*, Rankanidhi Samal 1, Mahesh Chandra Sahu 2

1Department of Otorhinolaryngology, IMS and SUM Hospital, Siksha ‘‘O’’ Anusandhan University, Odisha, India2Central Research Laboratory, IMS and SUM Hospital, Siksha ‘‘O’’ Anusandhan University, Odisha, India

p e d i a t r i a p o l s k a 9 0 ( 2 0 1 5 ) 3 2 3 – 3 2 7

a r t i c l e i n f o

Article history:

Received: 14.04.2015

Accepted: 29.04.2015

Available online: 15.05.2015

Keywords:� Glial heterotopia� Nasal glioma� Nasal septum� Encephalocele

a b s t r a c t

Glial heterotopia is a rare condition in which mature neuroglial tissue is found in the

body other than central nervous system (CNS). It manifests as a mass of extra-cranial

cerebral tissue unconnected with the brain. Clinically, these masses are firm and incom-

pressible. Histopathologically, they consist of neuroglial cells and astrocytes embedded

in fibrous and vascular connective tissue. Radiological investigations such as computed

tomography (CT) or magnetic resonance imaging (MRI) should be done to rule out intrac-

ranial extension. We report one case of nasal glial heterotopia in a 3-month-baby which

has an unusual attachment over the nasal septum. The mass was completely resected

endoscopically which was attached to the anterior part of the nasal septum.

© 2015 Polish Pediatric Society. Published by Elsevier Sp. z o.o. All rights reserved.

Available online at www.sciencedirect.com

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Introduction

Nasal glial heterotopia is also known as nasal glioma.These are rare, benign congenital nasal masses moreaccurately referred to as sequestered glial tissue. Schmidtwas the first to present a comprehensive description ofthis lesion and coined the term glioma in 1900 [1].According to Bradley et al. nasal glioma is reported toaccount for five of 109 congenital nasal masses [2]. Nasalglial heterotopia is frequently diagnosed in newborninfants, but rarely found in adults. It is habitually diagno-sed right after birth and may be detected in the neonatalperiod in 60% of the cases [3]. Nasal glial heterotopia maybe extranasal or intranasal. Extranasal gliomas usually

* Corresponding author at: Department of Otorhinolaryngology, IMS aTel.: +91 9556524887.

E-mail address: santoshvoltaire@yahoo.co.in (S.K. Swain).http://dx.doi.org/10.1016/j.pepo.2015.04.0140031-3939/© 2015 Polish Pediatric Society. Published by Elsevier Sp. z

appear at the root of the nose. These are one of thecongenital midline masses, a category which also includenasal dermoids and encephaloceles. An encephalocele isa protrusion of brain substance connected to the rest ofthe brain by a pedicle with associated osseous defectwhereas nasal glial heterotopia has no communicationwith the subarachnoid space or the central nervoussystem (CNS) [4]. They may deform the bones of the nasalfossa and extend through the nasal bones, cribriform plateor the foramen caecum. Calcifications are rarely seen.Cystic changes inside the nasal glioma are occasionallyseen. They may protrude out of the nostril and rarelyattach to the nasal septum. Our case was showing attach-ment of glioma to the anterior part of nasal septum whichis an extremely rare ectopic site inside the nasal cavity.

nd SUM Hospital, Kalinga Nagar, Bhubaneswar 3, Odisha, India.

o.o. All rights reserved.

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Case report

A 3-month-old girl baby was referred to outpatient depart-ment of otorhinolaryngology for right nasal mass. The masshad been present since birth. The baby was born at full termand had a normal vaginal delivery. Her elder sibling had nocongenital abnormalities and the family history was unre-markable.

Fig. 1 – Nasal mass coming from right nostril

Fig. 2 – CT scan showing nasal mass arising from the nasalseptum

On examination, the baby had no facial and nasalswelling except a polyp like mass blocking the right nasalcavity which was protruding from the right nostril (Fig. 1). Itwas pale in colour and firm in consistency. It was neithertender, non-pulsatile, non-compressible nor with negativeFurstenberg sign. On probing test with a swab stick, masswas attached to the anterior part of the nasal septum. Therewas no history of cerebrospinal fluid (CSF) leak or meningi-tis. Computed tomography (CT) (Fig. 2) and magnetic reso-nance imaging (MRI) (Fig. 3) of the brain showed a right sideintranasal mass with no direct connection to the brain andmass showing attachment to the septum. The lesion wascompletely resected endoscopically. The nose was packedwith merocele nasal pack which was removed after 72 h.The post-operative period was uneventful.

Histopathological examination of the nasal mass confir-med the diagnosis of nasal glioma. It consisted of matureglial tissue, astrocytes with fibrous components (Fig. 4). Theimmunohistochemistry revealed that S-100 and glial fibril-lary acidic protein (GFAP) were positive. At 6 months follow-up the patient was doing well with no evidence of residualor recurrence in diagnostic nasal endoscopic examination.

Discussion

The incidence of congenital nasal masses is 1 in every20 000–40 000 live births [5]. The differential diagnosis of

Fig. 3 – MRI brain showing no connection to nasal mass tothe brain

Fig. 4 – HPE showing (a) glial tissue in fibrovascular stroma, (b) glial elements in Masson's trichrome, (c) phosphotungstic acidhaematoxylin stain and (d) gliofibrillary acid protein stain

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congenital nasal masses includes dermoid, hemangioma,glioma and encephalocele. Nasal glioma and encephalocelesare the results of abnormal embryologic development.During second gestational month, a diverticulum of the duraextends through the pre-nasal space and comes in contactwith the superficial ectoderm in the area that will becomethe nose. As the naso-frontal area develops, the nasalprocesses of the frontal bone grow and surround the duralprojection and form the foramen caecum. Under normalcircumstances, the dural projection involutes into a fibrousstructure, which fills the foramen caecum. Nasal gliomaresults from aberration of this normal developmental pro-cess. Nasal glial heterotopia is the congenital mass compo-sed of mature brain tissue isolated from the cranial cavityor spinal canal. It is a rare condition, thought to be derivedfrom either entrapped neuroectodermal tissue during theclosure of the covering of the brain or a nasal encephalocelewhich is covered by dura, pia and arachnoid and laterdisconnected from the intracranial cavity during subsequent

development. Nasal glioma occurs sporadically with nofamilial tendency or sex predilection [6]. The symptomsseen are nonspecific for nasal cavity mass: nasal obstruc-tion, nasal discharge and chronic otitis media. These lesionsusually present as a red or bluish mass at or along thenaso-maxillary groove, or as an intranasal mass. They arecharacteristically firm, non-compressible, do not increase insize with crying or coughing and do not transilluminate.They may be associated with a widened nose or withhypertelorism, secondary to growth of the mass. Imagingstudies are needed before excision of this mass to differen-tiate from encephalocele. An encephalocele is a protrusionof brain substance connected to the rest of the brain bya pedicle with associated osseous defect whereas nasal glialheterotopia has no communication with subarachnoid spaceor the central nervous system [7]. Biopsy or aspiration ofthese nasal masses is contraindicated because of the risk ofmeningitis or injury of functional brain tissue within anencephalocele [8]. Nasal gliomas are made of neuroglial

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elements consisting of glial cells in a connective tissuematrix with or without a fibrous connection to the dura.Usually they consist of fibrillary neuroglial tissue witha prominent network of glial fibres, gemistocytic typeastrocytes in background of neuropil, representing classicneuroglial tissue. It may be arranged in a lobular patternand cystic structures may be present as well [9]. There is nofluid filled space connected to the subarachnoid space.About 90% of the reported nasal gliomas do not containneurons, explained by either inadequate supply of oxygen toprovide support the neurons or failure of the neurons todifferentiate from the embryonic neuroectoderm in anintranasal glioma or both [10]. The presence of abundantneurons raises the possibility of an encephalocele. The glialnature of the cells can be confirmed by immunohistochemi-cal demonstration of S-100 protein and GFAP. These twoproteins can demonstrate neuroglial cells with high specifi-city, and help to distinguish nasal gliomas from othertumours such as meningiomas and granular cell tumours[11]. In our patient, both S-100 and GFAP were positive.Intranasal glioma most commonly arises from the lateralnasal wall near the middle turbinate [12] whereas our caseshowing attachment to the anterior part of the nasalseptum which is extremely rare.

The diagnosis of heterotopia versus encephalocele needsthe knowledge of radiologic and operative findings of thepatients [13]. CT scan and conventional radiographic studieshave limitations in detecting small bony defects or narrowstalk like connections across the skull base. MRI is conside-red as the investigation of choice for evaluation of congeni-tal nasal masses, especially those having intracranial con-nection [14]. Nasal endoscopy should be done in all cases toget precise location, origin and pulsatility [15]. The preferredtreatment of nasal glioma is complete surgical excision,although recurrences after surgery rarely occur. Intranasalendoscopic approach is strongly recommended for theremoval of intranasal gliomas [16]. With suspected bonydefects, an intranasal endoscopic approach is considered asthe procedure of choice [17]. Early surgical intervention isessential in view of the potential risk of cerebrospinal fluidleak and meningitis. Delaying of the treatment may causedistortion of the septum and nasal bone or infection in thenasal cavity.

Conclusion

Nasal glial heterotopia is rare, benign and a congenitallesion. Ectopic presentation of glial tissue over the nasalseptum is extremely rare as in our case. Evaluation shouldinvolve mandatory pre-operative imaging with a thin cutcoronal and axial CT scan and/or multi-planar MRI toexclude any intracranial connection before doing an inva-sive procedure. Treatment requires endoscopic excision incase of intra-nasal glioma. Conservative endoscopic excisionis done as it is slow growing, rarely recurrent and has nomalignant potential. It is very important to close follow-upof these patients because of the possibility of postoperativeCSF rhinorrhea and infection.

Authors' contributions/Wkład autorów

According to order.

Conflict of interest/Konflikt interesu

None declared.

Financial support/Finansowanie

None declared.

Ethics/Etyka

The work described in this article has been carried out inaccordance with The Code of Ethics of the World MedicalAssociation (Declaration of Helsinki) for experiments invol-ving humans; EU Directive 2010/63/EU for animal experi-ments; Uniform Requirements for manuscripts submitted toBiomedical journals.

r e f e r e n c e s / p i �s m i e n n i c t w o

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