|Year : 2020 | Volume
| Issue : 2 | Page : 290-292
Rare case report of nasal glioma
Deekshith Rajmohan, S Sai Manohar
Department of Otorhinolaryngology, Yenepoya Medical College, Mangalore, Karnataka, India
|Date of Submission||11-May-2020|
|Date of Decision||16-May-2020|
|Date of Acceptance||19-Jun-2020|
|Date of Web Publication||23-Dec-2020|
Dr. Deekshith Rajmohan
Department of Otorhinolaryngology, Yenepoya Medical College, Mangalore City - 575 018, Karnataka State
Source of Support: None, Conflict of Interest: None
A child aged 14 months presented with facial disfigurement due to an extra nasal swelling at the root and dorsum of the nose, present since birth, and increasing in size. The swelling was firm in consistency, noncompressible, and nonpulsatile. Cough impulse was absent. Magnetic resonance imaging was suggestive of nasal glioma without intranasal or intracranial extension. Following complete surgical excision, primary reconstruction was required with a local transposition forehead flap. Histopathology confirmed the diagnosis of nasal glioma.
Keywords: Congenital nasal mass, encephalocele, fonticulus frontalis, foramen cecum, nasal gliomas
|How to cite this article:|
Rajmohan D, Manohar S S. Rare case report of nasal glioma. Arch Med Health Sci 2020;8:290-2
| Introduction|| |
Nasal glioma is a rare and benign form of midline congenital lesion made up of a heterotopic mass of neuroglial tissue, confined to the craniofacial region. Various theories have been proposed in the literature about the embryological origin. These lesions are believed to be the consequence of incomplete closure of the anterior fontanelle between the frontal and nasal bones resulting in an abnormal connection between embryonic ectodermal and neuroectodermal elements. Although gliomas are benign in nature, they often lead to cosmetic deformity requiring total excision.
In this report, we present a rare case of extranasal glioma who presented with facial disfigurement for its diagnostic and therapeutic challenges.
| Case Report|| |
A male child aged 14-month presented with an external nasal swelling since birth which was gradually increasing in size. On examination, it was a 2 cm × 3 cm swelling confined to the root and dorsal aspect of the nose more toward the left lateral aspect [Figure 1]. The swelling was firm; noncompressible, nonpulsatile, and cough impulse were absent. Contrast-enhanced magnetic resonance imaging (MRI) of the face showed an enhancing lesion on T1-weighted images suggestive of glioma. There was no intranasal extension or intracranial communication [Figure 2].
|Figure 2: Magnetic resonance imaging of the face showing T1-weighted with contrast (asterisk indicates nasal glioma enhancing on contrast)|
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With this work up, excision was planned using an external approach followed by primary closure of the defect under general anesthesia. Intraoperatively, the swelling was firm in consistency with skin fixity in certain areas necessitating unplanned skin removal along with the lesion. This was addressed by the primary reconstruction using local transposition forehead flap [Figure 3]. Transnasal endoscopic examination was done to rule out the nasal mucosal defect. There were no perioperative or postoperative complications and wound healed well.
|Figure 3: Intraoperative picture (arrow indicates transposition flap of the forehead)|
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Histopathology showed the presence of glial tissue with fibrovascular connective tissue confirming nasal glioma [Figure 4]. The patient was followed up for a period of 1-year and there was no evidence of recurrence of the lesion.
|Figure 4: Hematoxylin and eosin staining of the specimen (asterisk showing astrocytes with fibrosis)|
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| Discussion|| |
Nasal glioma was first described by Reid in 1852. The term glioma was coined in 1900 by Schmidt., The term gliomas are actually a misnomer and are often mistaken for the tumors of the brain. Nasal gliomas are rare benign masses originating from heterotopic neural tissue. These lesions are associated with intracranial complications due to their connection with the central nervous system (CNS). The incidence of nasal gliomas is around 1 in 20,000–40,000 births, as reported in the literature. There is a male preponderance., They often manifest as an extranasal (60%), intranasal (20%), or mixed (10%)., Very rarely, it can be located in other places such as the lip, tongue, scalp, orbit, frontal sinus, nasopharynx, and oropharynx, In about 10%–25% of cases, there may be a fibrous tract connecting to the subarachnoid space.,
Usual clinical presentation is of an external swelling limited to the upper one-third of the nose. A very large swelling can cause facial disfiguration. However, there can be both extra and intranasal components coexisting many a time in the same patient. There are often bony deformities seen around the mass due to pressure changes and remodeling of the nasal and facial bones leading to hypertelorism and obstruction of the nasolacrimal duct with epiphora on the affected side.,, Intranasal glioma often manifests as a pale mass within the nasal cavity with protrusion from the nostril. A large swelling with intranasal extension can cause nasal obstruction leading to respiratory distress. In some cases, it can cause visual impairment or failure to thrive.
One has to differentiate nasal gliomas from encephalocele. Encephalocele is the herniation of neural tissue with meninges through the developmental or acquired defects along the skull base, whereas gliomas are sequestered encephaloceles along the developmental tract line. Encephalocele generally contains one of the neural elements, such as meninges (meningocele) or brain matter (meningoencephalocele). Radiologically, both gliomas and encephalocele are distinguished based on the presence or absence of a connection between the mass and the intracranial cavity. Encephaloceles may pulsate and expand when the jugular vein is compressed (positive Furstenberg's sign) with impulse being seen on crying and coughing as compared to nasal glioma which is nonpulsatile and gives a negative Furstenberg's sign.,, Intranasal gliomas are reported to be more frequently connected with the CNS than the extra nasal or combined ones., In general, a biopsy of lesions having intracranial connection carries a risk of meningitis or cerebrospinal fluid (CSF) leak. Hence, it is better to avoid biopsy.
The skull base develops from two different tissues-neural crests and mesoderm at around the 4th month of gestation. The anterior skull base develops from neural crest cells, whereas the posterior skull base develops from the mesoderm. Two transient spaces develop around the nasofrontal region, called as fonticulus frontalis and prenasal space. The fonticulus frontalis is located in the midline between the paired frontal and nasal bone, and the prenasal space is located between the nasal capsule and the nasal bone. As gestation continues, a diverticulum of dura extends through the prenasal space and contacts the superficial ectoderm in the area that will become the nose. The prenasal space becomes smaller with the growth of the adjacent bone structures, eventually being reduced to a small canal anterior to the crista galli known as the foramen cecum., During embryologic development, the foramen cecum transmits dural diverticulum from the developing anterior cranial fossa to the dermal surface of the nose, which involutes completely and foramen cecum fills in with fibrous tissue with varying ossifications.
Many theories have been postulated for the development of nasal gliomas such as sequestration of glial tissue of the olfactory bulb entrapped during cribriform plate fusion, ectopic neural tissue cells, encephaloceles with lost intracranial connection, and meningeal continuity and inappropriate closure of the anterior neuropore (fonticulus frontalis).,,
Imaging studies such as MRI and computed tomography scan help in differentiating between other swellings. On MRI T1-weighted images, gliomas are hypointense or isointense to grey matter. On T2-weighted images, hyperintensity is often observed within the mass.,, It can be nonenhancing to slightly enhancing with contrast.
Histology of glioma consists of mature glial tissue, astrocytes, and fibrosis with fibrovascular tissues., On immunohistochemistry, nasal gliomas are strongly positive for S100 protein and glial fibrillary acid protein.
The differential diagnosis for external midline masses includes nasal dermoid, encephaloceles, teratoma, hemangiomas, lipomas, and nasolacrimal cysts. For intranasal gliomas, nasal polyps and encephaloceles are the common differentials. The treatment of these masses is surgical excision with primary closure of the defect., For extensive lesions, a multidisciplinary team involving otolaryngologist, neurosurgeon, ophthalmologist, and plastic surgeon may be required. Gliomas can slowly grow and cause cosmetic problems. Hence, early surgical intervention is needed. Extranasal gliomas can be excised through lateral rhinotomy, external rhinoplasty, midline nasal incision, or a bicoronal incision.,, Intranasal gliomas are usually excised using the endoscopic approach as it allows a clear view of each wall of the nasal cavity and precise excision with minimal damage to normal tissue. The endoscopic approach minimizes the chances of CSF leak and craniofacial resection surgeries.
This case exemplifies an extra nasal type of glioma without a intranasal or intracranial extension presenting as a cosmetic deformity. The patient was planned for surgical excision and primary closure through external approach. However, due to tissue loss and primary defect being larger in size, the forehead transposition flap was required for reconstruction.
| Conclusion|| |
Although nasal glioma is a benign nasal mass, it may prove quite challenging for the diagnosis and surgical resection. It may require a multidisciplinary team approach when larger in size for better functional and cosmetic results. Radioimaging enables presurgical planning, thereby reducing the complications associated with the surgery.
Declaration of patient
The authors certify that consent has been obtained for publication from the patient's guardian. The patient's parents have given consent for images and other clinical information to be reported in the journal. They have been informed that identifiable information will not be published and anonymity will be maintained.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]