Year : 2005 | Volume
: 15 | Issue : 4 | Page : 511--516
Heterotopic central Nervous tissue - nasal glioma : A case report
LG Krishna, R Uppoor, KN Rao, K Harish
Dept. of Radiology, Vijaynagar Inst. of Medical Science, Bellary, India
Dept. of Radiology, Vijayanagar Institute of Medical Sciences, Conturment. Bellary - 583104
|How to cite this article:|
Krishna L G, Uppoor R, Rao K N, Harish K. Heterotopic central Nervous tissue - nasal glioma : A case report.Indian J Radiol Imaging 2005;15:511-516
|How to cite this URL:|
Krishna L G, Uppoor R, Rao K N, Harish K. Heterotopic central Nervous tissue - nasal glioma : A case report. Indian J Radiol Imaging [serial online] 2005 [cited 2020 Apr 3 ];15:511-516
Available from: http://www.ijri.org/text.asp?2005/15/4/511/28785
Nasal gliomas are rare congenital midline tumours often misdiagnosed as encephalocele, midline dermoids and even as hemangiomas. By a proper systematic approach clinically, sonologically & including CT/MRI evaluation, a near definitive diagnosis can be made; however the gold standard is surgical excision and histopathological confirmation.
Case Report (Materials & Methods)
A 3 months old female bsaby presented with a swelling at the root of the nose (more towards left). The swelling was firm-to hard in consistency, non-tender, non pulsatile, non-compressible, non-reducible with posture/pressure. This swelling was covered by bluish red colored skin. The swelling gradually increased to attain the size of about 5 x3cms at the time of presentation.
The other biochemical investigations were within normal limits.
Ultrasonography of this extra nasal midline mass was performed with SEIMENS SONOLINE OMNIA USG machine using 2.5- 5.0 MHz convex probe and 5- 9 MHz linear probe. A lobulated heterogeneous mass lesion, predominantly hypoechoic with interspersed septations was noted at the root of the nose, which had an arterial supply through the bony defect in the frontal region. The vessel showed pulsatile flow and arterial waveform pattern on color & spectral Doppler tracings. Transcranial USG was normal.
CT scan was done with Toshiba Asteion single slice spiral CT. A well defined soft tissue attenuation lesion, at the root of the nose was seen with an underlying bony defect measuring 4mm and absent crista galli. A thick soft tissue cord like linear stalk was seen coursing towards the floor of the anterior cranial fossa from the above mentioned bony defect which appeared to merge with the basal frontal lobe. The lesion measured 2.3 x 1.9 x 2.5cm. The lesion showed mild heterogenous enhancement. No evidence of fat/calcification/fluid was noted within the lesion. Intracranial neuroparenchyma and ventricular systems were unremarkable. The lesion was diagnosed as nasal glioma with a thick connecting stalk extending to the base of the frontal lobe.
Surgical excision and subsequent histopathological evaluation, confirmed the lesion as low grade fibriallary astrocytoma .
Developmental midline nasal masses in children are rare lesions that occur in about one in 20,000-40,000 live births. Neuroimaging is essential to characterise these lesions, to determine the exact location of the lesion and most importantly to exclude a possible intracranial extension or connection. Neuroimaging was evaluated for (a) lesion location/size, (b) indirect (bifid or deformed crista-galli, widened foramen caecum, defect of the cribriform plate) and direct (identification of intracranially located lesion components or signal alterations) imaging signs of intracranial extension, (c) secondary complications and (d) associated malformations. Surgical and histological findings serve as gold standard.
Nasal gliomas are CNS masses of neurogenic origin, which have lost their intracranial connections and present as an obvious extranasal or intranasal mass at birth without associated surgical symptoms of encephalocele. Nasal gliomas arise from a skull defect, originating from the defective closure of the anterior neuroporus. They represent encephaloceles, which have lost their intracranial connection. The tumor is considered to arise from ectopic neuroectodermal tissue projected through the foramen cecum into the developing nasofrontal cranium and sequestered there on closure of the cranial sutures. Careful evaluation is required to confirm the diagnosis and appropriate management.
To understand the development of congenital midline nasal masses, knowledge of the normal embryological development of the nose is important.
The face develops during first twelve weeks of fetal growth. The fonticulus nasofrontalis is the space between the frontal and nasal bones while the prenasal space lies between the nasal bones and the nasal capsule and forms the precursor of the septum and nasal cartilages. During fetal development these spaces are normally closed by fusion and ossification. Abnormal development of these structures is thought to be involved in the formation of dermoids, gliomas, and encephaloceles of the nose. Gliomas however are thought to occur as a result of abnormal closure of the fonticulus nasofrontalis which leads to an ectopic rest of glial tissue being left extracranially. This is similar to the mechanism for the formation of encephaloceles, however there is not always an intracranial connection to a glioma unlike an encephalocele which, by definition retains an intracranial connection.
Gliomas are made of neuroglial elements consisting of glial cells in a connective tissue matrix with or without a fibrous connection to the dura. Essentially they consist of fibrillary neuroglial tissue with a prominent network of glial fibres, gemistocytic type astrocytes in background of neuropil, representing classic neuroglial tissue. There is no fluid filled space connected to the subarachnoid space. These lesions usually present as a red or bluish lump at or along the nasomaxillary suture, or as an intranasal mass. They are characteristically firm, non compressible, do not increase in size with crying, and do not transilluminate. The overlying skin may have telangiectasias. They can be associated with a widened nose or with hypertelorism secondary to growth of the mass.
The interpretation of CT and MR images can be difficult but is useful in differentiating nasal gliomas from other congenital nasal masses. The presence of a fibrous stalk may be associated with cranial defects and CSF leak.
Types of nasal gliomas:
• Extra nasal (60%) : lie external to the nasal bones and nasal cavity and most commonly occur slightly off the midline at the bridge of the nose
• Intranasal (30%) : found within the nasal or nasopharyngeal cavity, the
oral cavity or rarely the pterygopalatine fossa.
• Combined (10%) : consists of a communication between the extranasal and intranasal components which occurs via a defect in the nasal bones or around the lateral edges of the nasal bones
Associated congenital anomalies
Anomalies occur in 5 - 41% of cases which include aural atresia, mental retardation, spinal column abnormalities, hydrocephalus, hypertelorism, hemifacial microsomia, albinism, corpus callosal agenesis, cerebral atrophy, lumbar lipoma, dermal cyst of the frontal lobe, coronary artery anomaly, cleft lip and palate, tracheoesophageal fistula, cardiac, genital, and cerebral anomalies. There is no known syndromic association of these anomalies.
Ultrasound is useful for determining the mass as cystic or solid. Doppler flow studies of nasal gliomas reveal a characteristic arterial flow of low velocity during the end-diastolic phase. CT/ MRI characterize these lesions more accurately with their intracranial extension/ associated anomalies.
Our case is an extra nasal type of nasal glioma with a fibrous stalk/ pedicle and defect in cribriform plate of ethmoid bone
Differential diagnosis :
Encephalocele ethmoid/nasal :
Frontal encepahalocele is most common in Southeast Asia, Malaysia and Russia. It may present as a mass along the nose, orbital margin or forehead. Small lesions may go undetected. When large enough to cause separation of the midline structures they cause hypertelorism. Fronto nasal encephalocele represents herniation of brain substance into the nasal cavity through a congenital defect in the skull, just anterior to the crista galli. This may extend extranasally through the frontonasal suture. If such an encephalocele becomes pinched off and isolated from the cranial cavity, it is nasal glioma.
Nasal dermoids :
Nasal dermoids are thought to result from a lack of regression of a diverticulum of dura that extends through the foramen ceacum between the developing nasal cartilage and nasal bone. Depending on the patency of the diverticulum and its contents, the lesion can be a sinus, dermoid cyst, nasal glioma or encephalocele. It
may be sporadic or familial. The mean age of presentation is 3 yrs with no sexual predeliction. They may be found at any location between the glabella and the base of the columella.
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