¡¡¡¡Optic nerve glioma was first described in 1821 by PANIZA, and over 700 optic nerve gliomas have been described in the literature since. Approximately 75% of cases have occurred in patients less than 10 years of old; most present between the ages of 5 and 8. Optic nerve gliomas account for approximately 5% to 10% of pediatric orbital tumors. This tumor may occur as an isolated finding or as a part of neurofibromatosis. In patients with neurofibromatosis, the incidence of optic nerve glioma is between 12% and 38%. Conversely, approximately 25% to 50% of patients with optic nerve glioma have neurofibromatosis[11]. In our study, there were 7 cases of optic nerve gliomas of age range in 4ª²13 years and were comprised 10.29% of total 68 primary benign orbital tumors. Five presented with unilateral axial proptosis, two with unilateral nonª²axal proptosis. The proptosis was insidious, nonª²reducible, nonª²pulsatile, and with mild to moderate tenderness in all cases. Visual loss was moderate to severe (unilateral 5 cases, bilateral 2 cases) and with strabismus in 2 cases. The pupil showed relative afferent pupillary defect in 5 cases, afferent pupilliary defect in 2 cases and fundus showed disc hyperemia in 2 cases, disc pallor in 2 cases, disc swelling with blurred margin in 3 cases and chorioª²retinal folds at posterior pole in 4 cases. The CTª²scan showed conical shaped mass surrounding the intra orbital part of optic nerve in 3 cases, fusiform shaped mass around the orbital and cranial part of optic nerve in 2 cases, oval shaped mass along the chiasm in 1 case and multiple, small, round masses along the chiasm and optic tract in 1 case. The 4 (57.1%) out of 7 patients showing caf¨¦ª²auª²lait spots on the skin of back, leg, arms, buttocks, neck (Figure 6)and 2 of them were associated with Neurofibroma. One case of orbital glioma with minimal proptosis (2mm), stationary vision was observed for 5 years and more, and 2 patients of orbital glioma with moderate (2ª²4mm) proptosis, decreasing vision were underwent for excision of tumor through lateral orbitotomy (Figure 6). Two patients of orbitoª²cranial gliomas were referred to neurosurgeon where pre and para chiasmal tumors were treated by enblock surgical excision of the involved nerve just anterior of chiason and finally 2 cases of chiasmal, diffuse multifocal gliomas with significant proptosis and visual loss were treated by chemotherapy.

¡¡¡¡Optic nerve sheath meningioma is the most common adult optic nerve tumor. Meningiomas account for approximately 20% of the central nervous system tumors; less than 15% secondarily involve the orbit. Orbital involvement by meningiomas can occur as a result of primary intraorbital neoplasm, almost always arising from the optic nerve sheath, or secondarily from a meningioma that involves the orbit by contiguous spread from the central nervous system. Orbital involvement by meningiomas, both primary and secondary, occurs more frequently in females[12]. Similarly we have seen optic nerve sheath meningiomas in 3 middle aged females and in 2 males with symptoms and signs of decreased vision, headache, diplopia, relevant afferent pupillary defect, optic disc edema in 3 cases, optocilliary shunt vessels in 2 cases, minimal axial proptosis in 4 cases and significant nonaxial

¡¡¡¡Figure 7  Meningioma  £¨ÂÔ£©

¡¡¡¡A: Right post-craniotomy phtograph of  62 years old male with meningioma-(biopsy proven) showing right side proptosis and right temporalis fossa excavation; B: Right post-craniotomy CT scan brain (axial view) of same patient showing hyperdense recurrent mass and excavation at right frontotemporal region

¡¡¡¡Figure 8  Schwannoma £¨ÂÔ£©

¡¡¡¡A: Sixteen years old girl showing downward and inward proptosis of right eye; B: The CT scan of orbit (axial view) of same patient showing the conical shaped mass occupying the superiolateral part of the right orbit; C: Photomicrograph (x200) of mass biopsy of same patient showing neuroglial tissue with mild pleomorphism and increased cellularity (double arrow). Endothelial cells and microcystic space (arrow) are seen proptosis in 1 case, enlargement of temporal fossa with late intracranial symptoms (headache, nausea, vomiting and altered consciousness) in 2 cases. The CT Scan and MRI (Brain + Orbit) shows involvement of middle and outer third of sphenoid 3 cases, olfactory groove 1 case, parasellar 1 case and involvement of orbital tissues in all cases. Out of these 5, 4 patients with small orbital optic nerve sheath meningiomas with minimal proptosis were referred to neurosurgeon where subª²total excision of tumor was done and then followed by local radiotherapy (5500 cGy over a 6 week period) ( Figure 7) and 1 patient with large orbital optic nerve sheath meningioma with significant proptosis and blind eye was treated by resection of orbital portion of optic nerve through lateral orbitotomy leaving an intact eye and then followed by local radiotherapy of intracranial mass.

¡¡¡¡Schwannomas (Neurilemomas) are pure proliferation of schwan cells. In the orbit they represent 1% to 2% of tumors and are wellª²defined, encapsulated, slow progressive tumors that develop as eccentric growths from peripheral nerves. They may or may not be associated with neurofibromatosis type 1. The tumors tend to become clinically  symptomatic after the first two decades of life[13]  and are therefore usually seen in young to middle aged adults as a cause of slowly progressive proptosis. Uncommonly, they present in childhood. Ocular complaints depend on the location of the tumor and its size[14]. We have seen two patients of 18 and 22 years old with minimal to moderate size eccentric proptosis of slow onset with decreased vision, relative afferent pupillary defect and restricted extraocular movements of affected eye which were managed by excision of tumor through lateral and superior orbitotomy respectively. The excised mass biopsy shows schwanoma (Figure 8).

¡¡¡¡Neurofibroma histologically, are quite distinct from schwanomas. Schwanomas are pure proliferations of schwan cells, where as neurofibromas consist of a mixture of schwan cells, peripheral nerve axons, endoneurial fibroblasts, and perineural cells. There are three typical subtypes of orbital neurofibromas: localized, plexiform and diffuse. In our study a young female of localized neurofibroma was presented with a superior localized soft tissue left orbital swelling, pain, diplopia, proptosis due to mass effect (Figure 8). Through superior orbitotomy the excised mass biopsy confirm localized neurofibroma. Two patients of plexiform neurofibroma were presented with thickened, pendulous soft tissues of the lid, periorbita, and face with varying degree of proptosis and facial disfigurement. The affected overlying skin called as Eleª²phantiasis Neuromatosa and underlying tortuous, ropey, tangled nerves produce a characteristic "Palpable Bag of Worms" feelings on palpation[15]. The slit lamp examination shows charaª²cteristic round, dome shaped, avascular, gray colour nodules called Lisch Iris Nodules. In this case debulking surgery for cosmetic purpose was performed (Figure 9).

¡¡¡¡Figure 9  Localizad neurofibroma  A: Sixteen years old girl with left eye proptosis and supraª²orbital swelling. Through superior orbitotomy the excised mass biopsy showing  localized neurofibroma; B: CT scan orbit (axial view) of the same patient showing soft tissues mass, missing of sphenoid bone and displacement of the globe on the left side£¨ÂÔ£©

¡¡¡¡Figure 10  Plexiform neurofibroma. Thirteen years female after 3 years of superior orbitotomy showing recurrent soft tissue swelling of the right lids, periorbita and face. On the left side of neck caf¨¦ª²auª²lait patches are also visible

¡¡¡¡Figure 11  Acoustic neuroma  £¨ÂÔ£©

¡¡¡¡A: Fourtyª²eight years old female with acoustic neuroma showing left eye loss of corneal sensitivity; B: The CT scan brain (axial view) of same patient showing hyperdense mass along the left side of brain. The biopsy showed acoustic neuroma

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