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Figure 7 Meningioma （略）
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 （略）
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 subtotal 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 welldefined, 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 Elephantiasis Neuromatosa and underlying tortuous, ropey, tangled nerves produce a characteristic "Palpable Bag of Worms" feelings on palpation[15]. The slit lamp examination shows characteristic 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 supraorbital 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（略）
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 caféaulait patches are also visible
Figure 11 Acoustic neuroma （略）
A: Fourtyeight 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
Neurofibromatosis is primarily a neuroectodermal dysplasia characterized by tumor like formations derived from proliferation of peripheral nerve elements. It was identified as a distinct entity by VonRecklinghausen in 1882, after whom this disease is named. Two forms of neurofibromatosis have been described. The classic VonRecklinghausen disease as neurofibromatosis 1 (NF1) and bilateral acoustic neurofibromatosis 2 (NF2)[16]. NF1 (formerly known as peripheral neurofibromatosis) is the most common gene disorder affecting central nervous system (1 in 3000). It is autosomal dominant with variable expressivity and has a high spontaneous mutation rate. It is on the long arm of chromosome 17. The malignant lesion in NF1 may involve deletion of both copies of the tumor suppressor gene. The major ocular manifestations are Lisch iris nodules and orbital features of plexiform neurofibromas, dysplasia of the sphenoid wing, and optic nerve glioma. Rare features are prominent corneal nerves, perilimbal neurofibromas, congenital megaglobus, glaucoma, pigmentary hamartomas of the uveal tract, retinal astrocytoma and hamangiomas. The cutaneous manifestations are neurofibromas and caféaulait patches. Similarly we have seen 3 patients of NF1 with lisch iris nodules in one case, caféaulait patches in two cases in association of optic nerve glioma, plexiform neurofibroma (Figure 6, 10). NF2 (formerly called central neurofibromatosis) is characterized by the development of coustic neuromas, and less frequently, meningiomas, spinal nerve root schwanomas, and presenile posterior subcapsular lens opacities. It is autosomal dominant but ten times more rare than NF1. The gene for NF2 is situated on chromosome 22. The major findings of NF2 are bilateral acoustic neuromas or unilateral acoustic neuroma associated with any of two of the following neurofibroma, meningioma, glioma schwanoma, juvenile posterior subcapsular lens opacities[17]. In our study there was one case of unilateral neurofibromatosis type 2 presented with slow progressive dimness of vision in both eyes, decreasing hearing, headache and decreased corneal sensitivity. The CT scan showed left side acoustic neuroma (Figure 11). The patient referred to the neurosurgeon for further treatment. Lacrimal gland benign mixed cell Tumor (pleomorphic adenoma) is called as pleomorphic because of its widely varying histologic picture. In our study, 5 patients with benign mixed cell tumor of lacrimal gland were presented in between 2nd to 5th decade of life with slight female to male preponderance of 3:2, Sshaped lid swelling, mild to moderate tender superolateral mass, downward and inward displacement of globe of gradual onset, mass induced diplopia, decreasing vision. The CT scan demonstrates pressure indentation and expansion of the lacrimal fossa in most cases. The complete extirpation via a modified lateral orbitotomy with intact capsule was done in three cases and with accidental rupture of capsule in two cases. The excised mass biopsy confirms pleomorphic adenoma (Figure 12).
Figure 12 Lacrimal gland pleomorphic adenoma (benign mixed cell tumor) （略）
A: Postlateral orbitotomy photograph of 42 years old female with superiolateral orbital swelling (left side); B: The CT scan (axial view) of the same patient showing lacrimal gland growth on the left side; C: Xray skull (PA view) of the same patient showing increased density on the superiolateral part of the left orbit
The development of multidisciplinary and microsurgical approaches has substantially improved management. The key issue is to develop a management plan that allows one to arrive at the correct diagnosis and minimize the risk of recurrence. In this study, we have concluded that early arrival, careful evaluation, proper diagnosis and treatment can prevent the patient from visual and life threatening complication.
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