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Introduction: Aim of the present study is to present an historical overview of the surgical techniques introduced for the treatment of the cancer of the optic nerve and orbit. Methods: From the late 19th and early twentieth century there were introduced many novel surgical techniques for the treatment of the cancer of the optic nerve and orbit. The majority of these techniques concerned for the cure of orbital malignance, while optic nerve cancer many times was treated by enucleation of the eyeball. Results: Except of the extraction of the malignant tumors the surgeons of the time tried to present the best aesthetic result, fact which points the progress of the surgery those years. Conclusions: Many of the techniques introduced those years are still in use.

Although orbital tumors involving the optic nerve are rare, it is well-known that they are very likely to cause serious visual impairment in a patient. Unfortunately, at present, there are no effective interventions that can reliably preserve visual function while controlling tumor growth into intracranial spaces. To ensure visual function of the non-affected side, transection of the optic nerve together with the tumors involved is necessary in some cases. For this procedure large craniotomy and orbital unroofing are commonly utilized. As an alternative, we propose a novel surgical intervention for transection of the optic nerve having optic nerve tumors, which utilizes a lateral orbitotomy approach. To evaluate the invasiveness of different surgical approaches, we compared the days of hospitalization after surgery across patients who underwent the transcranial, lateral, and anterior approaches, respectively. We successfully removed 2 optic nerve tumors using the lateral approach, which required significantly shorter hospitalization than the transcranial approach. The transection of the optic nerve together with tumor removal by the lateral approach may be one of the novel surgical interventions for optic nerve tumors as this method is considerably less invasive than the transcranial removal method., (Copyright © 2018 Elsevier Ltd. All rights reserved.)

Mesenchymal chondrosarcoma (MCS) is a rare tumor in the orbit. Although optic nerve displacement is a common finding in intraorbital MCS, optic nerve tissue involvement in tumor has rarely been reported in huge tumors associated with intracranial extension. Herein the authors report a patient with MCS involving optic nerve tissue without intracranial extension. A 59-year-old woman with a 2-month history of progressive proptosis and normal vision presented to us. Computed tomography revealed a clearly outlined heterogeneous mass with calcified foci in its center, which was attached to the optic nerve, magnetic resonance imaging showed the mass to be isointense to gray matter on T1- and T2-weighted images. She underwent lateral orbitotomy and partial tumor excision. Histopathologic study confirmed MCS. She refused exenteration till 1 year but the tumor recurred and her vision decreased to no light perception. Then exenteration was performed with obtaining free margin and she is now free of tumor after 6 months without radiotherapy or chemotherapy. Mesenchymal chondrosarcoma must be differentiated from more common calcified tumors attached to optic nerve like meningioma.

Optic nerve sheath meningiomas (ONSM) and intra-conal orbital lymphomas are common entities on the differential of a retrobulbar optic nerve involving space-occupying lesion. In this study, we compare the pre-surgical diagnosis, based on clinical presentation and neuroimaging, to the surgical pathology results of intra-conal orbital lymphomas and ONSM. This is an IRB approved retrospective chart review of orbital lymphomas and optic nerve sheath meningiomas biopsied by a single surgeon over a 4-year period at a single institution. Pre-surgical diagnosis and surgical pathology were compared. Fifteen cases of orbital lymphoma were identified. Fourteen were excluded based on extra-conal location. The single histologically confirmed intra-conal orbital lymphoma had a pre-surgical diagnosis of ONSM. Four cases of optic nerve sheath meningioma were identified. Three of the 4 cases of histologically confirmed ONSM had a pre-surgical diagnosis of ONSM. One of the 4 had a pre-surgical diagnosis of lymphoma. Diagnosis based on surgical pathology differed from the pre-surgical diagnosis in 2 out of 5 cases showing that clinical diagnosis does not always correlate with histologic diagnosis. Although both diseases are typically managed with radiation therapy, the treatment dosage and systemic disease implications are very different. These findings emphasis the importance of biopsy in the diagnosis of orbital lesions surrounding the optic nerve.

Objective: To retrospectively assess the clinicopathological characteristics of orbital granular cell tumours (GCTs)., Methods: A non-comparative review of the clinical characteristics, imaging, histopathological features, management, and prognosis of five cases of benign GCT and one case of malignant GCT (MGCT) was conducted, along with a review of the English language literature., Results: Among the six cases, four tumours were adherent to the extraocular muscle (EOM), and three tumours to the optic nerve (ON). Morphologic examinations revealed polygonal cells containing periodic-acid-Schiff-positive eosinophilic granules. All tumours (100%) were positive for VIM and NSE, five (83.3%) tumours were positive for S-100, and three (50%) tumours were positive for CD68. The follow-up examination of the MGCT witnessed recurrence and brain metastasis despite several thorough resections, but the patient remained alive; the follow-up examination of the four benign GCTs that had received incomplete excision revealed recurrence in one patient and dramatic shrinkage of the residual tumour in another; there was no recurrence in the other two patients., Conclusions: GCT should be considered in the differential diagnosis of orbital tumours, which may affect EOMs and ON. The natural course of GCT can include tumour progression, stability, or spontaneous regression. To avoid recurrence, complete resection is recommended for orbital GCT. To the best of our knowledge, primary orbital MGCT is reported for the first time.

Traumatic or amputation neuromas are neoformations developing after damage to a peripheral nerve. They are not proper tumors but rather a reactive process or a frustrated attempt of nerve regeneration. Traumatic neuromas are potentially found in every sensory peripheral nerve and often at the site of past surgical intervention, including orbital surgery. A 29-year-old Northern African migrant presented progressive exophthalmos and progressive loss of acuity in left eye, which had started about 6 months before after a cranio-facial trauma caused by a violent assault. MRI of the orbits showed a massive intra-orbital, intra-conical lesion, clearly compressing and dislocating the optic nerve and extending posteriorly to the orbital apex. Surgery was performed through lateral approach of Kroenlein and led to complete excision of the lesion. Histology revealed fibrotic, adipose and striated muscle tissues, a disordered, non-neoplastic overgrowth of small and large fascicles of nerves, inflammatory infiltrates, and fibrosis with sparse calcifications were diffusely observed in a background of fat, scar and striated muscle tissued. Patient was discharged on the fifth day in good health condition, without deficit of eye motion but without recovery of visual acuity. In conclusion, this case demonstrates that traumatic neuromas may arise in the orbit in patients with minor direct trauma to nerves and without previous surgical treatment.

Background: With the refinement of the technique, endoscopic endonasal surgery increases its field of indications. The orbital compartment is among the locations easily reached through the nostril. This anteromedial approach has been described primarily for inflammatory or traumatic diseases, with few data for tumoral diseases., Method: Since 2010, this route has been used at our institution either for decompression or for biopsy of orbital tumoral diseases., Findings/conclusions: Even if further studies are warranted, this strategy proved to be beneficial for patients, with improvements in visual outcome. In this article, the authors summarize their technique and their experience with endonasal endoscopic orbital decompression., Key Points: Nasal and sphenoidal anatomies determine the feasibility and risks for doing an efficient medial optic or orbit decompression. • Techniques and tools used are those developed for pituitary surgery. • A middle turbinectomy and posterior ethmoidectomy are mandatory to expose the medial wall of the orbit. • The Onodi cell is a key marker for the optic canal and must be opened up with caution. • The lamina papyracea is opened first with a spatula and the optic canal opened up by a gentle drilling under continuous irrigation from distal to proximal. • Drilling might always be used under continuous irrigation to avoid overheating of the optic nerve. An ultrasonic device can be used as well. • The nasal corridor is narrow and instruments may hide the infrared neuronavigation probe. To overcome this issue, a magnetic device could be useful. • Doppler control could be useful to locate the ICA. • The optic canal must be opened up from the tuberculum of the sella to the orbital apex and from the planum (anterior cranial fossa) to the lateral OCR or ICA canal • At the end of the procedure, the optic nerve becomes frequently pulsatile, which is a good marker of decompression.

In this article, the authors are presenting their experience and the results with the surgical treatment of intraorbital intraconal tumors based on a review of 33 constitutive cases. Our data were evaluated in comparison to other major series, and possible factors that might influence surgical outcome and survival are discussed. Thirty-three patients diagnosed with intraorbital intraconal tumors between 1998 and 2009 were treated by transcranial approach. Of these patients, there were 14 males (42.4 %) and 19 females (57.8 %). The age ranged between 2 and 70 years (mean = 36 ± 16.6 years). The follow-up period ranged between 2 and 13 years (mean = 7.3 ± 3.2 years). The most common presenting symptoms were exophthalmus and decreased visual acuity, which was seen in 21 (63.6 %) and 19 patients (57.6 %), respectively. Total resection was achieved in 23 patients (69.7 %) while subtotal resection was done in ten patients (30.3 %). Cavernoma and optic nerve sheath meningioma were the most common histologic variants, which were found in 11 (33.3 %) and 10 (30.3 %) patients, respectively. In the long-term follow-up, 54.5 % of the patients showed total ophthalmologic improvement, 9.1 % showed partial improvement, 21.2 % demonstrated unchanged ophthalmologic status, and 15.2 % showed worse ophthalmologic outcome. Transcranial approach for the treatment of intraorbital intraconal tumors is an effective approach for the management of these pathologies. The effectiveness is clearly demonstrated by the clinical results and outcomes of these patients' groups.

Exophthalmus is the leading sign of space-occupying lesions of the orbit. Patients may further present with lid swelling, impaired ocular motility and optic neuropathy including a relative afferent pupillary defect, compressive optic disc edema or optic atrophy. Orbital tumors can be classified into various categories depending on the etiology, as lymphoproliferative lesions (in particular non-Hodgkin's lymphoma as the most common malignant orbital tumor of adulthood), optic nerve and meningeal lesions, lacrimal gland lesions, secondary orbital tumors which extend to the orbit from neighboring structures and metastases. Slightly less common are vasculogenic and cystic lesions including cavernous hemangioma as the most common benign orbital tumor of adulthood and dermoid cysts as the most common benign orbital tumor of childhood. Rhabdomyosarcoma is the most common malignant orbital tumor of childhood but has a low total incidence. Orbital tumors might not only cause symptoms like pain, diplopia and loss of visual acuity but may also lead to esthetically disfiguring changes. Particular attention should be paid to underlying systemic diseases and generalized tumor diseases. This article illustrates the approach to a detailed clinical and neuroradiological assessment which is mandatory for the care of orbital tumor patients.

Intraorbital schwannomas and solitary neurofibromas account for 2 to 5% of all operated intraorbital tumors. Without any known neurofibromatosis, their diagnosis is only suggested with the MRI data, which displays a heterogeneous signal that may mimic a cavernous angioma. Their treatment is solely surgical., (Copyright 2010 Elsevier Masson SAS. All rights reserved.)

We present a case of benign mature orbital teratoma in a neonatal boy that involved the chiasmal area and cavernous sinus and displaced the nasopharynx. Visual potential was deemed to be good. Because of the chiasmal involvement, complete surgical resection, which would have compromised his visual function, was considered inadvisable. A debulking procedure, mainly of the cystic parts of the tumor, was performed. The patient remains stable at 3 years of age, with no further growth of the tumor and good vision in the affected eye.

A 36-year-old woman with bilateral optic neuropathy and orbital and central nervous system metastasis from breast carcinoma developed progressive bilateral vision loss with severe optic disc swelling. She underwent optic nerve sheath fenestration of the left eye resulting in modest improvement in vision and resolution of disc edema. Optic nerve sheath fenestration is a known treatment for papilledema, but its effectiveness as a treatment for an optic neuropathy caused by perineural or intrasheath metastasis is less clear. Optic nerve sheath fenestration should be considered as a treatment option for an optic neuropathy caused by perineural or intrasheath metastasis, especially in cases where alternative treatments are not tolerated and visual loss is severe at presentation.

We present an overview of the treatment and clinical outcome of 30 pediatric patients with orbital and optic pathway tumors cared for in our center from 1991 to 2002. Follow-ups were available for 48 months on average. Eight of 14 gliomas of the optic nerve or pathway were subtotally resected via a pterional approach. Two were totally resected with transection of the optic nerve, 2 were only biopsied and 2 were observed. One of 4 optic nerve sheath meningiomas in patients with good visual acuity was subtotally resected with decompression of the optic canal. Two were totally resected with transection of the prechiasmal optic nerve and 1 was only biopsied. Four infectious intra-/extraconal lesions were biopsied (transconjunctival approach) and treated with antibiotics. Two vascular intra-/extraconal tumors (hemangiomas) were removed via a transconjunctival approach and a lateral orbitotomy. The following histologies only occurred once: dermoid cyst, aneurysmal bone cyst, eosinophilic granuloma, fibrous dysplasia, osteopetrosis and rhabdomyosarcoma. The spectrum of pathology in pediatric orbital tumors is wide and requires multidisciplinary treatment., (Copyright 2003 S. Karger AG, Basel)