Any intrinsic or extrinsic compression anywhere along the optic nerve can produce compressive optic neuropathy (CON). Other than damage by compression, optic nerve damage can occur as a result of demyelination, ischemia, metabolic, and traumatic insult. The most common sign is a slow progressive monocular visual loss, sometimes associated with headaches. Bilateral visual loss can result from midline lesions (pituitary adenoma, craniopharyngioma, meningioma, giant aneurysms) or bilateral orbital lesions (thyroid disease, sarcoidosis). It is essential to correctly identify the cause of the CON as the differential diagnosis is broad, and management varies accordingly.
The optic nerve has over 1 million nerve fibers. This quantity of fibers demonstrates the complexity and importance that the visual system has had in our evolution. The visual pathway starts in the retina and ends in the visual cortex at the occipital lobe. The retina consists of two functional parts: the optic part and the non-visual retina. The optic part of the retina consists of the neural and pigmented layer. In contrast, the non-visual retina is an extension of the pigmented layer and ends in the ciliary and iridial parts of the retina.
The optic nerve begins in the lamina cribrosa of the sclera and exits the orbit through the optic canal. As it leaves the orbit, it is engulfed with an extension of the cranial dura and subarachnoid layer, which forms the optic nerve sheath. The axons of the ganglion cells project as the optic nerve and decussate in the optic chiasm and continue as the optic tract which courses to the lateral geniculate ganglion, which then projects to the primary visual cortex as the optic radiations. The optic nerve averages 50 mm in length (1 mm intraocular segment, 25 mm intraorbital segment, 9 mm intracanalicular segment, and 16 mm intracranial segment).
CON can be produced by an extrinsic or intrinsic lesion, causing a mass effect anywhere along the optic nerve. Rarely, an intrinsic lesion of the optic nerve (optic nerve glioma) can cause a slow compression damaging its axons. Many disorders can compress the optic nerve and can be categorized as follows:
Overall estimates from reported cases are 4 cases per 100,000 individuals per year. Gender, race, ethnicity, and age vary depending on the specific etiology.
Given that etiologies causing CON are ample, estimates of incidence vary with each etiology.
The optic nerve is part of the central nervous system (CNS). The mammalian CNS lacks the ability for regeneration and axonal growth. When the optic nerve is exposed to axonal damage, glial scars are formed that limits the diffusion of growth factors. Inhibitory proteins of myelin like Nogo and myelin-associated glycoprotein, low expression of growth factors, and lack of laminin are also some factors that hinder the ability for re-growth. The more proximal the damage is to the eye, the quicker the apoptosis of retinal ganglion cells will be. Apoptosis will lead to a cascade of p53 that will result in further cell death.
A CON can occur by compressing the vascular supply and causing ischemia to the nerve or directly causing mass effect upon the axons, thereby impairing axonal transport and signal transmission. The areas most susceptible to compression are where the nerve passes through small bone structures like the orbital apex and optic canal.
Optic nerve compression seen in exophthalmos secondary to thyroid disease occurs due to the enlargement of extraocular muscles due to the proliferation of fibroblasts, increased extracellular matrix, and adipocyte proliferation and differentiation.
Patients with CON usually present with chronic progressive vision loss. It can be in one or both eyes. They can present with headaches, nausea, vomiting, diplopia, dyschromatopsia, exophthalmos, afferent pupillary defect, photophobia, red-eye, or unexplained weight loss. Sudden or rapid visual loss are rare except for traumatic cases. These cases usually have a blunt trauma or a penetrating injury. The nerve can be injured at any part, but the orbital apex and the optic canal are the most susceptible areas to damage.
It is imperative to obtain a good history and physical exam to help narrow the broad differential diagnosis.
A full neurological examination followed by a complete ophthalmological evaluation should be performed. The exam will give a baseline visual acuity and monitor progression or improvement.
The eye with optic nerve compression will have reduced visual acuity. It can also show a deficiency in color vision (dyschromatopsia), which can be evaluated by using the Ishihara test plate. Proptosis or resistance to manual pressure, suggest an intraorbital lesion. Ocular motility abnormalities are checked. The optic disc can be atrophic or edematous but can also appear healthy. Optociliary shunt vessels can be seen due to obstruction in the venous return.
Laboratory studies include complete blood count, comprehensive metabolic panel, lipid panel, thyroid-stimulating hormone, T3, T4, luteinizing hormone, anti-thyroid antibodies, thyroid releasing hormone, follicle-stimulating hormone, prolactin, adrenocorticotropin hormone, insulin growth factor-1, cortisol, bone-specific alkaline phosphatase, and prostate-specific antigen. The angiotensin-converting-enzyme is usually elevated in more than half of the patients with active sarcoidosis.
Brain and orbit magnetic resonance imaging will show in detail the optic nerves, parasellar area, and orbital contents. Fat suppression images are needed to demonstrate enhancing lesions inside the orbit. Lesions involving the orbital bones should be examined using a computed tomographic (CT) scan of the head and orbit. It will demonstrate orbital fractures and concomitant injuries in traumatic cases. Ultrasonography can be used for intraorbital biopsy of anterior lesions.
The first step in management is to treat the underlying condition. Corticosteroids are beneficial for inflammation (sarcoidosis) and thyroid disease CON. For these conditions, withdrawal of the steroid treatment can cause acute deterioration of vision. Surgical orbital decompression can help CON caused by thyroid ophthalmoplegia. For tumors intimately attached to the optic nerve, like optic nerve meningiomas, surgery can cause further loss of vision. Radiation therapy is beneficial for aggressive recurrent tumors and those in areas adjacent to cranial nerves and eloquent brain. It can also be used for surgically difficult to reach tumors like the cavernous sinus. Radiation can cause irreversible optic nerve damage; therefore, it has to be used judiciously.
In traumatic cases, conservative treatment is appropriate in patients with mild deficits as spontaneous improvement is possible. Steroids have no benefit for trauma. Surgery is used for patients with radiological evidence of compression. Direct compression of the optic nerve by bone fragments or a subperiosteal hematoma is usually treated surgically. However, surgery carries the risk of complications such as postoperative cerebrospinal fluid leak and meningitis.
When evaluating for the specific etiology, the following common disorders have to be ruled out as their management differs:
The prognosis of CON depends on the pathophysiology (vascular insufficiency or axonal damage) and the time between presentation and treatment.
The recovery of visual symptoms is usually correlated with the length of time to decompress. The consensus in the literature is that the earlier the decompression, the better the outcome. Complete visual recovery has been seen as early as one week. Slow progressive vision improvements have also been reported. More than half of the patients improve irrespective of the pre-operative visual status. Early decompression is recommended, but good results can still be obtained even if decompression is performed delayed.
It is important to emphasize that prognosis of visual recovery varies given the etiology causing the optic nerve compression. For tumoral etiologies, negative predictive factors for visual improvement are the severity of visual loss, disc atrophy, recurrent tumor resection, an extension to the cavernous sinus, hard consistency of the tumor, absence of arachnoid plane, extensive tumor resection, and longer duration of visual loss. Overall, 60% of patients with tumors will experience some improvement after optic nerve decompression.
Complications of CON include papilledema, blurry vision, afferent pupillary defect, diplopia, nausea, vomiting, permanent vision loss, impaired extraocular movement, and impaired ability to perform activities of daily living due to impaired vision.
Treatment with decompressive surgery may lead to complications such as hemorrhage, infections, complete vision loss, prolonged intubation, coma, and death.
When vision is significantly affected, the patient needs to be educated to use safety glasses to protect the unaffected eye. All patients who developed CON should attend their regular ophthalmologist care visits to evaluate for visual improvement or deterioration.
As the etiologies of CON are multiple, the patient needs a complete evaluation by an interprofessional team to reach the cause and get urgent treatment to improve the outcome.
Early decompression is recommended, but good results can still be obtained even if decompression is delayed. Patients should see an ophthalmologist as soon as they have symptoms. More than half of the patients improve irrespective of the pre-operative visual status.
For tumoral etiologies, 60% of patients will experience some improvement after optic nerve decompression.
While the ophthalmologist is almost always involved in the care of patients with CON, it is essential to consult with an interprofessional team of specialists that include a neurosurgeon, neuro-ophthalmologist, neurologist, and endocrinologist. The nurses will assist with the education of the patient and family. In the postoperative period for eye care, the pharmacist will ensure that the patient is on adequate eye pharmacotherapy. The neuroradiologist plays a vital role in determining the cause. Many lesions have specific radiologic features that can distinguish from other differential diagnoses.
|||Bastakis GG,Ktena N,Karagogeos D,Savvaki M, Models and treatments for traumatic optic neuropathy and demyelinating optic neuritis. Developmental neurobiology. 2019 Aug; [PubMed PMID: 31297983]|
|||Jonas JB,Müller-Bergh JA,Schlötzer-Schrehardt UM,Naumann GO, Histomorphometry of the human optic nerve. Investigative ophthalmology [PubMed PMID: 2335441]|
|||Jonas JB,Schmidt AM,Müller-Bergh JA,Schlötzer-Schrehardt UM,Naumann GO, Human optic nerve fiber count and optic disc size. Investigative ophthalmology [PubMed PMID: 1582806]|
|||Sheremet NL,Khanakova NA, [Etiology and diagnostics of compressive optic neuropathies]. Vestnik oftalmologii. 2018; [PubMed PMID: 30721204]|
|||Brown P,Demaerel P,McNaught A,Revesz T,Graham E,Kendall BE,Plant G, Neuro-ophthalmological presentation of non-invasive Aspergillus sinus disease in the non-immunocompromised host. Journal of neurology, neurosurgery, and psychiatry. 1994 Feb; [PubMed PMID: 8126516]|
|||Khochtali S,Zayani M,Ksiaa I,Ben Meriem I,Zaouali S,Jelliti B,Khairallah M, [Idiopathic orbital inflammatory syndrome: Report of 24 cases]. Journal francais d'ophtalmologie. 2018 Apr; [PubMed PMID: 29685738]|
|||Lally E,Murchison AP,Moster ML,Bilyk JR, Compressive optic neuropathy from neurosarcoidosis. Ophthalmic plastic and reconstructive surgery. 2015 May-Jun; [PubMed PMID: 25427128]|
|||Saeed P,Tavakoli Rad S,Bisschop PHLT, Dysthyroid Optic Neuropathy. Ophthalmic plastic and reconstructive surgery. 2018 Jul/Aug; [PubMed PMID: 29927882]|
|||Daldoul N,Knani L,Gatfaoui F,Mahjoub H, [Management of moderate and severe dysthyroid orbitopathy: about 22 cases]. The Pan African medical journal. 2017; [PubMed PMID: 29187926]|
|||Yoshinaga T,Kurokawa T,Uehara T,Nitta J,Horiuchi T,Sekijima Y, [Optic neuropathy from connected intra- and extraorbital lesions in IgG4-related disease]. Rinsho shinkeigaku = Clinical neurology. 2019 Nov 8; [PubMed PMID: 31656266]|
|||Hwang G,Jin SY,Kim HS, IgG4-related disease presenting as hypertrophic pachymeningitis and compressive optic neuropathy. Joint bone spine. 2016 Oct; [PubMed PMID: 27068616]|
|||Oh JK,Chandhoke DK,Shinder R, Compressive optic neuropathy with vision loss due to IgG4-related orbital disease. Orbit (Amsterdam, Netherlands). 2019 Oct 8; [PubMed PMID: 31590594]|
|||Takeishi M,Oshitari T,Ota S,Baba T,Yamamoto S, The Case of IgG4-related Ophthalmic Disease Accompanied by Compressive Optic Neuropathy. Neuro-ophthalmology (Aeolus Press). 2018 Aug; [PubMed PMID: 30042797]|
|||Fukiyama Y,Oku H,Hashimoto Y,Nishikawa Y,Tonari M,Sugasawa J,Miyachi S,Ikeda T, Complete Recovery from Blindness in Case of Compressive Optic Neuropathy due to Unruptured Anterior Cerebral Artery Aneurysm. Case reports in ophthalmology. 2017 Jan-Apr; [PubMed PMID: 28611648]|
|||Tan AC,Farooqui S,Li X,Tan YL,Cullen J,Lim W,Leng SL,Looi A,Tow S, Ocular manifestations and the clinical course of carotid cavernous sinus fistulas in Asian patients. Orbit (Amsterdam, Netherlands). 2014 Feb; [PubMed PMID: 24195725]|
|||Patel SR,Rosenberg JB,Barmettler A, Interventions for orbital lymphangioma. The Cochrane database of systematic reviews. 2019 May 15; [PubMed PMID: 31094450]|
|||Berthout A,Jacomet PV,Putterman M,Galatoire O,Morax S, [Surgical treatment of diffuse adult orbital lymphangioma: two case studies]. Journal francais d'ophtalmologie. 2008 Dec; [PubMed PMID: 19107078]|
|||Hwang CS,Lee S,Yen MT, Optic neuropathy following endovascular coiling of an orbital varix. Orbit (Amsterdam, Netherlands). 2012 Dec; [PubMed PMID: 22571224]|
|||Young SM,Kim KH,Kim YD,Lang SS,Park JW,Woo KI,Lee JI, Orbital apex venous cavernous malformation with optic neuropathy: treatment with multisession gamma knife radiosurgery. The British journal of ophthalmology. 2019 Oct; [PubMed PMID: 30612095]|
|||Caton MT Jr,Zamani AA,Du R,Prasad S, Optic Neuropathy Due to Compression by an Ectatic Internal Carotid Artery Within the Orbital Apex. Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society. 2020 May 1; [PubMed PMID: 32366760]|
|||Fargen KM,Blackburn S, Surgical decompression for optic neuropathy from carotid artery ectasia: case report with technical considerations. World neurosurgery. 2014 Jul-Aug; [PubMed PMID: 23851213]|
|||Sabermoghaddam A,Sardabi M,Kiarudi MY,Shokoohi-Rad S, The role of early office-based needle aspiration in the management of subperiosteal hematoma. Orbit (Amsterdam, Netherlands). 2019 Oct; [PubMed PMID: 30335540]|
|||Zhilin G,Huoniu O,Zhihua C,Guorong D, Wide optic nerve canal decompression for the treatment of blindness resulting from an indirect optic nerve injury. The Journal of craniofacial surgery. 2011 Jul; [PubMed PMID: 21772157]|
|||Kong DS,Shin HJ,Kim HY,Chung SK,Nam DH,Lee JI,Park K,Kim JH, Endoscopic optic canal decompression for compressive optic neuropathy. Journal of clinical neuroscience : official journal of the Neurosurgical Society of Australasia. 2011 Nov; [PubMed PMID: 21924915]|
|||Kang H,Takahashi Y,Nishimura K,Yasuda M,Akutsu H,Kakizaki H, Improved Vision from Severe Compressive Optic Neuropathy by Apical Cavernous Hemangioma. Case reports in ophthalmology. 2016 Jan-Apr; [PubMed PMID: 27099610]|
|||Bulters DO,Shenouda E,Evans BT,Mathad N,Lang DA, Visual recovery following optic nerve decompression for chronic compressive neuropathy. Acta neurochirurgica. 2009 Apr; [PubMed PMID: 19255716]|
|||Dalle Ore CL,Magill ST,Rodriguez Rubio R,Shahin MN,Aghi MK,Theodosopoulos PV,Villanueva-Meyer JE,Kersten RC,Idowu OO,Vagefi MR,McDermott MW, Hyperostosing sphenoid wing meningiomas: surgical outcomes and strategy for bone resection and multidisciplinary orbital reconstruction. Journal of neurosurgery. 2020 Mar 6; [PubMed PMID: 32114535]|
|||Keilani C,Abada S, An uncommon case of symptomatic multiple meningiomas with bilateral compressive optic neuropathy rapidly induced under cyproterone acetate treatment. Current drug safety. 2017 May 23; [PubMed PMID: 28545357]|
|||Jiang L,Shi J,Liu W,Kang J,Wang N, [Clinical feature of chronic compressive optic neuropathy without optic atrophy]. [Zhonghua yan ke za zhi] Chinese journal of ophthalmology. 2014 Dec; [PubMed PMID: 25619179]|
|||Ziaei MM,Ziaei H, Compressive Optic Neuropathy Caused by Orbital Non-Hodgkin's Lymphoma. Case reports in ophthalmological medicine. 2012; [PubMed PMID: 22606505]|
|||Lee MJ,Kang MH,Yeom JH,Yeom H,Seong M,Cho H,Shin YU, Compressive optic neuropathy secondary to Ewing sarcoma in the paranasal sinus: A case report. Medicine. 2018 Nov; [PubMed PMID: 30383663]|
|||Mohammad Razali A,Mohd Zain A,Bt Wan Abdul Halim WH,Md Din N, Good Visual Outcome Following Corticosteroid Treatment for Compressive Optic Neuropathy Secondary to Sinonasal Carcinoma. Cureus. 2020 Apr 18; [PubMed PMID: 32440379]|
|||Cheour M,Mazlout H,Agrebi S,Falfoul Y,Chakroun I,Lajmi H,Kraiem A, [Compressive optic neuropathy secondary to a pituitary macroadenoma]. Journal francais d'ophtalmologie. 2013 Jun; [PubMed PMID: 23623769]|
|||Bianchi-Marzoli S,Rizzo JF 3rd,Brancato R,Lessell S, Quantitative analysis of optic disc cupping in compressive optic neuropathy. Ophthalmology. 1995 Mar; [PubMed PMID: 7891982]|
|||Tannan A,Jhaveri M,Moftakhar R,Munich S,Harbhajanka A,Cohen AJ, Compressive optic neuropathy secondary to a lateral rectus muscle dermoid cyst. Ophthalmic plastic and reconstructive surgery. 2015 May-Jun; [PubMed PMID: 24833454]|
|||DeKlotz TR,Stefko ST,Fernandez-Miranda JC,Gardner PA,Snyderman CH,Wang EW, Endoscopic Endonasal Optic Nerve Decompression for Fibrous Dysplasia. Journal of neurological surgery. Part B, Skull base. 2017 Feb; [PubMed PMID: 28180039]|
|||MacNally SP,Ashida R,Williams TJ,King AT,Leatherbarrow B,Rutherford SA, A case of acute compressive optic neuropathy secondary to aneurysmal bone cyst formation in fibrous dysplasia. British journal of neurosurgery. 2010 Dec; [PubMed PMID: 20649393]|
|||Eretto P,Krohel GB,Shihab ZM,Wallach S,Hay P, Optic neuropathy in Paget's disease. American journal of ophthalmology. 1984 Apr; [PubMed PMID: 6720822]|
|||Pletcher SD,Metson R, Endoscopic optic nerve decompression for nontraumatic optic neuropathy. Archives of otolaryngology--head [PubMed PMID: 17709616]|
|||Loke JY,Mohd Ali H,Kamalden TA, Osteopetrosis craniopathy: a rare cause of bilateral compressive optic neuropathy and facial nerve palsy. Postgraduate medical journal. 2019 Sep; [PubMed PMID: 31292279]|
|||Medsinge A,Sylvester C,Tyler-Kabara E,Stefko ST, Bilateral endoscopic optic nerve decompression in an infant with osteopetrosis. Journal of AAPOS : the official publication of the American Association for Pediatric Ophthalmology and Strabismus. 2019 Feb; [PubMed PMID: 30171897]|
|||Bae JW,Kim YH,Kim SK,Wang KC,Shin HY,Kang HJ,Park SH,Phi JH, Langerhans cell histiocytosis causing acute optic neuropathy. Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery. 2015 Apr; [PubMed PMID: 25566821]|
|||Tzamalis A,Diafas A,Riga P,Konstantinidis I,Ziakas N, Onodi Cell Mucocele-Associated Optic Neuropathy: A Rare Case Report and Review of the Literature. Journal of current ophthalmology. 2020 Jan-Mar; [PubMed PMID: 32510023]|
|||Li E,Howard MA,Vining EM,Becker RD,Silbert J,Lesser RL, Visual prognosis in compressive optic neuropathy secondary to sphenoid sinus mucocele: A systematic review. Orbit (Amsterdam, Netherlands). 2018 Aug; [PubMed PMID: 29303386]|
|||Bhattacharjee H,Soibam R,Deori N, Sphenoethmoidal mucocele presenting with unilateral visual loss. Eye and brain. 2010; [PubMed PMID: 28539770]|
|||Sivadasan A,Alexander M,Mathew V,Mani S,Patil AK, Radiological evolution and delayed resolution of an optic nerve tuberculoma: Challenges in diagnosis and treatment. Annals of Indian Academy of Neurology. 2013 Jan; [PubMed PMID: 23661979]|
|||Tanda ML,Piantanida E,Liparulo L,Veronesi G,Lai A,Sassi L,Pariani N,Gallo D,Azzolini C,Ferrario M,Bartalena L, Prevalence and natural history of Graves' orbitopathy in a large series of patients with newly diagnosed graves' hyperthyroidism seen at a single center. The Journal of clinical endocrinology and metabolism. 2013 Apr [PubMed PMID: 23408569]|
|||Hiromatsu Y,Eguchi H,Tani J,Kasaoka M,Teshima Y, Graves' ophthalmopathy: epidemiology and natural history. Internal medicine (Tokyo, Japan). 2014 [PubMed PMID: 24583420]|
|||Bishokarma S,Shrestha S,Ranabhat K,Koirala S,Shrestha D,Panth R,Gongal DN, Outcome of Surgical Resection of Craniopharyngioma:Single Center 12 Years' Experience. Kathmandu University medical journal (KUMJ). 2018 Oct.-Dec. [PubMed PMID: 31729348]|
|||[PubMed PMID: 23966029]|
|||Haik BG,Karcioglu ZA,Gordon RA,Pechous BP, Capillary hemangioma (infantile periocular hemangioma). Survey of ophthalmology. 1994 Mar-Apr [PubMed PMID: 8009426]|
|||Léauté-Labrèze C,Harper JI,Hoeger PH, Infantile haemangioma. Lancet (London, England). 2017 Jul 1 [PubMed PMID: 28089471]|
|||Hattori Y,Tahara S,Aso S,Matsui H,Fushimi K,Yasunaga H,Morita A, Pituitary surgery's epidemiology using a national inpatient database in Japan. Acta neurochirurgica. 2020 Jun [PubMed PMID: 32125502]|
|||[PubMed PMID: 25057976]|
|||Revilla-Pacheco F,Escalante-Seyffert MC,Herrada-Pineda T,Manrique-Guzman S,Perez-Zuniga I,Rangel-Suarez S,Rubalcava-Ortega J,Loyo-Varela M, Prevalence of Incidental Clinoid Segment Saccular Aneurysms. World neurosurgery. 2018 Jul [PubMed PMID: 29656153]|
|||Etminan N,Rinkel GJ, Unruptured intracranial aneurysms: development, rupture and preventive management. Nature reviews. Neurology. 2016 Dec [PubMed PMID: 27808265]|
|||Shum JW,Liu K,So KF, The progress in optic nerve regeneration, where are we? Neural regeneration research. 2016 Jan; [PubMed PMID: 26981073]|
|||Gibson A,Czyz CN, Graves Disease, Orbital Decompression 2020 Jan; [PubMed PMID: 29261978]|
|||Woods RSR,Pilson Q,Kharytaniuk N,Cassidy L,Khan R,Timon CVI, Outcomes of endoscopic orbital decompression for graves' ophthalmopathy. Irish journal of medical science. 2020 Feb; [PubMed PMID: 31203506]|
|||Boboridis KG,Bunce C, Surgical orbital decompression for thyroid eye disease. The Cochrane database of systematic reviews. 2011 Dec 7; [PubMed PMID: 22161415]|
|||Kumaran AM,Sundar G,Chye LT, Traumatic optic neuropathy: a review. Craniomaxillofacial trauma [PubMed PMID: 25709751]|
|||Wilhelm H, [Traumatic optic neuropathy]. Laryngo- rhino- otologie. 2009 Mar; [PubMed PMID: 19247896]|
|||Yu-Wai-Man P,Griffiths PG, Surgery for traumatic optic neuropathy. The Cochrane database of systematic reviews. 2005 Oct 19; [PubMed PMID: 16235388]|
|||Jayaraman M,Gandhi RA,Ravi P,Sen P, Multifocal visual evoked potential in optic neuritis, ischemic optic neuropathy and compressive optic neuropathy. Indian journal of ophthalmology. 2014 Mar; [PubMed PMID: 24088641]|
|||Carlson AP,Stippler M,Myers O, Predictive factors for vision recovery after optic nerve decompression for chronic compressive neuropathy: systematic review and meta-analysis. Journal of neurological surgery. Part B, Skull base. 2013 Feb; [PubMed PMID: 24436885]|
|||Cook C, Emergency management: optic nerve compression. Community eye health. 2018; [PubMed PMID: 30487682]|