Gross-total resection in optic nerve sheath meningiomas: minimally invasive and cosmetic pleasing.

PURPOSE: The optic nerve sheath meningioma (ONSM) is one of the most challenging tumors in orbital surgery. From the perspective of mental health and patient needs, we analyzed the necessity and importance of the endoscopic transnasal approach (ETA) combined with optic nerve transection (ONT) in gross-total resection (GTR) in ONSM patients with residual vision and aim to broaden the use of ONT for specific people. METHODS: The authors included patients with ONSMs who were treated between 2014 and 2022. We divided those cases into two groups named ETA group and lateral orbitotomy approach (LOA) group. We present the application of ETA and analyze the preoperative indication of the ONT and compared the advantages and disadvantages between ETA and LOA. The degree of tumor resection was based on imaging and surgical evaluation. RESULTS: A total of 23 patients with ONSM were included. Sixteen patients underwent ETA, and seven underwent LOA. Among ETA cases, GTR was achieved in 14 patients with ONT and most patients maintained normal eye movement function (75%) and morphology (93.75%). In the ETA group, 14 patients experienced vision loss, while two other patients saw improvements in vision. And proptosis was alleviated (5.20 ± 2.34 vs 0.27 ± 0.46, p < 0.0001). Six patients with blindness and proptosis of the LOA group resulted in GTR with ONT and ophthalmectomy. Although intracranial extension and recurrence included no cases in the two groups, a significant psychological gap was presented due to cosmetic problems. CONCLUSIONS: Under the premise of reducing damage and improving aesthetics, the selection of ETA combined with ONT to gross-total resect ONSMs successfully provides a minimally invasive access with acceptable complications. As an important adjunct to GTR in the surgical treatment of ONSM, the scope of ONT application should be expanded to relieve the patient's psychological burden.

Optic nerve decompression through pterional and supraorbital approaches in the treatment of severe traumatic optic neuropathy.

To investigate the effectiveness of optic nerve decompression (OND) in the treatment of severe traumatic optic neuropathy (TON) through pterional and supraorbital approaches, and to identify the prognostic factor for postoperative visual acuity (VA) following OND. Patients with severe TON treated with OND through either pterional or supraorbital approach in our institute from September 2019 to June 2022 were retrospectively reviewed in this study. Demographic information, trauma factors, the interval between trauma and complete blindness, the interval between trauma and surgery, and the associated craniofacial traumas were recorded. Hospitalization days and the postoperative VA of patients in two groups were compared. There were 54 severe TON patients with NLP included in this study; 21 patients underwent OND through the pterional approach, and the other 33 underwent the supraorbital approach. Respectively, in groups of pterional and supraorbital approaches, the average hospitalization days were 9.8 ± 3.2 and 10.7 ± 2.9 days (p = 0.58), the mean durations of follow-up were 18.9 ± 4.3 and 20.8 ± 3.7 months (p = 0.09), and the average circumference of OND were 53.14 ± 15.89 ◦ (range 220 ◦ -278◦) and 181.70 ± 6.56◦ (range 173 ◦ -193◦) (p<0.001). The overall improvement rates of pterional and supraorbital approaches are 57.1% and 45.5% (p = 0.40), respectively. Optic canal fracture (OCF) was revealed to be significantly associated with postoperative VA in the supraorbital approach (Binary: p = 0.014, CI: 1.573-57.087; Ordinal: p = 0.003, CI: 1.517-5.503), but not in the pterional approach. In the group of supraorbital approach, patients with OFC had a higher rate of a better outcome (78.6%) than those without (21.4%). Patients with severe traumatic TON may benefit from OND through either the pterional or supraorbital approach. OCF is a potential prognostic factor for postoperative VA following OND through the supraorbital approach.

Improved optic nerve visualization and treatment planning through a dedicated optic nerve MRI protocol.

OBJECTIVE: This study describes an innovative optic nerve MRI protocol for better delineating optic nerve anatomy from neighboring pathology. METHODS: Twenty-two patients undergoing MRI examination of the optic nerve with the dedicated protocol were identified and included for analysis of imaging, surgical strategy, and outcomes. T2-weighted and fat-suppressed T1-weighted gadolinium-enhanced images were acquired perpendicular and parallel to the long axis of the optic nerve to achieve en face and in-line views along the course of the nerve. RESULTS: Dedicated optic nerve MRI sequences provided enhanced visualization of the nerve, CSF within the nerve sheath, and local pathology. Optic nerve sequences leveraged the "CSF ring" within the optic nerve sheath to create contrast between pathology and normal tissue, highlighting areas of compression. Tumor was readily tracked along the longitudinal axis of the nerve by images obtained parallel to the nerve. The findings augmented treatment planning. CONCLUSIONS: The authors present a dedicated optic nerve MRI protocol that is simple to use and affords improved cross-sectional and longitudinal visualization of the nerve, surrounding CSF, and pathology. This improved visualization enhances radiological evaluation and treatment planning for optic nerve lesions.

Effect of different positive end expiratory pressure levels on optic nerve sheath diameter in patients with or without midline shift who are undergoing supratentorial craniotomy.

PURPOSE: In general, high levels of PEEP application is avoided in patients undergoing craniotomy to prevent a rise in ICP. But that approach would increase the risk of secondary brain injury especially in hypoxemic patients. Because the optic nerve sheath is distensible, a rise in ICP is associated with an increase in the optic nerve sheath diameter (ONSD). The cutoff value for elevated ICP assessed by ONSD is between 5.6 and 6.3 mm. We aimed to evaluate the effect of different PEEP levels on ONSD and compare the effect of different PEEP levels in patients with and without intracranial midline shift. METHODS: This prospective observational study was performed in aged 18-70 years, ASA I-III, 80 patients who were undergoing supratentorial craniotomy. After the induction of general anesthesia, the ONSD's were measured by the linear transducer from 3 mm below the globe at PEEP values of 0-5-10 cmH2O. The ONSD were compered between patients with (n = 7) and without midline shift (n = 73) at different PEEP values. RESULTS: The increases in ONSD due to increase in PEEP level were determined (p < 0.001). No difference was found in the comparison of ONSD between patients with and without midline shift in different PEEP values (p = 0.329, 0.535, 0.410 respectively). But application of 10 cmH2O PEEP in patients with a midline shift increased the mean ONSD value to 5.73 mm. This value is roughly 0.1 mm higher than the lower limit of the ONSD cutoff value. CONCLUSIONS: The ONSD in adults undergoing supratentorial tumor craniotomy, PEEP values up to 5 cmH2O, appears not to be associated with an ICP increase; however, the ONSD exceeded the cutoff for increased ICP when a PEEP of 10 cmH2O was applied in patients with midline shift.

Endoscopic endonasal decompression of the optic nerve in the setting of compressive lesions: how I do it.

BACKGROUND: Many lesions in the anterior skull base may compress the optic nerve (ON), leading to vision loss, and even irreversible blindness. Although decompression of the optic nerve has traditionally been achieved transcranially, the endoscopic endonasal approach (EEA) is gaining traction as a minimally invasive approach recently. METHOD: We describe the key steps of an EEA ON decompression. The relevant surgical anatomy with illustration is described. Additionally, a video detailing our technique and instruments on an illustrative case is provided. CONCLUSION: Endoscopic endonasal approach ON decompression with a straight feather blade is a feasible, minimally invasive procedure to decompress the ON in the setting of anterior skull base mass lesions.

Optic nerve sheath diameter correlates with both success and failure of hydrocephalus treatment in pediatric patients with pineal region lesions.

BACKGROUND: Pineal region lesions in children are heterogenous pathologies often symptomatic due to occlusive hydrocephalus and thus elevated intracranial pressure (ICP). MRI-derived parameters to assess hydrocephalus are the optic nerve sheath diameter (ONSD) as a surrogate for ICP and the frontal occipital horn ratio (FOHR), representing ventricle volume. As elevated ICP may not always be associated with clinical signs, the adjunct of ONSD could help decision making in patients undergoing treatment. The goal of this study is to assess the available magnetic resonance imaging (MRI) of patients with pineal region lesions undergoing surgical treatment with respect to pre- and postoperative ONSD and FOHR as an indicator for hydrocephalus. METHODS: Retrospective data analysis was performed in all patients operated for pineal region lesions at a tertiary care center between 2010 and 2023. Only patients with pre- and postoperative MRI were selected for inclusion. Clinical data and ONSD at multiple time points, as well as FOHR were analyzed. Imaging parameter changes were correlated with clinical signs of hydrocephalus before and after surgical treatment. RESULTS: Thirty-three patients with forty operative cases met the inclusion criteria. Age at diagnosis was 10.9 ± 4.6 years (1-17 years). Hydrocephalus was seen in 80% of operative cases preoperatively (n = 32/40). Presence of hydrocephalus was associated with significantly elevated preoperative ONSD (p = 0.006). There was a significant decrease in ONSD immediately (p < 0.001) and at 3 months (p < 0.001) postoperatively. FOHR showed a slightly less pronounced decrease (immediately p = 0.006, 3 months p = 0.003). In patients without hydrocephalus, no significant changes in ONSD were observed (p = 0.369). In 6/6 patients with clinical hydrocephalus treatment failure, ONSD increased, but in 3/6 ONSD was the only discernible MRI change with unchanged FOHR. CONCLUSIONS: ONSD measurements may have utility in evaluating intracranial hypertension due to hydrocephalus in patients with pineal region tumors. ONSD changes appear to have value in assessing hydrocephalus treatment failure.

Visual Outcomes Following Surgical Debulking in Exophytic Optic Nerve Sheath Meningiomas.

PURPOSE: To report a case of a globular primary optic nerve sheath meningioma managed surgically with improvement in vision and review the literature for outcomes of purely intraorbital exophytic-globular primary optic nerve sheath meningiomas managed surgically. METHODS: A literature review was conducted using Google Scholar and PubMed with the search terms "primary optic nerve sheath meningioma," "surgery," "exophytic," and "globular." Articles were included if they were available in English. Individual cases from the reviewed articles were included if the tumor was purely intraorbital with a globular or exophytic morphology, was managed with total or subtotal surgical excision, and visual outcomes were reported. Cases were excluded if the tumor extended intracanalicularly or intracranially, tumor morphology was unknown, or surgical management consisted of biopsy, optic nerve sheath decompression, or optic canal decompression rather than tumor debulking. RESULTS: A total of 28 patients with intraorbital globular-exophytic primary optic nerve sheath meningiomas managed surgically have been reported in the literature. Vision improved in 29% (n = 8/28) and remained stable in 43% (n = 12/28) of patients. Furthermore, patients with good (Snellen notation ≥ 0.5) vision (n = 10) typically retained good vision postoperatively and at follow-up, with 1 patient experiencing a decline to poor (Snellen ≤0.1) vision at the last follow-up (92 months postoperatively). Similarly, patients with fair (Snellen notation >0.1 and <0.5) vision (n = 5) often improved to good vision (n = 3) or stayed at fair vision (n = 1), with 1 declining to poor vision at postoperative hospital discharge. CONCLUSIONS: Surgical management of exophytic or globular optic nerve meningiomas does not universally lead to vision loss and may be appropriate in select patients.

Endoscopic endonasal surgical anatomy of the optic canal: key anatomical relationships between the optic nerve and ophthalmic artery.

PURPOSE: A detailed understanding of the neurovascular relationships between the optic nerve (ON) and the ophthalmic artery (OA) in the optic canal (OC) is paramount for safe surgery. We focused on the neurovascular anatomy of this area from both an endoscopic endonasal and transcranial trajectories to compare the surgical exposures and perspectives offered by these different views and provide recommendations to increase the intraoperative safety. METHODS: Twenty sides of ten formalin-fixed, latex-injected head specimens were utilized. The surgical anatomy and anatomical relationships of the OA in relationship to the ON along their intracranial and intracanalicular segments was studied from endoscopic endonasal and transcranial perspectives. RESULTS: Three types of OA-ON relationships at the origin of the OA were identified: inferomedial (type 1, 35%), inferior (type 2, 55%), and inferolateral (type 3, 10%). The endoscopic endonasal trajectory offers an inferomedial perspective of the ON-OA neurovascular complex, in which the OA, especially when located inferomedially, is first encountered. When comparing with the transcranial view, all OA were covered by the nerve, type 1 was located below the medial third, type 2 below the middle third, and type 3 below the lateral third of the OC. The mean extension of the intracanalicular portion of both OA and ON was 8.9 mm, while the intracranial portion of the OA and ON were 9.3 mm and 12.4 mm, respectively. The OA, endoscopically, is located within the inferior half of the OC, and occupies 39%, 43%, and 42% of the OC height at its origin, mid, and end points, respectively. The mean distance between the superior margin of the OC at its origin and superior margin of the OA is 1.4 mm. CONCLUSIONS: Detailed anatomical understanding of the OC, and the ON and OA at their intracranial and intracanalicular segments is paramount to safe surgery. When opening the OC dura endoscopically, our results suggest that a medial incision along the superior third of the OC with a proximal to distal direction is recommended to avoid injury of the OA.

Optic nerve sheath diameter as a quantitative parameter associated with the outcome in patients with traumatic brain injury undergoing hematoma removal.

PURPOSE: To determine the association between optic nerve sheath diameter (ONSD) and outcome in patients with traumatic brain injury (TBI) who undergo hematoma removal (HR). METHODS: This study was a retrospective analysis of data from a single center between 2016 and 2021. Adult patients with TBI who underwent HR within 24 h after admission were included in this study. Preoperative and postoperative ONSD of the surgical side and the mean ONSD of both sides were measured for analysis. The primary outcome was mortality at 30 days. Receiver operating characteristic curve analysis was performed to calculate the area under the curve (AUC) and 95% confidence interval (CI) for 30 days mortality. RESULTS: Sixty-one patients were enrolled in the study. Among them, 48 (78.7%) survived for 30 days after admission. The AUC and 95% CI of the postoperative mean ONSD on both sides and postoperative/preoperative mean of the ONSD ratio on both sides were 0.884 [0.734-0.955] and 0.875 [0.751-0.942], respectively. The postoperative mean of both ONSDs of 6.0 mm had high accuracy as a cut-off value with a sensitivity of 85%, specificity of 83%, positive likelihood ratio (LR) of 5.0, and negative LR- of 0.18. CONCLUSION: This study demonstrated that postoperative ONSD and the postoperative/preoperative ONSD ratio were associated with postoperative outcome in patients with TBI who underwent HR.

Residual Apical Lamina Papyracea as a Cause for Persistent Dysthyroid Optic Neuropathy After Orbital Decompression.

Two patients had persistent compressive dysthyroid optic neuropathy after decompression of the medial orbital wall and floor. In both cases, there was ~3 mm of unresected lamina papyracea anterior to the Annulus of Zinn, and removal of this residual bone led to resolution of the neuropathy. These illustrative cases suggest that, in some patients, even small amounts of residual crowding at the orbital apex can critically embarrass optic nerve perfusion, with resulting continued ischemic optic neuropathy.

Primary optic nerve sheath schwannoma: a case report.

BACKGROUND: The optic nerve is an unusual site of schwannoma as it lacks Schwannoma cells on it. We report a primary optic sheath schwannoma and to review the literature. CASE REPORT: A 29-year old female presented with progressive painless non-pulsatile proptosis. Ocular examination revealed only axial proptosis. Imaging showed a well-defined intraconal mass abutting optic nerve. A left frontal craniotomy with orbitotomy and tumor excision was done. The tumor was well encapsulated, posteriorly attached to optic nerve without any plane, probable site of origin. The postoperative duration was uneventful without any complications. The histopathology examination confirms the diagnosis of schwannoma. CONCLUSIONS: We suggest to consider orbital optic nerve schwannoma in differential diagnosis of orbital tumors despite its exceedingly rare occurrence.

Alternative Path for Optic Nerve Decompression in Pseudotumor Cerebri With Full Endoscopic Lateral Transorbital Approach.

Endonasal endoscopic approaches are the most preferred surgical methods in patients with pseudotumor cerebri because of easy access to the optic nerve, but the choice of this technique may not apply to all endoscopic endonasal cases. Moreover, there may be difficulties in practice in some cases, including the coronavirus disease 2019 pandemic. This study aimed to suggest an alternative endoscopic approach by lateral orbitotomy for optic nerve decompression in patients with pseudotumor cerebri. The study was performed using 5 fresh-frozen cadaver heads (bilaterally, total of 10 sides) injected intravenously with colored silicone preserved in the cold chain. An average of 2.5 cm skin incision was made to fit the lateral orbitotomy. The lengths of the recurrent meningeal artery (mm), the meningo-orbital band (mm), and the optic nerve (mm) to the orbital margin were measured. After these morphometric measurements, optic nerve decompression was performed endoscopically, and the length of the decompression was measured (mm). The average length (mm) between the orbital rim and meningeal recurrent artery (or meningolacrimal branch) was 16.2 mm, between the orbital rim and the meningo-orbital band was 18.5 mm, and between the orbital rim and optic nerve was 44.1 mm. The average optic nerve decompression length was 4.4 mm. The endoscopic lateral orbitotomy approach provides easy access to the optic nerve by anatomically following the recurrent meningeal artery and the meningo-orbital band. It can be a safe second-line approach after endonasal approaches for optic nerve decompression in pseudotumor cerebri.

Anatomical Study and Clinical Application of Optic Canal Decompression Via Transethmoid-sphenoid Approach Under Endoscope.

This study aimed to evaluate the safety and accuracy of the endoscopic transethmoid-sphenoid approach for optic canal decompression. Twelve sides of 6 adult cadaveric heads fixed in formalin were selected to simulate optic canal decompression using the endoscopic transethmoid-sphenoid approach. Furthermore, this approach was used for optic canal decompression in 10 patients (11 eyes) with optic nerve canal injury. Related anatomical structures were observed using a 0-degree endoscope, and the anatomical characteristics as well as the surgical data were collected. The maximum effective widths of the cranial opening, orbital opening, and middle segment of the canal that could be drilled open endoscopically were 7.82±2.63, 8.05±2.77, and 6.92±2.01 mm, respectively. The angle between the line linking the center point of the tubercular recess with the midpoint of the cranial opening of the optic canal and the horizontal coordinate was 17.23±1.34 degrees. At the orbital opening of the optic canal, the ophthalmic artery was located directly inferior to the optic nerve in 2 cases (16.7%) and laterally inferior to the optic nerve in 10 cases (83.3%). Six of the operational eyes were effective while the remaining 5 were ineffective. No postoperative complications such as bleeding, infection, or cerebrospinal fluid leakage were observed during the follow-up period (6-12 mo). In conclusion, optic canal decompression positively impacts the prognosis of partial traumatic optic neuropathy. Furthermore, the endoscopic transethmoid-sphenoid approach for optic canal decompression is a minimally invasive procedure that provides direct access and adequate decompression. This technique is easy to master and suitable for clinical applications.

Endoscopic Endonasal Optic Nerve Decompression in a Patient With Invasive Fungal Sinusitis Invasion Into the Orbital Apex: Intraoperative Video and Literature Review.

Invasive fungal sinusitis (IFS) is rare but potentially fatal in immunocompromised patients. Orbital extension of IFS can cause visual loss or ophthalmoplegia, and the rate of recovery of visual acuity can be low even after treatment with antifungal agents and sinus surgery. In this case, optic nerve decompression with the endoscopic endonasal approach was successful for the treatment of visual loss in a patient with IFS with orbital apex extension. The authors describe the recovery of visual acuity after optic nerve decompression in a patient with IFS who had exhibited visual loss caused by invasion into the orbital apex. The authors also provide an intraoperative video and literature review.

A technique for excisional optic nerve biopsy.

PURPOSE: To describe a transconjunctival technique for full-thickness (excisional) optic nerve biopsy. METHOD: A medial transconjunctival approach to the optic nerve with disinsertion of the medial rectus is used. A small right-angle Mixter forcep is used to clamp the optic nerve far posteriorly, and then a microscalpel is directed metal-on-metal to cut the posterior optic nerve. The cut nerve is then rotated anteriorly to complete the proximal nerve cut. RESULT: A full-thickness specimen of 11 mm of more can be obtained without undue traction on the globe. The globe remains viable. CONCLUSION: A long length, excisional optic nerve biopsy can be readily and safely performed without endoscopic techniques.

Visual recovery after unilateral optic nerve sheath fenestration for pseudotumor cerebri syndrome.

PURPOSE: To evaluate the effect of optic nerve sheath fenestration (ONSF) on the rate of visual function improvement in patients with pseudotumor cerebri syndrome (PTCS). METHODS: Retrospective chart review of patients with PTCS who underwent ONSF between 1998 and 2017. Visual function was evaluated by evaluating visual field (VF), mean deviation (MD), retinal nerve fiber layer (RNFL) thickness, papilledema grade, and visual acuity (VA) prior to and after ONSF. RESULTS: Seventeen female patients aged 17 to 36 years underwent unilateral ONSF. Follow-up averaged 40.1 months. VF MD improved steadily in both eyes up to 12 months. Average RNFL thickness improved in the operated eye from 347 ± 166 mm to 92 ± 27 mm (p < .001) and the non-operated eyes from 306 ± 165 mm to 109 ± 46 mm (p < .001). The grade of papilledema improved in the operated eye from 3.3 ± 1.3 to 0.3 ± 0.7 and the non-operated eye from 3.0 ± 1.6 to 0.18 ± 0.4. There was an exponential rate of improvement in papilledema and RNFL thickness, with the greatest improvement occurring within the first 30 days. Average visual acuity remained intact in both eyes before and after surgery. CONCLUSIONS: ONSF in appropriately selected patients leads to rapid improvement in papilledema and a steady recovery in VF.

Traumatic optic neuropathy: a review of current studies.

Traumatic optic neuropathy (TON) is a serious complication of craniofacial trauma that directly or indirectly damages the optic nerve and can cause severe vision loss. The incidence of TON has been gradually increasing in recent years. Research on the protection and regeneration of the optic nerve after the onset of TON is still at the level of laboratory studies and which is insufficient to support clinical treatment of TON. And, due to without clear guidelines, there is much ambiguity regarding its diagnosis and management. Clinical interventions for TON include observation only, treatment with corticosteroids alone, or optic canal (OC) decompression (with or without steroids). There is controversy in clinical practice concerning which treatment is the best. A review of available studies shows that the visual acuity of patients with TON can be significantly improved after OC decompression surgery (especially endoscopic transnasal/transseptal optic canal decompression (ETOCD)) with or without the use of corticosteroids. And new findings of laboratory studies such as mitochondrial therapy, lipid change studies, and other studies in favor of TON therapy have also been identified. In this review, we discuss the evolving perspective of surgical treatment and experimental study.

The Effect of Optic Nerve Sheath Fenestration on Intraocular Pressure in Patients With Idiopathic Intracranial Hypertension.

BACKGROUND: To evaluate the change in intraocular pressure (IOP) in patients with idiopathic intracranial hypertension (IIH) who underwent optic nerve sheath fenestration (ONSF) and to determine if radiographic evidence of posterior scleral or globe indentation influenced IOP. METHODS: This is a retrospective analysis of IOP in IIH patients who underwent ONSF. The study included all patients from September 2010 to March 2018 operated on by a single surgeon (R.M.). IOPs preoperatively and postoperatively were recorded along with the acetazolamide dosage and whether there was evidence of posterior scleral or globe indentation on preoperative MRI. RESULTS: A total of 29 patients (35 eyes) with IIH underwent ONSF. The average reduction in IOP among all patients was 1.24 mm Hg (P = 0.0218), but this increased to 2.69 mm Hg (P = 0.004) in patients who were maintained on the same dosage of acetazolamide in the preoperative and postoperative period. Furthermore, the reduction in IOP in those with posterior scleral or globe indentation was 2.5 mm Hg (P = 0.0095). When the perioperative period was evaluated, the mean decrease in IOP was 1.83 mm Hg (P = 0.0217). CONCLUSIONS: Reducing the cerebral spinal fluid pressure (CSFP) at the level of the intraorbital optic nerve through an ONSF can slightly reduce the IOP. In those with evidence of posterior globe or scleral indentation/flattening, the reduction in IOP was higher, which supports the theory that CSF pressure indents the globe and leads to an increase in IOP. Although these changes in IOP are small, this study provides further evidence for a connection between IOP and CSFP.

Cavernous Malformation of the Optic Nerve and Chiasm: Prompt Suspicion and Surgery Matter.

BACKGROUND: Cavernous malformations (CMs) of the optic nerve and chiasm are extremely rare, accounting for less than 1% of all intracranial CMs. Acute, subacute, or progressive visual loss from CM may occur with or without hemorrhage. Prompt surgical excision of the CM offers the best hope to improve or stabilize vision. Given its rarity, optic nerve and chiasm CMs may not be readily suspected. We provide 3 cases of optic nerve and chiasm CM, highlighting key neuroimaging features and the importance of expedited intervention. METHODS: Case records of the neuro-ophthalmology clinics of the Bascom Palmer Eye Institute and the University of Colorado, and literature review of reported cases of optic CM. RESULTS: A 49-year-old woman reported acute progressive painless vision loss in the right eye. MRI showed a suprasellar mass with heterogeneity in signal involving the right prechiasmatic optic nerve. Surgical excision of the CM 5 days after onset of visual loss improved vision from 20/300 to 20/30. A 29-year-old woman with acute painless blurred vision in the right eye had anterior chiasmal junctional visual field defects corresponding to a heterogeneously minimally enhancing mass with blood products enlarging the optic chiasm and proximal right optic nerve. Surgical excision of the CM 8 weeks after onset of visual loss improved vision from 20/40 to 20/15 with improved visual fields. A 33-year-old woman with a history of familial multiple CMs, diagnosed at age 18, reported new-onset severe headache followed by blurred vision. MRI showed a hemorrhagic lesion of the optic chiasm and right optic tract. She was 20/20 in each eye with a reported left superior homonymous hemianopia. No intervention was recommended. Vision of the right eye worsened to 20/400 2 months later. The patient was followed over 13 years, and the MRI and visual function remained unchanged. Literature review yielded 87 optic CM cases occurring across gender and nearly all ages with visual loss and headache as the most common presenting symptoms. Optic chiasm is the most common site of involvement (79%). Nearly 95% of reported CM cases were treated with surgery with 81% with improved vision and 1% with worsened vision. CONCLUSION: MRI features are critical to the diagnosis of optic nerve and chiasm CM and may mimic other lesions. A high index of suspicion by the neuro-ophthalmologist and neuroradiologist leads to early recognition and intervention. Given optic CM displaces and does not infiltrate neural tissue, expedited surgical resection by a neurosurgeon after consideration of other diagnostic possibilities improves visual function in most cases.

Bilateral chiasm and optic nerve micro-decompression in a child with extensive cranial fibrous dysplasia: how I do it.

BACKGROUND: Surgical decompression to the optic-chiasmatic region in craneofacial fibrous dysplasia (CFD) must be performed safely to improve or stabilize visual loss. METHOD: We describe a technical nuance when facing on a huge, deformed skull with potentially imbricated dura mater. Craniectomy was performed in concentric arches allowing to expose surgical field and elevated step by step. Bilateral micro-decompression was performed after without difficulties. CONCLUSIONS: Decompressing both optic nerves using this technique is safe and relatively simple to perform.