Optic Pathway Glioma MRI

Optic Pathway Glioma MRI

Usually showing low T1 and high central T2 signal on MRI images, enhancement is variable.

MR imaging is optimal for showing the relationship of the mass to the hypothalamusoptic chiasm, and infundibulum as well as the intraorbital and intracanalicular components of the mass. Large tumours are typically heterogeneous with cystic and solid components.

T1: enlargement, often iso to hypointense compared to the contralateral side

T2: hyperintense centrally

thin low-signal at the periphery representing the dura 1).

FLAIR: hyper intense

T1 C+ (Gd): enhancement is variable

Patients with chiasmatic-hypothalamic low-grade glioma (CHLGG) have frequent MRIs with gadolinium-based contrast agents (GBCA) for disease monitoring. Cumulative gadolinium deposition in the brains of children is a potential concern. The purpose of a study of Malbari et al. was to evaluate whether MRI with GBCA is necessary for determining radiographic tumor progression in children with CHLGG.

Children who were treated for progressive CHLGG from 2005 to 2019 at Texas Children’s Cancer Center were identified. Pre- and post-contrast MRI sequences were separately reviewed by one neuroradiologist who was blinded to the clinical course. Three-dimensional measurements and tumor characteristics were evaluated. Radiographic progression was defined as a 25% increase in size (product of two largest dimensions) compared with baseline or best response after initiation of therapy.

A total of 28 patients with progressive CHLGG were identified with a total of 683 MRIs with GBCA reviewed (mean 24 MRIs/patient; range, 11-43 MRIs). Radiographic progression was observed 92 times, 91 (99%) on noncontrast and 90 (98%) on contrast imaging. Sixty-seven progressions necessitating management changes were identified in all (100%) noncontrast sequences and 66 (99%) contrast sequences. Tumor growth > 2 mm in any dimension was identified in 184/187 (98%) noncontrast and 181/187 (97%) with contrast imaging. Metastatic tumors were better visualized on contrast imaging in 4/7 (57%).

MRI without GBCA effectively identifies patients with progressive disease. When imaging children with CHLGG, eliminating GBCA should be considered unless monitoring patients with metastatic disease 2)

Gadolinium might not be needed for these exams to inform management decisions. Secondary benefits of a non-contrast follow-up protocol include decreased cost and risk to the patient 3).

MR examinations of 91 patients with OPG (47 with NF and 44 without) were reviewed at presentation and during follow-up. The images were evaluated for size and extension of tumor, and imaging parameters. Statistical bivariate analysis was used to compare the patients with and those without NF, and Pearson correlation was used to evaluate the correlation between the different imaging parameters and prognosis. Kappa values were calculated to determine intraobserver and interobserver variability.

Results: The most common site of involvement in the NF group was the orbital nerve (66%), followed by the chiasm (62%). In the non-NF group, the chiasm was the most common site of involvement (91%); the orbital nerves were involved in only 32%. Extension beyond the optic pathway at diagnosis was uncommon in the NF group (2%) but frequent in the non-NF group (68%). In the NF group, the tumor was smaller and the original shape of the optic pathways was preserved (91% vs. 27% in the non-NF group). The presence of cystic components was significantly more common in the non-NF patients (66% vs. 9% in the NF group). During follow-up, half the NF patients remained stable, in contrast to 5% of the non-NF group. No statistical correlation was found between imaging features and biological behavior of the tumor.

Conclusion: NF-OPG is a separate entity from non-NF-OPG, with different imaging features and prognosis, thereby warranting a specific diagnostic, clinical, and therapeutic approach 4).


Müller-Forell WS, Boltshauser E. Imaging of Orbital and Visual Pathway Pathology. Springer Verlag. (2005) ISBN:3540279881.

Malbari F, Chintagumpala MM, Wood AC, Levy AS, Su JM, Okcu MF, Lin FY, Lindsay H, Rednam SP, Baxter PA, Paulino AC, Orzaiz GA, Whitehead WE, Dauser R, Supakul N, Kralik SF. Gadolinium is not necessary for surveillance MR imaging in children with chiasmatic-hypothalamic low-grade glioma. Pediatr Blood Cancer. 2021 Jun 16:e29178. doi: 10.1002/pbc.29178. Epub ahead of print. PMID: 34133064.

Maloney E, Stanescu AL, Perez FA, Iyer RS, Otto RK, Leary S, Steuten L, Phipps AI, Shaw DWW. Surveillance magnetic resonance imaging for isolated optic pathway gliomas: is gadolinium necessary? Pediatr Radiol. 2018 Sep;48(10):1472-1484. doi: 10.1007/s00247-018-4154-4. Epub 2018 May 22. PMID: 29789890.

Kornreich L, Blaser S, Schwarz M, Shuper A, Vishne TH, Cohen IJ, Faingold R, Michovitz S, Koplewitz B, Horev G. Optic pathway glioma: correlation of imaging findings with the presence of neurofibromatosis. AJNR Am J Neuroradiol. 2001 Nov-Dec;22(10):1963-9. PMID: 11733333; PMCID: PMC7973845.

Optic nerve sheath diameter

Optic nerve sheath diameter

Dilatation of the optic nerve sheath has been shown to be a much earlier manifestation of ICP rise 1) 2).

For Liu et al. ONSD measured via head CT correlates with ICP and can predict the requirement for surgery in patients with TBI following admission to the emergency department 3).

In a study of Agrawal et al. optic nerve sheath diameter demonstrated a modest, statistically significant correlation with intracranial pressure, a predetermined level of diagnostic accuracy to justify routine clinical use as a screening test was not achieved. Measurement of optic disc elevation appears promising for the detection of elevated intracranial pressure, however, verification from larger studies is necessary 4).

Optic nerve sheath diameter ultrasonography

see Optic nerve sheath diameter ultrasonography.


1) Hansen HC, Helmke K. Validation of the optic nerve sheath response to changing cerebrospinal fluid pressure: Ultrasound findings during intrathecal infusion tests. J Neurosurg. 1997;87:34–40.2) Helmke K, Hansen HC. Fundamentals of transorbital sonographic evaluation of optic nerve sheath expansion under intracranial hypertension. I. Experimental study. Pediatr Radiol. 1966;26:701–5.3) Liu M, Yang ZK, Yan YF, Shen X, Yao HB, Fei L, Wang ES. Optic nerve sheath measurements by computed tomography to predict intracranial pressure and guide surgery in patients with traumatic brain injury. World Neurosurg. 2019 Oct 17. pii: S1878-8750(19)32683-X. doi: 10.1016/j.wneu.2019.10.065. [Epub ahead of print] PubMed PMID: 31629929.4) Agrawal D, Raghavendran K, Zhao L, Rajajee V. A Prospective Study of Optic Nerve Ultrasound for the Detection of Elevated Intracranial Pressure in Severe Traumatic Brain Injury. Crit Care Med. 2020 Oct 13. doi: 10.1097/CCM.0000000000004689. Epub ahead of print. PMID: 33048902.

Optic nerve sheath diameter ultrasonography

Optic nerve sheath diameter ultrasonography

Optic nerve sheath diameter ultrasonography is strongly correlated with invasive ICPmeasurements and may serve as a sensitive and noninvasive method for detecting elevated ICP in TBI patients after decompressive craniectomy 1).

Optic nerve sheath diameter measured by transorbital ultrasound imaging is an accurate method for detecting intracranial hypertension that can be applied in a broad range of settings. It has the advantages of being a non-invasive, bedside test, which can be repeated multiple times for re-evaluation 2).

Evolution of ultrasound technology and the development of high frequency (> 7.5 MHz) linear probes with improved spatial resolution have enabled excellent views of the optic nerve sheath.

The optic nerve sheath diameter (ONSD), measured at a fixed distance behind the retina has been evaluated to diagnose and measure intracranial hypertension in traumatic brain injury and intracranial hemorrhage 3) 4).

The optic nerve sheath is fairly easy to visualize by ultrasonography by insonation across the orbit in the axial plane. A-mode ultrasonography was used to view the optic nerve sheath more than four decades ago; B-mode scanning was performed subsequently to assess intraocular lesions 5).

Shirodkar et al., studied the efficacy of ONSD measurement by ultrasonography to predict intracranial hypertension. The case mix studied included meningoencephalitis, stroke, intracranial hemorrhage and metabolic encephalopathy. Using cut-off values of 4.6 mm for females, and 4.8 mm for males, they found a high level of sensitivity and specificity for the diagnosis of intracranial hypertension as evident on CT or MRI imaging 6).

There is wide variation reported in the optimal cut-off values, when ONSD was compared with invasive ICP monitoring, ranging from 4.8 to 5.9 mm7) 8).

Padayachy et al present a method for assessment of optic nerve sheath ONS pulsatile dynamics using transorbital ultrasound imaging. A significant difference was noted between the patient groups, indicating that deformability of the ONS may be relevant as a noninvasive marker of raised ICP 9).

Of the studied ultrasound noninvasive intracranial pressure monitoringoptic nerve sheath diameter (ONSD), is the best estimator of ICP. The novel combination of optic nerve sheath diameter ultrasonography and venous transcranial Doppler (vTCD) of the straight sinus is a promising and easily available technique for identifying critically ill patients with intracranial hypertension 10).

The optic nerve sheath diameter has been verified by various clinical studies as a non-invasive indicator of intracranial hypertension 11).

Correlations between ICP and Optic nerve sheath diameter (ONSD) using CT and MRI have been observed in adult populations.

Ultrasound methods has been proposed as an alternative safe technique for invasive ICP measuring methods 12).

Admission ONSD in decompressive craniectomy (DC) patients is high but does not predict mortality and unfavorable outcomes 13).

Intracranial pressure (ICP) can be noninvasively estimated from the sonographic measurement of the optic nerve sheath diameter (ONSD) and from the transcranial Doppler analysis of the pulsatility (ICPPI) and the diastolic component (ICPFVd) of the velocity waveform 14).

Where pediatric patients present with an ONSD of over 6.1mm following a TBI, ICP monitoring should be implemented 15).

Padayachy et al present a method for assessment of ONS pulsatile dynamics using transorbital ultrasound imaging. A significant difference was noted between the patient groups, indicating that deformability of the ONS may be relevant as a noninvasive marker of raised ICP 16).

While the ultrasonographic mean binocular ONSD (>4.53 mm) was completely accurate in detecting elevated ICP, color Doppler indices of the ophthalmic arteries were of limited value 17).

Bedside ultrasound may be useful in the diagnosis of midline intracranial shift by measurement of ONSD 18).

In patients with SAH and acute hydrocephalus after aneurysm rupture, the ONSD remains expanded after normalization of ICP. This is most likely due to an impaired retraction capability of the optic nerve sheath. This finding should be considered when using transorbital sonography in the neuromonitoring of aneurysmal SAH 19).

ONSD >5.5 mm yielded a sensitivity of 98.77% (95% CI: 93.3%-100%) and a specificity of 85.19% (95% CI: 66.3%-95.8%).In conclusion, the optimal cut-off point of ONSD for identifying IICP was 5.5 mm. ONSD seen on ocular US can be a feasible method for detection and serial monitoring of ICP in Korean adult patients 20).

Systematic review

The aim of a systematic review and meta-analysis will be to examine the accuracy of ONSD sonography for increased ICP diagnosis.

Koziarz et al. will include published and unpublished randomised controlled trials, observational studies, and abstracts, with no publication type or language restrictions. Search strategies will be designed to peruse the MEDLINE, Embase, Web of Science, WHO Clinical Trials, ClinicalTrials.gov, CINAHL, and the Cochrane Library databases. We will also implement strategies to search grey literature. Two reviewers will independently complete data abstraction and conduct quality assessment. Included studies will be assessed using the Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool. We will construct the hierarchical summary receiver operating characteristic curve for included studies and pool sensitivity and specificity using the bivariate model. We also plan to conduct prespecified subgroup analyses to explore heterogeneity. The overall quality of evidence will be rated using Grading of Recommendations, Assessment, Development and Evaluations (GRADE).

Research ethics board approval is not required for this study as it draws from published data and raises no concerns related to patient privacy. This review will provide a comprehensive assessment of the evidence on ONSD sonography diagnostic accuracy and is directed to a wide audience. Results from the review will be disseminated extensively through conferences and submitted to a peer-reviewed journal for publication 21).

Case series



Wang J, Li K, Li H, Ji C, Wu Z, Chen H, Chen B. Ultrasonographic optic nerve sheath diameter correlation with ICP and accuracy as a tool for noninvasive surrogate ICP measurement in patients with decompressive craniotomy. J Neurosurg. 2019 Jul 19:1-7. doi: 10.3171/2019.4.JNS183297. [Epub ahead of print] PubMed PMID: 31323632.

Beare NA, Kampondeni S, Glover SJ, Molyneux E, Taylor TE, Harding SP, Molyneux ME. Detection of raised intracranial pressure by ultrasound measurement of optic nerve sheath diameter in African children. Trop Med Int Health. 2008 Nov;13(11):1400-4. doi: 10.1111/j.1365-3156.2008.02153.x. Epub 2008 Oct 13. PubMed PMID: 18983275; PubMed Central PMCID: PMC3776606.

Geeraerts T, Merceron S, Benhamou D, Vigué B, Duranteau J. Non-invasive assessment of intracranial pressure using ocular sonography in neurocritical care patients. Intensive Care Med. 2008;34:2062–7.

Moretti R, Pizzi B. Optic nerve ultrasound for detection of intracranial hypertension in intracranial hemorrhage patients: Confirmation of previous findings in a different patient population. J Neurosurg Anesthesiol. 2009;21:16–20.

Gangemi M, Cennamo G, Maiuri F, D’Andrea F. Echographic measurement of the optic nerve in patients with intracranial hypertension. Neurochirurgia (Stuttg) 1987;30:53–5.

Shirodkar CG, Rao SM, Mutkule DP, Harde YR, Venkategowda PM, Mahesh MU. Optic nerve sheath diameter as a marker for evaluation and prognostication of intracranial pressure in Indian patients: An observational study. Ind J Crit Care Med. 2014;18:728–734

Rajajee V, Vanaman M, Fletcher JJ, Jacobs TL. Optic nerve ultrasound for the detection of raised intracranial pressure. Neurocrit Care. 2011;15:506–15.

Geeraerts T, Launey Y, Martin L, Pottecher J, Vigué B, Duranteau J, et al. Ultrasonography of the optic nerve sheath may be useful for detecting raised intracranial pressure after severe brain injury. Intensive Care Med. 2007;33:1704–11.
9) , 16)

Padayachy L, Brekken R, Fieggen G, Selbekk T. Pulsatile Dynamics of the Optic Nerve Sheath and Intracranial Pressure: An Exploratory In Vivo Investigation. Neurosurgery. 2016 Jul;79(1):100-7. doi: 10.1227/NEU.0000000000001200. PubMed PMID: 26813857; PubMed Central PMCID: PMC4900421.

Robba C, Cardim D, Tajsic T, Pietersen J, Bulman M, Donnelly J, Lavinio A, Gupta A, Menon DK, Hutchinson PJA, Czosnyka M. Ultrasound non-invasive measurement of intracranial pressure in neurointensive care: A prospective observational study. PLoS Med. 2017 Jul 25;14(7):e1002356. doi: 10.1371/journal.pmed.1002356. eCollection 2017 Jul. PubMed PMID: 28742869.

Choi SH, Min KT, Park EK, Kim MS, Jung JH, Kim H. Ultrasonography of the optic nerve sheath to assess intracranial pressure changes after ventriculo-peritoneal shunt surgery in children with hydrocephalus: a prospective observational study. Anaesthesia. 2015 Nov;70(11):1268-73. doi: 10.1111/anae.13180. Epub 2015 Aug 24. PubMed PMID: 26299256.

Karami M, Shirazinejad S, Shaygannejad V, Shirazinejad Z. Transocular Doppler and optic nerve sheath diameter monitoring to detect intracranial hypertension. Adv Biomed Res. 2015 Oct 22;4:231. doi: 10.4103/2277-9175.167900. eCollection 2015. PubMed PMID: 26645016; PubMed Central PMCID: PMC4647120.

Waqas M, Bakhshi SK, Shamim MS, Anwar S. Radiological prognostication in patients with head trauma requiring decompressive craniectomy: Analysis of optic nerve sheath diameter and Rotterdam CT Scoring System. J Neuroradiol. 2016 Feb;43(1):25-30. doi: 10.1016/j.neurad.2015.07.003. Epub 2015 Oct 20. PubMed PMID: 26492980.

Robba C, Bragazzi NL, Bertuccio A, Cardim D, Donnelly J, Sekhon M, Lavinio A, Duane D, Burnstein R, Matta B, Bacigaluppi S, Lattuada M, Czosnyka M. Effects of Prone Position and Positive End-Expiratory Pressure on Noninvasive Estimators of ICP: A Pilot Study. J Neurosurg Anesthesiol. 2016 Mar 18. [Epub ahead of print] PubMed PMID: 26998650.

Young AM, Guilfoyle MR, Donnelly J, Scoffings D, Fernandes H, Garnett MR, Agrawal S, Hutchinson PJ. Correlating optic nerve sheath diameter with opening intracranial pressure in pediatric traumatic brain injury. Pediatr Res. 2016 Aug 11. doi: 10.1038/pr.2016.165. [Epub ahead of print] PubMed PMID: 27513519.

Tarzamni MK, Derakhshan B, Meshkini A, Merat H, Fouladi DF, Mostafazadeh S, Rezakhah A. The diagnostic performance of ultrasonographic optic nerve sheath diameter and color Doppler indices of the ophthalmic arteries in detecting elevated intracranial pressure. Clin Neurol Neurosurg. 2016 Feb;141:82-8. doi: 10.1016/j.clineuro.2015.12.007. Epub 2015 Dec 15. PubMed PMID: 26771156.

Kazdal H, Kanat A, Findik H, Sen A, Ozdemir B, Batcik OE, Yavasi O, Inecikli MF. Transorbital Ultrasonographic Measurement of Optic Nerve Sheath Diameter for Intracranial Midline Shift in Patients with Head Trauma. World Neurosurg. 2016 Jan;85:292-7. doi: 10.1016/j.wneu.2015.10.015. Epub 2015 Oct 17. PubMed PMID: 26485420.

Bäuerle J, Niesen WD, Egger K, Buttler KJ, Reinhard M. Enlarged Optic Nerve Sheath in Aneurysmal Subarachnoid Hemorrhage despite Normal Intracranial Pressure. J Neuroimaging. 2016 Mar-Apr;26(2):194-6. doi: 10.1111/jon.12287. Epub 2015 Aug 17. PubMed PMID: 26278326.

Lee SU, Jeon JP, Lee H, Han JH, Seo M, Byoun HS, Cho WS, Ryu HG, Kang HS, Kim JE, Kim HC, Jang KS. Optic nerve sheath diameter threshold by ocular ultrasonography for detection of increased intracranial pressure in Korean adult patients with brain lesions. Medicine (Baltimore). 2016 Oct;95(41):e5061. PubMed PMID: 27741121; PubMed Central PMCID: PMC5072948.

Koziarz A, Sne N, Kegel F, Alhazzani W, Nath S, Badhiwala JH, Rice T, Engels P, Samir F, Healey A, Kahnamoui K, Banfield L, Sharma S, Reddy K, Hawryluk GWJ, Kirkpatrick AW, Almenawer SA. Optic nerve sheath diameter sonography for the diagnosis of increased intracranial pressure: a systematic review and meta-analysis protocol. BMJ Open. 2017 Aug 11;7(8):e016194. doi: 10.1136/bmjopen-2017-016194. PubMed PMID: 28801417.

Update: Optic chiasma cavernous malformation

Optic chiasma cavernous malformation


Suprasellar occurrences of cavernous malformations (CM) in the optic chiasm are extremely uncommon, representing less than 1% of all CNS CMs 1) 2).
To the best of the knowledge of Abou-Al-Shaar et al. less than 80 cases have been reported in the literature 3).

Clinical features

Patients with these lesions typically present with chiasmal apoplexy, characterized by sudden visual lossacute headaches, retroorbital pain, and nausea 4).
These symptoms typically occur after a period of transient blurry vision and headaches. In addition, hypopituitarism from direct compression of the pituitary stalk has been reported in the literature 5) 6).


On CT scan, optic pathway CMs appear as well-demarcated hyperdense lesions with or without calcifications 7).


MR imaging of cavernous hemangioma of the optic chiasm 8)
MRI is considered the most sensitive and specific imaging modality for the diagnosis of CM 9).
On T1-weighted images, CMs of the optic pathway demonstrate a hypointense to isointense appearance, whereas on T2-weighted images, they appear as heterogeneous “popcorn” lesions with mixed hyperintense and hypointense signals.
The hypointensity can be delineated further in the gradient-echo T2* images due to hemosiderin deposition in and around the CM. In addition, following intravenous gadolinium administration, minimal or no enhancement can be observed in the CM 10) 11).
It has been reported that CMs of the optic nerve and tract may show nerve thickening on coronal views, whereas CMs of the optic chiasm often appear as focal round masses 12).
Angiography is usually not helpful in diagnosing CMs because it does not delineate the lesion due to the low internal flow and high incidence of thrombosis 13).

Differential diagnosis

CMs of the optic pathway are commonly misdiagnosed as optic neuritisoptic gliomameningiomacraniopharyngiomavenous angiomaarteriovenous malformation, thrombosed intracranial aneurysm, and pituitary apoplexyhistiocytosishypothalamic gliomatuber cinereum hamartoma and metastasis 14) 15).
Cavernoma should be considered when a solid suprasellar mass has hemorrhage (mimicking cystic- adamantinomatous craniopharingioma).

Cavernoma and suprasellar meningioma are rarely associated. Holland and Symon report a patient, whose recovery after removal of the meningioma was complicated by haemorrhage from the cavernoma. This occurrence has not been previously reported 16).


Surgical removal is the recommended treatment to restore or preserve vision and to eliminate the risk of future hemorrhage. However, the anatomical location and eloquence of nearby neural structures can make these lesions difficult to access and remove.
The surgical approach should allow optimal exposure of the lesion using the shortest route and with minimal brain retraction. Various surgical approaches have been reported in the literature including pterional, orbitozygomatic, supraorbital, subfrontal, and transbasal interhemispheric approaches. Almost half of the cases reported in the literature were managed through the frontotemporal approach 17).
Biopsy is contraindicated for these lesions due to the high risk of bleeding and symptomatic worsening 18) 19).



In their meticulous review of the literature, Lehner et al. found 42 previously reported patients with vascular malformations within optic nerves, chiasm, or optic tracts, 30 of them being cavernous hemangiomas. The optic chiasma was involved in 38 patients (90.5%) and a total excision of the tumor was performed in 21 cases 20).

Case reports


A 33-year-old female presented 3 months postpartum with a headache of moderate severity and progressive visual loss in both eyes. On examination, the patient’s Glasgow coma scale (GCS) was 15/15. Visual field examination showed left homonymous incomplete hemianopia. Her visual acuity was 20/25 in the right eye and 20/30 in the left eye. Her discs and macula were healthy bilaterally. Extraocular movements were intact and pupils were reactive. The rest of her examination was unremarkable. Complete endocrine workup was normal.
Magnetic resonance imaging (MRI) revealed a large heterogeneous, hyperintense, hemorrhagic right suprasellar extra-axial complex cystic structure measuring 31 × 30 × 90 mm on T1-weighted images. There was mass effect on the adjacent hypothalamus and third ventricle displacing them toward the left and superiorly in addition to the optic pathway. The pituitary stalk was displaced toward the left. The lesion encased the right posterior cerebral artery and displaced the right carotid artery laterally.
Computed tomography (CT) arteriography demonstrated a completely thrombosed center. The imaging findings were compatible with suprasellar CM.
The patient underwent right frontal craniotomy and gross total resection of her suprasellar intrachiasmatic large infiltrative hemorrhagic CM. Organizing blood clots with reactive fibrohistiocytic and inflammatory reaction admixed with some ectatic vascular channels suggestive of a vascular malformation were noted. There were small foci admixed with granulation tissue, showing some dilated cavernous spaces that would be compatible with a vascular malformation such as cavernous angioma. On immunohistochemistry, the lesion was CD163+, CD20 rare, CD3+, CD34+, CD31+, CD38+, CTK−, EMA plasma cells, GFAP−, S100 dendritic cells, SMA vascular smooth muscle.
The patient had an uneventful operative course. Her visual acuity improved to 20/20 in both eyes. Extraocular muscles showed mild limitation of both eyes in an upward gaze. Otherwise, she was stable with no neurological deficits. Follow-up MRI at 12 months revealed complete removal of the suprasellar hemorrhagic CM with no evidence of a residual lesion or recurrence 21).

Cavernous malformation of the optic chiasm: Neuro-endoscopic removal 22).

Trentadue et al. report a case in which the finding was incidentally detected in a 49-year-old man. They describe the imaging characteristics of the lesion in such a rare location, highlighting the role of magnetic resonance imaging (MRI) (specifically 3 Tesla) in the management of asymptomatic patients 23).


A 48-year-old female presented with an insidious history of progressive visual loss. Magnetic resonance imaging (MRI) showed a CM in the suprasellar region. The patient was operated via a right pterional approach with a complete lesion removal. The postoperative course was uneventful. Early postoperative ophthalmological examination revealed minimal improvement of the vision in the left eye 24).


The case of a 60-year-old woman from our institution with acute-on-chronic visual disturbance secondary to visual pathway CM is presented. Including the current patient, 70 cases of anterior visual pathway CM have been published to our knowledge. The average patient age is 34.8 ± standard deviation of 14.2 years, with a female preponderance (n = 37, 52.9%). The majority of patients had an acute (n = 44; 62.9%; 95% confidence interval [CI] 0.51-0.73) onset of symptoms. In at least 55.6% (n = 40) of patients, the cause of visual disturbance was initially misdiagnosed. The majority (91.4%; n = 64) of patients underwent craniotomy, with complete resection and subtotal resection achieved in 53.1% (n = 34; 95%CI 0.41-0.65) and 17.2% (n = 11; 95%CI 0.10-0.28) of all surgical patients, respectively. Comparing surgically managed patients, complete resection improved visual deficits in 59.0% (n = 20; 95%CI 0.42-0.75), while subtotal resection improved visual deficits in 50.0% (n = 5; 95%CI 0.24-0.76; p = 0.62). CM is an important differential diagnosis for suprasellar lesions presenting with visual disturbance. A high index of suspicion is required in its diagnosis. Expeditious operative management is recommended to improve clinical outcomes 25).


Ning et al. report a 28-year-old male presenting with left homonymous hemianopsia. Magnetic resonance imaging (MRI) revealed an occupied lesion located in the right side of the optic chiasm, and a clinical diagnosis of chiasmal CM was made. Microsurgical excision was performed via anterolateral pterional craniotomy. The patient showed good recovery with slight improvement of the visual field deficits after the operation. No CM recurrence was discovered during the follow-up MRI scans 26).


Rheinboldt and Blase report the case of a 31-year-old male who presented to the ER with a 1-week history of progressively worsening, throbbing, left retro-orbital headache, ptosis, and subjective worsening of short-term memory function. Initial review of systems and laboratory data were noncontributory. Non-contrasted CT demonstrated a large hyperdense mass centered in the suprasellar cistern without evidence of dissecting extra-axial hemorrhage. Though the initial appearance mimicked a basilar tip aneurysm or another primary extra-axial suprasellar pathology such as a hemorrhagic or proteinaceous craniopharyngioma, germinoma, or optic glioma, a second smaller, clearly intra-axial, hyperdense lesion was observed in the left periventricular forceps major white matter. Consideration for multiple cavernomas versus hypervascular metastatic disease such as renal malignancy, thyroid malignancy, or melanoma was raised. CTA confirmed normal intracranial vasculature. Subsequent MRI images showed an acutely hemorrhagic mass centered at the left paramedian hypothalamus and tuber cinereum with numerous secondary foci, demonstrating mature hemorrhagic elements and confirming the diagnosis of multiple cavernomas 27).


A 33-year-old female who suffered from a recurrence of an intrachiasmatic cavernous malformation is presented. She had already undergone surgery in 1991 and 2001 and was admitted to our hospital with reduced vision in the right eye. After MRI, and diagnosis of recurrence of the cavernoma, a neurosurgical operation was performed using the pterional approach. The intraoperative situation was documented with micro photographs. The postoperative course was uneventful. The female described a minimal improvement of her vision. No postoperative complications were observed. To our knowledge, microsurgically complete extirpation of a recurrence of an intrachiasmatic cavernoma has not yet been reported in the literature 28).


Santos-Ditto et al. present the case of a female patient who developed chiasmatic apoplexy and menstrual alterations. CT scanning showed a suprasellar hemorrhage. She underwent surgery with the presumptive diagnosis of pituitary tumor. At surgery, we find a brown-grayish lesion involving left optic nerve and chiasm. Cavernous angioma was diagnosed by histopathology. Cavernous angiomas constitute nearly 15% of all central nervous system vascular malformations. Location at the optic pathway is very rare, but must to be ruled out in the diagnosis of a patient with chiasmatic and/or optic apoplexy. Surgery is useful in preventing worsening of the previous deficit or a new visual defect 29).

A 15-year-old boy presented with an extremely rare optochiasmatic cavernous angioma. He was admitted to a special hospital with the complaint of blurred vision persisting for 1 month. Magnetic resonance imaging and biopsy of the lesion were inconclusive. He was admitted to the neurosurgical clinic after worsening of the visual symptoms 9 months later. Repeat magnetic resonance imaging showed optochiasmatic cavernous angioma which had doubled in size. The lesion was removed completely without any problem. Postoperatively his visual complaints remained stable, but had improved after 1 year. Optochiasmatic cavernous malformation should be treated by surgical excision, whereas biopsy is useless and may result in enlargement 30).

A 38-year-old male patient who suffered from acute onset of severe headache and progressive loss of vision. The vascular malformation of the optic pathways was completely removed via a pterional approach. This is the first reported instance of complete resection of a cavernoma involving the optic nerve, the chiasm, and the optic tract 31).


Muta et al. report a 14-year-old boy with cavernous malformation of the optic chiasm. He had a 2-year history of gradually worsening visual disturbance. Computed tomography (CT) and magnetic resonance imaging (MRI) revealed a suprasellar mass, findings compatible with craniopharyngioma. The mass was biopsied and histological examination confirmed cavernous malformation. On the second day after the biopsy, he suffered chiasmal apoplexy due to intratumoural haemorrhage, lost visual acuity and developed a field cut. Cavernous malformations arising from the optic nerve and chiasm are extremely rare; only 29 cases have been reported to date. Most patients manifested acute visual acuity and visual field disturbances. Although MRI findings of cavernous malformations in the brain parenchyma have been reported, MRI findings on the optic nerve and chiasm may not be completely diagnostic. Of the 29 documented patients, 16 underwent total resection of the lesion without exacerbation of their preoperative symptoms; in some cases, resection was complicated by risk of damage to the surrounding neural tissue. As patients may suffer intratumoural haemorrhage after biopsy or partial removal of the lesion, the advisability of surgical treatment of cavernous malformations of the optic nerve and chiasm must be considered carefully 32).

In their meticulous review of the literature, Lehner et al. found 42 previously reported patients with vascular malformations within optic nerves, chiasm, or optic tracts, 30 of them being cavernous hemangiomas. The optic chiasma was involved in 38 patients (90.5%) and a total excision of the tumor was performed in 21 cases. Lehner et al. published a patient with a cavernous haemangioma of the optic chiasma and left optic tract who presented with an acute defect of the right visual field and severe retro-orbital pain. They succeeded in total excision of the malformation via a neuronavigationally guided approach. In the postoperative course, vision of our patient improved immediately and was found to be completely normal three months after the surgical intervention. Considering this patient and the published cases in the literature, they are of the opinion that microsurgical excision is a safe and efficient treatment for these rare pathologies 33).


Shkarubo et al. describe a rare case of chiasmatic apoplexy whose cause was chiasmatic cavernoma. In addition to acute visual disorders suggesting the involvement of the left optic nerve, chiasma, and left visual pathway, 23-year-old patient had endocrine disorders as polyuria, polydipsia, which first suggests craniopharyngioma and glioma of the chiasma. A capsule and hematomic clots were removed from the thickened left optic nerve and left chiasmatic half during surgery. Only did a morphological study involving immunohistochemical analysis permit identification of the process as hemorrhage from cavernous micromalformation with the formation of hematoma 34).


Three patients with cavernomas of the optic nerve, chiasm, or optic tract are presented. All suffered progressive visual loss due to local hemorrhage and the space-occupying effects of the vascular malformation. Computed tomography scans revealed small lesions with mild contrast enhancement in the suprasellar and parasellar cisterns, whereas angiography was unremarkable. Magnetic resonance imaging was helpful in our cases both for diagnosis and for planning surgical approach, showing typical signs of cavernomas as confirmed by subsequent surgery and histological examination. The clinical and intraoperative findings are presented 35).


Buonaguidi et al. report a very rare case of an intrasellar cavernous hemangioma mimicking, clinically and neuroradiologically, the presence of a nonfunctioning pituitary adenoma. It was possible to diagnose this benign, congenital vascular malformation only through a histological examination36).
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Abou-Al-Shaar H, Bahatheq A, Takroni R, Al-Thubaiti I. Optic chiasmal cavernous angioma: A rare suprasellar vascular malformation. Surg Neurol Int. 2016 Aug 1;7(Suppl 18):S523-6. doi: 10.4103/2152-7806.187495. eCollection 2016. PubMed PMID: 27583178; PubMed Central PMCID: PMC4982351.
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Rheinboldt M, Blase J. Exophytic hypothalamic cavernous malformation mimicking an extra-axial suprasellar mass. Emerg Radiol. 2011 Aug;18(4):363-7. doi: 10.1007/s10140-011-0940-0. Epub 2011 Feb 9. PubMed PMID: 21305332.

Shibuya M, Baskaya MK, Saito K, Suzuki Y, Ooka K, Hara M. Cavernous malformations of the optic chiasma. Acta Neurochir (Wien). 1995;136(1-2):29-36. Review. PubMed PMID: 8748824.

Tien R, Dillon WP. MR imaging of cavernous hemangioma of the optic chiasm. J Comput Assist Tomogr. 1989 Nov-Dec;13(6):1087-8. PubMed PMID: 2584493.

Rigamonti D, Drayer BP, Johnson PC, Hadley MN, Zabramski J, Spetzler RF. The MRI appearance of cavernous malformations (angiomas). J Neurosurg. 1987 Oct;67(4):518-24. PubMed PMID: 3655889.

Campbell PG, Jabbour P, Yadla S, Awad IA. Emerging clinical imaging techniques for cerebral cavernous malformations: a systematic review. Neurosurg Focus. 2010 Sep;29(3):E6. doi: 10.3171/2010.5.FOCUS10120. Review. PubMed PMID: 20809764; PubMed Central PMCID: PMC3708641.

de Champfleur NM, Langlois C, Ankenbrandt WJ, Le Bars E, Leroy MA, Duffau H, Bonafé A, Jaffe J, Awad IA, Labauge P. Magnetic resonance imaging evaluation of cerebral cavernous malformations with susceptibility-weighted imaging. Neurosurgery. 2011 Mar;68(3):641-7; discussion 647-8. doi: 10.1227/NEU.0b013e31820773cf. PubMed PMID: 21164377.
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Ozer E, Kalemci O, Yücesoy K, Canda S. Optochiasmatic cavernous angioma: unexpected diagnosis. Case report. Neurol Med Chir (Tokyo). 2007 Mar;47(3):128-31. PubMed PMID: 17384496.

Holland AJ, Symon L. Spontaneous haemorrhage in a cavernoma associated with a suprasellar meningioma: an unusual post-operative complication. Br J Neurosurg. 1994;8(1):109-11. PubMed PMID: 8011186.

Deshmukh VR, Albuquerque FC, Zabramski JM, Spetzler RF. Surgical management of cavernous malformations involving the cranial nerves. Neurosurgery. 2003 Aug;53(2):352-7; discussion 357. Review. PubMed PMID: 12925251.
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Lehner M, Fellner FA, Wurm G. Cavernous haemangiomas of the anterior visual pathways. Short review on occasion of an exceptional case. Acta Neurochir (Wien). 2006 May;148(5):571-8; discussion 578. Epub 2006 Mar 2. Review. PubMed PMID: 16505967.

Venkataramana NK, Rao SA, Arun LN, Krishna C. Cavernous malformation of the optic chiasm: Neuro-endoscopic removal. Asian J Neurosurg. 2016 Jan-Mar;11(1):68-9. doi: 10.4103/1793-5482.145114. PubMed PMID: 26889286; PubMed Central PMCID: PMC4732249.

Trentadue M, Pozzi Mucelli R, Piovan E, Pizzini FB. Incidental optochiasmatic cavernoma: Case report of an unusual finding on 3 Tesla MRI. Neuroradiol J. 2016 Aug;29(4):289-94. doi: 10.1177/1971400916648335. Epub 2016 May 4. PubMed PMID: 27145992; PubMed Central PMCID: PMC4978328.

Alafaci C, Grasso G, Granata F, Cutugno M, Marino D, Salpietro FM, Tomasello F. Cavernous malformation of the optic chiasm: An uncommon location. Surg Neurol Int. 2015 Apr 16;6:60. doi: 10.4103/2152-7806.155256. eCollection 2015. PubMed PMID: 25949848; PubMed Central PMCID: PMC4405893.

Ning X, Xu K, Luo Q, Qu L, Yu J. Uncommon cavernous malformation of the optic chiasm: a case report. Eur J Med Res. 2012 Aug 14;17:24. doi: 10.1186/2047-783X-17-24. PubMed PMID: 22892383; PubMed Central PMCID: PMC3488017.

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Update: Optic pathway glioma

Optic pathway glioma

Optic pathway gliomas or suprasellar gliomas.
These tumours have sometimes been divided into optic pathway gliomas and hypothalamic gliomas (not to be confused with hypothalamic hamartomas). In cases where the tumour is confined to the optic nerves (Dodge stage 1), they can safely be referred to as optic nerve gliomas. Often, however, they are either centred on or extend to involve the chiasm and optic radiations. In such cases, they are difficult to distinguish from hypothalamic gliomas and such a distinction is in most instances artificial. In such more posterior cases the term hypothalamic-optochiasmatic glioma is perhaps more accurate although it certainly does not roll off the tongue.
As such, generally, the term optic pathway glioma is favoured, recognising that there may be involvement of the hypothalamus 1).
Optic pathway/hypothalamic gliomas (OPHGs) are generally benign but situated in an exquisitely sensitive brain region, and may involve the optic nerves, the optic chiasm, and the optic tracts.
Martin and Cushing (1923) first noted the difficulty of determining the site of origin of optic nerve gliomas, since these tend to extend up and down the optic pathways, often making it impossible to decide even at postmortem examination whether the growth originated in the chiasm and extended to the hypothalamus, or vice versa.


Gliomas of the optic chiasm and hypothalamus account for 10-15% of supratentorial tumors in children. Males and females are approximately equally affected.
Between 20 and 50% of patients with hypothalamic gliomas have a positive family history of neurofibromatosis type 1 (NF-1). Gliomas of the optic chiasm and hypothalamus in children with NF-1 usually have a more indolent course. Tumours may grow more slowly and occasionally regress spontaneously.
A retrospective, single-center, cohort study of 176 patients (93 boys), aged 6 years (range, 0.2-18 years), with hypothalamic-pituitary lesions showed craniopharyngioma (n = 56), optic pathway glioma (n = 54), suprasellar arachnoid cyst (n = 25), hamartoma (n = 22), germ cell tumor (n = 12), and hypothalamic-pituitary astrocytoma (n = 7). The most common presenting symptoms were neurologic (50%) and/or visual complaints (38%), followed by solitary endocrine symptoms (28%). Precocious puberty led to diagnosis in 19% of prepubertal patients (n = 131), occurring earlier in patients with hamartoma than in patients with optic-pathway glioma (P < .02). Isolated diabetes insipidus led to diagnosis for all germ-cell tumors. For 122 patients with neuro-ophthalmic presenting symptoms, the mean symptom interval was 0.5 year (95% CI, 0.4-0.6 year), although 66% of patients had abnormal body mass index or growth velocity, which preceded the presenting symptom interval onset by 1.9 years (95% CI, 1.5-2.4 years) (P < .0001) and 1.4 years (95% CI, 1-1.8 years) (P < .0001), respectively. Among them, 41 patients were obese before diagnosis (median 2.2 years [IQR, 1-3 years] prior to diagnosis) and 35 of them had normal growth velocity at the onset of obesity. The sensitivity of current guidelines for management of childhood obesity failed to identify 61%-85% of obese children with an underlying hypothalamic-pituitary lesion in this series 2).
These masses are, however, rare in adults and require tissue sampling for diagnosis 3).


Dodge classification
The most widely accepted classification of optic pathway gliomas was first proposed by Dodge et al in 1958, and divides these tumours into just three groups based on anatomical localisation:
stage 1: optic nerves only
stage 2: chiasm involved (with or without optic nerve involvement)
stage 3: hypothalamic involvement and/or other adjacent structures
A more recent modification of the Dodge classification has been proposed which further subdivides each stage.


Most are juvenile pilocytic astrocytomas, but their imaging characteristics are not specific with regard to their histologic features. Varying degrees of cystic change and enhancement are demonstrated. The tumours may appear smooth, fusiform, eccentric, or lobulated.
see hypothalamic pilocytic astrocytoma

Clinical features

Clinical features may include:
Decreased visual acuity / optic nerve atrophy ≈50%
Diencephalic syndrome 4) 5) 6).
Obesity; sexual precocity; endocrine dysfunction (short stature)
Diabetes insipidus.


They are characterised on imaging by an enlarged optic nerve seen either on CT or MRI.


Usually showing low T1 and high central T2 signal on MRI images, enhancement is variable.
MR imaging is optimal for showing the relationship of the mass to the hypothalamus, optic chiasm, and infundibulum as well as the intraorbital and intracanalicular components of the mass. Large tumours are typically heterogeneous with cystic and solid components.
T1: almost always hypo intense

T2: hyper intense

FLAIR: hyper intense

T1 C+ (Gd): solid components of large tumours often contrast enhance.

Differential diagnosis

The main differential is that of optic nerve sheath meningioma, however, the potential list is much longer including most causes of optic nerve enlargement.
The absence of calcification can be used to differentiate optic nerve glioma from optic nerve sheath meningioma.
Additionally, when the bulk of the tumour is located at the chiasm, the differential should include pituitary region masses.
They usually present earlier (first 5 years of age) than craniopharyngioma. Hypothalamic glioma poses a diagnostic dilemma with craniopharyngioma and other hypothalamic region tumors, when they present with atypical clinical or imaging patterns. Neuroimaging modalities especially MRI plays a very important role in scrutinizing the lesions in the hypothalamic region.
The main differentiating features between craniopharyngioma and hypothalamic glioma are the presence of mixed intensity cysts on T1 and calcification in craniopharyngioma and the relative young age of presentation in hypothalamic gliomas. Yet, it is not always possible to differentiate craniopharyngiomas from hypothalamic glioma. Thus, obtaining a tissue diagnosis via biopsy may be the right course of action in planning further management, whenever diagnosis is in doubt. 7).


If it is isolated to one optic nerve and does not extend to the chiasm, then resection is curative (albeit with the loss of vision in that eye). If the tumour extends to the chiasm or more posteriorly, then curative resection is not possible, with resection reserved for treatment of mass effects (proptosis, intracranial mass effect)
En-block removal of intraorbita tumor is recommended in cases with disfiguring exophthalmos and loss of vision.
Surgical resection of intraorbital optic nerve (ON) poses the risks of permanent ptosis and globe atrophy.

Optimal management of optic pathway/hypothalamic glioma (OPHG) remains an ongoing challenge. Little is known about the natural history, management strategies, and outcomes in adolescents.
see Anterior interhemispheric approach
Radiotherapy continues to be the mainstay of progressive OPHG management strategies. In the 2000s, chemotherapy has emerged as a therapeutic option for those <10 years of age aiming to avoid or at least postpone radiation to the immature brain 8).
Carboplatin-based chemotherapy is a useful modality in younger children, delaying radiation to their immature brains. National trials have focused on younger children and excluded adolescents from studies evaluating the role of chemotherapy.
In fact, many institutions continue to consider irradiation as the treatment of choice for progressive OPHGs in patients >10 years of age.
Chemotherapy is a valuable treatment modality for the achievement of disease control even in adolescents; their progression-free survival compares favorably with that in younger children. Chong et al. propose that chemotherapy be considered as a first-line modality in adolescents, avoiding potential radiation-associated morbidities 9).


Presenting at <1 year of age, diencephalic features, non-NF1 status, and location along the posterior pathway have been typically associated with a more aggressive disease course 10) 11).
Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. 12).

Case series


A retrospective case series study was led for 3 patients diagnosed with primary optic nerve and chiasm glioblastoma (GBM), coming from two referral neurosurgical centers. 2) An electronic search was conducted on MEDLINE via PUBMED, COCHRANE, from October 1973 to April 2016. Cohort, case reports, and case series were screened for investigating treatment and overall survivals of malignant optic nerve gliomas. Pooled means and 95% confidence intervals of overall survival for each treatment were generated.
1) From our retrospective case series, all patients had initial visual impairment (2 women and 1 man). The histological diagnosis was done by biopsy. The patients’ mean age of was 67.3 years (SD 18.5). The disease was rapidly lethal for all patients: median overall survival (OS) was 5 months (SD: 15.1). Two patients underwent chemotherapy by single cure of temozolomide, while the third one was treated with a radio-chemotherapy protocol. 2) Due to the fact that there is no gold standard treatment as first choice treatment, a large heterogeneity in first-choice oncological treatment is observed. However, we did not find any significant differences for overall survival between WHO grade III and grade IV optic gliomas.
Malignant optic glioma is a rare and fatal disease in adults. Despite the modalities of treatment, the treatment outcomes remain unsatisfactory. There is no significant difference in the median overall survival of patients with malignant optic nerve, as compared to those diagnosed with other supratentorial glioblastoma. Chemoradiotherapy with temozolomide currently remains the best treatment in terms of overall survival. Advances in the understanding of tumor biology have yet failed to translate into effective treatment regimens 13).


Ten patients managed surgically utilising ioMRI at Alder Hey Children’s Hospital between 2010 and 2013 were retrospectively identified. Demographic and relevant clinical data were obtained. MRI was used to estimate tumour volume pre-operatively and post-resection. If ioMRI demonstrated that further resection was possible, second-look surgery, at the discretion of the operating surgeon, was performed, followed by post-operative imaging to establish the final status of resection. Tumour volume was estimated for each MR image using the MRIcron software package.
Control of tumour progression was achieved in all patients. Seven patients had, on table, second-look surgery with significant further tumour resection following ioMRI without any surgically related mortality or morbidity. The median additional quantity of tumour removed following second-look surgery, as a percentage of the initial total volume, was 27.79 % (range 11.2-59.2 %). The final tumour volume remaining with second-look surgery was 23.96 vs. 33.21 % without (p = 0.1).
OPHGs are technically difficult to resect due to their eloquent location, making them suitable for debulking resection only. IoMRI allows surgical goals to be reassessed intra-operatively following primary resection. Second-look surgery can be performed if possible and necessary and allows significant quantities of extra tumour to be resected safely. Although the clinical significance of additional tumour resection is not yet clear, we suggest that ioMRI is a safe and useful additional tool, to be combined with advanced neuronavigation techniques for partial tumor resection 14).


Forty-two patients were treated between 1998 and 2011. Their median age at diagnosis was 5 years 7 months. Nineteen patients were positive for neurofibromatosis Type 1 (NF1) and 23 patients were negative for NF1. The median duration of follow-up was 77 months (range 21.8-142.3 months). Presenting symptoms included visual impairment (in 50% of cases), headache (in 24%), and hypothalamic/pituitary dysfunction (in 29%). Twenty-two debulking procedures were performed in 21 patients. Four biopsies (3 open, 1 endoscopic) were also performed. The histological diagnosis was pilocytic astrocytoma in 21 patients and pilomyxoid astrocytoma in 2 patients. Ten patients (Group 1) had primary surgical debulking alone and were then observed. Four patients (Group 2) had surgical debulking, plus planned chemotherapy within 3 months. Seven patients (Group 3) required surgical debulking for progressive disease following a variety of treatments. Patient age had the greatest impact on subsequent tumor progression. In total, 13 patients received chemotherapy, 4 on initial presentation, 4 in combination with surgery, and 5 for further tumor progression. Five patients were treated with radiotherapy, 3 prior to referral to Alder Hey. Eleven patients required shunt insertion for hydrocephalus. Vision was stabilized for 74% of patients. The number of patients with hypothalamic/pituitary dysfunction increased from 12 at presentation to 16 by the end of treatment. The overall survival rate was 93%. Three patients died-1 from tumor progression, 1 from infective complications from tumor biopsy, and 1 from a spontaneous posterior fossa hemorrhage. NF1 was associated with improved outcome-fewer patients required active intervention and rates of visual impairment and/or or hypothalamic/pituitary dysfunction were lower.
Good long-term survival and functional outcomes can be achieved in children with OPHG. Tumor control was achieved through an individualized approach using surgery, chemotherapy, or radiotherapy in varied combinations. The authors aim to limit radiotherapy to cases involving older children in whom other therapies have failed, due to the well-described and often devastating late effects associated with midline cranial irradiation. Surgery has a clear role for diagnosis, tumor control, and relief of mass effect. In particular, primary surgical debulking of tumor (without adjuvant therapy) is safe and effective. Recent advances in intraoperative MRI may add value and need further assessment 15).

Zoli et al. analyze their experience with hypothalamic gliomas treated via the endoscopic endonasal approach. Methods Consecutive cases of hypothalamic gliomas treated since 2007 via an endoscopic endonasal approach were reviewed. Preoperative and postoperative neuroimaging as well as endocrinological, neurological, and visual symptoms were analyzed to assess the surgical outcome. Signs and symptoms of hypothalamic dysfunction including body mass index (BMI), memory, sleep-wake rhythm, and polyphagia were prospectively collected pre- and postoperatively to assess hypothalamic function. Quality of life was evaluated using the Katz scale. Results In the initial phase the endoscopic endonasal approach was adopted in 3 cases with a palliative intent, to obtain a biopsy sample or for debulking of the mass followed by radio- or chemotherapy. In 2 later cases it was successfully adopted to achieve gross-total tumor resection. Complications consisted of 2 postoperative CSF leaks, which required an endoscopic endonasal reintervention. Visual deficit improved in 3 cases and normalized in the other 2. Four patients developed diabetes insipidus, and 3 an anterior panhypopituitarism. All patients had a moderate increase in BMI. No patients presented with any other signs of hypothalamic damage, and their quality of life at follow-up is normal. Conclusions Despite the limitations of a short follow-up and small sample, the authors’ early experience with the endoscopic endonasal approach has revealed it to be a direct, straightforward, and safe approach to third ventricle astrocytomas. It allowed the authors to perform tumor resection with the same microsurgical technique: dissecting the tumor with 2 hands, performing a central debulking, and controlling the bleeding with bipolar coagulation. The main limitations were represented by some anatomical conditions, such as the position of the chiasm and the anterior communicating artery complex and, finally, by the challenge of watertight plastic repair. To definitively evaluate the role of this approach in hypothalamic gliomas, a comparison with transcranial series would be necessary, but due to the rarity of these cases such a study is still lacking. The authors observed that more aggressive surgery is associated with a worse endocrinological outcome; thus they consider it to be an open question (in particular in prepubertal patients) whether radical removal is an advisable goal for hypothalamic gliomas. 16).

A retrospective review of patients diagnosed with suprasellar glioma between 2000-October 2012, included patients diagnosed with optic pathway glioma based on radiological features (with or without biopsy) and those who had a biopsy confirming pilocytic astrocytoma.
Fifty-three patients included (sporadic tumours 24 and NF1 related 29). Fifteen sporadic and four NF1 patients were biopsied. Twelve sporadic and 13 NF1 patients were initially treated with chemotherapy while only 1 patient had radiotherapy initially. Progression was noted in 58 % of the sporadic group and 24 % of the NF1 group. The only significant factor for progression was NF1 status (p = 0.026).
Management should be guided by individual patient circumstance. In our cohort, chemotherapy did not significantly improve progression free survival; however, NF1 status significantly correlated with the decreased progression 17).


A total of 101 patients with optic glioma newly diagnosed between 1975 and 2008 were evaluated retrospectively. COPP (cyclophosphamide, vincristine, procarbazine, prednisolone) and cisplatin plus etoposide were the most commonly used chemotherapy regimens. Radiotherapy was administered in patients with progressive or unresponsive disease.
The median age at the time of diagnosis was 6 years, and the male/female ratio was 1.15. The most common referral complaint was strabismus. The most common site of optic glioma was the hypothalamic-chiasmatic region (31.7%). Fifty-three patients (52.5%) had neurofibromatosis type 1 (NF-1). Treatment consisted of surgery, radiotherapy, and chemotherapy. Forty-nine patients (48.5%) underwent surgery, which was predominantly subtotal resection, radiotherapy was administered to 39.4%, and 30 patients received chemotherapy. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 65.8% and 88.4%, respectively, and the 10-year PFS and OS were 54.2% and 83.4%, respectively, with an 8-year median follow-up. OS was significantly lower in patients with hypothalamo-chiasmatic involvement and significantly higher in patients with NF-1. The 5- and 10-year PFS rates were significantly higher in patients 10 years or older at diagnosis (P=0.0001) and in patients with intraorbital involvement (P=0.032). Eighteen patients (17.8%) died of disease.
Patients with NF-l and those older than 10 years have a better prognosis, whereas patients younger than 3 years and those with hypothalamic-chiasmatic optic glioma have a worse outcome. Further studies are needed to find appropriate treatment strategies 18).


Diencephalic syndrome and its relation to opticochiasmatic glioma: review of twelve cases 19).


Twenty children with hypothalamic gliomas from the Hospital for Sick Children, Great Ormond Street, were reviewed in an attempt to determine the appropriate management. If the child’s condition at the time of diagnosis is such that survival for several months is likely, the long-term prognosis for good survival is excellent and is enhanced by treatment. It is concluded that radiotherapy has a definite beneficial effect and should be given to every child not presenting in poor or critical condition. These are best left untreated. An exception is a child with the diencephalic syndrome whose general condition is poor but there is little or no impairment of consciousness; such a child should be treated by radiotherapy sometimes preceded by a shunt operation. If there is increased intracranial pressure with radiological evidence to suggest that the obstruction may be relieved by operation, then partial removal should be carried out. If it is judged that obstruction cannot be relieved, a shunting procedure is required. Craniotomy is also indicated if there is anything in the clinical or radiological examination to suggest that the lesion may be extracerebral. Otherwise, biopsy through a burr hole may be adequate for confirmation of the diagnosis 20).

Case reports


Loh et al. present here the case of a 4-year-old boy with exophthalmos and near blindness due to an intraorbital OPG. Despite chemotherapy he showed progressive exophthalmos and vision loss. Bony orbital decompression with ON transection temporally reduced his exophthalmos. OPG resection was required later for recurrence of his exophthalmos secondary to tumor progression. Post operatively, he had preserved oculomotor nerve functions but developed globe ischemia. Unusually, his ischemic globe caused him to have pain and severe photophobia, which later lead to enucleation. Photophobia has been reported in blind patients. Animal models and MRI functional imaging showed activation of trigeminal pathway during photophobia in completely transected ON. However, the exact neuro-ophthalmology pathway requires further study.
This is the first described case of photophobia after excision of OPG with ON denervation. Photophobia can be a serious side effect that significantly lowers the patient’s quality of life 21).


Cavicchiolo et al. describe the case of a 3-year-old child, diagnosed with familial neurofibromatosis type 1 (NF1) and asymptomatic optic pathway tumor at the age of two, who developed diencephalic syndrome (DS) due to tumor progression 1 year after diagnosis. Magnetic resonance imaging disclosed an enlarging hypothalamic contrast-enhanced mass. Because of the tumor progression, in terms of tumor volume and DS, chemotherapy (CT) treatment was started according to the international protocol for progressive low-grade glioma, with rapid clinical improvement in terms of gain weight and DS resolution. Interestingly, tumor volume was unchanged after CT.
This case report highlights the following facts: (1) optic pathway glioma (OPG) in young children with NF1 may have definitive growth potentials and thus, they are worth an accurate clinical follow-up; (2) also, OPG occurring in NF1 patients can be responsible for DS in case of hypothalamus involvement; (3) consequently, the child’s growth pattern must be included among the clinical parameters, which must be specifically evaluated during the follow-up of children, with or without NF1, bearing an OPG; and, finally, (4) that DS can improve after CT, even in face of a stable tumor volume 22).

Vyas et al. report a case of a hypothalamic glioma masquerading as a craniopharyngioma on imaging along with brief review of both the tumors 23).

Diffusion tensor imaging localization of the pyramidal tract and spectroscopy in diencephalic pilocytic astrocytoma: a case report 24).


the authors present an unusual radiographic appearance of a Pilomyxoid astrocytoma (PMA) in an 11-year-old child. Preoperative images suggested a dural-based, homogenously enhancing lesion coupled with an enlarged optic nerve. Surgery revealed an intraparenchymal lesion of the right temporal lobe. There was hyperintensity on T2 MRI sequences, suggesting infiltration of the tumor along the optic tracts 25).


A retrospective, single-center, cohort study of 176 patients (93 boys), aged 6 years (range, 0.2-18 years), with hypothalamic-pituitary lesions was performed. The lesions were craniopharyngioma (n = 56), optic pathway glioma (n = 54), suprasellar arachnoid cyst (n = 25), hamartoma (n = 22), germ cell tumor (n = 12), and hypothalamic-pituitary astrocytoma (n = 7). The most common presenting symptoms were neurologic (50%) and/or visual complaints (38%), followed by solitary endocrine symptoms (28%). Precocious puberty led to diagnosis in 19% of prepubertal patients (n = 131), occurring earlier in patients with hamartoma than in patients with optic-pathway glioma (P < .02). Isolated diabetes insipidus led to diagnosis for all germ-cell tumors. For 122 patients with neuro-ophthalmic presenting symptoms, the mean symptom interval was 0.5 year (95% CI, 0.4-0.6 year), although 66% of patients had abnormal body mass index or growth velocity, which preceded the presenting symptom interval onset by 1.9 years (95% CI, 1.5-2.4 years) (P < .0001) and 1.4 years (95% CI, 1-1.8 years) (P < .0001), respectively. Among them, 41 patients were obese before diagnosis (median 2.2 years [IQR, 1-3 years] prior to diagnosis) and 35 of them had normal growth velocity at the onset of obesity. The sensitivity of current guidelines for management of childhood obesity failed to identify 61%-85% of obese children with an underlying hypothalamic-pituitary lesion in this series.

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