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.

References

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

References

1)

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.
2)

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.
3)

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.
4)

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.
5)

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.
6)

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
7)

Rajajee V, Vanaman M, Fletcher JJ, Jacobs TL. Optic nerve ultrasound for the detection of raised intracranial pressure. Neurocrit Care. 2011;15:506–15.
8)

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.
10)

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.
11)

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.
12)

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.
13)

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.
14)

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.
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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.
17)

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.
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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.
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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.
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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.
21)

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

Epidemiology

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).

Diagnosis

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

MRI

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).

Treatment

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).

Reviews

2006

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

2016

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).

2015

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).

2014

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).

2012

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).

2011

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).

2008

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).

2007

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).

2006

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).

2005

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).

1989

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).

1984

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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