Acute management of ruptured cavernous malformation of the optic nerve: illustrative case

Philip Kawalec Section of Neurosurgery, Department of Surgery, University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba, Canada

Search for other papers by Philip Kawalec in
Current site
jns
Google Scholar
PubMed
Close
 MD
,
Marc R Del Bigio Department of Pathology, University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba, Canada; and
Children’s Hospital Research Institute of Manitoba, University of Manitoba, Winnipeg, Manitoba, Canada

Search for other papers by Marc R Del Bigio in
Current site
jns
Google Scholar
PubMed
Close
 MD,PhD
, and
Anthony M Kaufmann Section of Neurosurgery, Department of Surgery, University of Manitoba, Health Sciences Centre, Winnipeg, Manitoba, Canada

Search for other papers by Anthony M Kaufmann in
Current site
jns
Google Scholar
PubMed
Close
 MD
Open access

BACKGROUND

A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve cavernous malformations is generally surgical, although the timing of surgery is controversial.

OBSERVATIONS

A 47-year-old female experienced the sudden loss of vision in her left eye. Examination showed that this eye was nearly blind, and her right eye had a temporal field defect. Neuroimaging showed hemorrhage in her left optic nerve and optic chiasm. She was taken to the operating room on an emergent basis where the optic canal was decompressed, the hemorrhage was evacuated, and a vascular malformation with features of a cavernoma was removed from the optic nerve. Over the next 2 days, the vision in her right eye significantly recovered.

LESSONS

CMONs remain rare, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, the authors recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic nerve decompression, hematoma evacuation, and cavernoma resection to improve chances of vision recovery and prevent further vision loss.

ABBREVIATIONS

CMON = cavernous malformation of the optic nerve; CT = computed tomography; MRI = magnetic resonance imaging

BACKGROUND

A cavernous malformation of the optic nerve (CMON) is a rare condition that often presents with an abrupt decline in vision. Acute management of ruptured optic nerve cavernous malformations is generally surgical, although the timing of surgery is controversial.

OBSERVATIONS

A 47-year-old female experienced the sudden loss of vision in her left eye. Examination showed that this eye was nearly blind, and her right eye had a temporal field defect. Neuroimaging showed hemorrhage in her left optic nerve and optic chiasm. She was taken to the operating room on an emergent basis where the optic canal was decompressed, the hemorrhage was evacuated, and a vascular malformation with features of a cavernoma was removed from the optic nerve. Over the next 2 days, the vision in her right eye significantly recovered.

LESSONS

CMONs remain rare, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, the authors recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic nerve decompression, hematoma evacuation, and cavernoma resection to improve chances of vision recovery and prevent further vision loss.

ABBREVIATIONS

CMON = cavernous malformation of the optic nerve; CT = computed tomography; MRI = magnetic resonance imaging

Cavernous malformations of the optic nerve (CMONs) are rare conditions, making up less than 1% of intracranial cavernous malformations.1,2 They can present with acute visual decline or, less commonly, with progressive visual decline over a longer period.3 Management is often surgical; however, there is no consensus on the timing of surgery, whether it be emergent, urgent, or elective. Here, we present the extremely rare case of a previously healthy female who developed acute visual deficits and was found to have hemorrhage in her left optic nerve and optic chiasm.

Illustrative Case

History and Examination

A 47-year-old female with no past medical history awoke in the early morning with a sudden-onset, moderate headache. She took ibuprofen and the headache resolved; however, approximately 1 hour later, she began to rapidly lose vision in her left eye. Over the next 2 hours, she also noticed changes in peripheral vision in her right eye.

She was first seen in a community hospital emergency department, where a contrast-uninfused computed tomography (CT) scan of the brain and a contrast-infused CT scan of the circle of Willis were ordered to rule out a stroke. These showed an expansile hyperdensity extending along the course of the left optic nerve into the optic chiasm. There was no evidence of subarachnoid hemorrhage, aneurysm, or large vessel occlusion (Fig. 1A and B).

FIG. 1
FIG. 1

A: Preoperative axial contrast-uninfused CT showing hyperdense acute blood in the optic nerve (red arrow) compressing the optic chiasm. B: A coronal contrast-uninfused CT once again showing the hemorrhage in the left optic nerve (red arrow). C: Coronal T1-weighted MRI showing dilatation of the left prechiasmatic optic nerve up to 8 mm and resultant compression of the optic chiasm. D: Axial T2-weighted MRI showing the enlarged left optic nerve with an area of relative hypointensity that correlates with the area of acute hemorrhage seen on earlier CT.

Urgent magnetic resonance imaging (MRI) confirmed marked expansion of the prechiasmatic optic nerve up to 8 mm in diameter, extending into the optic chiasm and superiorly into the left optic tract. There was an area of decreased T2-weighted signal intensity in the left optic nerve that correlated to the area of hyperdensity on CT; this was consistent with hemorrhage (Fig. 1C and D) as well as surrounding optic nerve, chiasm, and tract edema seen on fluid-attenuated inversion recovery sequences. There was no significant enhancement after the administration of gadolinium. The differential diagnosis at the time remained broad, with spontaneous optic nerve hemorrhage and hemorrhage into an optic nerve glioma reported as top possibilities.

The patient was later transported to our tertiary care center neurosurgical service. She had a Glasgow Coma Scale score of 15/15 and was neurologically intact aside from her vision. There was near-complete loss of vision in her left eye (able to discern the presence of bright light but unable to count fingers). She also had a dense temporal visual field defect in her right eye, which was worse in the superior temporal field. The central vision and nasal visual fields in her right eye were intact. She reported that her vision had stabilized after the initial deterioration 9 hours earlier.

A decision was made to perform emergent surgery. The intended goals were to decompress the left optic canal, evacuate the hematoma from the nerve, and obtain a tissue sample for diagnosis.

Operation

A traditional left pterional craniotomy was performed with extradural removal of the sphenoid ridge down to the anterior clinoid base. A wide sylvian dissection was performed together with an opening of the optic and carotid cisterns that facilitated elevation of the frontal lobe with minimal retraction (Video 1). The dural falciform ligament was opened, the optic canal roof was removed with a drill, and the optic sheath was incised.

VIDEO 1. Clip showing dissection of the sylvian fissure, evacuation of left optic nerve hematoma, and resection of the CMON. Click here to view.

The left optic nerve was swollen with an area of dark discoloration bulging inferolaterally. The thin layer of epineurium overlying this hematoma was incised sharply, and a semiliquid blood clot was evacuated using microsuction. While exploring the hematoma cavity for residual clot, an area of abnormal tissue was noticed in the anterior portion of the cavity. This tissue was firmer and had a reddish-brown color in comparison with the surrounding neural tissue. Grossly, it had the appearance of a cavernous malformation. It was dissected from the optic nerve, removed in one large 0.9 × 0.6 cm piece, and sent to pathology (Fig. 2).

FIG. 2
FIG. 2

A: Intraoperative photograph showing a swollen left optic nerve with a focal, inferolaterally facing, discolored outpouching of the nerve secondary to the hematoma (arrowhead), as well as more subtle areas of discoloration due to the underlying hematoma (arrow). The planned incision to decompress the optic nerve is indicated by the dashed line. B: Intraoperative photograph showing the left optic nerve after decompression and resection of the cavernoma.

Postoperative Course

Within 6 hours after surgery, the patient began to report improvements in the previously dense temporal field defect in the right eye. Over the next 48 hours, her right eye vision continued to improve to a point subjectively comparable to her previous vision. Unfortunately, the vision in her left eye did not improve. Twenty-four–hour postoperative MRI showed diminished bulk of the left optic nerve and complete resection of the lesion. Two-month follow-up MRI showed no discernible lesion and resolving products of hemorrhage (Fig. 3).

FIG. 3
FIG. 3

Twenty-four–hour (A) and 2-month (B) postoperative coronal high-resolution T2-weighted MRI showing complete evacuation of the hemorrhage and left optic nerve lesion. Note the hypointensity within the optic nerve caused by hemosiderin staining.

At the 2-month follow-up with a neuro-ophthalmologist, the patient had a residual superior temporal visual field deficit in her right eye and a large central scotoma in her left eye. Her central vision had improved only slightly; on Humphrey peripheral field testing, she scored a mean deviation of −27 dB 2 months postsurgery compared with −30 dB 1 month postsurgery. However, her left-eye peripheral vision significantly improved, to the point that she could count fingers in all peripheral fields. Her vision was sufficient to meet the requirements for a full driver’s license.

Histopathology

Microscopic examination of the fragmented optic nerve lesion (approximately half of the total specimen) showed collections of abnormal blood vessels (up to 200 µm in diameter; (approximately half of the total specimen). Some were packed together, and others were isolated within the surrounding nerve. Some vessel walls were collagenous (up to 200 µm thick) and lacked an elastic lamina. Congo red stain showed no amyloid. Immunostaining for CD34 also showed a network of collapsed, thin-walled vascular channels up to 400 µm in diameter. The vessels were surrounded by myelinated optic nerve axons. Immunostaining for amyloid precursor protein showed clusters of damaged axons in the optic nerve. Abundant hemosiderin was demonstrated by Perls Prussian blue stain. The final interpretation was vascular malformation, with features of a cavernous malformation (Fig. 4).

FIG. 4
FIG. 4

Pathology of the optic nerve lesion. A: Abnormal thick-walled blood vessel surrounded by myelinated axons. Myelin is stained blue. Solochrome cyanin and eosin stain, original magnification ×100. B: Abnormal thick-walled blood vessels with mainly collagenous walls. Collagen is stained pink. Humberstone, original magnification ×100. C: Extensive hemosiderin (blue) in optic nerve tissue. Perls Prussian blue, original magnification ×200. D: Abnormal large- and small-diameter, thin-walled blood vessels. Endothelial cells are brown. CD34 immunostain and hematoxylin, original magnification ×100.

Patient Informed Consent

The necessary patient informed consent was obtained in this study.

Discussion

Observations

Intracranial cavernous malformations occur in 0.4% to 0.8% of the population, and CMONs are estimated to make up less than 1% of all central nervous system cavernous malformations.1–4 Patients with CMON often present with sudden-onset visual disturbances termed “chiasmal apoplexy” due to extralesional hemorrhage, as in our case.1,5–10 Some patients present less acutely with progressive visual disturbances, likely secondary to progressive expansion caused by intralesional hemorrhage.11–15 Although these two groups of patients are not described as separate entities in the literature, we believe they warrant individualized treatment. Acute hemorrhage into the optic nerve, whether from a CMON or other pathology, should be treated with emergency decompression of the optic nerve to preserve any remaining vision, whereas a slowly progressing CMON can be treated on a more urgent-elective basis. Indeed, it is likely that many CMONs that do not present acutely go undiagnosed, because more than two-thirds of cases in the literature present with acute extralesional hemorrhage.3

MRI features of CMON are less stereotypical than with brainstem or cerebral cavernomas. In one large review of anterior visual pathway cavernomas, more than one-third of the cases were initially misdiagnosed after MRI.16 Generally, MRI features of CMON include lesion hyperintensity on T1-weighted MRI and hypodensity on T2-weighted MRI. The differential diagnosis for undifferentiated cases with such imaging features is often broad and can include optic nerve gliomas, craniopharyngiomas, other vascular malformations, as well as nonsurgical pathologies such as optic neuritis or demyelination disorders.3,16

There is no consensus on the optimal treatment of these rare lesions. Most reported cases have been treated surgically,3 although conservative management is likely underreported.17,18 In general, most authors of previous reports in the literature have supported operative management. In a review of 70 symptomatic cases found in the literature, 91% had some sort of surgical intervention, although there was no consensus on the timing of surgery.3 We undertook an emergent approach to evacuate the optic nerve hematoma and decompress the chiasm in an effort to prevent further vision loss in our patient, with good results.

In general, surgical management of CMONs presenting with extralesional hemorrhage yields positive results.3 Gross-total resection usually prevents further visual decline, and some cases even improve quite rapidly, as in our case. This improvement is likely secondary to decompression of the optic nerve fibers and chiasm.

Our patient had a significant and early recovery after emergent optic canal decompression, hematoma evacuation, and gross-total lesion resection from the optic nerve. This case shows that early surgery for CMON-related hematoma can lead to improved outcomes and a decreased risk of future harm.

Lessons

CMONs remain rare lesions, and it is unlikely that enough cases can be gathered to form a larger trial to compare the role and timing of surgery. On the basis of our experience with this case, we recommend that acute CMON-related hematomas should be treated as a surgical emergency and managed with acute optic canal decompression, hematoma evacuation, and cavernoma resection to improve the chances of vision recovery and prevent further vision loss.

Author Contributions

Conception and design: Kaufmann, Kawalec. Acquisition of data: all authors. Analysis and interpretation of data: all authors. Drafting the article: Kaufmann, Kawalec. Critically revising the article: all authors. Reviewed submitted version of manuscript: Kaufmann, Kawalec. Approved the final version of the manuscript on behalf of all authors: Kaufmann. Administrative/technical/material support: Kaufmann, Kawalec. Study supervision: Kaufmann.

Supplemental Information

Videos

References

  • 1

    Hempelmann RG, Mater E, Schröder F, Schön R Complete resection of a cavernous haemangioma of the optic nerve, the chiasm, and the optic tract. Acta Neurochir (Wien). 2007;149(7):699703.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Batra S, Lin D, Recinos PF, Zhang J, Rigamonti D Cavernous malformations: natural history, diagnosis and treatment. Nat Rev Neurol. 2009;5(12):659670.

  • 3

    Tan T, Tee JW, Trost N, McKelvie P, Wang YY Anterior visual pathway cavernous malformations. J Clin Neurosci. 2015;22(2):258267.

  • 4

    Srinivasan VM, Koester SW, Wang MS, et al. Cavernous malformations of the optic nerve and optic pathway: a case series and systematic review of the literature. Oper Neurosurg (Hagerstown). 2021;21(5):291302.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Newman H, Nevo M, Constantini S, Maimon S, Kesler A Chiasmal cavernoma: a rare cause of acute visual loss improved by prompt surgery. Pediatr Neurosurg. 2008;44(5):414417.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Regli L, de Tribolet N, Regli F, Bogousslavsky J Chiasmal apoplexy: haemorrhage from a cavernous malformation in the optic chiasm. J Neurol Neurosurg Psychiatry. 1989;52(9):10951099.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Son DW, Lee SW, Choi CH Cavernous malformation of the optic chiasm: case report. J Korean Neurosurg Soc. 2008;44(2):8890.

  • 8

    Campero A, Casas-Parera I, Villalonga JF, Baldoncini M Microsurgical resection of a chiasmatic cavernoma: 3-dimensional operative video. Oper Neurosurg (Hagerstown). 2021;21(1):E46E47.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Deshmukh VR, Albuquerque FC, Zabramski JM, Spetzler RF Surgical management of cavernous malformations involving the cranial nerves. Neurosurgery. 2003;53(2):352357.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Kostic M, Subramanian PS, Falcone SF, et al. Cavernous malformation of the optic nerve and chiasm: prompt suspicion and surgery matter. J Neuroophthalmol. 2022;42(1):108114.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    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;17(1):24.

  • 12

    Özer E, Kalemci O, Yücesoy K, Canda S Optochiasmatic cavernous angioma: unexpected diagnosis. Case report. Neurol Med Chir (Tokyo). 2007;47(3):128131.

  • 13

    Stellon MA, Elliott RJ, Taheri MR, Jean WC Cavernous malformation of the intracranial optic nerve with operative video and review of the literature. BMJ Case Rep. 2020;13(12):e236550.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Sun XY, Yu F Microsurgical resection of a cavernous angioma that involves the optic pathway using a pterional approach: a case report and literature review. Br J Neurosurg. 2012;26(6):882885.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Voznyak O, Lytvynenko A, Maydannyk O, Kalenska O, Hryniv N Cavernous hemangioma of the chiasm and left optic nerve. Cureus. 2020;12(5):e8068.

  • 16

    Liu JK, Lu Y, Raslan AM, Gultekin SH, Delashaw JB Jr. Cavernous malformations of the optic pathway and hypothalamus: analysis of 65 cases in the literature. Neurosurg Focus. 2010;29(3):E17.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Marnat G, Gimbert E, Berge J, Rougier MB, Molinier S, Dousset V Chiasmatic cavernoma haemorrhage: to treat or not to treat? Concerning a clinical case. Neurochirurgie. 2015;61(5):343346.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Panczykowski D, Piedra MP, Cetas JS, Delashaw JB Jr. Optochiasmatic cavernous hemangioma. Br J Neurosurg. 2010;24(3):301302.

  • Collapse
  • Expand
  • FIG. 1

    A: Preoperative axial contrast-uninfused CT showing hyperdense acute blood in the optic nerve (red arrow) compressing the optic chiasm. B: A coronal contrast-uninfused CT once again showing the hemorrhage in the left optic nerve (red arrow). C: Coronal T1-weighted MRI showing dilatation of the left prechiasmatic optic nerve up to 8 mm and resultant compression of the optic chiasm. D: Axial T2-weighted MRI showing the enlarged left optic nerve with an area of relative hypointensity that correlates with the area of acute hemorrhage seen on earlier CT.

  • FIG. 2

    A: Intraoperative photograph showing a swollen left optic nerve with a focal, inferolaterally facing, discolored outpouching of the nerve secondary to the hematoma (arrowhead), as well as more subtle areas of discoloration due to the underlying hematoma (arrow). The planned incision to decompress the optic nerve is indicated by the dashed line. B: Intraoperative photograph showing the left optic nerve after decompression and resection of the cavernoma.

  • FIG. 3

    Twenty-four–hour (A) and 2-month (B) postoperative coronal high-resolution T2-weighted MRI showing complete evacuation of the hemorrhage and left optic nerve lesion. Note the hypointensity within the optic nerve caused by hemosiderin staining.

  • FIG. 4

    Pathology of the optic nerve lesion. A: Abnormal thick-walled blood vessel surrounded by myelinated axons. Myelin is stained blue. Solochrome cyanin and eosin stain, original magnification ×100. B: Abnormal thick-walled blood vessels with mainly collagenous walls. Collagen is stained pink. Humberstone, original magnification ×100. C: Extensive hemosiderin (blue) in optic nerve tissue. Perls Prussian blue, original magnification ×200. D: Abnormal large- and small-diameter, thin-walled blood vessels. Endothelial cells are brown. CD34 immunostain and hematoxylin, original magnification ×100.

  • 1

    Hempelmann RG, Mater E, Schröder F, Schön R Complete resection of a cavernous haemangioma of the optic nerve, the chiasm, and the optic tract. Acta Neurochir (Wien). 2007;149(7):699703.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 2

    Batra S, Lin D, Recinos PF, Zhang J, Rigamonti D Cavernous malformations: natural history, diagnosis and treatment. Nat Rev Neurol. 2009;5(12):659670.

  • 3

    Tan T, Tee JW, Trost N, McKelvie P, Wang YY Anterior visual pathway cavernous malformations. J Clin Neurosci. 2015;22(2):258267.

  • 4

    Srinivasan VM, Koester SW, Wang MS, et al. Cavernous malformations of the optic nerve and optic pathway: a case series and systematic review of the literature. Oper Neurosurg (Hagerstown). 2021;21(5):291302.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5

    Newman H, Nevo M, Constantini S, Maimon S, Kesler A Chiasmal cavernoma: a rare cause of acute visual loss improved by prompt surgery. Pediatr Neurosurg. 2008;44(5):414417.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6

    Regli L, de Tribolet N, Regli F, Bogousslavsky J Chiasmal apoplexy: haemorrhage from a cavernous malformation in the optic chiasm. J Neurol Neurosurg Psychiatry. 1989;52(9):10951099.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7

    Son DW, Lee SW, Choi CH Cavernous malformation of the optic chiasm: case report. J Korean Neurosurg Soc. 2008;44(2):8890.

  • 8

    Campero A, Casas-Parera I, Villalonga JF, Baldoncini M Microsurgical resection of a chiasmatic cavernoma: 3-dimensional operative video. Oper Neurosurg (Hagerstown). 2021;21(1):E46E47.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9

    Deshmukh VR, Albuquerque FC, Zabramski JM, Spetzler RF Surgical management of cavernous malformations involving the cranial nerves. Neurosurgery. 2003;53(2):352357.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 10

    Kostic M, Subramanian PS, Falcone SF, et al. Cavernous malformation of the optic nerve and chiasm: prompt suspicion and surgery matter. J Neuroophthalmol. 2022;42(1):108114.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11

    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;17(1):24.

  • 12

    Özer E, Kalemci O, Yücesoy K, Canda S Optochiasmatic cavernous angioma: unexpected diagnosis. Case report. Neurol Med Chir (Tokyo). 2007;47(3):128131.

  • 13

    Stellon MA, Elliott RJ, Taheri MR, Jean WC Cavernous malformation of the intracranial optic nerve with operative video and review of the literature. BMJ Case Rep. 2020;13(12):e236550.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 14

    Sun XY, Yu F Microsurgical resection of a cavernous angioma that involves the optic pathway using a pterional approach: a case report and literature review. Br J Neurosurg. 2012;26(6):882885.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15

    Voznyak O, Lytvynenko A, Maydannyk O, Kalenska O, Hryniv N Cavernous hemangioma of the chiasm and left optic nerve. Cureus. 2020;12(5):e8068.

  • 16

    Liu JK, Lu Y, Raslan AM, Gultekin SH, Delashaw JB Jr. Cavernous malformations of the optic pathway and hypothalamus: analysis of 65 cases in the literature. Neurosurg Focus. 2010;29(3):E17.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17

    Marnat G, Gimbert E, Berge J, Rougier MB, Molinier S, Dousset V Chiasmatic cavernoma haemorrhage: to treat or not to treat? Concerning a clinical case. Neurochirurgie. 2015;61(5):343346.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18

    Panczykowski D, Piedra MP, Cetas JS, Delashaw JB Jr. Optochiasmatic cavernous hemangioma. Br J Neurosurg. 2010;24(3):301302.

Metrics

All Time Past Year Past 30 Days
Abstract Views 0 0 0
Full Text Views 302 302 30
PDF Downloads 174 174 15
EPUB Downloads 0 0 0