Abstract

Background: Cavernous sinus meningiomas are rare, and radiotherapy is considered because the risk of postoperative complications is high. Radiotherapy is useful for short-term control of meningiomas, but hydrocephalus may appear as a long-term complication.

Case Description: A 71-year-old male patient suffered from a cavernous sinus meningioma with orbital involvement and communicating hydrocephalus. Radiotherapy was administered thrice due to tumor progression. Right intraorbital meningioma increased markedly, and right eye bulging and visual deterioration were aggravating. Tumor removal with orbital exenteration was performed to prevent left visual impairment due to tumor progression. The pathology was atypical meningioma (WHO grade 2). Postoperative cerebrospinal fluid (CSF) leakage from the right orbit, so-called oculorrhea, was difficult to repair due to hydrocephalus, requiring eyelid sutures and a lumboperitoneal shunt. The tumor never developed into the opposite cavernous sinus.

Conclusion: Radiation-induced hydrocephalus can lead to intractable postoperative CSF leakage from orbit after tumor removal with orbital exenteration and require surgery. In these cases, hydrocephalus treatment may lead to a cure for intractable CSF oculorrhea.

Keywords: Cavernous sinus meningioma, Cerebrospinal fluid oculorrhea, Hydrocephalus tumor resection, Orbital exenteration

INTRODUCTION

Cavernous sinus meningiomas are rare, accounting for approximately 1% of all meningiomas,[ 4 ] and most are benign.[ 13 ] The risk of postoperative complications is high, and radiotherapy is considered in asymptomatic cases with tumor growth and in symptomatic cases of small-size tumors and elderly patients.[ 7 ] Radiotherapy is the standard treatment for cavernous sinus meningiomas, but hydrocephalus may occur as a long-term complication.[ 6 ] It is known to be difficult to control cerebrospinal fluid (CSF) leakage when hydrocephalus is present, requiring shunt treatment.[ 10 ]

CSF leakage into/or the orbit to the exterior is termed “oculorrhea,” as compared with otorrhea and rhinorrhea.[ 21 ] Here, we present a rare case of right cavernous sinus meningioma with CSF oculorrhea, requiring a lumboperitoneal shunt for hydrocephalus after tumor removal with orbital exenteration.

CASE DESCRIPTION

A 71-year-old male patient with a history of hypertension and diabetes mellitus presented transient visual field abnormality as his main complaint. He was referred to our department with bilateral superior quadrantanopia on physical examination and confirmed right cavernous sinus meningioma (22 mm) [ Figure 1a ]. Brain magnetic resonance imaging (MRI) revealed no ventricular enlargement [ Figure 1b ]. MRI revealed an increased size to 26 mm and extension into the right sphenoid bone and optic nerve tract 10 months later; thus, a gamma knife surgery was performed with a 12 Gy prescription dose. The tumor size reduced to 23 mm after 13 months. The patient did not visit our hospital in his self-determination for a while, but he had difficulty opening his right eye after 5 years. MRI showed an increased tumor size of 32 mm [ Figure 1c ]; hence, stereotactic radiotherapy (SRT) was performed (50.4 Gy/28 Fr). The tumor continued to grow slowly, and a second SRT was performed 8 years later (36 Gy/12 Fr) due to the appearance of right eyelid ptosis and movement disorder, tumor extension into the right orbit, and right sphenoid wing tumor enlargement. The tumor was 50 mm in diameter. Nine years later, he presented with right exophthalmos and completely lost his right sight. MRI showed a marked increase in the right intraorbital tumor and a slight increase in the right sphenoid wing lesion, with a tumor diameter of 73 mm [ Figure 1d ]. In addition, the patient presented with gait apraxia by communicating hydrocephalus [ Figures 1e and f]. The tap test was performed, and his quality and speed of gait were improved. The CSF pressure was 25 cmH₂O, and the spinal fluid had a high protein concentration of 208 mg/dL upon CSF examination, suggesting radiotherapy-associated spinal fluid malabsorption. Considering the risk of infection associated with tumor removal, the treatment plan was to perform tumor removal before hydrocephalus surgery. The following month, orbitozygomatic craniotomy was performed to remove the tumor extending into the right orbit and sphenoid wing. Preoperative examination revealed a completely blind right eye, so orbital exenteration was performed [ Figure 2a ]. Concurrently, part of the right middle skull base dura mater to which the tumor had adhered was resected [ Figure 2b ]. DuraGen^® (Integra Life Sciences Corp., Princeton, NJ, USA), which is an artificial dural substitute, was placed over the optic nerve canal for intraorbital repair, and the osteoperiosteal flap was laid and covered over the intraorbital floor, then fixed with fibrin glue [ Figures 2c and d ]. The postoperative pathology was atypical meningioma [ Figures 2e and f ]. The intraorbital tumor was completely removed, leaving the right cavernous sinus, but there was a postoperative CSF leakage in the orbit [ Figures 3a and b ]. The day after surgery, a CSF leakage from the right eye appeared and was difficult to treat. The patient was treated with eyelid sutures and spinal drainage for 7 days after the CSF leakage onset, but without improvement; thus, a CSF leakage closure was performed. The middle fossa dural defect and the opened optic nerve canal were replaced with an artificial dural substitute during the initial operation, but they did not adhere and became defective, resulting in a leakage [ Figure 3c ]. Therefore, the dural defective region was sutured as much as possible, and the intraorbital space was occupied with fat and fascial graft, which was not performed in the initial operation [ Figures 3d and e ]. The CSF leakage was subsequently reduced but persisted. Hydrocephalus was considered a possible cause of the CSF leakage. Finally, eyelid sutures and lumboperitoneal shunt were performed for hydrocephalus, which eliminated the CSF leakage and improved the hydrocephalus [ Figure 3e ]. The patient was discharged home 2 months postoperatively after rehabilitation. He continues to be followed up on an outpatient basis. It has been 16 months since the primary surgery with no recurrence of CSF leakage. The tumor never progressed to the opposite cavernous sinus, and the left visual field was preserved. Twenty months after the primary surgery, he died from aspiration pneumonia-induced sepsis, unrelated to complications from the tumor removal and hydrocephalus surgery.

Figure 1:

The images of magnetic resonance imaging (MRI) revealed that the size of the cavernous tumor had increased in the process remarkably. (a and b) The image of MRI at the time of the initial examination. (a) The mass within the cavernous sinus is contrasted on enhanced T1-weighted images (yellow arrowheads). (b) Evan’s index was 0.29 at the time of the initial examination of the fluid-attenuated inversion recovery image. (c) The image of MRI before the initial stereotactic radiotherapy. The tumor extends to the right middle cranial fossa. (d-f) The image of MRI before the initial surgery. (d) The tumor extends into the orbit and right middle cranial fossa-enhanced T1-weighted image. (e) Evan’s index was 0.35 at the time of the appearance of hydrocephalus. Ventriculomegaly and sylvian fissure dilation were confirmed. (f) On the coronal image, the narrowing of the high convexity/midline subarachnoid spaces and steepening of the callosal angle were confirmed at the time of the appearance of hydrocephalus.

Figure 2:

Operative findings of the initial surgery and immunohistology were revealed. (a) After removing as much of the optic nerve and periocular tumor as possible, the optic nerve transection was performed. (b) The tumor in the middle cranial fossa was removed. (c) Findings in the surgical field after tumor removal. Dura mater was sutured directly as much as possible. (d) Finally, DuraGen^® was used to repair the dura mater. (e and f) Pathologic findings of the tumor. (e) Hematoxylin and eosin staining of the tumor revealed meningioma, and original magnification ×200, and (f) The MIB-1 labeling index was high at 25%. The immunohistology revealed anaplastic meningioma. The scale bar in (e) and (f) indicates 100 μm. Rt: Right, FL: frontal lobe, TL: Temporal lobe, ICA: Internal carotid artery.

Figure 3:

(a) The day after his initial surgery, cerebrospinal fluid (CSF) leakage from the right orbit appeared. (b) The tumor was removed except those in the cavernous sinus on enhanced T1-weighted images (left: axial image, right: sagittal image). On the other hand, there was a CSF retention in the orbit (asterisk). (c) Operative findings of the CSF leakage closure. The DuraGen^® was not adherent to the optic nerve, and the frontal lobe was exposed. (d) The intraorbital was filled with fat and fascial. (e) Postoperative magnetic resonance imaging confirmed that the orbit was filled with fat (yellow arrowhead). FL: frontal lobe.

DISCUSSION

In the present case, the patient was administered thrice radiotherapy for a right cavernous sinus meningioma, but the tumor progressed. Although the patient was elderly, a tumor resection was performed to prevent total blindness due to tumor progression. The progression-free survival rate at 5 years for radiation therapy for cavernous sinus meningioma was reported to be over 95%, making it effective.[ 17 ] On the other hand, it was also reported that 3–5% of cases still grow after radiation therapy, and in our case, the patient ultimately required a craniotomy.

To our knowledge, this is the first report of refractory CSF leakage from orbit after tumor resection with orbital exenteration that ultimately required shunting surgery. There have been 21 previous reports of CSF leakage with orbital exenteration.[ 2 , 18 ] Primary diseases include trauma and debridement for mucormycosis and only two cases have been reported to have occurred after tumor removal.[ 25 ] The first case is a patient with recurrent squamous cell carcinoma of the tight medial canthus expanding deep into the orbital apex. The other case was a patient with hemangioblastoma, which almost completely occupied the left orbit and presented with severe vision loss. Treatment for CSF leakage includes dural sutures, local pericranial flap, fat-packing, lumbar drainage, and intravenous antibiotic therapy, although spontaneous closure was observed in some cases.[ 2 , 3 , 9 , 16 , 20 , 24 ]

In this case, the patient developed a refractory CSF oculorrhea postoperatively. The first reason for the failure of CSF leakage closure was the preoperative presence of hydrocephalus, which was extravasated by the intracranial forces. Because of the anatomic risk of internal carotid artery and cranial nerve (oculomotor, pulmonary, trigeminal, and abducens nerve) damage and uncontrolled venous hemorrhage, the intracavernous surgical procedure was abandoned in favor of radiotherapy.[ 1 ] In this case, the patient underwent three sessions of radiotherapy before undergoing surgery for 9 years after the tumor was first noted. Hydrocephalus is a known radiotherapy complication. A previous report of 91 patients with atypical meningioma revealed hydrocephalus in one case after radiotherapy, but the symptoms improved with a ventriculoperitoneal shunt.[ 8 ] Another study of cerebellar pontine angle tumors revealed that 1.4% of meningiomas treated with radiotherapy alone without tumor resection developed hydrocephalus after treatment.[ 15 ] Communicating hydrocephalus in our case was mainly because of poor CSF resorption due to increased CSF proteins, which is consistent with a previous report of hydrocephalus after radiotherapy for meningiomas.[ 14 ] Radiotherapy can cause irradiated tissue necrosis, blood–brain barrier disruption, and blood vessel degeneration, leading to serum protein leakage from the blood vessels into the CSF cavity, resulting in elevated CSF protein levels and consequent hydrocephalus.[ 23 ] It has been previously shown that hydrocephalus can be a risk factor for postoperative spinal fluid leakage, such as in transnasal endoscopic surgery.[ 5 ] Furthermore, hydrocephalus is the only risk factor for CSF leakage after intradural brain surgery in children.[ 22 ] In our case, the hydrocephalus improved, and the CSF oculorrhea disappeared after the lumboperitoneal shunt surgery, indicating intracranial pressure control as an important factor in CSF leakage closure.

Another major factor contributing to the patient’s refractory postoperative CSF oculorrhea was the external tumor extension beyond the cavernous sinus, particularly into the orbit and middle cranial fossa through the optic nerve tract. This extra-cavernous sinus lesion was intraoperatively resected from the infraorbital to the middle cranial fossa, which was difficult to close with sutures due to partial skull base dura mater removal, and the CSF route eventually opened into the orbit through the optic nerve canal. As in the present case, the maximum possible removal of the meningioma, including the dura mater, is desirable, especially in the case of a meningioma that is likely not a benign tumor based on its course. In fact, the present case was diagnosed as atypical meningioma, not a benign tumor. The loss of tissue from the orbit and midface increases the volume, surface area, and structural demands and makes it more difficult to support the central nervous system, so orbital exenteration, and intracranial tumor reduction could increase the complexity of skull base reconstruction. Actually, middle cranial fossa exposure has been reported as the only risk factor for CSF leakage after microvascular reconstruction of complex craniofacial defects with orbital resection in the past,[ 12 ] as in the present case.

A third cause of refractory CSF leakage is inadequate wound healing due to radiation therapy. One of the adverse effects of radiation therapy is its interference with surgical wound healing.[ 19 ] The effect of irradiation sometimes involves the dura, which can lead to CSF leakage.[ 11 ] In the present case, we attempted to prevent postoperative CSF leakage using DuraGen^® during the first surgery and fat and fascia during the second surgery, but these were unsuccessful. Insufficient adhesion of DuraGen^® to the surrounding tissue was observed during the second surgery, which may have been related to inadequate tissue healing due to radiation. This may be related to inadequate healing of the tissue due to radiation.

CONCLUSION

Radiotherapy for cavernous sinus meningiomas can be controlled in the short term; hydrocephalus is a possible long-term complication. Patients with hydrocephalus have a high risk of developing intractable CSF leakage after tumor removal with orbital exenteration; in these cases, early surgical treatment for hydrocephalus may be necessary for intractable CSF oculorrhea.

Ethical approval

Institutional Review Board approval is not required.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Use of artificial intelligence (AI)-assisted technology for manuscript preparation

The authors confirm that there was no use of artificial intelligence (AI)-assisted technology for assisting in the writing or editing of the manuscript, and no images were manipulated using AI.

Disclaimer

The views and opinions expressed in this article are those of the authors and do not necessarily reflect the official policy or position of the Journal or its management. The information contained in this article should not be considered to be medical advice; patients should consult their own physicians for advice as to their specific medical needs.

References

1. Amelot A, van Effenterre R, Kalamarides M, Cornu P, Boch AL. Natural history of cavernous sinus meningiomas. J Neurosurg. 2019. 130: 435-42

2. Arora K, Mehta A, Virk RS, Saini M. Cerebrospinal fluid leak from lateral orbit during exenteration for mucormycosis. BMJ Case Rep. 2020. 13: e237575

3. Bartley GB, Garrity JA, Waller RR, Henderson JW, Ilstrup DM. Orbital exenteration at the Mayo Clinic 1967-1986. Ophthalmology. 1989. 96: 468-73

4. Bhat AR, Wani MA, Kirmani AR, Ramzan AU. Histologicalsubtypes and anatomical location correlated in meningeal brain tumors (meningiomas). J Neurosci Rural Pract. 2014. 5: 244-9

5. Carrau RL, Snyderman CH, Kassam AB. The management of cerebrospinal fluid leaks in patients at risk for high-pressure hydrocephalus. Laryngoscope. 2005. 115: 205-12

6. Cayuela N, Domínguez-Lizarbe M, Plans G, Alemany M, Sánchez JJ, Andrés B. Normal pressure hydrocephalus following cranial radiation: Identification of shunting responders. Cancers (Basel). 2023. 15: 1949

7. Corniola MV, Roche PH, Bruneau M, Cavallo LM, Daniel RT, Messerer M. Management of cavernous sinus meningiomas: Consensus statement on behalf of the EANS skull base section. Brain Spine. 2022. 2: 100864

8. Dawley T, Rana Z, Abou-Al-Shaar H, Goenka A, Schulder M. Major complications from radiotherapy following treatment for atypical meningiomas. Neurosurg Focus. 2019. 46: E5

9. De Conciliis C, Bonavolontà G. Incidence and treatment of dural exposure and CSF leak during orbital exenteration. Ophthalmic Plast Reconstr Surg. 1987. 3: 61-4

10. Filippidis AS, Kalani MY, Nakaji P, Rekate HL. Negative-pressure and low-pressure hydrocephalus: The role of cerebrospinal fluid leaks resulting from surgical approaches to the cranial base. J Neurosurg. 2011. 115: 1031-7

11. Fujioka M. Surgical reconstruction of radiation injuries. Adv Wound Care (New Rochelle). 2014. 3: 25-37

12. Gill KS, Hsu D, Tassone P, Pluta J, Nyquist G, Krein H. Postoperative cerebrospinal fluid leak after microvascular reconstruction of craniofacial defects with orbital exenteration. Laryngoscope. 2017. 127: 835-41

13. Hekmatpanah J. Evidence-based treatment of cavernous sinus meningioma. Surg Neurol Int. 2019. 10: 228

14. Kajiwara M, Yamashita K, Ueba T, Nishikawa T. Normal pressure hydrocephalus after radiosurgery for sphenoid ridge meningioma. J Clin Neurosci. 2009. 16: 162-4

15. Kim J, Woo B, Ji S, Hwang K, Kim YH, Han JH. Communicating hydrocephalus following treatment of cerebellopontine angle tumors. World Neurosurg. 2022. 165: e505-11

16. Kuo CH, Gao K, Clifford A, Shannon K, Clark J. Orbital exenterations: An 18-year experience from a single head and neck unit. ANZ J Surg. 2011. 81: 326-30

17. Leroy HA, Tuleasca C, Reyns N, Levivier M. Radiosurgery and fractionated radiotherapy for cavernous sinus meningioma: A systematic review and meta-analysis. Acta Neurochir (Wien). 2018. 160: 2367-78

18. Massoud VA, Fay A, Yoon MK. Cerebrospinal fluid leak as a complication of oculoplastic surgery. Semin Ophthalmol. 2014. 29: 440-9

19. Powers WE, Ogura JH, Palmer LA. Radiation therapy and wound healing delay. Animals and man. Radiology. 1967. 89: 112-5

20. Rahman I, Cook AE, Leatherbarrow B. Orbital exenteration: A 13 year Manchester experience. Br J Ophthalmol. 2005. 89: 1335-40

21. Salame K, Segev Y, Fliss DM, Ouaknine GE. Diagnosis and management of posttraumatic oculorrhea. Neurosurg Focus. 2000. 9: e3

22. Slot EM, van Doormaal TP, van Baarsen KM, Krayenbühl N, Regli L, Germans MR. Incisional CSF leakage after intradural cranial surgery in children: Incidence, risk factors, and complications. J Neurosurg Pediatr. 2023. 31: 313-20

23. Szeifert GT, Atteberry DS, Kondziolka D, Levivier M, Lunsford LD. Cerebral metastases pathology after radiosurgery: A multicenter study. Cancer. 2006. 106: 2672-81

24. Wulc AE, Adams JL, Dryden RM. Cerebrospinal fluid leakage complicating orbital exenteration. Arch Ophthalmol. 1989. 107: 827-30

25. Yuen HK, Cheng AC, Auyeung KC. Late-onset occult cerebrospinal fluid leakage after orbital exenteration. Ophthalmic Plast Reconstr Surg. 2008. 24: 238-40