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Prosthetic Replacement of the Ocular Surface Ecosystem Treatment of Ocular Surface Disease After Skull Base Tumor Resection
Original Article
Prosthetic Replacement of the Ocular Surface Ecosystem Treatment of Ocular Surface Disease After Skull Base Tumor Resection Carolyn E. Kloek1, Karen W. Jeng-Miller1, Deborah S. Jacobs1,2, Ian F. Dunn3
BACKGROUND: Prosthetic replacement of the ocular surface ecosystem (PROSE) treatment is an effective, nonsurgical therapeutic option for patients with ocular surface disease related to cranial nerve deficits secondary to skull base tumor resection.
-
METHODS: This case series describes the impact of PROSE treatment in patients with symptomatic exposure keratopathy or neurotrophic keratitis after skull base tumor surgery.
-
RESULTS: All patients improved symptomatically and functionally with PROSE treatment, and have had sustained improvement for as long as 3 years.
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CONCLUSIONS: In postneurosurgical cases in which neurologic function may recover, PROSE treatment offers a safe, nonsurgical treatment option to support the ocular surface during the period of observation awaiting neurologic recovery.
-
INTRODUCTION
R
esection of skull base lesions can result in cranial nerve (CN) deficits that can have a detrimental effect on the ocular surface. Ophthalmic sequelae are well described after resection of skull base tumors. Specifically, trigeminal (CN V) nerve dysfunction may lead to deinnervation of the cornea and neurotrophic keratitis, and facial (CN VII) nerve dysfunction— even if temporary—can lead to incomplete lid closure and
Key words - Neurotrophic keratopathy - PROSE - Skull base tumor surgery Abbreviations and Acronyms cc: With correction CN: Cranial nerve PH: Pinhole PROSE: Prosthetic Replacement of the Ocular Surface Ecosystem sc: Without correction SPK: Superficial punctate keratitis VA: Visual acuity
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exposure keratopathy. These ophthalmic complications can confer significant morbidity from corneal ulceration, infection, melting, and perforation; although rare, infection requiring enucleation of the eye has been reported after vestibular schwannoma and other skull base tumor resection.1-5 Management of the neurotrophic or exposed cornea after surgery varies widely. Options include use of lubricant drops, gels, and ointments and surgical interventions such as weight implantation in the affected eyelid, lower lid tightening, or partial or complete tarsorrhaphy.6,7 Prosthetic replacement of the ocular surface ecosystem (PROSE) treatment is another option for the management of the vulnerable cornea. The prosthetic devices used in PROSE treatment were Food and Drug Administration approved in 1994 for daily wear in the management of distorted cornea and certain disorders of the ocular surface. The clinical impact and cost-effectiveness of PROSE treatment has been well-established.8,9 The devices used in PROSE treatment are made of a rigid gas permeable polymer. The shape is customized to an individual’s eye with an optic portion that vaults the cornea entirely and a haptic portion that is aligned with and rests on the sclera. The PROSE device is filled with artificial tears just prior to insertion, and when placed on the eye, results in the appearance of a hard contact lens with an aqueous reservoir that encompasses the entire corneal surface to provide constant lubrication and protection of the ocular surface (Figure 1). The customization process for the devices used in PROSE treatment involves evaluation of physiologic function and subjective tolerance of 1, 3, and 6 hours of wear. After the customization process is complete, patients return for interval examination generally on a yearly basis.10 Maintenance of the device requires daily application and removal of the device and a regimen of daily cleaning and overnight disinfection (Figure 2). As of January 2015, there are 12 specialty eye care institutes located in
From the 1Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston; 2 BostonSight, Needham; and 3Department of Neurosurgery, Brigham and Women’s Hospital, Boston, Massachusetts, USA To whom correspondence should be addressed: Carolyn E. Kloek, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2018) 110:e124-e128. https://doi.org/10.1016/j.wneu.2017.10.111 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2017 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111
ORIGINAL ARTICLE CAROLYN E. KLOEK ET AL.
Figure 1. Prosthetic replacement of the ocular surface ecosystem. (A) Devices used in prosthetic replacement of the ocular surface ecosystem treatment have an optic portion that vaults on the cornea and a haptic portion that rests on the sclera, creating a reservoir filled with artificial tears that provides constant
PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
lubrication of the ocular surface. (B) Image of prosthetic replacement of the ocular surface ecosystem device on the ocular surface with the upper lid retracted. PROSE, prosthetic replacement of the ocular surface ecosystem.
academic medical centers and clinics throughout the United States that offer PROSE treatment. There are reports of PROSE treatment and scleral lenses as treatment options for patients with corneal complications of ocular surface disease such as corneal opacification, ulceration, and melting after vestibular schwannoma resection resection.11,12 In our experience, patients without frank cranial nerve palsy or corneal breakdown after resection of skull-based tumor may nevertheless be plagued by persistent, irritation and blurry vision only partially relieved by frequent instillation of lubricants. This dry eye syndrome may occur despite enhancement of CN VII function with upper lid weights and may interfere with patients’ activities of daily living. Herein, we describe the impact of PROSE treatment device in patients with symptomatic exposure keratopathy or neurotrophic keratitis after skull base tumor surgery. METHODS Institutional review board approval was obtained from the Massachusetts Eye and Ear Infirmary Human Studies Committee. Medical records of patients who were seen by an ophthalmologist (C.E.K.) between January 1, 2010, and January 1, 2015, with diagnoses of exposure keratopathy, lagophthalmos, facial nerve disorder, neurotrophic cornea, and Bell’s palsy, were reviewed. Representative cases were selected for this case series. CASE REPORTS
Figure 2. Application and removal of the prosthetic replacement of the ocular surface ecosystem (PROSE) device. PROSE treatment requires daily application and removal, and cleaning and disinfection of the device. Most patients can be trained to care for the device and perform application and removal independently.
WORLD NEUROSURGERY 110: e124-e128, FEBRUARY 2018
Case 1 A 52-year-old woman presented with complaints of diplopia with right gaze that ultimately led to the diagnosis of a right petroclival meningioma. The patient underwent a combined petrosal approach for resection of the meningioma 2 months after onset of symptoms. She developed right CN III and VI nerve palsies postoperatively and right hyposthesia and hypoalgesia in all CN V distributions 1 month after surgery.
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PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
Figure 3. Ocular surface before and after initiation of the prosthetic replacement of the ocular surface ecosystem (PROSE) device. (A) Prior to initiation of PROSE treatment, keratopathy and corneal epithelial staining imaged with slit lamp biomicroscopy using cobalt blue filter after instillation of fluorescein eye
The patient was referred to ophthalmology 9 months after resection of the meningioma with ophthalmic complaints of decreased vision, dryness, and a burning sensation in her right eye for which she used artificial tears 3 times a day. Visual acuity (VA) was 20/300 without correction (sc) and 20/50 pin hole (PH) in the right eye; her VA in the right eye 1 year prior was 20/25 sc. On examination, there was 4þ confluent coarse superficial punctate keratitis (SPK) without frank erosion, geographic epithelial defect, ulceration, or thinning of the right cornea. Right corneal sensation was tested using the wisp of a cotton-tipped applicator and found to be reduced in the right eye compared with the left. There was good blink and no lagophthalmos, leading to a diagnosis of neurotrophic keratopathy. Other ophthalmic findings noted on examination included mild cataracts but no retinal pathology. The patient was instructed to increase artificial tear usage to 10 times daily with mild improvement in her VA (20/100 sc, PH 20/30-2), but no change in the superficial keratitis was noted on examination. She was treated with punctal plugs in her right upper and lower lid, prescribed cyclosporine ophthalmic emulsion 0.05% twice daily, and advised increased artificial tear usage to every 30 minutes. VA improved to 20/30 sc, PH 20/20-1, but the patient had continued eye irritation and examination showed diffuse SPK without thinning or ulceration, but surface breakdown had coalesced in the interpalpebral fissure (Figure 3A).
Figure 4. Prosthetic replacement of the ocular surface ecosystem treatment on the ocular surface in various gazes. The device rests on the
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drops is shown. Regions of corneal epithelial breakdown appear green. (B) Postinitiation of PROSE treatment, notable improvement in the keratopathy and decrease in corneal staining with the same imaging technique is shown.
The patient underwent PROSE treatment, wearing the device daily for 14 months after meningioma resection. She cited significant improvement in all of her ophthalmic symptoms. Her VA was 20/25-2 sc, PH 20/20-2, and her cornea was clear with no staining on slit lamp examination. Nine months after customizations, she was wearing the PROSE device 12 hours daily and remained asymptomatic while the device was in place (Figure 3B). Case 2 A 58-year-old woman with a large left vestibular schwannoma underwent left transtemporal craniotomy with partial mastoidectomy. Intraoperative neuromonitoring was performed showing intact CN VII function throughout the case at the brainstem and in the internal auditory canal. Ten days postoperatively, her CN VII function was a House-Brackmann grade IV.13 A left upper lid gold weight was implanted 20 days after vestibular schwannoma resection to preserve ocular surface health. The patient was referred to ophthalmology 3 weeks after left upper lid gold weight placement, at which time her ophthalmic complaints included irritation and blurry vision from frequent ophthalmic ointment application, which impaired her ability to work. She was using artificial tears/gel multiple times per day and ointment at night. On examination, she was found to have left facial nerve weakness, an incomplete blink, 1e2 mm of
sclera of the eye in contrast with a standard contact lens.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111
ORIGINAL ARTICLE CAROLYN E. KLOEK ET AL.
lagophthalmos, a positive Bell’s reflex, and diffuse SPK on her inferior cornea without geographic epithelial defect, ulceration, or thinning. Corneal sensation was noted as intact. VA was 20/300þ1 with correction (cc) and PH acuity of 20/80þ1 in the left eye. Other ophthalmic findings noted on examination included mild cataracts. The patient was referred for PROSE treatment for exposure keratopathy. At initial consultation, placement of a trial device was found to relieve symptoms, and the patient was declared a candidate for PROSE treatment. At ophthalmic evaluation 4 months later, she reported use of the PROSE device on a daily wear basis. The patient’s VA improved to 20/20-1 with the device in place, and she cited significant improvement in all of her ophthalmic symptoms. The patient’s facial nerve function overall had improved to a House-Brackmann grade II. The eyelid weight was removed 1 year after tumor resection. She continued to experience chronic irritation when not wearing her PROSE device. At 3-year follow-up, the patient continues with daily wear of a PROSE device with no complications. Figure 4 shows the PROSE device on the ocular surface in several directions of gaze. Vision remains 20/20-1 cc in the left eye with report of relief of eye symptoms with the PROSE device in place. Case 3 A 29-year-old woman was diagnosed with a left vestibular schwannoma after experiencing left facial weakness and hearing loss. The patient sought care with ophthalmology prior to tumor resection, at which time her ophthalmic complaints included ocular pain found to be secondary to exposure keratopathy from a seventh nerve palsy. The patient’s VA was 20/20; there was 5e6 mm lagophthalmos and diffuse SPK involving the inferior two-thirds of the cornea corresponding to the area of lagophthalmos. There was no geographic corneal epithelial defect, ulceration, or thinning or any other ocular pathology found on examination. The patient was advised to use artificial tears and lubricant gel. She underwent tumor resection via suboccipital craniotomy at another institution 4 months after the onset of her symptoms. After surgery, the patient continued to experience left facial weakness rated a House-Brackmann grade IV, lagophthalmos, and exposure keratopathy. The patient had a thin platinum upper eyelid weight implanted 2 weeks after surgery, which was subsequently removed 11 months after surgery because of partial recovery of left facial tone. Ongoing treatment included artificial tears and lubricant gel application every 2 hours, and eyelid taping at night for 1 year after surgery. The patient, however, continued to complain of a dry and an occasionally painful left eye and decreased vision caused by the lubricant gel. Nearly 1 year after tumor resection, the patient’s VA worsened to 20/400 attributed to exposure keratopathy and blur from lubricant gel. Slit lamp examination continued to reveal facial nerve weakness, 2 mm of lagophthalmos, poor blink, and diffuse SPK without corneal erosion, ulceration, or thinning. There continued to be no other ocular pathology evident on examination. The patient was referred for PROSE treatment. After daily wear of the PROSE device 15 months after vestibular schwannoma resection, VA improved to 20/20 with significant improvement in all ophthalmic symptoms. Four millimeters of
WORLD NEUROSURGERY 110: e124-e128, FEBRUARY 2018
PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
lagophthalmos persisted, but no corneal pathology or complications from PROSE developed over the following 3 years. DISCUSSION PROSE treatment is an effective, nonsurgical therapeutic option for patients with ocular surface disease related to CN deficits secondary to skull base tumor resection. In this representative case series, 2 patients developed exposure keratopathy that was insufficiently addressed with lid weights, whereas a third patient suffered from corneal epithelial breakdown because of a neurotrophic cornea from trigeminal nerve manipulation. All patients improved symptomatically and functionally with PROSE treatment, and have had sustained improvement for as long as 3 years. In postneurosurgical cases in which neurologic function may recover, PROSE treatment offers a safe, nonsurgical treatment option to support the ocular surface during the period of observation awaiting neurologic recovery. In patients with chronic cranial neuropathies or more severe ocular pathology, the PROSE device also offers an option to tarsorrhaphy that is more cosmetically appealing to patients. In cases of trigeminal nerve dysfunction it offers a stand-alone treatment approach; in cases where lagophthalmos or incomplete blink results from facial nerve palsy, it may be combined with a lid weight procedure. PROSE is an innovative, Food and Drug Administratione approved prosthetic device for ocular surface disease. Most contact lenses and scleral lenses are not labeled for therapeutic use and may be contraindicated in ocular surface disease. PROSE treatment efficacy and safety has been reported in various corneal pathologies, seen here with neurotrophic corneas secondary to skull base tumor resections, and in others such as an adjunct treatment for those after head and neck malignancy resection,14 chronic ocular graft-versus-host disease,10 severe dry eye,15 and corneal ectasias.16 Furthermore, PROSE efficacy as measured through the National Eye Institute Visual Functional Questionnaire-25 showed improved visual functioning in 88% of patients after 6 months8; sustained improvement in visual functioning was still present at a 5-year follow-up.17 Although PROSE is beneficial for a range of corneal pathologies, complications have been reported including bacterial keratitis, exacerbation of corneal edema, and degradation of vision from tear debris accumulation in the fluid compartment of the PROSE device.18 However, most recently, in a large 5-year retrospective review of PROSE treatment, complications from PROSE treatment were uncommon, occurring in 2.5% of the studied cohort, and consisted of redness, corneal graft failure and elevated intraocular pressure, and opacification.17 It is important for clinicians to incorporate comprehensive and continued education about PROSE treatment, device hygiene, and regular interval checkups to enable long-term continuation with device wear and mitigate any possible adverse effects of the device. PROSE treatment requires a degree of cognitive function, and manual dexterity for application and removal of the device; some patients with limited ability make require an assistant or coach to comply with PROSE treatment. Moreover, although PROSE treatment is available at centers throughout the United States, patients located outside of one of these cities will need to travel to a site that offers the treatment. Although the cost of PROSE
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treatment is comparable or less than that for alternative treatment options, including implantation of a lid weight or tarsorrhaphy, third-party coverage varies; therefore, the cost of PROSE treatment may be a barrier for some patients. Almost 80% of the patients treated at the Massachusetts-based PROSE center in 2011 were covered in part or in full for PROSE treatment through their insurance providers and/or financial assistance.19 Commercially available scleral lenses may be a less expensive option for some patients; however, none are labeled for therapeutic use.
REFERENCES 1. Abolfotoh M, Bi WL, Hong CK, Almefty KK, Boskovitz A, Dunn IF, et al. The combined microscopic-endoscopic technique for radical resection of cerebellopontine angle tumors. J Neurosurg. 2015;123:1301-1311. 2. Hunter JB, Weaver KD, Thompson RC, Wanna GB. Petroclival meningiomas. Otolaryngol Clin North Am. 2015;48:477-490. 3. Khanna JN, Natrajan S, Galinde J. Skull base tumors: a kaleidoscope of challenge. J Neurol Surg Rep. 2014;75:e11-e21. 4. Mulhern MG, Aduriz-Lorenzo PM, Rawluk D, Viani L, Eustace P, Logan P. Ocular complications of acoustic neuroma surgery. Br J Ophthalmol. 1999; 83:1389-1392. 5. Rogers NK, Brand CS. Acoustic neuroma and the eye. Br J Neurosurg. 1997;11:292-297. 6. Sacchetti M, Lambiase A. Diagnosis and management of neurotrophic keratitis. Clin Ophthalmol. 2014;8:571-579. 7. Sohrab M, Abugo U, Grant M, Merbs S. Management of the eye in facial paralysis. Facial Plast Surg. 2015;31:140-144. 8. Stason WB, Razavi M, Jacobs DS, Shepard DS, Suaya JA, Johns L, et al. Clinical benefits of the Boston Ocular Surface Prosthesis. Am J Ophthalmol. 2010;149:54-61.
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Neurosurgeons and ophthalmologists should consider PROSE treatment for patients with symptoms or signs of ocular surface disease after skull base tumor resection. We hope this case series raises awareness among neurosurgeons of the importance of managing the ocular surface in patients with keratopathy after skull base neurosurgical procedures and presents a treatment alternative to the traditional management strategies of tarsorrhaphy and weight implantation in the upper lid. More experience is needed to understand how PROSE best fits into existing treatment algorithms.
9. Shepard DS, Razavi M, Stason WB, Jacobs DS, Suaya JA, Cohen M, et al. Economic appraisal of the Boston Ocular Surface Prosthesis. Am J Ophthalmol. 2009;148:860-868.e2.
17. Agranat JS, Kitos NR, Jacobs DS. Prosthetic replacement of the ocular surface ecosystem: impact at 5 years. Br J Ophthalmol. 2016;100: 1171-1175.
10. Jacobs DS, Rosenthal P. Boston scleral lens prosthetic device for treatment of severe dry eye in chronic graft-versus-host disease. Cornea. 2007;26: 1195-1199.
18. Rosenthal P, Croteau A. Fluid-ventilated, gaspermeable scleral contact lens is an effective option for managing severe ocular surface disease and many corneal disorders that would otherwise require penetrating keratoplasty. Eye Contact Lens. 2005;31:130-134.
11. Gire A, Kwok A, Marx DP. PROSE treatment for lagophthalmos and exposure keratopathy. Ophthal Plast Reconstr Surg. 2013;29:e38-e40. 12. Weyns M, Koppen C, Tassignon MJ. Scleral contact lenses as an alternative to tarsorrhaphy for the long-term management of combined exposure and neurotrophic keratopathy. Cornea. 2013;32: 359-361. 13. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93:146-147. 14. Lin SJ, Jacobs DS, Frankenthaler R, Rubin PA. An ocular surface prosthesis as an innovative adjunct in patients with head and neck malignancy. Otolaryngol Head Neck Surg. 2008;139:589-591. 15. Dimit R, Gire A, Pflugfelder SC, Bergmanson JP. Patient ocular conditions and clinical outcomes using a PROSE scleral device. Cont Lens Anterior Eye. 2013;36:159-163.
19. BostonSight. About costs and insurance. Available at: http://www.bostonsight.org/PROSE-treatment/ New-to-PROSE-FAQs/About-Costs-and-Insurance. Accessed October 5, 2015.
Conflict of interest statement: D.S. Jacobs is a salaried, fulltime employee of BostonSight, a 501(c)3 nonprofit organization, and has no financial or proprietary interest in any contact lens or prosthetic devices. Received 27 June 2017; accepted 20 October 2017 Citation: World Neurosurg. (2018) 110:e124-e128. https://doi.org/10.1016/j.wneu.2017.10.111 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com
16. Baran I, Bradley JA, Alipour F, Rosenthal P, Le HG, Jacobs DS. PROSE treatment of corneal ectasia. Contact Lens Anterior Eye. 2012;35:222-227.
1878-8750/$ - see front matter ª 2017 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111
Prosthetic Replacement of the Ocular Surface Ecosystem Treatment of Ocular Surface Disease After Skull Base Tumor Resection Carolyn E. Kloek1, Karen W. Jeng-Miller1, Deborah S. Jacobs1,2, Ian F. Dunn3
BACKGROUND: Prosthetic replacement of the ocular surface ecosystem (PROSE) treatment is an effective, nonsurgical therapeutic option for patients with ocular surface disease related to cranial nerve deficits secondary to skull base tumor resection.
-
METHODS: This case series describes the impact of PROSE treatment in patients with symptomatic exposure keratopathy or neurotrophic keratitis after skull base tumor surgery.
-
RESULTS: All patients improved symptomatically and functionally with PROSE treatment, and have had sustained improvement for as long as 3 years.
-
CONCLUSIONS: In postneurosurgical cases in which neurologic function may recover, PROSE treatment offers a safe, nonsurgical treatment option to support the ocular surface during the period of observation awaiting neurologic recovery.
-
INTRODUCTION
R
esection of skull base lesions can result in cranial nerve (CN) deficits that can have a detrimental effect on the ocular surface. Ophthalmic sequelae are well described after resection of skull base tumors. Specifically, trigeminal (CN V) nerve dysfunction may lead to deinnervation of the cornea and neurotrophic keratitis, and facial (CN VII) nerve dysfunction— even if temporary—can lead to incomplete lid closure and
Key words - Neurotrophic keratopathy - PROSE - Skull base tumor surgery Abbreviations and Acronyms cc: With correction CN: Cranial nerve PH: Pinhole PROSE: Prosthetic Replacement of the Ocular Surface Ecosystem sc: Without correction SPK: Superficial punctate keratitis VA: Visual acuity
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exposure keratopathy. These ophthalmic complications can confer significant morbidity from corneal ulceration, infection, melting, and perforation; although rare, infection requiring enucleation of the eye has been reported after vestibular schwannoma and other skull base tumor resection.1-5 Management of the neurotrophic or exposed cornea after surgery varies widely. Options include use of lubricant drops, gels, and ointments and surgical interventions such as weight implantation in the affected eyelid, lower lid tightening, or partial or complete tarsorrhaphy.6,7 Prosthetic replacement of the ocular surface ecosystem (PROSE) treatment is another option for the management of the vulnerable cornea. The prosthetic devices used in PROSE treatment were Food and Drug Administration approved in 1994 for daily wear in the management of distorted cornea and certain disorders of the ocular surface. The clinical impact and cost-effectiveness of PROSE treatment has been well-established.8,9 The devices used in PROSE treatment are made of a rigid gas permeable polymer. The shape is customized to an individual’s eye with an optic portion that vaults the cornea entirely and a haptic portion that is aligned with and rests on the sclera. The PROSE device is filled with artificial tears just prior to insertion, and when placed on the eye, results in the appearance of a hard contact lens with an aqueous reservoir that encompasses the entire corneal surface to provide constant lubrication and protection of the ocular surface (Figure 1). The customization process for the devices used in PROSE treatment involves evaluation of physiologic function and subjective tolerance of 1, 3, and 6 hours of wear. After the customization process is complete, patients return for interval examination generally on a yearly basis.10 Maintenance of the device requires daily application and removal of the device and a regimen of daily cleaning and overnight disinfection (Figure 2). As of January 2015, there are 12 specialty eye care institutes located in
From the 1Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston; 2 BostonSight, Needham; and 3Department of Neurosurgery, Brigham and Women’s Hospital, Boston, Massachusetts, USA To whom correspondence should be addressed: Carolyn E. Kloek, M.D. [E-mail: [email protected]] Citation: World Neurosurg. (2018) 110:e124-e128. https://doi.org/10.1016/j.wneu.2017.10.111 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com 1878-8750/$ - see front matter ª 2017 Elsevier Inc. All rights reserved.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111
ORIGINAL ARTICLE CAROLYN E. KLOEK ET AL.
Figure 1. Prosthetic replacement of the ocular surface ecosystem. (A) Devices used in prosthetic replacement of the ocular surface ecosystem treatment have an optic portion that vaults on the cornea and a haptic portion that rests on the sclera, creating a reservoir filled with artificial tears that provides constant
PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
lubrication of the ocular surface. (B) Image of prosthetic replacement of the ocular surface ecosystem device on the ocular surface with the upper lid retracted. PROSE, prosthetic replacement of the ocular surface ecosystem.
academic medical centers and clinics throughout the United States that offer PROSE treatment. There are reports of PROSE treatment and scleral lenses as treatment options for patients with corneal complications of ocular surface disease such as corneal opacification, ulceration, and melting after vestibular schwannoma resection resection.11,12 In our experience, patients without frank cranial nerve palsy or corneal breakdown after resection of skull-based tumor may nevertheless be plagued by persistent, irritation and blurry vision only partially relieved by frequent instillation of lubricants. This dry eye syndrome may occur despite enhancement of CN VII function with upper lid weights and may interfere with patients’ activities of daily living. Herein, we describe the impact of PROSE treatment device in patients with symptomatic exposure keratopathy or neurotrophic keratitis after skull base tumor surgery. METHODS Institutional review board approval was obtained from the Massachusetts Eye and Ear Infirmary Human Studies Committee. Medical records of patients who were seen by an ophthalmologist (C.E.K.) between January 1, 2010, and January 1, 2015, with diagnoses of exposure keratopathy, lagophthalmos, facial nerve disorder, neurotrophic cornea, and Bell’s palsy, were reviewed. Representative cases were selected for this case series. CASE REPORTS
Figure 2. Application and removal of the prosthetic replacement of the ocular surface ecosystem (PROSE) device. PROSE treatment requires daily application and removal, and cleaning and disinfection of the device. Most patients can be trained to care for the device and perform application and removal independently.
WORLD NEUROSURGERY 110: e124-e128, FEBRUARY 2018
Case 1 A 52-year-old woman presented with complaints of diplopia with right gaze that ultimately led to the diagnosis of a right petroclival meningioma. The patient underwent a combined petrosal approach for resection of the meningioma 2 months after onset of symptoms. She developed right CN III and VI nerve palsies postoperatively and right hyposthesia and hypoalgesia in all CN V distributions 1 month after surgery.
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PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
Figure 3. Ocular surface before and after initiation of the prosthetic replacement of the ocular surface ecosystem (PROSE) device. (A) Prior to initiation of PROSE treatment, keratopathy and corneal epithelial staining imaged with slit lamp biomicroscopy using cobalt blue filter after instillation of fluorescein eye
The patient was referred to ophthalmology 9 months after resection of the meningioma with ophthalmic complaints of decreased vision, dryness, and a burning sensation in her right eye for which she used artificial tears 3 times a day. Visual acuity (VA) was 20/300 without correction (sc) and 20/50 pin hole (PH) in the right eye; her VA in the right eye 1 year prior was 20/25 sc. On examination, there was 4þ confluent coarse superficial punctate keratitis (SPK) without frank erosion, geographic epithelial defect, ulceration, or thinning of the right cornea. Right corneal sensation was tested using the wisp of a cotton-tipped applicator and found to be reduced in the right eye compared with the left. There was good blink and no lagophthalmos, leading to a diagnosis of neurotrophic keratopathy. Other ophthalmic findings noted on examination included mild cataracts but no retinal pathology. The patient was instructed to increase artificial tear usage to 10 times daily with mild improvement in her VA (20/100 sc, PH 20/30-2), but no change in the superficial keratitis was noted on examination. She was treated with punctal plugs in her right upper and lower lid, prescribed cyclosporine ophthalmic emulsion 0.05% twice daily, and advised increased artificial tear usage to every 30 minutes. VA improved to 20/30 sc, PH 20/20-1, but the patient had continued eye irritation and examination showed diffuse SPK without thinning or ulceration, but surface breakdown had coalesced in the interpalpebral fissure (Figure 3A).
Figure 4. Prosthetic replacement of the ocular surface ecosystem treatment on the ocular surface in various gazes. The device rests on the
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drops is shown. Regions of corneal epithelial breakdown appear green. (B) Postinitiation of PROSE treatment, notable improvement in the keratopathy and decrease in corneal staining with the same imaging technique is shown.
The patient underwent PROSE treatment, wearing the device daily for 14 months after meningioma resection. She cited significant improvement in all of her ophthalmic symptoms. Her VA was 20/25-2 sc, PH 20/20-2, and her cornea was clear with no staining on slit lamp examination. Nine months after customizations, she was wearing the PROSE device 12 hours daily and remained asymptomatic while the device was in place (Figure 3B). Case 2 A 58-year-old woman with a large left vestibular schwannoma underwent left transtemporal craniotomy with partial mastoidectomy. Intraoperative neuromonitoring was performed showing intact CN VII function throughout the case at the brainstem and in the internal auditory canal. Ten days postoperatively, her CN VII function was a House-Brackmann grade IV.13 A left upper lid gold weight was implanted 20 days after vestibular schwannoma resection to preserve ocular surface health. The patient was referred to ophthalmology 3 weeks after left upper lid gold weight placement, at which time her ophthalmic complaints included irritation and blurry vision from frequent ophthalmic ointment application, which impaired her ability to work. She was using artificial tears/gel multiple times per day and ointment at night. On examination, she was found to have left facial nerve weakness, an incomplete blink, 1e2 mm of
sclera of the eye in contrast with a standard contact lens.
WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111
ORIGINAL ARTICLE CAROLYN E. KLOEK ET AL.
lagophthalmos, a positive Bell’s reflex, and diffuse SPK on her inferior cornea without geographic epithelial defect, ulceration, or thinning. Corneal sensation was noted as intact. VA was 20/300þ1 with correction (cc) and PH acuity of 20/80þ1 in the left eye. Other ophthalmic findings noted on examination included mild cataracts. The patient was referred for PROSE treatment for exposure keratopathy. At initial consultation, placement of a trial device was found to relieve symptoms, and the patient was declared a candidate for PROSE treatment. At ophthalmic evaluation 4 months later, she reported use of the PROSE device on a daily wear basis. The patient’s VA improved to 20/20-1 with the device in place, and she cited significant improvement in all of her ophthalmic symptoms. The patient’s facial nerve function overall had improved to a House-Brackmann grade II. The eyelid weight was removed 1 year after tumor resection. She continued to experience chronic irritation when not wearing her PROSE device. At 3-year follow-up, the patient continues with daily wear of a PROSE device with no complications. Figure 4 shows the PROSE device on the ocular surface in several directions of gaze. Vision remains 20/20-1 cc in the left eye with report of relief of eye symptoms with the PROSE device in place. Case 3 A 29-year-old woman was diagnosed with a left vestibular schwannoma after experiencing left facial weakness and hearing loss. The patient sought care with ophthalmology prior to tumor resection, at which time her ophthalmic complaints included ocular pain found to be secondary to exposure keratopathy from a seventh nerve palsy. The patient’s VA was 20/20; there was 5e6 mm lagophthalmos and diffuse SPK involving the inferior two-thirds of the cornea corresponding to the area of lagophthalmos. There was no geographic corneal epithelial defect, ulceration, or thinning or any other ocular pathology found on examination. The patient was advised to use artificial tears and lubricant gel. She underwent tumor resection via suboccipital craniotomy at another institution 4 months after the onset of her symptoms. After surgery, the patient continued to experience left facial weakness rated a House-Brackmann grade IV, lagophthalmos, and exposure keratopathy. The patient had a thin platinum upper eyelid weight implanted 2 weeks after surgery, which was subsequently removed 11 months after surgery because of partial recovery of left facial tone. Ongoing treatment included artificial tears and lubricant gel application every 2 hours, and eyelid taping at night for 1 year after surgery. The patient, however, continued to complain of a dry and an occasionally painful left eye and decreased vision caused by the lubricant gel. Nearly 1 year after tumor resection, the patient’s VA worsened to 20/400 attributed to exposure keratopathy and blur from lubricant gel. Slit lamp examination continued to reveal facial nerve weakness, 2 mm of lagophthalmos, poor blink, and diffuse SPK without corneal erosion, ulceration, or thinning. There continued to be no other ocular pathology evident on examination. The patient was referred for PROSE treatment. After daily wear of the PROSE device 15 months after vestibular schwannoma resection, VA improved to 20/20 with significant improvement in all ophthalmic symptoms. Four millimeters of
WORLD NEUROSURGERY 110: e124-e128, FEBRUARY 2018
PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
lagophthalmos persisted, but no corneal pathology or complications from PROSE developed over the following 3 years. DISCUSSION PROSE treatment is an effective, nonsurgical therapeutic option for patients with ocular surface disease related to CN deficits secondary to skull base tumor resection. In this representative case series, 2 patients developed exposure keratopathy that was insufficiently addressed with lid weights, whereas a third patient suffered from corneal epithelial breakdown because of a neurotrophic cornea from trigeminal nerve manipulation. All patients improved symptomatically and functionally with PROSE treatment, and have had sustained improvement for as long as 3 years. In postneurosurgical cases in which neurologic function may recover, PROSE treatment offers a safe, nonsurgical treatment option to support the ocular surface during the period of observation awaiting neurologic recovery. In patients with chronic cranial neuropathies or more severe ocular pathology, the PROSE device also offers an option to tarsorrhaphy that is more cosmetically appealing to patients. In cases of trigeminal nerve dysfunction it offers a stand-alone treatment approach; in cases where lagophthalmos or incomplete blink results from facial nerve palsy, it may be combined with a lid weight procedure. PROSE is an innovative, Food and Drug Administratione approved prosthetic device for ocular surface disease. Most contact lenses and scleral lenses are not labeled for therapeutic use and may be contraindicated in ocular surface disease. PROSE treatment efficacy and safety has been reported in various corneal pathologies, seen here with neurotrophic corneas secondary to skull base tumor resections, and in others such as an adjunct treatment for those after head and neck malignancy resection,14 chronic ocular graft-versus-host disease,10 severe dry eye,15 and corneal ectasias.16 Furthermore, PROSE efficacy as measured through the National Eye Institute Visual Functional Questionnaire-25 showed improved visual functioning in 88% of patients after 6 months8; sustained improvement in visual functioning was still present at a 5-year follow-up.17 Although PROSE is beneficial for a range of corneal pathologies, complications have been reported including bacterial keratitis, exacerbation of corneal edema, and degradation of vision from tear debris accumulation in the fluid compartment of the PROSE device.18 However, most recently, in a large 5-year retrospective review of PROSE treatment, complications from PROSE treatment were uncommon, occurring in 2.5% of the studied cohort, and consisted of redness, corneal graft failure and elevated intraocular pressure, and opacification.17 It is important for clinicians to incorporate comprehensive and continued education about PROSE treatment, device hygiene, and regular interval checkups to enable long-term continuation with device wear and mitigate any possible adverse effects of the device. PROSE treatment requires a degree of cognitive function, and manual dexterity for application and removal of the device; some patients with limited ability make require an assistant or coach to comply with PROSE treatment. Moreover, although PROSE treatment is available at centers throughout the United States, patients located outside of one of these cities will need to travel to a site that offers the treatment. Although the cost of PROSE
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ORIGINAL ARTICLE CAROLYN E. KLOEK ET AL.
PROSE TREATMENT AFTER SKULL BASE TUMOR RESECTION
treatment is comparable or less than that for alternative treatment options, including implantation of a lid weight or tarsorrhaphy, third-party coverage varies; therefore, the cost of PROSE treatment may be a barrier for some patients. Almost 80% of the patients treated at the Massachusetts-based PROSE center in 2011 were covered in part or in full for PROSE treatment through their insurance providers and/or financial assistance.19 Commercially available scleral lenses may be a less expensive option for some patients; however, none are labeled for therapeutic use.
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Neurosurgeons and ophthalmologists should consider PROSE treatment for patients with symptoms or signs of ocular surface disease after skull base tumor resection. We hope this case series raises awareness among neurosurgeons of the importance of managing the ocular surface in patients with keratopathy after skull base neurosurgical procedures and presents a treatment alternative to the traditional management strategies of tarsorrhaphy and weight implantation in the upper lid. More experience is needed to understand how PROSE best fits into existing treatment algorithms.
9. Shepard DS, Razavi M, Stason WB, Jacobs DS, Suaya JA, Cohen M, et al. Economic appraisal of the Boston Ocular Surface Prosthesis. Am J Ophthalmol. 2009;148:860-868.e2.
17. Agranat JS, Kitos NR, Jacobs DS. Prosthetic replacement of the ocular surface ecosystem: impact at 5 years. Br J Ophthalmol. 2016;100: 1171-1175.
10. Jacobs DS, Rosenthal P. Boston scleral lens prosthetic device for treatment of severe dry eye in chronic graft-versus-host disease. Cornea. 2007;26: 1195-1199.
18. Rosenthal P, Croteau A. Fluid-ventilated, gaspermeable scleral contact lens is an effective option for managing severe ocular surface disease and many corneal disorders that would otherwise require penetrating keratoplasty. Eye Contact Lens. 2005;31:130-134.
11. Gire A, Kwok A, Marx DP. PROSE treatment for lagophthalmos and exposure keratopathy. Ophthal Plast Reconstr Surg. 2013;29:e38-e40. 12. Weyns M, Koppen C, Tassignon MJ. Scleral contact lenses as an alternative to tarsorrhaphy for the long-term management of combined exposure and neurotrophic keratopathy. Cornea. 2013;32: 359-361. 13. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93:146-147. 14. Lin SJ, Jacobs DS, Frankenthaler R, Rubin PA. An ocular surface prosthesis as an innovative adjunct in patients with head and neck malignancy. Otolaryngol Head Neck Surg. 2008;139:589-591. 15. Dimit R, Gire A, Pflugfelder SC, Bergmanson JP. Patient ocular conditions and clinical outcomes using a PROSE scleral device. Cont Lens Anterior Eye. 2013;36:159-163.
19. BostonSight. About costs and insurance. Available at: http://www.bostonsight.org/PROSE-treatment/ New-to-PROSE-FAQs/About-Costs-and-Insurance. Accessed October 5, 2015.
Conflict of interest statement: D.S. Jacobs is a salaried, fulltime employee of BostonSight, a 501(c)3 nonprofit organization, and has no financial or proprietary interest in any contact lens or prosthetic devices. Received 27 June 2017; accepted 20 October 2017 Citation: World Neurosurg. (2018) 110:e124-e128. https://doi.org/10.1016/j.wneu.2017.10.111 Journal homepage: www.WORLDNEUROSURGERY.org Available online: www.sciencedirect.com
16. Baran I, Bradley JA, Alipour F, Rosenthal P, Le HG, Jacobs DS. PROSE treatment of corneal ectasia. Contact Lens Anterior Eye. 2012;35:222-227.
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WORLD NEUROSURGERY, https://doi.org/10.1016/j.wneu.2017.10.111