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Year : 2020  |  Volume : 2  |  Issue : 1  |  Page : 7

Multidisciplinary management of neurotrophic and exposure keratopathy secondary to clivus chordoma resection: An ocular surface challenge

Tecnológico de Monterrey, School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Monterrey, México

Date of Submission13-Dec-2019
Date of Acceptance26-Feb-2020
Date of Web Publication24-Mar-2020

Correspondence Address:
Alejandro Rodriguez-Garcia
School of Medicine and Health Sciences, Institute of Ophthalmology and Visual Sciences, Zambrano - Hellion Hospital – TecSalud Batallon de San Patricio 112, CP 66278, San Pedro Garza Garcia, Nuevo Leon
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/PAJO.PAJO_27_19

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The purpose is to report the ocular complications observed after compound cranial nerve palsies secondary to multiple clivus chordoma resection surgeries. A 38-year-old man was referred to our clinic with diplopia and foreign-body sensation. Examination revealed a right peripheral facial paralysis with paralytic lagophthalmos leading to exposure keratopathy and a right sixth nerve palsy with esotropia. Subsequently, the patient developed a longitudinal fusiform-shaped corneal epithelial defect in the right eye. The corneal sensation was reduced due to a fifth nerve palsy leading to neurotrophic keratopathy. The combination of neurotrophic with exposure keratopathy is one of the worst scenarios for corneal integrity.

Keywords: Clivus chordoma, exposure keratopathy, facial nerve palsy, neurotrophic keratopathy, trigeminal nerve section

How to cite this article:
Morales-Mancillas N, Bustamante-Arias A, Paez-Garza JH, Marines-Sanchez H, Rodriguez-Garcia A. Multidisciplinary management of neurotrophic and exposure keratopathy secondary to clivus chordoma resection: An ocular surface challenge. Pan Am J Ophthalmol 2020;2:7

How to cite this URL:
Morales-Mancillas N, Bustamante-Arias A, Paez-Garza JH, Marines-Sanchez H, Rodriguez-Garcia A. Multidisciplinary management of neurotrophic and exposure keratopathy secondary to clivus chordoma resection: An ocular surface challenge. Pan Am J Ophthalmol [serial online] 2020 [cited 2023 Sep 27];2:7. Available from: https://www.thepajo.org/text.asp?2020/2/1/7/281370

  Introduction Top

Chordomas are rare tumors that arise from remnants of the primitive notochord with an incidence of <0.1/100,000 persons/year. Approximately 30% of the tumors arise from the skull base.[1]

Clivus chordoma treatment is challenging because of their location and their invasive nature. Currently, complete resection of the tumor and adjuvant radiation is the therapeutic goal; however, many patients often require more than one surgical procedure.[1],[2]

This clinical case aims to report the ocular complications that can be observed in compound cranial nerve palsies secondary to clivus chordoma resection. This patient had a persistent affectation of three cranial nerves (V, VI, and VII) after multiple surgical interventions. He presented with severe, unilateral exposure and neurotrophic keratopathy. Few case reports describe the ocular complications secondary to clivus chordomas.[3],[4],[5]

  Case Report Top

A 38-year-old male presented complaining of horizontal diplopia, foreign-body sensation, and red-eye after a second skull base surgery due to a clivus chordoma stage IB, T2 N0 M0. The patient had undergone a gross total resection of the tumor via the preauricular/transzygomatic/infratemporal approach 8 years ago. The immunohistochemical analysis of the tumor demonstrated a positive epithelial membrane antigen (EMA), brachyury, and pan-K, confirming the diagnosis. He received 71.1 cobalt-Gy equivalents with protons for 4 months. Since completing the radiotherapy, the initial diplopia, headaches, nausea, and vomiting resolved completely. The ophthalmologic examination revealed a best-corrected visual acuity (BCVA) of 20/20 in both eyes (OU) and normal extraocular movements. The pupil photo motor reflex was intact OU with no relative afferent pupillary defect, and the confrontation visual field test was normal. The slit-lamp examination was unremarkable, showing clear corneas, normal corneal sensation, deep anterior chamber, and normal fundus examination.

The clivus chordoma relapsed 8 years later, requiring a subsequent surgical intervention [Figure 1]. At that time, the ophthalmic examination revealed an uncorrected visual acuity of 20/30 in the right eye (OD) and 20/20 in the left eye (OS). His right eye position was 60 prismatic diopters of esotropia in primary position with limited abduction (−4) and compensatory left head turn. A diagnosis of the right sixth nerve palsy was made. The slit-lamp examination of the right cornea showed 2 + superficial punctate keratitis and patchy epitheliopathy. The qualitative esthesiometry showed normal corneal sensitivity. Another significant finding was a right peripheral facial paralysis with the development of a paralytic lagophthalmos, leading to a new diagnosis of exposure keratopathy. At this point, a lateral tarsorrhaphy was not indicated due to the preexistent CN-VI palsy, which could have exacerbated the corneal epithelial exposure.
Figure 1: Magnetic resonance imaging of the brain. T2-weighted axial magnetic resonance imaging showing a hyperintense lesion (recurrent chordoma) right to the middle line behind the clivus. In the axial plane, it measures 23.0 mm × 23.6 mm, and in the sagittal plane 25 mm

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The patient was treated with preservative-free 0.15% hyaluronic acid eye drops four times a day and 5% dexpanthenol gel at night. During subsequent ophthalmologic visits, the right cornea remained clear with nonocular surface staining.

After repeated botulinum toxin injections of the right medial rectus muscle, a total reduction of 40 prismatic diopters of esotropia was achieved. Nonetheless, the patient continued to experience diplopia and right esotropia, requiring a simple vertical (superior and inferior) rectus muscle transposition.

One year later, the patient had a third intracranial intervention due to a relapse of the clivus chordoma in the cavernous sinus. Microsurgical resection of the tumor from the cavernous sinus and petroclival area (preauricular/middle fossa/zygomatic approach) was performed. Two weeks after the surgery, the patient complained of decreased vision and burning sensation without ocular pain. His uncorrected visual acuity was 20/30 OD. There was a longitudinal fusiform-shaped corneal epithelial defect (6 mm × 1.5 mm) in the inferior quadrant of the right eye [Figure 2]a. The corneal esthesiometry showed a reduced right corneal sensation, leading to a diagnosis of Mackie Stage-II neurotrophic keratopathy. At this point, a lateral tarsorrhaphy was offered to the patient, which he refused due to the cosmetic implications. Therefore, the patient was advised to apply preservative-free 0.15% hyaluronic acid eye drops hourly; a therapeutic contact lens was applied with 0.5% moxifloxacin three times a day as antibiotic prophylaxis. Following a week of treatment, the corneal epithelial defect markedly improved (4 mm × 1 mm), and after 1 month, a complete resolution of the epithelial defect with a minimal linear corneal opacity was observed [Figure 2]b.
Figure 2: (a) Inferior corneal epithelial defect. (b) Inferior corneal epithelial defect after 1 month of treatment

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One month later, the patient developed a right consequent 25 prism diopters exotropia and secondary ptosis with partial pupil obstruction [Figure 3]. Therefore, a 5 mm medial rectus plication was performed to avoid compromising the anterior segment vascular supply [Figure 4]. As for the secondary ptosis with poor levator muscle function, a conservative frontalis suspension surgery using GORE-TEX® combined with a lateral tarsal strip to improve the blinking mechanics and cosmetic appearance [Figure 5].
Figure 3: Right consequent exotropia and secondary ptosis with poor elevator function

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Figure 4: Postoperative of right medial rectus plication

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Figure 5: Frontalis suspension combined with a lateral tarsal strip

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During subsequent visits, the patient remained stable. The visual acuity oscillated between 20/30 and 20/50 due to the corneal surface irregularity. He had another tumor recurrence, requiring a fifth neurosurgical intervention. Subsequently, the patient complained of photophobia and red-eye OD. On examination, the visual acuity was 20/70, he developed a fusiform corneal epithelial defect (4 mm × 1 mm), with anterior stromal edema around the lesion, which was treated with a combination of 3% trehalose +0.15% sodium hyaluronate, and 5% dexpanthenol gel every night with subsequent resolution. Then, the patient developed multiple episodes of inferior corneal epithelial defects; hence, we decided to add a bandage contact lens (140 Dk/t and 24% water content silicone hydrogel lens), 20% autologous serum (AS) eye drops four times a day, and a silicone punctal plug in his right lower lacrimal punctum. After several months of follow-up, the patient presented a sterile corneal ulcer with 30% corneal thinning, 2+ anterior chamber inflammatory cell reaction, and posterior iris synechiae [Figure 6]. The ulcer was successfully treated with prophylactic 0.5% moxifloxacin three times a day, 1% atropine once a day, 0.15% sodium hyaluronate once every 4 hours, 2% AS four times a day, and overnight eye patching.. Currently, the patient has a BCVA of 20/40; the cornea remains markedly hypoesthetic and shows inferior thinning and scarring with superficial vascularization but with a clear visual axis [Figure 7]. He continues on preservative-free 0.15% HA +3% chondroitin sulfate, 20% AS, and patching overnight.
Figure 6: (a) Sterile corneal ulceration with thinning, neovascularization, and inferior scarring. (b) Positive fluorescein staining of the sterile ulcer

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Figure 7: Cornea with a clear visual axis, inferior stromal thinning, and scarring with superficial vascularization

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  Discussion Top

Chordomas are rare, locally aggressive tumors with a high risk for local recurrence. They can be identified by their immunohistochemical profile (cytokeratin, S-100, EMA, and 5×-nucleotidase positive). Brachyury is expressed in 90% of chordomas.[6] Our patient was positive for EMA, pan-K, and brachyury.

Clivus chordomas usually affect the cranial nerves due to their proximity to the brain stem and their local invasion. Regarding midline location chordomas, the surgical strategy must support approaches that do not involve the cranial nerves and great vessels; nevertheless, there is a high incidence of postoperative nerve palsies. About one-half of the patients experience transient deficits of one or more nerves, while others suffer from a persistent deficit.[5],[7] In our case, the patient had a permanent affectation of three cranial nerves (V, VI, and VII).

In this patient, the presence of lagophthalmos led to the corneal desiccation, causing exposure keratopathy combined with the permanent corneal anesthesia leading to decreased metabolism of epithelial cells with a consequent persistent epithelial defect (PED). Chronic epithelial breakdown allows proteolytic enzymes to degrade the extracellular matrix components leading to stromal melting. This condition may lead to significant corneal thinning, perforation, and eventually, loss of the eye.[8],[9]

The combination of neurotrophic with exposure keratopathy is one of the worst scenarios for corneal integrity and function. This entity may be visually disabling, so proper treatment should be given as soon as it is detected.[9] First-line therapy is frequent topical lubrication, as well as prophylactic antibiotics. The main therapeutic goal is the recovery of the corneal epithelium integrity. Restoration of eyelid closure is crucial to reduce corneal exposure and prevent mechanical injuries.[8] After the combined strabismus and oculoplastic surgery to align the eye globe and improve the ptosis, the patient continued to have recurrent epithelial defects despite aggressive unpreserved lubricant and regenerative therapy along with night-long patching. Moreover, he could not wear a bandage contact lens anymore due to intolerance and the eye conditions already explained. After a PED installed, the patient was offered a permanent lateral tarsorrhaphy to reduce the corneal exposure exacerbated by the inadequate blinking and lagophthalmos, almost immobile eye, and anesthetic cornea. Despite warnings about the potential of devastating ocular complications, due to cosmetic issues, the patient declined the procedure. This decision brought a PED with moderate stromal melt, inferior vascularization, anterior chamber inflammatory reaction with the development of posterior iris synechiae, and a final visual acuity of 20/40 OD. Today, the patient remains stable with aggressive unpreserved lubricant and regenerative eye drops, 20% AS eye drops, and overnight patching [Figure 7].

This case remarks on the ocular surface complications that can be observed in compound cranial nerve palsies secondary to clivus chordoma resection. Successful management of this condition depends on prompt and aggressive interventions directed to maintaining the integrity and functionality of the ocular surface. The patient's compliance and cooperation are critical to achieving such goals. Furthermore, it is imperative to have a close follow-up of the patient due to the high morbidity and relapses that may conduct sight-threatening severe consequences.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Chugh R, Tawbi H, Lucas DR, Biermann JS, Schuetze SM, Baker LH. Chordoma: The nonsarcoma primary bone tumor. Oncologist 2007;12:1344-50.  Back to cited text no. 1
Gay E, Sekhar LN, Rubinstein E, Wright DC, Sen C, Janecka IP, et al. Chordomas and chondrosarcomas of the cranial base: Results and follow-up of 60 patients. Neurosurgery 1995;36:887-96.  Back to cited text no. 2
Bagan SM, Hollenhorst RW. Ocular manifestations of intracranial chordomas. Trans Am Ophthalmol Soc 1980;78:148-55.  Back to cited text no. 3
Harada T, Ohashi T, Ohki K, Sawamura Y, Yoshida K, Ito T, et al. Clival chordoma presenting as acute esotropia due to bilateral abducens palsy. Ophthalmologica 1997;211:109-11.  Back to cited text no. 4
Bowyer J, Natha S, Marsh I, Foy P. Visual complications of proton beam therapy for clival chordoma. Eye (Lond) 2003;17:318-23.  Back to cited text no. 5
Goodwin R, Liang L, Zadnik P, Sciubba D. Chordomas and chondrosarcomas. In: Richard W, editor. Youmans and Winn Neurological Surgery. Vol. 155. Philadelphia, USA: Elsevier; 2017. p. 1243-50.  Back to cited text no. 6
Walvekar R, Culicchia F, Nuss D. Surgery of the anterior and middle cranial base. In: Mark R, Paul F, editors. Cummings Otolaryngology. Vol. 174. Philadelphia, USA: Elsevier; 2015. p. 2671-700.  Back to cited text no. 7
Semeraro F, Forbice E, Romano V, Angi M, Romano MR, Filippelli ME, et al. Neurotrophic keratitis. Ophthalmologica 2014;231:191-7.  Back to cited text no. 8
Grey F, Carley F, Biswas S, Tromans C. Scleral contact lens management of bilateral exposure and neurotrophic keratopathy. Cont Lens Anterior Eye 2012;35:288-91.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]


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