anchester Royal Eye Hospital, Manchester Academic Health Science Centre, Oxford Road, Manchester, United Kingdom
r t i c l e i n f o
rticle history:eceived 25 January 2012eceived in revised form 24 July 2012ccepted 26 July 2012
eywords:cleral lenses
a b s t r a c t
We report an interesting case of therapeutic scleral lens management of bilateral exposure and neu-rotrophic keratopathy resulting from bilateral cranial nerve (CN) palsies including V, VI and VII, whichcaused lagophthalmos and anaesthetic corneas. Subsequent development of severe exposure keratitiswith vascularisation and keratinisation of the inferior cornea was previously treated with intensive ocu-lar lubrication, botulinum toxin injections to the upper eyelid levator muscle, temporary tarsorrhophies,bilateral amniotic membrane grafts, punctal plugs, lid taping, gold eyelid weights and soft bandage con-
tact lenses. Corneal integrity was re-established but visual acuity remained significantly compromised bycorneal vascularisation, scarring and keratin deposits. Visions on presentation to the contact lens depart-ment were R 1.90 log MAR, L 1.86 log MAR. Therapeutic, high Dk, non-fenestrated, saline filled, sclerallenses were fitted. Daily wear of these lenses have protected and hydrated the cornea, enabling cornealsurface recovery whilst retaining visual and social function. The visual acuities 6 months post-scleralfitting with lenses in situ are R 0.90 log MAR and L log MAR 0.70.
Scleral lenses are most commonly used for achieving opticalorrection and visual improvement in eyes with highly irregularorneal surfaces [1,2] but they may also serve a secondary thera-eutic function in cases of corneal disease. In situ, the lenses protect
njured or diseased corneal tissue and aid the return to a normalnatomical and functional state [3].
Exposure keratopathy refers to drying of the cornea with sub-equent epithelial breakdown. Severe exposure keratopathy mayead to significant visual loss due to corneal scarring, neovascu-arisation, stromal thinning, ulceration and ultimately perforation.he corneal exposure arises from incomplete or inadequate lidlosure.
Several therapeutic and surgical treatment options are available,or example performing a tarsorrhaphy, but these options have lim-tations, and may be inconvenient to maintain, cosmetically poornd visually disabling while attaining corneal coverage.
This patient was fitted with Innovative Sclerals (Innovative Scle-
als Ltd., Hertfordshire, UK) RGP scleral lenses which were fittedligned to the sclera with clearance over the limbal area and cornea.he area of clearance was filled with saline creating a fluid bandage
∗ Corresponding author at: Optometry Department, Manchester Royal Eye Hospi-al, Oxford Road, Manchester M13 9WL, United Kingdom. Tel.: +44 0161 276 5255.
h Contact Lens Association. Published by Elsevier Ltd. All rights reserved.
lens. The cornea is therefore protected from lid friction as well asdesiccation [4]. This method has been shown to be not only effectivebut visual function is maintained during treatment.
2. Case report
HL presented with a diagnosis of a low grade astrocytomaaged 4 years and underwent surgical de-bulking and radiotherapytreatments for the tumour. Further surgery 6 years after initial pre-sentation was followed by a significant deterioration in her physicalhealth due to extensive brain stem involvement which resulted inbilateral V, VI, VII, and VIII cranial nerve palsies and a correspond-ing bulbar palsy involving bilateral impairment of function of thelower cranial nerves (IX, X, XI and XII). She was rendered virtuallyquadriplegic and required mechanical ventilation.
Involvement of the sensory branch of the trigeminal nerve (CNV) led to anaesthetic corneas and the development of bilateral neu-rotrophic corneal ulceration.
Abducens nerve (CN VI) involvement resulted in an intermit-tent right esotropia. Communication was limited at this stage hencethere was difficulty elucidating symptoms.
Facial nerve (CN VII) involvement caused weakness of theorbicularis oculi muscles resulting in significant lagophthalmos
and marked corneal exposure which was exacerbated due to theabsence of a Bell’s phenomenon. The innervation to the lacrimalgland runs along CN VII so reduced tear production also exacerbatedcorneal dehydration.
F. Grey et al. / Contact Lens & Anterior Eye 35 (2012) 288– 291 289
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Fig. 1. R and L corneas on
As a result of the above HL developed bilateral severe exposureeratitis with a neurotrophic element leading to vascularisationnd keratinisation of the inferior corneas which was worse in theight eye compared to the left. Aged 14 she was referred to theanchester Royal Eye Hospital.Over the course of 18 months HL underwent a series of treat-
ents.Preservative free artificial tears were instilled on an hourly basis
o improve the corneal surface by hydration. Temporary tarsorrha-hies were performed to reduce the area of exposure. Botulinumoxin injections to the upper eyelid levator muscle induced pto-is and consequently achieved corneal lid coverage. Gold eyelideights were fitted to the upper lids. There was still significant
agophthalmos in the day but the eyelids closed almost com-letely at night. Lid taping at night was advised but HL was notompliant.
Despite the above measures the patient’s eyes continued dete-iorating, noting worse vision and increased hyperaemia. It wasifficult to assess the patient’s vision due to her poor health and
nability to communicate but she was found to fix and follow lightith each eye.
On referral to the corneal team punctal plugs were fitted andmniotic membrane grafts arranged. Soft bandage lenses (Purevi-ion 8.60/14.0/plano) were fitted but displaced easily. The fittingf a larger soft lens was discussed but not pursued due to markedagophthalmos and anticipated desiccation. Corneal integrity wastabilised but visual acuity was significantly reduced by cornealeratin deposits. The cornea was thinned but not thought to bet risk of perforation. The right conjunctiva was hyperaemic and
nflamed. HL was referred to the Contact Lens Clinic for visual reha-ilitation with therapeutic scleral contact lens fitting.
Refraction was not possible on presentation as retinoscopyas obstructed by corneal opacification and subjective refraction
Fig. 2. Scleral lenses in situ 3 m
ral to contact lens clinic.
was not feasible. Visions on presentation were R 1.90 log MAR, L1.86 log MAR (Fig. 1).
Though hyperaemic due to exposure, the bulbar conjunctivaewere sufficiently regular to allow for a sealed fit of the lenses. Totalcorneal clearance over the raised keratin deposits was achieved.
Preservative free saline was used to fill the lenses prior to inser-tion. HL had limited use of her upper and lower limbs so insertionand removal was completed by her mother.
On review it was noted that the right lens was less comfortableafter 3 h wear. The lens was steepened to increase corneal clearanceand symptoms resolved by the next follow up.
R) Innovative sclerals therapeutic scleral N5 7.73/23.00/planostandard material CT 1.05
Due to reduced tear production the use of artificial tears wasadvocated over the lenses three or four times a day.
The lenses were dry when removed in the evening so a drop ofpreservative free carmellose sodium 0.5% was added to the saline
prior to lens insertion. Wear was intermittent initially but increasedto include all waking hours 8 weeks after initial fitting. During shortperiods of ill health wear was suspended but resumed on recovery.After 6 months of wear the results were very positive. Return of
onths after initial fitting.
290 F. Grey et al. / Contact Lens & Anterior Eye 35 (2012) 288– 291
sclera
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Fig. 3. R and L corneas at 6 months of
entral corneal clarity to the left eye has enabled retinoscopy andncorporation of refractive power to the lens.
L) Innovative sclerals scleral N4 7.98 23 mm +3.00 standard mate-rial CT 1.15
The corneas were visibly more hydrated. The keratin depositsad markedly reduced and were now translucent. Conjunctivalyperaemia was much improved in the right eye (Fig. 3).
Vision was notably improved at RVA 0.90 log MAR and LVA.70 log MAR with the lenses in situ.
A significant improvement to quality of life has occurred. HL isow able to decipher large print books and is able to enjoy reading.
Ongoing contact lens follow up is required but it is hoped that inime the lagophthalmos will resolve with continued recovery andhe patient will become less reliant on contact lens therapy.
. Discussion
This case highlights the ocular surface complications that cane seen in multiple cranial nerve palsies. The presence of sig-ificant lagophthalmos led to desiccation of the corneal surfaceausing severe exposure keratitis. The principle of managing expo-ure keratopathy is to restore corneal integrity, thereby stabilisingorneal thinning and minimising the risk of corneal perforation4]. The prognosis for exposure keratopathy is worse if associatedith corneal anaesthesia as loss of corneal sensory innervation
eads to a decrease in vitality, metabolism, and mitosis of epithe-ial cells and consequently to epithelial breakdown and ulceration5].
Conventional treatments are highly effective but may have lim-tations in specific cases. Hourly application of ocular lubrications difficult to maintain on a long term basis, particularly for outatients. Due to the bilaterality of this case the obstruction of vision
nduced by bilateral botulinum toxin injections would be visuallyisabling and distressing for the patient and would interfere wither physical rehabilitation. The same would be true for tarsorrha-hies, and, though not significant in this case, consideration shouldlso be given to the psychological effect on a patient with this dis-guring therapy.
Amniotic membrane grafts reduce inflammation and promoteorneal healing [6]. However, they are vulnerable to dehydration inases of extreme lagophthalmos. Soft contact lenses may also dehy-rate in the absence of lid coverage so are not effective if significant
agophthalmos is present [7]. Soft lenses also displaced easily, as
ight occur post eyelid gold weight implantation surgery, and were
herefore discontinued due to limited success. Gold eyelid weightsere surgically inserted into the upper eye lid. The weights allow
he lids to close by gravity when muscles relax but do not affect
l lens wear (imaged without lenses).
the ability to open the eyes normally when muscles are tense [8].Lagophthalmos was reduced but the corneas remained exposed inthe day, despite closing at night, due to facial paresis.
Eyelids may be taped closed at night but there were some asso-ciated behavioural difficulties which rendered the application ofthis treatment unsuccessful for this patient.
In this case scleral lenses were successfully used to man-age severe corneal exposure and neurotrophic keratopathy. Theintegrity of the corneal epithelium is improved and the lensesare effective at increasing corneal surface hydration and pro-moting corneal healing in a manner that is relatively convenientand without detrimental effect on visual function or cosmeticappearance.
The potential applications of scleral lens therapy in corneal sur-face disease management are numerous. Successful treatment ofocular surface disease with scleral lenses has also been reportedin the management of neurotrophic keratitis secondary to herpeszoster ophthalmicus [9] and exposure keratopathy associated withsevere craniofacial trauma [7].
Lenses are ideally fitted with total corneal clearance and a sealedfit. The introduction of rigid gas permeable materials enables thesaline reservoir to remain oxygenated throughout the day and asealed fit is relatively stable with minimal settling back [10,11].There was some seepage of saline during the day leading to thelenses drying out by evening. The addition of higher viscositycarmellose sodium (Celluvisc®) 0.5% to the saline reservoir sig-nificantly preserved the fluid reservoir and the patient did notcomplain of any visual degradation with this management. HL’smother was also advised that lenses could be removed and refilledduring the day but this was difficult on weekdays when the patientwas at school.
Regular aftercare will be required as there is still risk of hypoxiawith prolonged wear and in this case, the anaesthetic corneas couldleave the patient unaware of symptoms of ulceration. Daily wearwas advised due to the significant risk of microbial keratitis withextended wear modality [4]. A strict cleaning regime is also essen-tial.
Severe exposure keratopathy can be extremely visuallydisabling. The successful management of the condition hasdramatically improved quality of life, aided education andallowed HL to actively partake in much enjoyed recreationalactivities.
In summary, we report a case of severe, bilateral exposureand neurotrophic keratopathy as a consequence of multiple cra-nial nerve palsies. Restoration of corneal integrity and clarity was
achieved with the aid of therapeutic scleral lenses and were foundto be a highly effective treatment modality for our patient. Wewould advocate the use of such lenses as a management optionfor patients with similar ocular surface disease.
Ante
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F. Grey et al. / Contact Lens &
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