Rajasthan Journal of Ophthalmology 2012 Page 1

Rajasthan Journal of Ophthalmology 2012


RAJASTHAN JOURNAL OF OPHTHALMOLOGY 2012

ROS Executive Committee 2011-2012

President Dr S.K. Nair

President Elect

Dr S.P. Vyas

Past President Dr V.K .Sharma

Hony. Gen Secretary

Dr Mahesh Punjabi

Treasurer

Dr J.S.Saroya

Chairman Scientific Committee Dr Mukesh Sharma

Editor Journal Dr Sudhir Singh

Members Dr Ajeet Jakhar

Dr Neeraj Khungar Dr Anil Sharma Dr Vivek Jain

Dr Harshul Tak

Advisors Dr K Lal

Dr L.K. Nepalia


RAJASTHAN JOURNAL OF OPHTHALMOLOGY

2012

Contents

1. Editorial

2. To Evaluate The Efficiency Of Fibrin Glue To Secure

Conjunctival Autograft For Pterygium Surgery In

Comparison With Sutures 5

Dr. L.S.Jhala, Dr. Snehdeep Shrikar; Dr. Abhishek

Saraf

3. Effect Of Muscle Displacement Of Horizontal Recti

In Pattern Strabismus 14

Dr Amit Mohan, M.S.; Dr Sudhir Singh, M.S.

4. Keratoconus- Treatment Options 19

Dr Vikas Gupta M.S., Dr Sonu Goel D.N.B., Dr Chitra

Sitaraman M.S., Dr Anurag Mathur M.S.,Dr. Vaishali

Mathur M.S.,

5. Rise Of Intraocular Pressure After Intravitreal

Injection Triamcinolone Acetonide 28

Dr Manish Joshi, MS; Dr Mona Vaish, MS

6. Orbital Implants: Our Experience 34

Dr Seema Laad, DOMS, Dr Amit Mohan, MS; Dr Sudhir

Singh

7. Current Biologic Therapies In Management Of

Uveitis 38

Dr Pankaj Dhaka, MD, Dr Amarendra Samal, MD

8. Case Report Of Isolated Schwannoma A Rare

Tumour Of Eyelid 53

Dr Sudhir Singh, M.S, Dr Subodh Saraf ,M.S,

DrvDivyesh Goswami , M.D

9. Goldenhar Syndrome: Dermoid Excision Reduces

Acquired Astigmatism 58

Dr Amit Mohan,M.S.; Dr Sudhir Singh, M.S.

Editor Dr Sudhir Singh

Advisors

Prof. P.K Mathur Dr Pavan Shorey

Editorial Board

Dr Anshoo Choudhary

Dr Arun Kshetrapal Dr Gulam Ali Kamdar Dr L S Jhala Dr Mayank Agrawal Dr Mukesh Sharma Dr Sandeep Arora Dr Sonu Goel Dr Subodh Saraf Dr Sukesh Tandon Dr Sunil Gupta

Dr Suresh Kumar Pandey

Dr Swati Tomar Dr Virendra Agrawal

Editor's Office

Dr Sudhir Singh

Sr. Consultant & Head Dept of Ophthalmology JW Global Hospital & Research Centre Mount Abu Rajasthan 307501 [email protected]


To evaluate the efficiency of fibrin glue to secure conjunctival autograft for pterygium surgery in comparison with sutures L.S.Jhala

; Snehdeep Shrikar; Abhishek Saraf

Correspondence Author Dr LS Jhala Alakh Nayan Mandir Ashokpura Udaipur

Methods: A prospective randomized case control study was conducted on 50 patients visiting the OPD at

Alakh Nayan Mandir eye institute, Udaipur. Patients were divided into two groups comprising 25 patients

each by simple random method. Group A underwent pterygium excision with conjunctival autograft

transplantation with the help of 8-0 Vicryl sutures and in group B surgery was done using fibrin glue. The

operative time, postoperative pain and foreign body sensation and intra and postoperative complications

were compared between both the groups.

Results: Our study revealed that the use of fibrin glue in pterygium surgery significantly reduces the average operating time in comparison to vicryl suture (p= 0.001). The degree of pain was much less in case of fibrin glue group (p=0.02), and also recovery from the same was much faster than the suture group (p= 0.042).

Conclusion: The use of fibrin glue for the attachment of conjunctival autografts in pterygium surgery

seems to be safe and efficacious.

Key Words: Pterygium, conjunctival autograft, sutures, fibrin glue.

Financial disclosure: No author has a financial or proprietary interest in any material or method mentioned.

Introduction:

Pterygium derived from the Greek, pterygion meaning “wing”1 was introduced to the English language in 1875 by Walton2. Clinically, it appears as a fleshy, vascular mass that occurs in the interpalpebral fissure. Pterygium typically develops in patients who have been living in hot tropical climates, outdoor workers and may represent a response to chronic dryness and ultraviolet exposure 3.

The progression of the pterygium onto the cornea can lead to decreased vision either due to induced astigmatism or disruption of pre corneal tear film or

involvement of the visual axis3. The indications for surgery include decreased vision, foreign body sensation, redness, watering, recurrent inflammation and cosmetic purpose.

In recent years, Conjunctival autograft transplantation has evolved as a procedure of choice in which free conjunctival graft is secured over the bare sclera with the help of either vicryl sutures or fibrin glue. Suturing is a time consuming task in ophthalmology and suture induced irritation and redness are frequent problems. Postoperative wound infection and corneal graft rejection are examples of possible suture related complications. To prevent these


complications, ophthalmic surgeons are switching to sutureless surgery, i.e. using fibrin glue, a recent innovation in pterygium surgery.

Methods:

A prospective randomized case control study was conducted on 50 patients visiting the OPD at Alakh Nayan Mandir eye institute, Udaipur were evaluated and operated for pterygium at base hospital. Patients were divided into two groups comprising 25 patients each by simple random method. All the surgeries were performed by the single surgeon (AS). Suture removal in all cases (group A) was done at seventh day from surgery. This was primarily for the purpose of observing the recovery time.

Group A underwent pterygium excision with conjunctival autograft transplantation with the help of 8-0 Vicryl sutures and in group B surgery was done using fibrin glue. Patients were explained about both the procedures and patient had the freedom to choose between the two.

Harvesting of Conjunctival autograft

Application of Fibrin Glue over the bare area

Conjunctival Autograft secured with fibrin

Conjunctival autograft in situ glue over the bare area.


Results: Out of 50 patients, 17(34%) were male and 33(66%) were female. The average age in our study was 43 years and majority of the patients (68%) belonged to the 20-50 years age group. Our study revealed that 92% of cases had a history of pterygium for duration under 3 years. The length of pterygium (corneal encroachment) was measured in each case and majority (72%) of cases were found to have pterygium size between 1-4 mm. Out of the 50 eyes operated 23 were right eye and 27 were left eye.

Study variable age, sex, operated eye and the grade of pterygium did not bring out any significant statistical correlation ship.

The operative time was measured for each surgery in both the groups (Table 1). The average time in group A (suture) subjects was 27.9 minutes and 21.9 minutes for group B (Fibrin glue). The t- test indicated a significant difference in surgery time between both the groups (p= 0.001).

Pterygium recurrence was observed in one (4%) patient in group A (suture), whereas no recurrence was seen in group B (Fibrin glue). On Chi-Square analysis, the difference between both the groups was not significant (p= 0.318)

Patient comfort was significantly higher in Fibrin glue group than the vicryl suture group. The Chi-Square analysis indicates that the pain was dependent upon the type of surgery (p=0.02). In Fibrin glue group all the patients had mild pain whereas in suture group 24%

had moderate pain and 4% had severe pain.

The Chi-Square analysis on foreign body sensation also showed a significant difference in both the groups (p=0.002). In Fibrin glue group 88% of patients had mild foreign body sensation whereas in suture group 48% had moderate and 4% had severe foreign body sensation.

The average age in our study group was 43 yrs. 68% of patients belonged to 20-50yrs of age group.


Males comprised 34% of cases while

female were 66%.

Our study revealed that 92% of cases had a history of pterygium less than 3 years


In our study 72% of cases had pterygium size between 1-4 mm

The average operating time in group A subjects was 27.9 minutes and 21.9 minutes for group B (p=0.001).


There was one pterygium recurrence in suture group, however, no recurrence was observed in fibrin glue group

The chi-square analysis indicates that the pain was dependent upon the type of surgery (p=0.02). In fibrin glue group all the patients had mild pain whereas in

suture group 24% had moderate pain and 4% had severe pain

The chi-square analysis indicates that the foreign body sensation was dependent upon the type of surgery (p=0.002). In fibrin glue group 88% of the patients had mild foreign body sensation whereas in suture group 48% had moderate and 4% had severe foreign body sensation

Mann Whitney U test analysis revealed significant difference between both the groups (p=0.039), in graft retraction. It was present in 16% of cases in fibrin glue group


Mann- Whitney U Test showed

significant difference in the recovery

from pain in both the groups at 30 days

(p=0.042). On 30th day, there was

complete recovery in 96% of cases in

fibrin glue group, whereas in the suture

group 20% of patients still had mild pain.

In the follow- up at the end of six months

both groups showed extinction of the

same.

Mann- Whitney U Test showed

significant difference in the recovery

from foreign body sensation in both the

groups at 30 days (p=0.001). On 30th

day, there was complete recovery in

80% of cases in fibrin glue group;

whereas in the suture group 56% had

mild and 12% had moderate degree of

foreign body sensation.

Mann Whitney U test analysis revealed no significant difference between both the groups’- Not Significant


Table 8 shows percentage wise distribution of Intra and Post operative complications. On interpretation of the parameters by Mann Whitney U test there was significant difference between the two groups (p= 0.039), in respect to graft retraction. It was present in 16% of patients in Fibrin glue group.

Buttonholing of the graft, graft loss, graft edema, sub-conjunctival hemorrhage, granuloma formation at the autograft donor area and post-operative surface irregularity did not bring out any significant relationship.

The recovery from post operative pain, when evaluated with the help of Mann-Whitney U test, showed significant difference in the recovery from pain in both the groups (p= 0.042) at 30 days. On 30th day, there was complete recovery in 96% of the cases in fibrin glue group, whereas in the suture group 20% of patients still had mild pain.

Similar evaluation was done for recovery from post-operative foreign body sensation, on 30th day, there was complete recovery in 80% of cases in fibrin glue group, where as in the suture group 56% had mild and 12% had moderate degree of pain.

On comparison for early and late post-operative complications by Mann-Witney U test, there was no significant difference between both the groups.

Conclusions:

Our study revealed that the use of fibrin glue in pterygium surgery significantly reduces the average operating time in comparison to vicryl suture. Use of fibrin glue results in a shorter operating time, less post operative discomfort and inflammation. Bahar et al4 found average operative time to be 16 minutes (range, 14-16 minutes) in the fibrin glue group and 20 minutes (range, 20-29 minutes) in the Vicryl suture group (P < 0.05). Study conducted by Ratnalingam et al5 found the mean duration required to complete surgery in the fibrin adhesive group was 16.93 +/- 2.85 minutes, whereas that of the suture group was 29.84 +/- 5.65 minutes, which was statistically significant (P < 0.001).

The degree of pain was much less in case of fibrin glue group and also recovery from the same was much faster than the suture group. Similar results were also obtained by the study conducted by Bahar et al.4 Our results was in accordance with the study conducted by Bahar et al4 which showed patient comfort was significantly higher in the fibrin glue group than the vicryl suture group.

Hence the use of fibrin glue for the attachment of conjunctival autografts in pterygium surgery seems to be safe and efficacious.


References:

1. Krachmer, Mannis and Holland. Cornea and external disease: Clinical diagnosis and management. Second edition. Philadelphia. Elsevier (Mosby). 2005: 1749-1761.

2. Walton HH. A practical treatise on diseases of the eye. 3rd ed. London: J and A Churchill, 1875.

3. Jack J. Kanski, Clinical Ophthalmology, Sixth edition. London:Butterworth-Heinemann, 2007: 242-244.

4. Bahar, Irit; Weinberger, Dov; Dan, Gaton; Avisar, Rahamim. Pterygium Surgery: Fibrin Glue Versus Vicryl Sutures for Conjunctival Closure. Cornea. 25(10):1168-1172, December 2006.

5. Ratnalingam, Vanitha; Keat Eu, Andrew Lim; Ng, Gim Leong;

Taharin, Rohana; John, Elizabeth. Fibrin Adhesive Is Better Than Sutures in Pterygium Surgery Cornea. 29(5):485-489, May 2010.

http://journals.lww.com/corneajrnl/Abstract/2006/12000/Pterygium_Surgery__Fibrin_Glue_Versus_Vicryl.8.aspx



http://journals.lww.com/corneajrnl/Abstract/2010/05000/Fibrin_Adhesive_Is_Better_Than_Sutures_in.1.aspx




Effect of muscle displacement of horizontal recti in pattern strabismus Amit Mohan, M.S.; Sudhir Singh, M.S. Correspondence Author Dr Amit Mohan Junior Consultant Global Hospital Institute of Ophthalmology Aburoad, Sirohi.Rajasthan Pin 307510 Sixteen cases of A & V pattern strabismus undergoing horizontal muscle surgery with half tendon width

vertical offsets and three patients undergoing two third to full tendon width offsets were retrospectively

studied. Half muscle width shift were found to be effective in cases of A & V pattern strabismus in which

oblique muscle dysfunction is inadequate & pattern is less than 25PD. For pattern greater than 25PD,

three quarter to full tendon with offset was effective in collapsing pattern.

Introduction:

A significant difference in size of

horizontal strabismic deviation in

defined positions of up gaze and

downgaze is termed A- or V- pattern

strabismus 1 .The co-existence of an A

or V pattern with horizontal strabismus

is seen in 12.5% to 50% of cases 2, 1, 3.

Surgical management of pattern is

complex because of its different

presentation. If oblique muscles are

significantly overacting or under acting,

they should be target of surgery. If there

is no oblique muscle dysfunction,

treatment of pattern by supraplacement

or infraplacement of the recessed or

resected horizontal rectus muscle can

be effective. The MR is always

transposed toward the apex of the V

(infraplaced) or the A (supraplaced).

The LR is supraplaced to correct V

pattern and infraplaced to correct A

patterns 4, 5.

In our study, the effectiveness of vertical

offsets of horizontal muscles were

studied on short term (4 weeks) and

long term (6 months) basis.

Material and Methods

Patient Selection:

Nineteen cases of AV pattern that

underwent muscle displacement surgery

in the squint clinic of Global hospital

institute of ophthalmology, Abu road

were selected for retrospective study to

evaluate the effect of vertical shifting. A

complete orthoptic evaluation was done

where deviations were measured in

primary position, 25 degree up gaze &

25 degree downgaze by tilting the head.

A difference of 15 prism diopter or more

in V phenomenon and 10 PD or more in

A phenomenon were taken as criterion

for these patterns. Measurement was

done with prism cover test and the tests

for binocularity were done with worth 4

dot test. Oblique over action were

clinically estimated on a scale of +1

through +4 6 and those with +1 or less

were considered for muscle

displacement surgery.

Surgical Technique

Monocular recession-resection

procedures were done for the deviation


in the primary position with vertical

displacement of horizontal recti. For V

pattern the medial rectus was

depressed and lateral rectus was

elevated and the reverse was done for A

pattern.5mm shift was done for pattern

less than 25PD and 8-10mm shift was

done for pattern more than 25 PD.

Patient were assessed 1st post operative

day and thereafter at 4 weeks and 6

months interval.

Results:

19 patients with pattern strabismus

underwent muscle displacement

surgery. The mean age at surgery in this

study was 16.15years with a range of 2

to 34 year. The male patients were

12(63.15 %) and female patients were

7( 36.84%)RE surgery was done in 2

case and L/E was in 7 cases. out of 19

patients 11 patients(57.89%) had

exotropia and 8(42.10%) has

esotropia.7 patients had V pattern

exotropia and 4 has A pattern exotropia

while 6 has V pattern esotropia and 2

had A pattern esotropia.16

patients(84.21%) had pattern less than

25PD while 3 patients(15.78%) had

pattern more than 25 PD.

One the basis of surgery performed,

patients were divided into two groups.

Group 1 consists of patients having

pattern <25PD and underwent 5mm

muscle displacement. (Table 1 shows

pre operative & post operative deviation

observed in cases of 5mm shift).

Group 2 consists of patients having

pattern >25PD and underwent 8-10mm

muscle shifting. (Table 2 shows

preoperative & postoperative deviation

in cases of 8-10mm shift).

In group 1 the initial correction to within

+/_ 10PD of pattern was 100% over all

with 93.75% remaining collapsed over 6

month follow up.

In group 2, 8-10mm muscle offsets were

effective in 66.67% cases in collapsing

pattern within +/_10PD in 6 months

follow up.

In group 1 mean preoperative pattern

was 16.18 prism diopter and

postoperative pattern was 4.25PD in 6

month. Average correction was 14.25PD

In group 2 mean preoperative pattern

was 28.33PD and postoperative pattern

was 9.33 PD in 6month with average

correction of 19PD.

Discussion:

Monocular vertical displacements of

horizontal recti were successful in

decreasing the A and V pattern. Surgical

procedures on different extra ocular

muscles and their transposition have

been practiced for many year and many

ophthalmologists agree that in cases

with no obvious oblique muscle

dysfunction , symmetrical vertical

displacement of horizontal recti muscle

should be performed 7,8,9. Goldstein 10


performed monocular vertical

displacement of horizontal recti,

comparing 5mm and 8mm shifting and

concluded that both the procedures

were effective for reducing the vertical

incomitance.Metze 11and Almedia 12performed only 5mm shift and claimed

that the procedure was effective in

reducing A and V phenomenon. Metz 11also reported 15 PD correction with

the 5mm shift. Our study confirmed the

finding of Almedia and Metz that 5mm

shift was effective in correcting AV

phenomenon. Scott 13 performed three

quarter to full tendon width offsets for

pattern greater than 30PD. Our study

also revealed that 8-10mm muscle

displacement is effective for pattern

greater than 25PD.

Dr. Pradeep Sharma 14 advocates a 5-

mm shift is as effective as an 8mm shift

done along with a monocular recession-

resection procedure to correct the A or

V phenomenon but 8mm shift causes

incomitances in extreme gazes, so in

our study we have done 8mm shifting

only in pattern greater than 25 PD.

To conclude a 5mm shift is effective

along with a monocular recession –

resection to correct the A or V pattern

less than 25 PD and 8mm shift is

effective for pattern more than 25 PD.


pattern preoperative pattern

postoperative pattern (4 week)

postoperative pattern 6months

primary upgaze downgaze pattern pattern left correction pattern left correction

1 V 40 45 30 15 0 15 0 15

2 V 30 45 25 20 5 15 0 15

3 V 35 40 25 15 0 15 0 15

4 V 40 40 18 22 4 18 7 15

5 V 45 50 30 20 0 70 5 15

6 V 20 35 16 19 0 19 6 13

7 A 30 25 45 20 5 15 5 15

8 A 45 40 55 15 0 15 0 15

9 A 40 20 45 25 10 15 12 13

10 V 35 25 45 20 5 15 5 15

11 V 40 35 50 15 0 15 0 15

12 V 30 20 40 20 4 16 6 19

13 V 40 30 45 15 0 15 0 15

14 V 35 35 50 15 0 15 0 15

15 V 25 20 40 20 5 15 8 12

16 A 45 50 30 20 7 13 9 11

GROUP-2 Table-2

s.no age/sex type pattern preoperative pattern postoperative pattern (4 week) postoperative pattern(6 months

primary upgaze downgaze pattern pattern leftcorrection pattern leftcorrection

1 18/M XT V 25 40 10 30 6 24 8 22

2 20/M XT A 25 15 45 30 10 20 12 18

3 4/F ET A 45 50 25 25 8 17 8 17


REFERENCES:

1- Knapp P: Vertically incomitant

horizontal strabismus,the so

called A & V syndromes.Trans

Am Ophthalmol Soc

1959;57:666

2- Harley RD,Manley DR: Bilateral

superior oblique tenectomy in A

pattern exotropia.Trans Am

Ophthalmol Soc 1969;67:324

3- Urist MJ: The etiology of the so

called A and V syndromes.Am

J Ophthalmol 1958;46:835.

4- Pratt-Johnson JA,Tilson G: The

patient with vertical

strabismus,A practical guide ,p

155.New York thieme Medical

Publishers,1994.

5- Von Noorden GK; A and V

patterns.In: Binocular Vision

and Ocular Motility:5th ed

p376.St Louis,Mosby,1996.

6- color atlas of strabismus

surgery -kenneth w. Wright, 17

IO weakening procedure p.167)

7- Burian HM, Cooper EL,

Costenbader FD. The A-V

pattern in strabismus. Trans

Am Acad Ophthalmol 68:375,

1964.

8- Noorden GK von, Olson CL.

Diagnosis and surgical

management of vertically

incomitant horizontal

strabismus. Am J Ophthalmol

60:433-442, 1965.

9- Prakash P, Menon V, Nath J.

Surgical management of A and

V patterns. Indian J ophthalmol

31:463, 1983

10- Goldstein GH. Monocular

vertical displacement of

horizontal rectus muscle in A

and V patterns. Am J

Ophthalmol 64:265, 1967.

11- Metz HS. The treatment of A

and V patterns by monocular

surgery. Arch Ophthalmol

95:251, 1978.

12- Almeida HC. Correction of A

and V syndrome acting upon

only one eye. Proceedings of

the International

Strabismological Association,

France, 1974, pp. 134-137.

13- Scott: Aust NZ J Ophthalmol

1989 Aug,17(3);281

14- Sharma P, Halder M, Prakash

P. Effect of monocular vertical

displacement of horizontal recti

in A V phenomena. Indian J

Ophthalmol 1995;43:9-11


Keratoconus- Treatment options

Vikas Gupta M.S., Sonu Goel D.N.B., Chitra Sitaraman M.S., Anurag Mathur M.S., Vaishali Mathur

M.S.,

Correspondence Author

Dr Vikas Gupta Anand Eye Hospital Civil Lines,Jaipur

Introduction

Keratoconus is a bilateral no

inflammatory corneal ectasia with an

incidence of approximately 1 per 2,000

in the general population.1, 2, 3It has well

described clinical signs, but early forms

of the disease may go undetected

unless the corneal topography is

studied. Classic histopathologic features

include stromal thinning, iron deposition

in the epithelial basement membrane,

and breaks in Bowman’s layer.

Keratoconus is most commonly an

isolated disorder although an

association with Down syndrome,

Leber’s congenital amaurosis, and mitral

valve prolapsed has been described.

The differential diagnosis of keratoconus

includes keratoglobus, pellucid marginal

degeneration and Terrien’s marginal

degeneration. Typically, the patients

present in early adulthood and visual

symptoms result from irregular

astigmatism and increasing myopia4, 5. It

is reported to have bilateral involvement

in over 90% of patients, with asymmetric

presentation. Most keratoconus patients

can be adequately corrected with

spectacles or contact lenses. However,

In recent years there has been a rapid

advancement in the therapeutic options

for keratoconus management.

Spectacle Correction

In the early stages of keratoconus, the

patient’s refractive error can often be

successfully managed with spectacle

lenses. It is important to communicate to

the patient that there is no evidence to

support the theory that early contact

lens intervention is of therapeutic benefit

in preventing or lessening the

progression of the disease. However,

wearing contact lenses typically

provides the patient with better visual

acuity than can be obtained with glasses

by neutralizing the regular and irregular

refractive errors induced by the

condition.

Contact lenses

As the condition progresses, spectacles

may fail to provide the patient with a

satisfactory degree of visual acuity.

Contact lenses improve vision by means

of tear fluid filling the gap between the


irregular corneal surface and the smooth

regular inner surface of the lens, thereby

creating the effect of a smoother cornea.

Traditionally, lenses for keratoconus

have been the “hard” or rigid gas-

permeable contact lens variety. For

most patients with keratoconus, a three

point touch contact lenses design is

ideal (figure 1), and is preferred over

apical clearance and apical touch

designs. The base curve should be

steep enough to provide a slight central

touch, shown by thinning of fluorescein,

at the corneal apex and slight touch

mid-peripherally at 3 and 9 o’clock along

the horizontal meridian. This creates

three points of lens touch along the

horizontal meridian. In mild to moderate

keratoconus, the lens diameter selected

is usually 7.5-8.5mm. A small size

facilitates tear exchange and allows a

steeper fit to accommodate the cone.

Central nipple cones do best with small

diameter lenses. When the cone is

displaced peripherally, as with oval and

globus cones, one usually ends up

fitting a larger, flatter lens. Several

specially designed contact lenses have

been developed to facilitate fitting in

advanced, difficult to fit keratoconus

cases. Soper lenses are one of the best

known lenses. This is a bicurve design

with a steep central curve to

accommodate the cone and peripheral

curve to align with the peripheral

cornea.

Image 1 Source

http://www.clspectrum.com/content/archive/2011/May/image

s/CLS_May_A20_fig3.jpg

Image Source 2 http://en.wikipedia.org/wiki/File:Keratoconus_contact_lens_size.jpg

http://www.clspectrum.com/content/archive/2011/May/images/CLS_May_A20_fig3.jpg

http://www.clspectrum.com/content/archive/2011/May/images/CLS_May_A20_fig3.jpg

http://en.wikipedia.org/wiki/File:Keratoconus_contact_lens_size.jpg

http://en.wikipedia.org/wiki/File:Keratoconus_contact_lens_size.jpg


Image Source http://pacificu.edu/optometry/ce/courses/15167/images/Slide54.jpg

http://pacificu.edu/optometry/ce/courses/15167/images/Slide55.jpg

They are fitted by varying the sagittal

depth which in tun is done by varying

the diameter of the lenses. Mcguire

lenses are modified Soper lenses. They

have central vaulting to minimise central

bearing and peripheral cornea bears the

major pressure. Nicone and Rose-K

(figure2) designs have also been

developed. The Rose K lens design is a

fully flexible lens that works well on early

to advanced keratoconus patients.

Complex lens geometry, combined with

the enhanced material benefits of

Boston ES™, makes the Rose K lens

the good fit enhancing patient comfort

and visual acuity. Multiple parameters

make fitting the Rose K lens possible for

most keratoconic eyes. Hybrid lenses

have been developed which are hard in

the centre and encompassed by a soft

skirt. Soft or hybrid lenses do not

however prove effective for every

patient. Some patients also find good

vision correction and comfort with a

“piggyback” lens combination (figure 3),

in which gas permeable rigid lenses are

worn over soft lenses, both providing a

degree of vision correction Wave

Custom Designed Contact Lens is

topography based designed contact

lens. The corneal map is loaded into a

lens designing software. This software is

then used to design a lens specifically

for that cornea.

Boston Scleral lenses Prosthetic

device (BSLPD)

It is a fluid-ventilated gas-permeable

contact lens that rests entirely on the

sclera creating a fluid-filled space over

the diseased cornea. They are

sometimes prescribed for cases of

advanced or very irregular keratoconus;

these lenses cover a greater proportion

of the surface of the eye and hence can

offer improved stability and comfort.

BSLPD has been worn with all day

wearing comfort in many RGP lens

intolerant patients5. High cost prohibits

widespread usage.

Image Source http://allaboutdryeye.com/wpcontent/uploads/2011/11/scleral_lenses.png





http://allaboutdryeye.com/wp-content/uploads/2011/11/scleral_lenses.png

http://allaboutdryeye.com/wp-content/uploads/2011/11/scleral_lenses.png


The Boston Mini Scleral lens device

(rests on peripheral cornea) has been

recently developed for keratoconus

patients.

Refractive surgery

Laser in situ keratomileusis (LASIK) or

photorefractive keratectomy (PRK) is

contraindicated in these patients

because of a greater risk for scarring

and excessive thinning leading to

possible post-LASIK ectasia. Thorough

topographic evaluation should be done

to rule out keratoconus fruste or suspect

before considering these procedures.

For similar reasons, radial keratotomy

has also generally not been used for

keratoconic patient, unlike refractive

procedures; phototherapeutic

keratectomy (PTK) has been helpful for

some selected keratoconus patients to

reduce steepness of the cone and for

nodular subepithelial scars in patients

who have become contact lens

intolerant. The resultant flattening of the

cone makes contact lens fitting easier.

The key to the safety of the procedure is

that the very shallow ablation is not

intended to have a refractive effect6.

Corneal Collagen Cross Linking With

Riboflavin (C3-R)

Corneal collagen crosslinking with

riboflavin (C3-R) is the name given to

the treatment that combines the use of

riboflavin (vitamin B2) with ultraviolet

light for the treatment of keratoconus.

The riboflavin 0.1%eyedrops in 20%

dextran are activated by approximately

30 minutes illumination with UV-A

(370nm) light.

Fig 5 Source

http://www.optyco.co.uk/wpcontent/uploads/2012/07/crosslin

king.png

This treatment is applied to

deepithelised cornea. The currently

used UVA radiant exposure of 5.4

mJ/cm2 and the corresponding

irradiance of 3 mW/cm2 are below the

known damage thresholds of UVA for

the corneal endothelium, lens, and

retina (figure 4)7 8.Recently The KXLTM

System for Accelerated Cross-linking

achieves speed by increasing the UVA

power and reducing the exposure time (

30mW/cm2for 3 minutes), thereby

maintaining the same energy on the eye

as standard cross-linking while reducing

crosslinking time by an order of

magnitude. C3-R augments the collagen

cross-links within the stroma(figure 5)

and so recovers some of the cornea’s

http://www.optyco.co.uk/wpcontent/uploads/2012/07/crosslinking.png

http://www.optyco.co.uk/wpcontent/uploads/2012/07/crosslinking.png


mechanical strength8 .The treatment has

been shown to slow or arrest the

progression of keratoconus, and in

some cases even reverses it10,11,12

particularly when applied in combination

with intracorneal ring segments9, 10. In

these cases, C3-R treatments stabilize

keratoconus from getting worse as well

as help the Intacs reverse the

keratoconus steepening that had

already occurred up to the time of the

treatment. The need for penetrating

keratoplasty might then be significantly

reduced in keratoconus. Average

flattening in central ‘K’ value reported in

literature following C3R is 1.5 – 2 D

which is responsible for improved

contact lens fitting in many patients in

whom it was not possible preoperatively.

Intrastromal Corneal Ring Segments

A recent surgical alternative to corneal

transplant is the insertion of intrastromal

corneal ring segments (figure 6). These

inserts are designed to be placed at a

depth of approximately two-thirds the

corneal thickness and are surgically

inserted through a small radial incision

into a track created within the corneal

stroma. The use of a femtosecond laser

for Intacs channel creation seems as

effective as mechanical dissection12, 13.

They are oriented horizontally in the

cornea at 12 and 6 o’clock (figure 7).

They shorten the corneal arc length and

have a net effect of flattening the central

cornea. The amount of flattening is

determined by the insert’s thickness.

Rings are available in thicknesses of

0.250, 0.275, 0.300, 0.325 and 0.350

mm (recently 0.400 and 0.450 are also

available).

Fig

6 Source

http://www.harvardeye.com/procedures/images/intacs_orang

e_county.png

Source http://www.fda.gov/ucm/groups/fdagovpublic/docume

nts/image/ucm080963.gif

http://www.harvardeye.com/procedures/images/intacs_orange_county.png

http://www.harvardeye.com/procedures/images/intacs_orange_county.png

http://www.fda.gov/ucm/groups/fdagov-public/documents/image/ucm080963.gif

http://www.fda.gov/ucm/groups/fdagov-public/documents/image/ucm080963.gif


Intacs are indicated for contact lens

intolerant patients with early

keratoconus who have minimal central

stromal scarring. The two principal types

of intrastromal rings available Intacs and

Ferrara rings. Intacs are flatter and less

centrally placed than the Ferrara rings.

Intacs implantation is being increasingly

considered and shown effective in early

keratoconus case. Potential

complications of intrastromal rings

include accidental penetration through

to the anterior chamber when forming

the channel, post-operative infection of

the cornea, and migration or extrusion of

the segments. The rings offer a good

chance of vision improvement even in

otherwise hard to manage eyes and can

always be a good option before taking

up the patient for surgery14,15. Contact

lenses may be needed for keratoconus

patients who have INTACS inserts and

have a role in augmenting their vision.

Contact lens tolerance was restored in

over 80% of cases in a study. Rigid gas-

permeable or toric soft lenses can be

used.

.

Corneal Transplant

Penetrating keratoplasty (PK) has been

the gold standard surgery for

keratoconus patients with success rates

of more than 90%17. Approximately10%

to 25% of cases of keratoconus will

progress to a point where vision

correction is no longer possible, thinning

of the cornea becomes excessive, or

scarring as a result of contact lens wear

causes problems of its own, and a

corneal transplantation becomes

required1, 2. In this procedure, the

keratoconic cornea is prepared by

removing the central area of the cornea,

and a full-thickness corneal button is

sutured in its place. Usually trephines

between 8.0-8.5 mm are used.

Fleischer’s ring can be used as the limit

of the conical cornea. Depending on the

criteria used to assess the success rate,

this surgery is 90% to 95% successful

(figure8). Most of these patients who are

grafted for keratoconus are younger

than the majority who are grafted for

other reasons. Contact lenses are often

required after this procedure for best

visual rehabilitation. Recently the

Femtosecond Laser was approved for

performing Corneal Transplants (also

known as I.E.K or Intralase Enabled

Keratoplasty). An alternative is lamellar

keratoplasty, a partial corneal

transplant. The cornea is removed to the

depth of posterior stroma, and the donor

button is sutured in place. This

technique is technically difficult, and

visual acuity is inferior to that obtained

after penetrating keratoplasty.


Lamellar keratoplasty has recently been

almost replaced by an alternative highly

rewarding procedure of deep anterior

lamellar keratoplasty (DALK) 18, 19, 20. In

DALK, the patient’s corneal endothelium

is retained, giving some additional

structural integrity to the post-graft

cornea. The chance of a rejection

episode is greatly reduced. DALK thus

provides lower postoperative

complications, faster postoperative

recovery, fewer graft rejections and

similar visual outcomes compared to PK

(figure9). It is however a technically

demanding procedure. A rarely

performed but once tried procedure,

thermokeratoplasty involved placing a

hot ring (Holmium yag laser, 2100nm)

along the base of the cone to heat and

traumatize the cornea21, resulting in a

corneal scar which reduces the corneal

curvature, and allows a flatter contact

lens to be fitted. The disadvantages of

the procedure were a transitory corneal

haze, development of corneal scarring

and the fact that it does not preclude

future keratoplasty. Epikeratoplastyis

primarily suited for contact-lens-

intolerant patients in whom scarring has

not yet occurred22. In this procedure, the

central host epithelium is debrided, and

the donor cornea is sewn over the

keratoconic cornea. This is a rarely

performed procedure today as the

outcome is generally less favourable.

Phakic intraocular lens implantation

It can be used to correct high myopia

and associated astigmatism of selected

keratoconus patients. Anterior chamber

phakic intraocular lens have also been

combined with intacs with good

results23. The Intacs implantation is

followed by toric phakic intraocular lens

implantation to correct the residual

myopic and astigmatic refractive error24.

Patients who are extremely nearsighted

more than -10D might benefit from

phakic intraocular lenses. Currently

there are two types of these lenses

approved by the FDA –the Verisys and

the Visian ICL. These are implantable

contact lens has been approved by the

FDA for up to -20Diopters.

Keratophakia

Is an operation in which a partial-

thickness corneal tissue graft is inserted

into the cornea. The graft or 'lenticule'

helps to restore the cornea to a normal

thickness, and may also cause a

change in the corneal surface profile,

and so alter the cornea's refractive

power. Keratophakia was originally

conceived over fifty years ago by Dr

José Barraquer as a treatment for the

correction of hypermetropia (long-

sightedness). It has also been used as a


treatment for myopia, and more recently

for the management of keratoconus and

corneal ectasia. In these latter

conditions, the lenticule may be

designed primarily to restore the corneal

thickness to normal, rather than to

change the corneal surface profile.

Thus to conclude, a number of

treatments have been tried in

keratoconus. Newer modalities like

INTACS and C3R are helping to achieve

visual rehabilitation and delaying the

need for penetrating keratoplasty. RGP

contact lenses are the mainstay in the

rehabilitation of keratoconus patients.

This holds true even though the patient

may have undergone other procedures

like keratoplasty, INTACS and C3R.

Recently a new microwave procedure,

Keraflex KXL TM, holds the promise of

treating keratoconus while also

correcting the associated refractive

error25. The procedure is carried out in

two steps. In the first step, microwave

energy is applied to a ring-shaped area

of the cornea using an annular

electrode. The energy penetrates the

superficial stroma, inducing localized

shrinkage of the collagen fibers. Step

two entails a riboflavin-UV cross-linking

procedure specifically within the

microwaved area. The center and

periphery of the cornea are masked

using UV-blocking corneal shields.

Riboflavin 0.1% is applied to the

annulus following removal of the

epithelium, and UV irradiation is carried

out for 30 minutes. “UV cross-linking is

used to improve the stability and extend

the lifetime of the flattening that Keraflex

induces. The two procedures work

synergistically.

References

1. Duke-Elder S, Leigh AG: System of

ophthalmology. Diseases of the outer

eye, Vol 8. London, Henry Kimpton,

1965, pp 964–976

2. Clinical and epidemiological features

of kerato-conus: genetic and external

factors in the pathogenesis of the

disease. Acta Ophthalmol 178(Suppl):5–

64, 1986

3. Iwaszkiewicz E: Keratoconus.

Coexisting diseases and theories on its

etiology and pathogenesis. Klin Oczna

91:210–211, 1989

4. Rabinowitz YS, Keratoconus. Survey

Ophthalmol 1998; 42:297– 319.

5. Visser ES, Visser R, van Lier HJ,

Otten HM. Modern scleral lenses part I:

clinical features.Eye Contact Lens. 2007

Jan;33(1):13-20

6.Lombardi M, Abbondanza M.

Asymmetric radial keratotomy for the

correction of keratoconus. J Refract

Surg. 1997 May-Jun; 13(3):302-7.

7. Spoerl, Eberhard , Mrochen, Michael ,

Sliney, David ,Trokel, Stephen , Seiler,

Theo.Safety of UVA-Riboflavin Cross-

Linking of the Cornea. Cornea.

26(4):385-389, May 2007.

8. Riboflavin/ultraviolet-a-induced

collagen crosslinking for the treatment of

keratoconus. Am J Ophthalmol. 2003.


9. Chan CC, Sharma M, Wachler BS.

Effect of inferior-segment Intacs with

and without C3-R on keratoconus. J

Cataract Refract Surg. 2007 Jan;

33(1):75-80.

10. Parasurgical therapy for

keratoconus by riboflavin-ultraviolet type

A rays induced cross-linking of corneal

collagen: preliminary refractive results in

an Italian study. J Cataract Refract

Surg. 2006.PMID: 16765803

11. Raiskup-Wolf F, Hoyer A, Spoerl E,

Pillunat LE. Collagen crosslinking with

riboflavin and ultraviolet-A light in

keratoconus: long-term results. J

Cataract Refract Surg. 2008

May;34(5):796-801.

12. Kymionis G, Portaliou D. Corneal

crosslinking with riboflavin and UVA for

the treatment of keratoconus.J Cataract

Refract Surg. 2007 Jul;33(7):1143-4.

13.Zare MA, Hashemi H, Salari MR

Intracorneal ring segment implantation

for the management of keratoconus:

safety and efficacy J Cataract Refract

Surg. 2007 Nov;33(11):1886-91

14. Colin J, Malet FJ. Intacs for the

correction of keratoconus: two-year

follow-up.J Cataract Refract Surg. 2007

Jan;33(1):69-74.

15. Kymionis GD, Siganos CS, Tsiklis

NS, Anastasakis A, Yoo SH, Pallikaris

AI, Astyrakakis N, Pallikaris IG. Long-

term follow-up of Intacs in keratoconus.

Am J Ophthalmol.2007 Feb; 143(2):

236-244.


Rise of intraocular pressure after intravitreal injection triamcinolone acetonide

Manish Joshi, MS; Mona Vaish, MS

Correspondence Author Dr Manish Joshi Vitreo-Retina & Phaco Surgeon Jodhpur Rajasthan

Abstract Purpose: To report the occurrences of rapid increases in intraocular (IOP) after single intravitreal Triamcinolone injection. Design: Observational case series: review of three cases (eyes) seen by author Results: In all three cases, a significant rise in IOP occurred within two weeks of intravitreal Triamcinolone injection for retinal disorders. In one patient, a white material was found in the angle on gonioscopy. All three cases required surgical intervention to reduce the IOP. Conclusions: Considering the early rapid rise in IOP in these three cases, we suggest that ophthalmologists should closely monitor patients after intravitreal triamcinolone injections for the development of acute glaucoma.

Introduction

Intravitreal triamcinolone acetonide (IVTA) has been widely used in various intraocular inflammatory, exudative, vascular, oedematous conditions. Increasing use of IVTA has caused investigation of rise of intraocular pressure post injection which can be from just immediate two days after injection. Although 4–8 mg doses drawn undiluted from the commercially available vial are commonly used, some ophthalmologists remove the diluent From the medication and concentrate it into a 20–25 mg dose in a small volume.

One of the side effects of intravitreal

steroid injection is rise of intraocular

pressure.Im l et al [1] reported in their

study a significant IOP rise in eyes after

a single intravitreal injection of 4 mg of

triamcinolone within 1 month of

injection. In this study, the most frequent

time point that required IOP treatment

was at 2-week post injection, suggesting

that early and frequent monitoring of

IOP should be considered. Eyes which

are at increased risk for post‐IVTA

elevation of IOP include those with a

baseline IOP >16 mm Hg, younger age,

repeated IVTA injections, pre‐existing

glaucoma, and an increased IOP

following provocative testing with a 400

μg test dose [ rhee et al 2 ].

Here we are reporting three cases of

acute rise of intraocular pressure after

intravitreal injection of triamcinolone.


Case 1 & 2

A 24 year old gentleman presented with

blurred vision, heaviness in head and

eye pain. He was taking treatment of

uveitis at an eye care centre. He was

given intravitreal injection of

Triamcinolone 4 mg there 1 week back.

His IOP on presentation to us was 29

mm Hg in right eye and 30 mm Hg in left

eye. He was on brimonidine and timolol

combination drop.

Anterior segment findings: both eyes

mild corneal edema with post

subcapsular cataract. Indirect

ophthalmoscopy revealed white

particulate material in inferior vitreous.

Gonioscopy showed open angles with

no particles. He was started with

dorzolamide eye drops and tab diamox

250 mg bid in sustained dose

formulation for two days. He came back

after 1 wk and his IOP was 40 mm Hg in

both eyes. Injection maniitol was given

and bimatoprost drops was added in

topical regimen and tablet diamox was

prescribed for 1 week. Two weeks later

his IOP still in range of 30 to 35 mm Hg

in both eyes. On basis of this we

decided to go for anti glaucoma surgery

in right eye. Surgery was uneventful and

post trabeculectomy IOP in right eye

was 14 mm Hg on 1st follow-up. As he

was not willing for surgery in left eye he

was on medical management for left

eye. One month follow-up IOP right eye

16 mm Hg left eye IOP 30 mm Hg.

Visual field left eye glaucomatous field

defect, and fundus left eye showing

pallor with 0.7 cupping vertically. Right

eye 0.4 cupping.

Left eye trabeculectomy done but after

significant damage. So it is important to

do follow up very regularly after IVTA

and take decision in time.

Visual field and fundus photo of patient


Right eye


Left Eye


Case 3:

A 47 year old male presented with pain

left eye with redness and blurred vision.

Known case of diabetic retinopathy, with

history of left eye intravitreal injection

triamcinolone 4 mg given 15 days back.

Left eye anterior segment findings

showing corneal edema, IOP 41 mm

Hg, pupil dilated, immature cataract, and

gonioscopy showing particles in angles.

Fundus examination left eye showing

CSME and triamcinolone particles.

Pt was prescribed brimonidine and

timolol combination drops, dorzolamide

eye drops and travoprost eye drops.

IOP was down to 29 mm Hg and not

becoming lower for almost one month.

Optic disc getting compromised with 0.7

C/D ratios. Other eye was having only 3

meter finger counting vision. We

considered left eye anti glaucoma

surgery. Left eye trabeculectomy was

done. Post trabeculectomy IOP 10 mm

Hg, maintained for long time in normal

range.


Observing the above mentioned cases,

young patients are more prone for high IOP

after intravitreal injection triamcinolone.

Surgical intervention should be considered

early if IOP is not controlled with

conservative management and optic disc is

getting compromised. As truly said more

eyes are lost due to avoidance of surgery

rather than because of surgery in glaucoma.

References:

1) J Glaucoma. 2008 Mar;17(2):128-32.A

prospective study of early intraocular

pressure changes after a single intravitreal

triamcinolone injection.

2) Rhee D J, Peck R E, Belmont J. et al

Intraocular pressure alterations following

intravitreal triamcinolone acetonide. Br J

Ophthalmol 2006.

http://www.ncbi.nlm.nih.gov/pubmed/18344759


Orbital implants: Our experience Seema Laad, DOMS, Amit Mohan, MS; Sudhir Singh

Correspondence Author Dr Seema Laad Global hospital institute of ophthalmology Abu Road, Sirohi, Rajasthan India 307501

Aim: To compare sizing methods and suturing techniques in 2 different groups. Their effect on the results

of the procedure.

Abstract: This is a retrospective study conducted between Nov 2009 to Feb 2012 at our institution. Where

in 16 cases of evisceration / enucleation for various causes done & lost orbital volume had been replaced

by orbital implants

Result: Zero rate of implant exposure in group with proper sizing and meticulous suturing.

Introduction:

Orbital Implants replaces volume lost

by enucleated /eviscerated eye, impart

motility to the prosthesis and

maintains cosmetic symmetry to the

fellow eye.

Types:

1. Non Integrated: Do not allow

direct or indirect integration with orbital

structures or prosthesis.

Cheaper well tolerated and have fewer

complications. E.g. Silicon & PMMA

Orbital Implant.

2. Semi integrated: Have direct

integration with Orbital structures but

not with prosthesis.

3. Integrated: Gets incorporated

into Orbital tissues & have attachment

with prosthesis through motility pegs.

In our study we used only Silicone

implants varying in diameter from 14

to 20 mm.

Material & Methods

This is a retrospective study

conducted between Nov 2009 Feb

2012 at our institution. Where in 16

cases of evisceration / enucleation for

various causes done & lost orbital

volume had been replaced by orbital

implants

Patient Selection:

1. Cases of panopthalmitis: 8

2. Anterior staphyloma & traumatic

perforation: 7

3. Old pthysical: 1

Age group:

From 10 years to 80 years


Sex Male 7 Female 9 Eye Right Eye 9 Left Eye 7 Demography: Sirohi, Pali, Jalore district.

Sizing of implant & suturing techniques

1. In group A (previous group) we

have used empirical methods of sizing.

After implanting we’ve sutured sclera&

conjunctiva in same direction.

2. In group B we used AXL of fellow

eye (AXL-2mm) to calculate diameter of

implant needed suturing we have closed

sclera with interrupted 6-0 vicryl suture

vertically and conjunctiva horizontally.

Group A

Sr.

No.

Age &

Sex RE/LE

Diagnosis

Follow up

4 weeks 6 weeks

1. 41/M LE Traumatic / Old Perforation Healthy

wound

Nicely healed

wound

2. 60/F RE Non responding perforated

corneal ulcer -do- Good

3. 18/M LE Injuries in prev. ant. Staphyloma -do- Healed wound

better prosthesis

4. 55/M LE Panophthalmitis -do- -do-

5. 50/M LE Panophthalmitis -do- Healed wound

better prosthesis

6. 30/F RE Ant. Staphyloma -do- Good cosmesis

7. 55/F RE Panophthalmitis

Wound

gaping &

Implant

exposure

Good results after

resuturing

8. 27/F RE Old Pthysical eye Implant

exposure

Has to be

explanted


Group – B

Sr.

No.

Age &

Sex RE/LE

Diagnosis

Follow up

4 weeks 6 weeks

1. 10/F LE Total Ant.

Staphyloma

Healthy wound

(comfortable

patients)

Nicely healed wound & nicely

accepted implant

(Artificial eye given

successfully)

2. 40/M LE Panophthalmitis -do- Nicely healed wound &

nicely accepted implant

3. 70/M RE Panophthalmitis -do- (Artificial eye given

successfully)

4. 45/F RE Panophthalmitis -do- Nicely healed wound &


5. 80/F LE

Panophthalmitis Healthy wound

(comfortable

patients)


successfully)

6. 60/F RE Total melting of

cornea

Healthy wound

(comfortable

patients)

Nicely healed wound &


7. 70/F RE Sloughed Corneal

ulcer

Healthy wound

(comfortable

patients)


successfully)

8. 20/M LE Traumatic

perforation

Healthy wound

(comfortable

patients)

Nicely healed wound &



Results:

Implant exposure found in 2 cases of

inpatients in group A. In one patient we

had explanted the sphere and in

another resuturing settled the implant.

Discussion:

It is found that sizing the silicone

implant using axial length of fellow eye

gives better result and less implant

exposure than empirical methods of

sizing. Suturing the conjunctiva &

sclera in criss cross direction than

same direction gives additional strength

to the wound resulting in decrease in

complication rate from 11.5 % to 0 %.

Sara A Kaltreider1 recommends a

preoperative A-scan and placement of

an implant with an appropriate diameter

to replace 70% to 80 % of the volume

removed. Sized spheres may be used to

reconfirm the implant diameter, but are

not recommended as a precise

guideline.

Too often, a fear of implant exposure

limits the diameter of implant used 2, 3.

Our study demonstrates that the A-scan

is a valuable tool in estimating an

appropriate sphere diameter (group-2)

and would have prevented oversized

prosthetics and exposure.

Conclusion:

Proper sizing of implant is crucial and

using axial length of fellow eye to

decide it and suturing in crisscross

direction provides additional strength to

wound .This reduced complication rate

to 0 %. Hence proper sizing and

meticulous wound closure minimized

the risk of implant exposure.

References

1. Sara A Kaltreider. Predicting the

ideal implant size , Ophthalmic

Plastic & Reconstruction

Surgeries , 1999 Vol. 15 , no.1

P.P. 37-43

2. Kaltreider SA, Newman.

Prevention and management of

complication associated with the

implant. Ophthalmic Plastic

Reconstruction Surgeries 1997;

vol.13 : 18-20

3. Narnery W Heinz GW, Bonnin JM

et al , exposure rate of implant in

an ophthalmic socket and

comparison with silicon sphere

implants .Ophthalmic Plastic

Reconstruction surgeries 1993;

vol. 9(2); 96-104

4. Implants in ophthalmology AIOS

CME Series (No.-15); Orbital

Implants:current practices and

recent trends -Dr Santosh G

Honavar,pp40-44


Current biologic therapies in management of uveitis Pankaj Dhaka, MD, Amarendra Samal, MD

Corresponding Author Dr.Pankaj Dhaka M.N Hospital and Research Centre,Bikaner Pin-334001, Phone no. 0151 -2230347, 9314806609 [email protected]

Introduction

The biologic agents are a group of

therapeutic agents targeting mediators

of inflammation including soluble factors

(e.g., cytokines, chemokines),

Cytokine/chemokine receptors, and

immune cell surface markers, have

been increasingly used in the treatment

of ocular inflammatory diseases. As the

pathogenic mechanisms underlying

uveitis and ocular inflammation continue

to be uncovered, the indications for

specific immunomodulatory agents such

as the biologic agents will likely continue

to expand.

Clinical studies evaluating the aqueous,

vitreous, and serum factors of patients

have also provided valuable information

about the various soluble and cellular

mediators of inflammation in specific

disease entities. Several agents have

been designed to antagonize the action

of tumor necrosis factor- -

alpha), and have been used for the

treatment of ocular inflammatory

diseases. These agents include

infliximab, adalimumab, and etanercept.

The interleukin-2 (IL-2) receptor

antagonist daclizumab has also been

utilized for the successful treatment of

endogenous uveitis. Several other

biologic agents, including alemtuzumab

and anakinra, have also been reported

in some series for ocular inflammatory

diseases. This chapter will focus on the

pharmacology, indications,

contraindications, and side-effects

associated with the TNF-alpha

-2 receptor antagonist

daclizumab, and other biologic agents,

which have been used for the treatment

of uveitis.

Tumor Necrosis Factor-Alpha

Antagonists

Histological studies have demonstrated

a uveal infiltration of macrophages and

T cells, resulting in an inflammatory

cytokine and cellular milieu with

subsequent loss of tissue architecture

and function.

TNF-

inflammatory cytokine involved in EAU1

and its presence has also been detected

in the aqueous humor of uveitis

patients.2 Thus, TNF-

an attractive target for therapy.

mailto:[email protected]


Three TNF- – infliximab,

adalimumab, and etanercept – have

been approved by the Food and Drug

Administration (FDA) for systemic

autoimmune indications, and all of these

agents have been used for the

treatment of ocular inflammatory

diseases.

Infliximab

Infliximab is a mouse-derived chimeric

monoclonal antibody, which antagonizes

TNF-alpha. Its inhibition is mediated via

interference of TNF binding to two

known receptors – TNFr1, which binds

to soluble TNF, and TNFr2, which binds

to membrane-bound TNF. A mean

terminal half-life between 9 and 12 days

has been reported at doses between 5

and 20 mg/kg. Following its

administration, infliximab has been

detected in most patient sera from 8 to

12 weeks after infusion.3 Infliximab is

administered via intravenous infusion in

doses ranging from 3 to 20 mg/kg for

both systemic and ophthalmic

indications.

Most studies in systemic autoimmune

diseases have reported dosing intervals

varying from 4- to 8-week dosing

intervals. In the ophthalmic literature,

doses vary from 3 to 10 mg/kg/dose.

However, higher doses of infliximab

have been reported for the treatment of

refractory pediatric uveitis.

Ophthalmic Indications For Infliximab

The efficacy of infliximab has been

evaluated for a number of ophthalmic

inflammatory diseases. A paucity of

prospective clinical trial data is available

for infliximab use in ocular disease;

however, its efficacy has been

demonstrated for select conditions in a

number of case reports and small case

series.

Conditions for which infliximab has

been used include Behçet’s disease-

associated panuveitis 4 and retinal

vasculitis, juvenile idiopathic arthritis

(JIA)-associated uveitis, human

leukocyte antigen (HLA)-B27-

associated uveitis, scleritis, and

peripheral ulcerative keratitis. Suhler et

al.5 reported their experience in a

prospective, phase II, open-label study

of infliximab for refractory autoimmune

uveitis. The various diagnoses from their

series included pars planitis,

sarcoidosis, Crohn’s disease, and

Behçet’s disease. Infliximab was

administered in 3- or 5-mg/kg doses via

intravenous infusion at weeks 0, 2, and

6 with clinical assessment at week-10

follow-up. If the patient is stable, the

regimen is then reduced to every 8

weeks. If the patient does not respond,

however, the dose can be increased up

to 10mg/kg and the interval decreased

to every 4 weeks. A total of 18 (78%) of

23 patients treated with acuity, two-step

decrease in intraocular inflammation,

ability to taper immunosuppression, or

decrease in inflammatory signs by

optical coherence tomography or


fluorescein angiography). Of the 14

patients maintained on infliximab

therapy for a full year, 7 (50%) patients

maintained successful grades. However,

the number of adverse events observed

in their series was concerning and

included congestive heart failure,

pulmonary embolus, lupus-like reaction,

and vitreous hemorrhage in 2 patients.

Antinuclear antibodies were also

identified in 15 (75%) of 20 patients who

received more than three infusions.

Based on this study, the authors

recommended additional long-term

studies to assess further the safety and

efficacy of infliximab for ophthalmic

inflammatory diseases.

Niccoli et al.6 prospectively evaluated

the efficacy of infliximab for Behçet’s

disease-associated posterior uveitis,

which had failed therapy with at least

one immunosuppressive medication

prior to enrollment. In their series of 12

Behçet’s disease patients, results were

encouraging, with 9 (75%) patients

demonstrating a complete remission at

12-month follow-up. The total number of

ocular attacks across all patients

decreased from 40 in the year prior to

infliximab therapy to five attacks in the

year following cessation of infliximab.6

Abu El-Asrar et al.7 reported their

experience with infliximab for refractory

Behçet’s disease-associated uveitis in 6

patients who had each failed at least on

other immunosuppressive medication. In

this small prospective trial, patients were

treated with infliximab at a dosage of 5

mg/kg infusions at weeks 0, 2, 6, and

every 8 weeks thereafter. All 6 patients

achieved remission by the 2-month time

period and 3 patients remained relapse

free during the follow-up period (range,

16–36 months, mean, 23.6 months).

From their study, it appeared that long-

term remission could be maintained by

repeated infusions.7 Tugal-Tutkun et al.8

reported encouraging results from a

prospective trial of infliximab for

Behçet’s disease-associated uveitis

resistant to azathioprine, cyclosporine,

and corticosteroids. Thirteen male

patients were treated with infliximab (5

mg/kg) at weeks 0, 2, 6, and 14 in this

trial, and uveitis exacerbations were

documented during an infusion period

(weeks 0–22) and observation period

(weeks 23–54). One patient

demonstrated a sustained remission

with no ocular attacks during both

infusion and observation periods, and 4

patients (30.8%) demonstrated

remission during the infusion period.

During the observation period, 36

attacks of uveitis in 12 patients were

documented, and the treatment protocol

was amended to allow the reinstitution

of infliximab infusions because of the

severity of sight-threatening ocular

attacks. Three of the 12 patients

required the reinstitution of infliximab

therapy. Of note, the mean daily

corticosteroid requirement and mean

number of uveitis attacks were lower in

the infusion period compared to the

observation period (weeks 23–54). No

serious adverse events were reported in

this study. Accorinti et al.9 also

described their experience with


infliximab in 12 patients with Behçet’s

disease-associated ocular inflammatory

disease. Of the 12 patients in their

series, 11 patients demonstrated a

decrease in the number of ocular

relapses per month in the follow-up

period. In addition, all 11 patients on

corticosteroids prior to therapy were

able to decrease their daily

corticosteroid requirement. Significant

side-effects observed included

tuberculosis, herpetic keratitis, severe

non-ocular herpetic infection, and

recurrent urinary tract infections. Two of

these 4 patients experienced uveitic

exacerbations when infliximab infusions

were delayed because of these side-

effects. Thus, while infliximab appeared

to benefit some patients, infectious

complications possibly related to the

medication in this series was

concerning.9

Other retrospective studies have

supported the efficacy of infliximab for

Behçet’s disease-associated panuveitis

and retinal vasculitis.10-14 Outcome

measures have differed between these

retrospective case series, making

comparisons between studies difficult.

Improvement in visual acuity, decreased

intraocular inflammation, and reduction

of daily corticosteroid requirements have

been reported. However, the

development of ocular and systemic

tuberculosis in 1 patient14 and an

episode of thoracic herpes zoster in

another patient13 required systemic

antimicrobial therapy.

Besides Behçet’s disease-associated

uveitis, sarcoid-associated uveitis has

also been treated successfully with

infliximab therapy. Cruz et al.15 reported

the successful use of infliximab (3 mg/kg

infusions given at day 0, week 2, and

week 6) in 2 patients with retinal

vasculitis and multisystem sarcoidosis.

Other patients with sarcoid associated

uveitis have also experienced

therapeutic benefit with infliximab.5, 12, 16

Other retrospective case series have

reported a therapeutic benefit of

infliximab for JIA-associated uveitis.17, 18

Richards et al.18 reported improved

control of intraocular inflammation in 6

of 6 patients treated with infliximab and

maintenance low-dose

immunosuppression. This study

suggested a role for infliximab as

adjunctive therapy in JIA associated

uveitis.

Rajaraman et al 17 also evaluated

infliximab for pediatric uveitis due to a

variety of etiologies, including JIA-

associated uveitis (3 patients), pars

planitis (1 patient), retinal vasculitis (1

patient), and idiopathic uveitis (1

patient). All patients demonstrated

improvements in intraocular

inflammation while on infliximab and 5 of

6 patients were able to wean completely

off corticosteroid therapy by the end of

the study period evaluated (mean

follow-up 48 weeks). Vitreous

hemorrhage developed in 1 patient

while another patient experienced a

transient upper respiratory infusion

reaction. The use of high-dose infliximab

(10–20 mg/kg) for pediatric patients with


refractory uveitis was reported by Kahn

et al. In 17 patients evaluated, 13

individuals demonstrated quiescence of

inflammation following two infliximab

infusions, and the remaining 4 patients

achieved this outcome after three to

seven infusions. All patients receiving

oral steroids before therapy

discontinued steroid treatment over a

period varying between 2 weeks and 2

years, and 15 of 17 patients were

tapered off topical corticosteroids. One

prospective non-comparative case

series of infliximab as monotherapy for

HLA-B27-associated anterior uveitis

demonstrated a rapid decrease in

anterior-chamber inflammation in 7

patients treated with infliximab (10

mg/kg) infusions. However, 1 patient

required a second infusion after 3 weeks

because of a flare-up, and median time

to relapse in the 4 patients was 5+/-

months. Besides these reports of its

efficacy for intraocular inflammation,

infliximab has also been successfully

utilized for the treatment of scleritis and

peripheral ulcerative keratitis due to a

variety of etiologies, including

Wegener’s granulomatosis, RA, and

relapsing polychondritis. The efficacy of

infliximab for posterior scleritis in a

pediatric patient has also been reported.

Infliximab Toxicity

Herpetic keratitis, 9 vitreous

hemorrhage, 5, 17 optic neuritis, and

ocular tuberculosis 14 have been

reported during infliximab therapy. A

number of systemic complications have

been associated with infliximab therapy,

the most concerning of which include

reactivation of latent tuberculosis,

exacerbation of congestive heart failure,

unmasking of demyelinating disease,

the development of autoantibody

formation (i.e., antinuclear antibodies,

anti-dsDNA antibodies), and the

formation of anti-infliximab antibodies.

Severe hepatotoxicity complicating

infliximab therapy has also been

reported both with and without

concomitant use of other medications

with known hepatotoxicity.

Fluorescein angiography of patient with

idiopathic retinal vasculitis and a history of

bilateral, consecutive branch retinal vein


occlusions (A) Segmental hyper fluorescence of

inferotemporal arcade (B). Following infliximab

therapy, most retinal hemorrhages have

resolved (B).

Adalimumab

Adalimumab is a fully human, anti-TNF-

blocks the interaction of TNF-

p55 and p75 cell surface receptors.

Adalimumab is typically administered as

a 20 or 40 mg dose via subcutaneous

injection either weekly or every other

week. The subcutaneous route of

administration may be favorable to

infliximab, which requires an

intravenous infusion. The terminal half-

life of adalimumab ranges from 15 to 19

days.

Ophthalmic Indications Of

Adalimumab

The efficacy of adalimumab for uveitis

has been reported in several

retrospective series for adults and

pediatric patients. Its efficacy for

Behçet’s disease-associated uveitis was

reported in two retrospective reports.19,

20 Mushtaq et al. reported 3 patients with

Behçet’s disease associated uveitis who

were successfully switched from

infliximab to adalimumab while in clinical

remission. Follow-up was variable in this

series, varying from 11 to 24 months;

however, intraocular inflammation

remained well controlled during the

follow-up period. Administration of

adalimumab for severe Vogt–Koyanagi–

Harada syndrome allowed the

successful tapering of corticosteroid and

cyclosporine in one case report.21

Vazquez-Cobian et al.22 reported the

successful treatment of pediatric uveitis

(9 JIA-associated and 5 idiopathic) with

adalimumab. In their series,

adalimumab was well tolerated with no

reports of serious adverse events. A

total of 21 of 26 (80%) eyes with

inflammation at baseline experienced a

decrease in intraocular inflammation

while 4 (15%) eyes remained stable and

1 (4%) eye worsened. A decrease in

topical corticosteroid use was observed

in 11 of 14 patients (79%) while 4 of 14

patients (29%) completely discontinued

topical medications. In addition, other

corticosteroid-sparing medications were

decreased in a number of patients

during the period of adalimumab

therapy.

Another retrospective series by Biester

et al.23 described the efficacy of

adalimumab for uveitis in 18 pediatric

patients. Seventeen patients were JIA-

associated while 1 was idiopathic.

Ophthalmic efficacy was demonstrated

in 16 of 18 patients (88%). Adalimumab

also appeared effective or mildly

effective for JIA-associated arthritis in

13 of 16 patients (81%) during the

follow-up period.

Herpes simplex keratitis has been

reported in one 5-year-old female

patient treated with adalimumab for

pauciarticular JIA-associated uveitis.23

Injection site reactions appear to be the

most commonly reported adverse event

and occur in up to 10% of patients


treated. Elevation of liver enzymes

requiring cessation of therapy has also

been reported.

Etanercept

The third FDA-approved TNF antagonist

is etanercept, which differs structurally

from the monoclonal antibody structure

of infliximab and adalimumab.

Etanercept is a recombinant TNF-alpha

constant (Fc) portion of human IgG1

and two copies of the extracellular

ligand-binding portion of TNF receptor

p75. Etanercept is given via

subcutaneous injection at a dose of 25–

50 mg twice weekly. Its elimination half-

life is 102 hours following a single

subcutaneous dose of 25 mg.24

Ophthalmic Indications Of Etanercept

Smith et al.25 reported the results of a

randomized, controlled, masked trial of

etanercept for JIA-associated anterior

uveitis. In their study, no apparent

difference in anterior-segment

inflammation was observed between

etanercept and placebo.26

Another prospective study of etanercept

for pediatric uveitis, including 7 juvenile

RA patients, reported a decrease in

anterior-chamber inflammation in 10 of

16 affected eyes (63%).27 Patients in

this study were treated with 0.4 mg/ kg

subcutaneous injection twice weekly for

12 weeks, and the dose was increased

to 25 mg twice weekly for patients with

an incomplete response. While the

majority of eyes experienced an

improvement in anterior-chamber

cellular reaction at 12 weeks, 7

responses were incomplete at 3-month

follow-up, and no further improvement

was demonstrated after 6 months of

therapy. Mild injection reactions were

observed, but no other significant

adverse events were reported in their

series. The successful use of etanercept

for sight-threatening scleritis and sterile

corneal ulceration has also been

previously reported.28 A retrospective

study by Guignard et al.29 evaluated the

efficacy of anti-TNF agents for the

prevention of uveitic flares. Their study

found a decrease in the risk of a uveitic

exacerbation in ankylosing spondylitis

patients treated with monoclonal

antibodies (i.e., infliximab, adalimumab)

targeting TNF-alpha, but no such benefit

was observed in patients treated with

etanercept. A retrospective comparison

of etanercept and infliximab for the

treatment of uveitis by Galor et al.30 was

consistent with these findings. In their

report, 17 of 18 (94%) patients on

infliximab showed a reduction in

intraocular inflammation at their final

follow-up, whereas 0 of 4 patients on

etanercept experienced a reduction in

intraocular inflammation.

Findings from questionnaires from

pediatric rheumatologists regarding the

differential efficacy of the TNF-alpha

-associated uveitis

therapy and the prevention of uveitic

exacerbations have reported greater


efficacy of infliximab when compared to

etanercept.31 In addition, although

arthritis appeared to respond to therapy

in 87% of patients, etanercept did not

appear to influence the frequency or

severity of uveitis episodes.32

Etanercept Toxicity

New-onset uveitis and acute

exacerbations of ocular inflammatory

disease (i.e., scleritis, uveitis, and

myositis) have been observed in

patients treated with etanercept.

Reports of optic neuritis and tuberculous

pan uveitis have also been reported.

The most commonly observed systemic

adverse effects in both children and

adults have been injection site

reactions, infection, headache, rhinitis,

and dizziness.24 Serious infections and

tuberculosis have been reported in

patients receiving etanercept in post

marketing surveillance.

Interleukin-2 Receptor Antagonists

Daclizumab

Daclizumab is a humanized monoclonal

recombinant IgG1 antibody targeting

Tac, a 55-kDa IL-2 alpha

subunit expressed by most T, B, and

natural killer (NK) cells following

activation by interaction with an antigen

or with IL-2. The IL-2 receptor (IL-2R)

system is a lymphokine receptor system

composed of three subunits (alpha, beta

and gamma) and plays a central role in

the induction of the immune response.

IL-2 binding to its receptor system

facilitates antibody formation, cell-

mediated immune responses, and NK

cell responses.

The association of the Tac subunit with

IL- ts forms a

high-affinity IL-2R complex, which is a

critical step in the activation of all T

cells, which are major contributors to

autoimmune disease and allograft

rejection. Doses of daclizumab 1

mg/kg in 2–4-week intervals have been

reported in published studies to date for

ocular inflammatory diseases, as 6-

week intervals led to uveitis

recurrences.

Doses of 2 mg/kg given in 4–5-week

intervals via intravenous infusion or

subcutaneous injection have been

utilized for maintenance

immunosuppression in a number of

patients.33

Ophthalmic Indications Ofdaclizumab

In the initial nonrandomized, open-label

pilot study of daclizumab for uveitis,

intravenous daclizumab therapy in up to

4-week intervals allowed the successful

tapering of immunosuppressive

medication (i.e., corticosteroids,

cyclosporine) in 8 of 10 patients enrolled

during the first 8 weeks of therapy. Of

note, daclizumab prevented the


expression of sight-threatening

inflammatory disease in these patients

treated over a 12-month follow-up

period.

Uveitic syndromes treated with

daclizumab included sarcoidosis, Vogt–

Koyanagi–Harada’s disease, idiopathic

intermediate uveitis, idiopathic

panuveitis, and multifocal choroiditis.26 A

longer-term (>4-year) phase I/II

interventional study of intravenous

daclizumab and a short-term phase II

study using subcutaneous daclizumab

further supported the efficacy of

daclizumab for the long term control of

uveitis. Of the 10 patients enrolled in

this study, 7 patients were able to taper

off all other immunosuppressive

medications and were maintained

exclusively on daclizumab for over 4

years.33 In the long-term study, a dosing

interval of 6 weeks resulted in recurrent

uveitis, whereas 2–4-week intervals did

not. In the short-term phase II study

evaluating the preliminary safety and

activity of subcutaneous daclizumab, 4

of 5 patients enrolled met their primary

study endpoint for success by 12 weeks

of therapy (i.e., 50% reduction in

immunosuppressive medication and

maintenance of visual acuity within 5

letters), and all 5 patients met this

endpoint by week 26. None of the

patients in the long-term study stopped

daclizumab due to adverse events

attributable to daclizumab. In a

randomized, double-masked, placebo-

controlled trial evaluating the efficacy of

daclizumab for Behçet’s disease, there

was no suggestion that daclizumab was

beneficial in comparison with placebo.

Specifically, efficacy outcomes (i.e.,

number of ocular attacks per year,

visual acuity change from baseline, and

immunosuppressive medication load)

were comparable between the

daclizumab and placebo arms.34 Several

other retrospective studies have

reported the use of daclizumab for a

variety of ocular inflammatory diseases

in both children and adults.35,36

Papaliodis et al.36 described the use of

daclizumab for 14 patients with a variety

of inflammatory conditions, including

scleritis, ocular cicatricial pemphigoid,

and panuveitis. An improvement in

visual acuity was seen in 12 of 27 eyes

(44%) and in 5 of 14 (36%) patients.

Intraocular inflammation improved in 16

of 27 eyes (59%), remained stable in 3

of 27 (11%) eyes, and worsened in 8 of

27 (30%) eyes. A decrease in ocular

inflammation was observed in 59% of

eyes in their series. Efficacy of

daclizumab has also been observed for

birdshot retino-choroidopathy, leading to

improvements in visual acuity and

resolution in vitreous inflammation in the

majority of patients treated. 37 Gallagher

et al.35 described the use of biologic

response modifier therapy in 23

pediatric patients with uveitis: 5 patients

in this series were treated with

daclizumab. Conditions treated with

daclizumab in this series included

sarcoidosis, panuveitis, keratouveitis,

and uveitis. Of these 5 patients, 4 of 10

eyes demonstrated improvements in

visual acuity and 8 of 10 eyes showed


improvements in ocular inflammatory

grade.

Fluorescein angiogram of patient with

birdshot retino-choroidopathy and

history of bilateral, recurrent, cystoid

macular edema despite multiple

partiocular corticosteroid injections.

Following repeat sub-tenon

triamcinolone and monthly daclizumab

infusions at a dosage of 2 mg/kg, the

cystoid macular edema has resolved

without recurrence.

Daclizumab Toxicity

No known ocular toxicities have been

reported in patients on daclizumab

therapy. No difference in serious

infectious complications or cancer has

been observed when comparing

patients receiving daclizumab or

placebo.

Other Biologic Agents

Other biologic agents, which have been

used in several retrospective series and

case reports for the treatment of uveitis,

include anakinra, alemtuzumab, and

interferon- -alpha).

Rituximab, an anti-CD20 monoclonal

antibody targeting B cells, has been

reported for the treatment of primary

intraocular lymphoma.

Anakinra , a is recombinant human IL-1

receptor antagonist that binds to IL-1

type 1 receptors, down-regulating the

proinflammatory effects of IL-1. Its

efficacy for the treatment of ocular

inflammation has been demonstrated in

murine models of uveitis.38 Recently,

several reports have described the

efficacy of anakinra for the treatment of

specific uveitic syndromes thought to be

IL-1-mediated. Specifically, pediatric

patients with uveitis associated with

chronic infantile neurologic, cutaneous,

and articular (CINCA) syndrome 39 and

the NOD2 gene-associated pediatric

granulomatous arthritis were treated

successfully with anakinra therapy.40

Alemtuzumab (Campath-1H), the anti-

CD52 monoclonal antibody that targets


T and B lymphocytes, has been used for

a variety of hematologic indications,

including myelodysplastic syndrome,

aplastic anemia, chronic lymphocytic

leukemia, and a number of T-cell

leukemias/ lymphomas.41-43 Dick et al.44

reported the use of alemtuzumab for

severe, recalcitrant ocular inflammation

in 10 patients with a variety of

ophthalmic inflammatory diseases,

which included Wegener’s

granulomatosis-associated peripheral

ulcerative keratitis and pseudotumor,

retinal vasculitis, sympathetic

ophthalmia, and Behçet’s disease.44 All

patients showed an initial improvement

following Campath-1H administration;

however 2 of 10 patients required

retreatment. Remission was observed in

8 patients who received Campath-1H,

and no opportunistic infections or

malignancies were observed at short

term Follow-up.

IFN-alpha

antineoplastic and antiviral effects;

however its precise mechanism of

action is unknown. Its use in the

prevention of ocular relapses, as well as

in the treatment of Behçet’s disease-

associated ocular inflammation in the

doses of 3 to 6 million IU by

subcutaneous injection daily to three

times per week has been reported

previously. 9,45 Kotter et al.45 reported

that IFN-

low-dose steroid led to remission of

ocular disease in 7 patients with

Behçet’s disease-associated panuveitis.

A more recent report suggested a

potential benefit of IFN-2alpha

treatment-resistant CME, with 6 of 8

patients responding to IFN-

resolution of CME during the first 6

months of therapy.46 Further studies into

IFN-

are required before its implementation

into routine clinical practice.

Rituximab, a monoclonal anti-CD20

antibody targeting B cells, has been

used systemically for the treatment of a

number of hematologic malignancies

and lymphoproliferative disease

processes, including primary central

nervous system lymphoma.47,48 Recent

studies have supported its use for ocular

adnexal lymphomas, 49, 50 and limited

case series have also reported its

benefit for the treatment of primary

intraocular lymphoma. While the use of

rituximab has been advocated for the

treatment of primary intraocular

lymphoma recurrences, vigilant central

nervous system surveillance,

management, and treatment with a

neuro-oncologist for systemic

chemotherapy are highly recommended.

Finally, abatacept (fusion protein that

binds the co stimulatory factor

B7,thereby inhibiting T-cell

activation),and tocilizumab (humanized

monoclonal antibody against IL-6) have

been found to have some efficacy

against rheumatoid arthritis, and

therefore may be under future

consideration for patients with ocular

inflammatory disease who fail with other

biologic agents.


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Case report of isolated schwannoma a rare tumour of eyelid

Sudhir Singh, M.S, Subodh Saraf, M.S, Divyesh Goswami, M.D

Correspondence Author Dr Sudhir Singh M.S. Ophthalmology Senior Consultant & HOD Dept of Ophthalmology JW Global Hospital & Research Centre Mount Abu Rajasthan 307501 [email protected] Abstract

Schwannoma (neurilemmoma) is a benign tumour of peripheral nerve arising from Schwann cells that

form the neural sheath. Schwannoma of ophthalmic interest is rare. Although it has been reported in

relation with the orbit, and less frequently with the uveal tract and conjunctiva but eyelid schwannoma is

extremely uncommon. We report a case of an 18-year-old male who developed eyelid schwannoma. The

mass was surgically removed by excisional biopsy. The histopathological examination showed

schwannoma

Keywords: Benign tumour, eyelid, histopathology, schwannoma

Case Report

An 18-year-old male presented to us

with a 6 month history of slowly

enlarging, painless mass in his right

upper lid resulting in progressive ptosis

(Fig.1). Ocular examination was

suggestive of a firm, non-tender nodule

of size 2 × 1 × 1 cm on the right upper

lid. The mass was non-adherent to the

skin and was mobile on palpation

(Fig.2). There were no clinical findings

indicative of neurofibromatosis.

Fig 1.Pre operative photograph of the patient

showing ptosis of right upper lid

mailto:[email protected]


Fig. 2 demonstration firm non tender nodule

with free mobile underlying skin.

The patient was operated under local

anaesthesia. Two silk sutures were

taken near lid margin to provide traction

if required during surgery. Globe was

secured with entropion plate. Lid crease

incision was given superficial to tarsal

plate. The lesion was isolated from the

surrounding tissue by blunt dissection.

Capsule was present over the lesion.

Dissection was easily done in the extra

capsular plane except for the lower

portion of around 2 × 2 mm which was

found adherent with tarsal plate. The

lesion was excised completely along

with some part of tarsal plate. No

communication with the supraorbital

nerve could be identified.

Fig. 3 Schwannoma nodule after dissection.

Fig. 4. Full thickness lid defect after excision

of schwannoma.

The tarsal plate was sutured with 6-0

vicryl continuous sutures. Skin was

sutured with interrupted 6-0 vicryl.

Eyelid movements were present on

table. Patient’s ptosis was recovered on

first post op day.


Fig.5 Improvement of the ptosis of the right

upper lid on the first post operative day.

Fig.6 first post operative day appearance of

the patient showing no lid lag.

Gross Examination-

The tumour was encapsulated nodular

lesion of size 2.4 X 1.7 X 1.5 cm. The

consistency was firm with whitish

appearance at cross section.

Fig.7 Specimen of the schwannoma.

Microscopic Examination

H & E stained Sections show alternating

areas of highly cellular (Antoni A) and

hypocellular (Antoni B). Antoni A cells-

spindle cells, containing elongated

nuclei arranged in fascicles, nuclei tend

to palisade.

Fig.8 H & E stained Sections H & E stained

Sections show alternating areas of highly

cellular (Antoni A) and hypocellular (Antoni

B).

Antoni B area- homogenous acellular

material, in which the cells were more

oval, and had rounded nuclei, clear

cytoplasm and less basement


membrane, and were loosely entwined

within a clear myxoid matrix

Discussion

Schwannoma is rare benign neurogenic

tumour made up of proliferating

Schwann cells of peripheral nerve. It is a

neoplasm which occurs wherever

Schwann cells are present, that is, in

any myelinated peripheral nerve. In

most cases, while Schwannoma is

sporadically manifested as a single

benign neoplasm, the presence of

multiple Schwannoma is usually

indicative of neurofibromatosis-2. Our

patient had isolated eyelid Schwannoma

with no family history or clinical findings

of neurofibromatosis-1 or

neurofibromatosis-2.

Clinically, the tumour is a solid, slowly

progressive and painless mass. Due to

its rarity and unusual location, eyelid

Schwannoma is frequently confused

with other diagnosis like chalazion or

inclusion cyst. In our case, patient

presented with solid slowly enlarging,

painless mass of 6 month duration.

Literature suggests that the tumour,

though rare, can be present in both

upper and the lower eyelids.

Macroscopically, they appear to be well

demarcated; they usually grow very

slowly and are asymptomatic.

Microscopically, they may demonstrate

a biphasic pattern with areas of highly

cellular (Antoni type A) and myxoid

matrix (Antoni type B) [6]. . The cells

tend to align themselves into compact

parallel rows, with intermittent dense

anucleate zones. In other locations, a

poor prognosis has been described if

the cells are fusiform, contain melanin

granules, or if epithelioid cells are

present [1]. Nevertheless, malignant

transformation has not been reported in

eyelid schwannomas.

The most important feature in its

diagnosis remains the strong reactivity

to S100 protein by

immunohistochemistry, particularly in

Antoni type A areas. Despite sometimes

striking cytologic atypia, mitotic figures

are rare. It is postulated that

degenerative changes occur due to the

long period of time over which large

schwannomas develop [7].

The age range in the adult group of

published cases (in 40 years) was

between 19 and 63.The size of the

tumour ranges from few millimetres to

3.5 cm. [2]

http://dermatology.cdlib.org/132/case_presentations/schwannoma/gutierrez.html#7



http://ukpmc.ac.uk/abstract/MED/19085294


Management of Schwannoma of the

eyelid is complete excision with clear

margin to establish the histopathological

diagnosis and prevent recurrence.

Incomplete removal is associated with

eventual recurrence and more

aggressive behaviour. There have been

anecdotal reports of malignant changes

in a previously incompletely excised

benign Schwannoma. The swelling also

tends to transgress tissue planes and

grow rapidly on incomplete excision. [4,

5] An attempt to preserve continuity of

nerve should be made, but this is not

always possible and does not appear to

have any major consequences at this

site.

References

1.http://dermatology.cdlib.org/132/case_

presentations/schwannoma/gutierrez.ht

ml#2

2.http://ukpmc.ac.uk/abstract/MED/1908

5294


748


025



ml#7



ml#8



















Case Report

Goldenhar syndrome: Dermoid excision reduces acquired astigmatism Amit Mohan, M.S.; Sudhir Singh, M.S.

Correspondence Author Dr Amit Mohan,M.S Global Hospital Institute of Ophthalmology, Abu Road,Sirohi Rajasthan 307501

Abstract

Goldenhar syndrome is a rare congenital anomaly which consist of a triad of an ocular dermoid cyst,preaural skin

tags and vertebral dysplasia. We report a case of Goldenhar syndrome diagnosed in a 19yr old girl with acquired

astigmatism of 3 Diopters against the rule and decreased to 1 diopter against the rule asigmatism after dermoid

excision.

Case Report

A 19 year old girl presented with congenital

lesions of the face and eyes. Ocular

examination revealed a dermoid nodule

located on the bulbur conjunctiva at

inferotemporal side of limbus in the Left Eye

(fig-1) .

Fig.1 A dermoid nodule located on the bulbur

conjunctiva at inferotemporal side of limbus in the

Left Eye

Refraction identified against the rule

astigmatism of 3 Diopters. BCVA was 6/6 in

right eye and 6/18 in left eye. Ocular motility

was normal.

On facial examination there was a preaural

tag near the tragus of pinna (fig.-2)

Fig.2. Showing pre aural tag near tragus of the

pinna of the left eye.

Skeletal examination showed micrognathia

and dental anomalies (fig-3)

Fig.3 Showing micrognathia and dental anomalies


Which was confirmed in x-ray film (fig-4 & 5).

These symptoms were consistant with the

diagnosis of Goldenhar syndrome.

Fig 4 X ray film showing dental abnormality

Fig 5. Fig 4 X ray film showing micrognathia

Complete excision of dermoid cyst was done

(fig-6).

Fig.6 Appearance after dermoid excision

Intraoperative and postoperative course was

uneventful. Postopertive Astigmatism

reduced to 1 diopter against the rule

astigmatism.

Discussion

Goldenhar Syndrome also known as

oculoauriculovertebral syndrome is a rare

congenital malformation involving the first and

second branchial arches 1.It was first

described by Goldenhar in 1952. If the limbal

dermoid are detected and excised early

astigmatism may be reduced. The main

ocular feature of Goldenhar syndrome is

epibulbar choriostoma in 30 to 60 %2,3. It

consist of a dermoid or lipodermoid. The

corneal and the sclera invasion by the tumour

are rare and lead to against the rule

astigmatism. Management of ocular dermoid

is surgical excision. Dermoid cyst is a benign

tumour that causes serious ophthalmological

sequels-Astigmatism, Amblyopia

&Strabismus. The ophthalmologist should

focus on visual consequences, treat early and

follow up the patient because dermoid causes

acquired astigmatism and amblyopia.

References

1) Bijal M, Nayak S, Shankar S-

Goldenhar syndrome with unusual

features,Indian –J.Dermatol,2008;74

254-256.

2) Andernson PJ, David DJ-Craniofacial

J.2005; 42 477-80.

3) Murat O, Mesut G, Aylekin G, Guven

O –Goldenhar syndrome associated

with bilateral ocular choriostoma and

cardiac abnormalities, Eur J Gen Med

2004; 1: 28-30.

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