Transcript International Outcomes of the Boston Type I Keratoprosthesis in

International Outcomes of the
Boston Type I Keratoprosthesis in
Stevens-Johnson Syndrome
April 15-17, 2015
World Cornea Congress VII
San Diego, California
Jamie K. Alexander, M.D.
Samar K. Basak, M.D.
Ma. Dominga B. Padilla, M.D.
Fei Yu, Ph.D.
Anthony J. Aldave, M.D.
The authors have no relevant financial interests to disclose.
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Purpose
Stevens-Johnson syndrome (SJS) can devastate the ocular surface.
There is currently no reliable, sustainable method for restoring sight
in these patients.
While initial studies of the Boston type I keratoprosthesis (KPro)
indicated that its results were poorest in patients with SJS, advances
in KPro design and post-operative management have improved
outcomes, including those in SJS.
Using international data and the world’s most widely used
keratoprosthesis, we present the largest study to date of the KPro in
SJS. We compare the device’s outcomes in SJS to those of patients
without SJS and seek to determine the factors influencing outcomes
of the KPro in SJS.
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Methods
•
Retrospective, interventional, comparative case series
•
Study approval: Institutional Review Board at UCLA
Ethics Committee at Disha Eye Hospitals
Institutional Ethics Review Committee at St. Luke’s Medical Center
•
Data was collected from 3 international academic medical centers on all
patients undergoing KPro surgery, including preoperative characteristics,
intraoperative data, and postoperative outcomes:
Stein Eye Institute
Los Angeles, California
May 2004 – Jul 2013
•
Disha Eye Hospitals
Kolkata, India
Mar 2009 – Jan 2014
St. Luke’s Medical
Center Global City
Manila, Philippines
Mar 2009 – Jan 2014
Statistical Analysis: SAS software version 9.4 (SAS, Inc., Cary, NC) (p=0.05)
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Results: Preoperative Characteristics
No. of procedures
No. of eyes
No. of patients
Gender (female; male)
Mean age (range), yrs
Follow-up (mos)
Mean (median)
Standard deviation (range)
Indication for keratoprosthesis
Failed corneal transplant
Stevens-Johnson syndrome
Limbal stem cell deficiency
Chemical injury
Repeat keratoprosthesis
Corneal vascularization
Mucous membrane pemphigoid
Aniridia
Thermal Burn
AKC
Other
Glaucoma
Previous glaucoma surgery
Previous corneal transplant(s)
0
1
2
3
>4
SJS
Non-SJS
P value
40
27
26
14; 12
50 (26-72)
194
182
175
75; 100
60 (3-95)
0.3
<0.001
17.6 (12.5)
16.2 (0.5-73.1)
29.3 (23.5)
22.8 (0.8-106.5)
-27 (68%)
--13 (33%)
------7 (26%)
4 (15%)
109 (56%)
-24 (12%)
18 (9%)
15 (8%)
11 (6%)
4 (2%)
4 (2%)
3 (2%)
2 (1%)
4 (2%)
130 (71%)
45 (25%)
18 (67%)
5 (19%)
2 (7%)
0
2 (7.4%)
42 (23%)
32 (18%)
54 (30%)
33 (18.1%)
21 (11.5%)
0.001
<0.001
<0.001
0.334
<0.001*
*Comparing <1 to >2 previous corneal transplants
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Results: CDVA for Most Recent KPro
Baseline
6 Months
1 Year
2 Years
3 Years
4 Years
5 Years
At last follow-up
No. of eyes
SJS
Non-SJS
20/20-20/50
SJS
Non-SJS
20/60-20/100
SJS
Non-SJS
20/125-20/200
SJS
Non-SJS
20/250-20/400
SJS
Non-SJS
CF
SJS
Non-SJS
HM
SJS
Non-SJS
LP
SJS
Non-SJS
NLP
SJS
Non-SJS
27
182
23
169
18
142
9
99
3
68
1
43
0
25
22
162
0 (0%)
1 (1%)
14 (61%)
53 (31%)
7 (39%)
45 (32%)
5 (56%)
31 (31%)
1 (33%)
21 (31%)
1 (100%)
15 (35%)
-9 (36%)
9 (41%)
49 (30%)
0 (0%)
0 (0%)
5 (22%)
49 (29%)
7 (39%)
34 (24%)
3 (33%)
18 (18%)
2 (67%)
9 (13%)
0 (0%)
5 (12%)
-2 (8%)
8 (36%)
33 (20%)
0 (0%)
9 (5%)
3 (13%)
18 (11%)
4 (22%)
16 (11%)
1 (11%)
18 (18%)
0 (0%)
13 (19%)
0 (0%)
5 (12%)
-4 (16%)
4 (18%)
20 (12%)
0 (0%)
7 (4%)
0 (0%)
9 (5%)
0 (0%)
6 (4%)
0 (0%)
4 (4%)
0 (0%)
3 (4%)
0 (0%)
4 (9%)
-0 (0%)
0 (0%)
12 (7%)
8 (30%)
53 (29%)
0 (0%)
27 (16%)
0 (0%)
21 (15%)
0 (0%)
11 (11%)
0 (0%)
6 (9%)
0 (0%)
3 (7%)
-4 (16%)
0 (0%)
19 (12%)
9 (33%)
61 (34%)
0 (0%)
7 (4%)
0 (0%)
8 (6%)
0 (0%)
12 (12%)
0 (0%)
10 (15%)
0 (0%)
9 (21%)
-1 (4%)
1 (5%)
11 (7%)
10 (37%)
51 (28%)
1 (4%)
4 (2%)
0 (0%)
7 (5%)
0 (0%)
2 (2%)
0 (0%)
3 (4%)
0 (0%)
1 (2%)
-4 (16%)
0 (0%)
9 (6%)
0 (0%)
0 (0%)
0 (0%)
2 (1%)
0 (0%)
5 (4%)
0 (0%)
3 (3%)
0 (0%)
3 (4%)
0 (0%)
1 (2%)
-1 (4%)
0 (0%)
9 (6%)
0 (0%)
10 (6%)
0.367
22 (96%)
120 (71%)
0.01
18 (100%)
95 (66.9%)
0.002
9 (100%)
67 (68%)
0.055
3 (100%)
43 (63%)
0.547
1 (100%)
25 (58%)
1
-15 (60%)
--
21 (96%)
102 (63%)
0.001
>20/200
SJS
Non-SJS
p-value
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Results: Complications and Secondary
Surgical Procedures
No. of eyes with >1 mo of follow-up
Retroprosthetic membrane
YAG laser membranotomy
Surgical membranectomy
Sterile corneal stromal necrosis
Keratoprosthesis replacement
Elevated IOP (>25 mmHg)
Glaucoma surgery
Corneal infiltrate
Persistent epithelial defect
Tarsorrhaphy
Infectious endophthalmitis
Vitreous tap and injections
Retinal detachment
Repair of retinal detachment
Sterile vitreitis
Vitreous tap and injection
Cystoid macular edema
Intravitreal injection
SJS
Non-SJS
p-value
27
10 (37%)
9 (33%)
1 (4%)
16 (59%)
9 (33%)
5 (189%)
1 (4%)
8 (30%)
16 (59%)
14 (52%)
0
0
3 (11%)
1 (4%)
0
0
2 (7%)
1 (4%)
181
89 (49%)
68 (38%)
13 (7%)
25 (8%)
8 (4%)
32 (18%)
11 (6%)
18 (10%)
44 (24%)
22 (12%)
4 (2%)
4 (2%)
21 (12%)
14 (8%)
15 (8%)
9 (5%)
23 (13%)
17 (9%)
0.30
0.83
1
<0.001
<0.001
1
1
0.009
<0.001
<0.001
1
1
1
0.70
0.23
0.61
0.75
0.48
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Results: Keratoprosthesis Retention Rate
SJS
Non-SJS
p-value
17.6 (0.5-73.1)
29.3 (0.8-106.5)
0.001
Keratoprostheses removed (eyes)
14 of 27 (51.9%)
28 of 182 (15.4%)
<0.001
Keratoprostheses removed (procedures)
18 of 40 (45.0%)
32 of 194 (16.5%)
<0.001
Mean follow-up of all procedures in mos (range)*
Retention failure
Retention failure rate (procedures)**
Time to failure in mos (mean, range)*
18 per 58.75 eye-years, 32 per 472.91 eye-years,
0.306/eye-year
0.068/eye-year
<0.001
14.1 (0.5-73.1)
19.9 (3.6-82.6)
0.011
Keratoprostheses retained (eyes)
22 of 27 (81.5%)
162 of 182 (89.0%)
0.335
Keratoprostheses retained (procedures)
22 of 40 (55.0%)
162 of 194 (83.5%)
<0.001
20.5 (5.8-59.5)
31.1 (0.8-106.5)
0.084
Retention success
Mean follow-up of retained procedures in mos (range)*
*Kruskall-Wallis test
** Log-rank test
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Results: Kaplan-Meier Survival of Last KPro
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Results: Characteristics of Patients with SJS
Associated with KPro Retention and Failure
Keratoprostheses Keratoprostheses
Retained
Removed
Number (eyes; procedures)
p-value
13; 22
14; 18
13.72
12.02
0.91
Perforation at or before time of KPro
4 (31%)
7 (50%)
0.44
Bulbar conjunctival keratinization
1 (8%)
6 (43%)
0.08
Lid malpositioning*
3 (23%)
4 (29%)
1
Tarsorrhaphy at time of keratoprosthesis
7 (32%)
5 (28%)
1
Preoperative immunosuppression
3 (13%)
2 (11%)
1
Postoperative immunosuppression
13 (59%)
8 (44%)
0.53
Sterile corneal necrosis
-
14
Infectious corneal necrosis**
-
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Pre-operative characteristics (eyes)
Time from SJS to initial procedure (yrs)
Peri-operative characteristics (procedures)
Indication for removal (procedure)
*requiring previous, concurrent or subsequent oculoplastics procedures
**organisms including: mixed (1), Candida (2), budding yeast without speciation (1)
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Results: Repeat KPro Results in SJS (example)
Slit lamp photomicrograph of a 67 year-old man with SJS. Donor
corneal necrosis developed 5 months following KPro surgery,
necessitating replacement of the KPro. At the time of the second
surgery, the donor cornea was covered with mobilized bulbar
conjunctiva and extensive medial and lateral tarsorrhaphies were
performed. Two years later, the donor cornea remains covered
by conjunctiva and the patient maintains 20/20 CDVA.
Results: SJS Patients in which KPro Not Retained
Pre-op
CDVA
# Kpros
Time Kpro
retained (mos)
HM
2
2.2
LP
1
6
LP
1
LP
HM
Procedure(s) Performed Following
Removal of Last Type I Kpro
Follow-up After
Removal of Last
Type I KPro (mos)
Current
CDVA
Boston type II Keratoprosthesis
99.7
20/40
Penetrating keratoplasty
4.9
LP
5.8
Penetrating keratoplasty (x 2)
42
LP
1
7.7
Penetrating keratoplasty
(repeat keratoprosthesis planned)
1.1
CF
1
5.7
Penetrating keratoplasty
(repeat keratoprosthesis planned)
2
CF
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Conclusions
The Boston type I keratoprosthesis is the most commonly used device of its kind worldwide. This study retrospectively analyzed the international outcomes of 40 KPro
procedures performed in 27 eyes of 26 patients with SJS in comparison to those of 194
procedures performed in 182 eyes of 175 patients without SJS.
Demographics
Patients undergoing KPro for SJS are more likely to be younger and less likely to have a
preoperative diagnosis of glaucoma or a history of multiple previous corneal transplants
compared to patients undergoing KPro for other indications. KPro procedures performed
in SJS patients are significantly more likely to be performed as repeat implantations of
the device (33% vs. 8%, p<0.001).
Visual Outcomes
Nearly all eyes undergoing KPro with SJS have a final CDVA > 20/200 when the KPro is
retained (96%).
- This is significantly higher than that of eyes without SJS (63%, p=0.001)
- This is likely due a significantly lower incidence of preoperative glaucoma.
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Conclusions
Complications
Several post-operative complications are more common in SJS patients following KPro:
sterile corneal stromal necrosis, persistent epithelial defect, and corneal infiltrate.
- No sight-threatening complications are more common.
- No endophthalmitis was encountered in SJS patients in this study.
Retention
Although retention failure is higher in SJS (relative risk 4.5), eyes with SJS are no less
likely to ultimately retain KPro following repeat implantation (82 vs. 89%, p=0.34).
Characteristics Affecting KPro Failure/Success in SJS
Based on available data, there are no identifiable pre- or postoperative characteristics
that lead to KPro failure or success in SJS.
- This may be due to the heterogeneous nature of the manifestations of SJS.
The Boston type I keratoprosthesis is an effective means to restore vision to
patients blinded by Stevens-Johnson syndrome.
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