Transcript Paget’s disease of the perianal region

Outcome of Penetrating
Keratoplasty for
Mucopolysaccharidoses
Alejandra Decanini MD, Erick D. Bothun MD, C. Gail Summers MD,
Paul J Orchard MD, Jakub Tolar MD, PhD
University of Minnesota
Department of Ophthalmology
The authors have no financial interest in the subject matter of this poster.
Introduction



Corneal opacification is a common eye finding in many
of the systemic mucopolysaccharidoses (MPS).
Clearing of the corneal opacity is uncommon despite
early systemic enzyme replacement therapy or bone
marrow transplantation.
Success of penetrating keratoplasty in this setting has
been variable and reports are limited.
Methods

A consecutive chart review of all patients who were
followed for at least a year after penetrating keratoplasty
in the setting of MPS between 1980 and 2009 at the
University of Minnesota.
MPS 1H: Hurler syndrome
MPS IV: Morquio syndrome
MPS VI: Marteaux-Lamy syndrome
Patient data
Eye
#
Sex
MPS
type
Eye
Age at
PK (y)
FU
(m)
VA pre PK
Snellen
(logMAR)
VA post PK
Snellen
(logMAR)
1
M
1-H
Right
11.7
13.0
20/80 (0.60)
20/70 (0.56)
2
F
1-H
Left
19.2
24.0
20/160 (0.92)
20/50 (0.40)
3
M
IV
Right
52.0
132.0
20/300 (1.16)
20/25 (0.10)
4
M
IV
Left
60.1
31.0
20/400 (1.30)
20/25 (0.10)
5
M
IV
Right
63.3
83.0
N/A
20/40 (0.30)
6
M
IV
Left
65.3
59.0
20/400 (1.30)
20/50 (0.40)
7
F
VI
Right
24.4
91.0
20/40 (0.30)
20/50 (0.40)
8
F
VI
Left
28.2
46.0
20/100 (0.70)
20/40 (0.30)
Results
Eight eyes of five patients met inclusion criteria.
Mucopolysaccharidoses type
4
Eye #
3
4
2
2
2
1
0
1-H
The two patients with MPS 1-H
had a bone marrow transplant
with 100% engraftment at the
time of the PK.
IV
VI
Results
Gender distribution
40%
60%
Age at PK
Female
70.0
60.0
Years
50.0
40.0
30.0
Mean:
26.3 years
20.0
10.0
0.0
1
2
3
4
Eye #
5
Male
6
7
8
Visual Acuity Pre/Post PK
1.40
1.20
VA (logMAR)
1.00
0.80
Pre PK
0.60
Post PK
0.40
0.20
0.00
1
2
3
4
6
7
8
9
Eye #
Visual acuity improved in seven out of eight eyes (p = 0.0017)
Results
Three patients had cataract extraction / intraocular lens
placement.
ACIOL
1
2
PCIOL
Results
All transplanted corneal grafts were clear at the last followup examination. No clearing of the surrounding host
cornea was identified.
Patient Follow up
Time (m)
144.0
Mean: 59 months
120.0
96.0
72.0
48.0
24.0
0.0
1
2
3
4
5
Eye #
6
7
8
Results


2/8 eyes (#1 and #2) presented signs of graft rejection
four and five months post PK. The patient was treated
with systemic and topical corticosteroids.
1/8 eyes (#2) developed recurrent episodes of rejection
that were resistant to treatment and a second PK was
required three years after the original procedure. The
second graft has remained clear in the follow-up period.
Conclusions


This study is the largest series of
keratoplasties for MPS in the literature.
penetrating
Stable, clear corneal grafts with improvement in vision
were obtained for each of the represented MPS
disorders (Hurler, Morquio, and Maroteaux-Lamy).