Irregular Astigmatism after DSAEK in case of Congenital Hereditary Endothelial Distrophy with Intrastromal Vacuolization - L. Avoni, L. Cappuccini, M. Busin

1. L.Avoni 1, L.Cappuccini 1, M.Busin 2
1 Department of Ophthalmology “Maggiore” Hospital – Bologna - Italy
2 Department of Ophthalmology “Villa Igea” Hospital – Forlì - Italy

2. Clinical Hystory
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CASE REPORT
Female - 15 Years Old
Misdiagnosis of CHED 1 in early life
(RE > LE)
No Nystagmus
Visual Acuity Previous Values
RE & LE 6/12 ( Snellen Fractions
Based on 6m) (i.e 5/10) with refr:
-3.00 sph -3.50 cyl x 180°
(Moderate Amblyopia)
Reported a Deterioration of Visual
Acuity over the years

3. CASE REPORT
At Our First Investigation:
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Visual Acuity RE & LE
rispectively 6/60 (i.e 1/10)
with refraction of:
-3.00 sph -3.50 cyl x 180° (?)
Anterior Segment RE & LE :
Diffuse Serious Bilateral Haze
Fundus OO: Red Reflex Visible

4. CHED: note the diffuse serious bilateral corneal haze
OD
OS

5. CHED: At this stage, which Clinical Strategies?
OD
OS

6. Tests Carried Out
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Complete Eye Examination.
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Photographs of Anterior Segment.
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Orbscan Topography and Pachymetry.
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Ultrasound Pachymetry.
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Anterior Segment OCT (Casia)
Sirius Topography, Tomography, Pachimetry.

7. Corneal Topography (Orbscan) at Our
First Investigation
SIMK’s Astigm 4,8 D @ 93°; MAX 45,7 D @93° / 40,9 D @
3°

8. CASIA at Our First Investigation
njnj

9. Strategies and Management
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DSAEK May 2011
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Post Surgical Therapy : Topic Antibiotic & Steroid
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Close Ophthalmological Examinations /Follow Up
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Assess the use of a RGP Contact Lens to improve Visual Acuity

10. Corneal topography (Orbscan) After Surgery
SIMK’s Astigm 18,3 D @ 65; MAX 53,1 D @65° / 34,8 D @
155°

11. Casia topography After Surgery

12. Casia Tomography After Surgery

13. CASIA Pachimetry after Surgery

14. Corneal Topography (Sirius) 3 months after
Surgery
Axial Algorithm

15. Corneal Topography (Sirius) 6 months after Surgery
Axial Algorithm

16. SIMK’s after Surgery
Axial Algorithm
SIMK’s Astigm 18,3 D @ 65; MAX 53,1 D @65° / 34,8 D @
155°
SIMK’s 3 months after Surgery
Axial Algorithm
SIMK’s 6 months after
Surgery
Axial Algorithm
SIMK’s Pre Surgery
Axial Algorithm
SIMK’s Astigm 4,8 D @ 93°;
MAX 45,7 D @93° / 40,9 D @ 3°

17. Corneal
Topography(Sirius) 3
Months
After Surgery
(Local Algorithm)
NOTE THE CORNEAL
SHAPE
REGULARIZATION
Corneal
Topography(Sirius) 6
Months
After Surgery
(Local Algorithm)

18. Corneal Pachymetry (Sirius) 6 Months After Surgery
i.e Thinnest Point

19. Corneal Pachymetry After Surgery
Thinnest Point : 840 μ
Corneal Pachymetry 6 months After
Surgery
Thinnest Point : 586 μ

20. Corneal Tomography (Sirius) 3 months after
Surgery (top) and 6 months after Surgery
(bottom): note the reduction and the
regularization of the corneal thickness.
OO
PP
PP
OO
PP
PP

21. Six Months After Surgery
Pre Surgery
Six months after
Surgery

22. Current Results
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Visual Acuity with RGPCL 6/12 (i.e 5/10)
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Visual Acuity with refraction 6/12 : +3,75 sph – 2,75 cyl X 170°
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IOP : 16mmhg
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Anterior Segment: Considerably Reduction of Corneal Edema
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Same Anterior Toricity as Before Surgical Treatment
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Central Pachymetry: 600 μ

23. Discussion
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CHED is characterized by a diffuse ground-glass appearance of both corneas and
markedly thickened (2-3 times thiker than normal) corneas from birth or infancy.
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Two types of CHED are recognized: CHED 1 (autosomal dominant - the gene
responsible 1 has been mapped to the pericentromeric region of chromosome 20)
and CHED 2 (autosomal recessive).
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CHED 1 becomes manifest during the first two years of life, but in contrast to CHED 2
nystagmus is absent.
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The treatment is primarly surgical.

24. Discussion
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Several Authors (Busin M.; Busin M. et al) noted that DSAEK offers key
benefits over PK in conditions with moderate risk of graft failure (corneal
dystrophy).

25. Conclusions
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In this patient DSAEK has resulted in a restoration of the corneal clarity with
a significant reduction of corneal astigmatism in few months.
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The young patient reported a subjective significant improvement of visual
performance reporting also a considerably clearer vision.
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An RGPCL with extremely high value of oxygen transmissibility was assed in
order to enhance the quality of vision.
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At the moment the young patients is waiting for DSAEK in left eye.

26. Thank You for your Kind Attention
L.Avoni , L.Cappuccini , M.Busin
luca.avoni@gmail.com