Date post: | 19-Jun-2015 |
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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
CASE REPORTClinical Hystory 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
CASE REPORTAt Our First Investigation:
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
CHED: note the diffuse serious bilateral corneal haze
OD OS
CHED: At this stage, which Clinical Strategies?
OD OS
Tests Carried Out Complete Eye Examination.
Photographs of Anterior Segment.
Orbscan Topography and Pachymetry.
Ultrasound Pachymetry.
Anterior Segment OCT (Casia)
Sirius Topography, Tomography, Pachimetry.
Corneal Topography (Orbscan) at Our First Investigation
SIMK’s Astigm 4,8 D @ 93°; MAX 45,7 D @93° / 40,9 D @ 3°
njnj
CASIA at Our First Investigation
Strategies and Management
DSAEK May 2011
Post Surgical Therapy : Topic Antibiotic & Steroid
Close Ophthalmological Examinations /Follow Up
Assess the use of a RGP Contact Lens to improve Visual Acuity
Corneal topography (Orbscan) After Surgery SIMK’s Astigm 18,3 D @ 65; MAX 53,1 D @65° / 34,8 D @ 155°
Casia topography After Surgery
Casia Tomography After Surgery
CASIA Pachimetry after Surgery
Corneal Topography (Sirius) 3 months after Surgery
Axial Algorithm
Corneal Topography (Sirius) 6 months after Surgery Axial Algorithm
SIMK’s after Surgery Axial Algorithm
SIMK’s 3 months after Surgery Axial Algorithm
SIMK’s 6 months after Surgery
Axial Algorithm
SIMK’s Astigm 18,3 D @ 65; MAX 53,1 D @65° / 34,8 D @ 155°
SIMK’s Pre SurgeryAxial Algorithm
SIMK’s Astigm 4,8 D @ 93°; MAX 45,7 D @93° / 40,9 D @ 3°
Corneal Topography(Sirius) 3 MonthsAfter Surgery(Local Algorithm)
NOTE THE CORNEAL SHAPE REGULARIZATION
Corneal Topography(Sirius) 6 MonthsAfter Surgery(Local Algorithm)
Corneal Pachymetry (Sirius) 6 Months After Surgery
i.e Thinnest Point
Corneal Pachymetry After Surgery
Corneal Pachymetry 6 months After Surgery
Thinnest Point : 840 μ
Thinnest Point : 586 μ
Corneal Tomography (Sirius) 3 months after Surgery (top) and 6 months after Surgery (bottom): note the reduction and the regularization of the corneal thickness.
OOPPPP
OOPPPP
Six Months After Surgery
Pre Surgery
Six months after Surgery
Current Results
Visual Acuity with RGPCL 6/12 (i.e 5/10)
Visual Acuity with refraction 6/12 : +3,75 sph – 2,75 cyl X 170°
IOP : 16mmhg
Anterior Segment: Considerably Reduction of Corneal Edema
Same Anterior Toricity as Before Surgical Treatment
Central Pachymetry: 600 μ
Discussion
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.
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).
CHED 1 becomes manifest during the first two years of life, but in contrast to CHED 2
nystagmus is absent.
The treatment is primarly surgical.
Discussion 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).
Conclusions
In this patient DSAEK has resulted in a restoration of the corneal clarity with
a significant reduction of corneal astigmatism in few months.
The young patient reported a subjective significant improvement of visual
performance reporting also a considerably clearer vision.
An RGPCL with extremely high value of oxygen transmissibility was assed in
order to enhance the quality of vision.
At the moment the young patients is waiting for DSAEK in left eye.