A 41-year-old white woman was referred to our Department to rule out the presence of a Fuch's corneal dystrophy. On slit-lamp biomicroscopy, small bilateral punctuate opacities appearing mostly in the posterior stroma were observed, suggesting a differential diagnosis of pre-Descemet's dystrophy as opposed to Cornea Farinata

Benusiglio, Enrico

Borrelli, Maria

Lanza, Michele

Rosa, Nicola

English

PubMed

Michele Lanza

Maria Borrelli

Enrico Benusiglio

Nicola Rosa

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In vivo confocal microscopy of an apparent deep stroma corneal dystrophy: a case report

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BioMed CentralCases Journal
ssOpen AcceCase Report
In vivo confocal microscopy of an apparent deep stroma corneal 
dystrophy: a case report
Michele Lanza1,2, Maria Borrelli2, Enrico Benusiglio2 and Nicola Rosa*1,2
Address: 1Department of Ophthalmology, Second University of Naples Via Pansini 5, Naples 80100 Italy and 2Centro Grandi Apparecchiature, 
Second University of Naples, Sezione di Ricerca Clinica in Oftalmologia, Via De Crecchio, 16 Naples, 80100 Italy
Email: Michele Lanza - michele.lanza@unina2.it; Maria Borrelli - nicola.rosa@unina2.it; Enrico Benusiglio - nicola.rosa@unina2.it; 
Nicola Rosa* - nicola.rosa@unina2.it
* Corresponding author    
Abstract
A 41-year-old white woman was referred to our Department to rule out the presence of a Fuch's
corneal dystrophy. On slit-lamp biomicroscopy, small bilateral punctuate opacities appearing
mostly in the posterior stroma were observed, suggesting a differential diagnosis of pre-Descemet's
dystrophy as opposed to Cornea Farinata.
Confocal microscopy in the central cornea of both eyes revealed the normal appearance of
superficial and basal epithelial layers. However throughout the full thickness of the cornea fine
highly refractive granules, localized both in the keratocytes cytoplasm and in the stroma matrix
were noted. In both eyes abnormal polymegatism and pleomorphism was observed.
Introduction
Several corneal dystrophies such as Cornea Farinata,
Punctuate Dystrophy, pre-Descement's Dystrophy, Fleck's
Dystrophy, Deep Filiform Dystrophy and Posterior Punc-
tiform Dystrophy have the clinical appearance of fine
opacities limited to the posterior corneal stroma. These
deposits are not associated with ocular or systemic dis-
eases, they often are asymptomatic and only very rarely
they interfere with visual acuity [1].
Sometimes the differential diagnosis between these dys-
trophies may prove challenging [1]. In vivo confocal
microscopy (CM) has been useful in differentiating sev-
eral cases and might help to bring about a better classifi-
cation and discover new entities [2]. In this paper we
report a case where (CM) appeared crucial for carrying out
diagnosis.
Case presentation
A 41-year old white woman was referred to us to rule out
the presence of a Fuch's corneal dystrophy.
Her family history did not reveal the presence of corneal
dystrophies; in both eyes her best corrected visual acuity
was 20/20, while intraocular pressure was within normal
limits, and the findings on external examination and
dilated pupil fundus examination were unremarkable. On
slit lamp biomicroscopy small bilateral punctuate opaci-
ties mostly located in the posterior stroma were observed,
suggesting the differential diagnosis between pre-
Descemet's dystrophy as against Cornea Farinata (Figure
1).
The patient underwent in vivo CM with Confoscan 3
(Nidek Technologies, Vigonza, Italy) in order to obtain
further details of her corneal disorder.
Published: 14 December 2009
Cases Journal 2009, 2:9317 doi:10.1186/1757-1626-2-9317
Received: 7 January 2009
Accepted: 14 December 2009
This article is available from: http://www.casesjournal.com/content/2/1/9317
© 2009 Lanza et al; licensee BioMed Central Ltd. 
This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), 
which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.Page 1 of 3
(page number not for citation purposes)
Cases Journal 2009, 2:9317 http://www.casesjournal.com/content/2/1/9317In both eyes it revealed a normal appearance of the super-
ficial and basal epithelial layers. Nevertheless throughout
the full thickness of the corneal stroma fine highly refrac-
tive granules, localized both in the keratocytes cytoplasm
and in the stromal matrix, were observed (Figure 2 and
Figure 3), while normal keratocytic nuclei with a typical
coffee-bean like appearance were noted. In the right eye
the corneal endothelial cell count was 2,512 cell/mm2
with abnormal polymegatism (46.1%) and pleomor-
phism (37.9%), whereas in the left eye the endothelial cell
count was 2,984 cell/mm2 with abnormal polymegatism
(49.9%) and pleomorphism (26.9%).
Discussion
Corneal dystrophies affecting the entire corneal stroma,
and in particular the posterior stroma, can often show a
similar appearance on slit-lamp examination, thus the dif-
ferential diagnosis may prove challenging and some of
these dystrophies may actually be a variation of the same
disease [1].
In vivo CM has been used by several authors in an attempt
to clarify the diagnosis of several deep stroma corneal dys-
trophies.
Cornea Farinata is an asymptomatic degenerative condi-
tion characterized by a myriad of fine, dust-like opacities
found bilaterally in the posterior stroma near Descemet's
membrane. They are best seen on retro illumination and
appear grey-brown to white colour. On CM the superficial
(dark and light cells) and the mid stroma layers appear
normal; highly reflective small particles can be found in
the cytoplasm of some keratocytes only in the deep
stroma adjacent to the corneal endothelial layer with no
abnormalities of the Descemet's membrane and endothe-
lial cell layers [1].
Pre-Descemet's dystrophy, a condition bearing a close
clinical resemblance to Cornea Farinata, on slit-lamp
examination shows larger and more polymorphous opac-
ities than those of Cornea Farinata, distributed through-
out the cornea but with a predilection for the deep stroma
[2]. CM demonstrates intra- and extra-cellular hyper-
reflective inclusions immediately anterior to Descemet's
membrane and prominent sub-basal nerves; the epithe-
Slit-lamp photography demonstrating the corneal changesF gure 1
Slit-lamp photography demonstrating the corneal 
changes.
In vivo confocal microscopic findings of the right eye showing hyperreflective dot-like deposits in the anterior corneal stromaFigu e 2
In vivo confocal microscopic findings of the right eye 
showing hyperreflective dot-like deposits in the ante-
rior corneal stroma.
In vivo confocal microscopic findings of the left eye showing hyperreflective dot-like depos ts  the deep stroma adjacent to the corneal endoth liumFigu e 3
In vivo confocal microscopic findings of the left eye 
showing hyperreflective dot-like deposits in the deep 
stroma adjacent to the corneal endothelium.Page 2 of 3
(page number not for citation purposes)
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lium, basement membrane and endothelium appear nor-
mal [3,4].
Fleck's dystrophy is a rare dominant autosomal corneal
dystrophy with discrete opacities similar to those of Cor-
nea Farinata and pre-Descemet dystrophy, but the opaci-
ties are located not only in the posterior but throughout
the entire corneal stroma [4]; intracellular hyper-reflective
dots of various shapes throughout the corneal stroma
have been reported on CM. Some authors have described
the involvement of sub-basal nerves characterized by mul-
tiple inclusions similar to those found in the keratocytes
[4] while others have suggested that the presumed nerves
and inclusions actually represent abnormal keratocytes
processes (5).
Deep Filiform dystrophy of Maeder and Danis consists of
multiple filiform gray opacities in the pre-Descemet area
that affect the entire width of the cornea except for the per-
ilimbal region. The original case occurred in a middle-
aged woman with keratoconus. This disorder may repre-
sent a degeneration rather than a dystrophy [5].
In Posterior Punctiform dystrophy punctiform elements
are present in small groups of filament some of which are
of a dendritic type, whereas others resemble canes or
comas, and are of a bluish-white colour, being visible to
direct and indirect illumination with ring or strictly axial
distribution of the opacities [5].
According to our findings our case report does not seem
to belong to any of the above described corneal dystro-
phies both on account of the appearance and distribution
of the deposits.
On the basis of our findings we suggest that the classifica-
tion of these dystrophies should be revised. The diagnosis
of pre-Descemet's dystrophies should be limited to those
dystrophies where CM rules out the involvement of the
anterior and mid-stroma, whereas those with an involve-
ment of the anterior and mid stroma, should be classified
differently.
This CM based classification, could be very important for
future genetic studies on corneal dystrophies. Clearly, fur-
ther longitudinal studies on a large number of patients
should be performed in order to rule out the possibility
that these dystrophies are not evolving pictures of a phe-
notypic variation of a unique dystrophy, as happened in
the so-called Avellino dystrophy case [6].
Consent
Written informed consent was obtained from the patient
for publication of this case report and accompanying
images. A copy of the informed consent is available for
review by the Editor-in-Chief of this journal.
Competing interests
The authors declare that they have no competing interests.
Authors' contributions
LM has been involved in drafting the manuscript and has
given final approval of the version to be published BM.
has been involved in drafting the manuscript and has
given final approval of the version to be published. BE has
made substantial contributions to conception and design,
acquisition of data and has given final approval of the ver-
sion to be published.
RN has made substantial contributions to conception and
design, acquisition of data and has given final approval of
the version to be published
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LANZA, Michele

Borrelli M

Benusiglio E

Rosa N.

Archivio Istituzionale della Ricerca - Università degli Studi della Campania "Luigi Vanvitelli"

In vivo confocal microscopy of an apparent deep stroma corneal dystrophy: a case report.

Acosta lEP, Malbran E: Punctiform and polychromatic pre-Descemet's dominant corneal dystrophy.

CNJ: Microstructural assessment of rare corneal dystrophies using real time in vivo confocal microscopy. Clin Exp Ophthalmol

Cornea farinata. Report of a case: clinical, histologic and ultrastuctural study.

HD: Phenotypic variation in combined granular/lattice (Avellino corneal dystrophy). Arch of Ophthalmol

Kazuhisa S: In vivo confocal microscophy in patients with cornea farinata. Cornea

Tervo TMT: In vivo confocal microscopy of Fleck Dystrophy and Pre-Descemet's membrane Corneal Dystrophy. Cornea

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In vivo confocal microscopy of an apparent deep stroma corneal dystrophy: a case report

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