Table of Contents - WOC 2012

WOC2012 Abstract Book
Anterior Segment Malformations
Thu 16 Feb 16:30 - 18:00 Hall 7 - Tokyo Hall
IS-PED-TH 45 (1)
Congenital Corneal Opacification - A New Classification
Nischal Ken
1. UPMC Childrens Hospital of Pittsburgh and Eye Center
Congenital corneal opacification (CCO) nomenclature has become extremely
confused 1. Clinically it has been shown that up to 40 % of clinical diagnoses
in such cases are proven to be wrong when examined by High Frequency
Ultrasound. 2. We have proposed a new system of classification of CCO3
which relies on accurate phenotypic description using clinical and ultrasound
examination. This classification helps explain the condition to the parents
and gives a prognosticating evaluation for the surgeon. Essentially CCO can
be considered as being primary or secondary. The only primary CCO›s are
corneal dystrophies (CHED, PPMD, CHSD and XL-ECD), corneal dermoid
and true sclerocornea (i.e. CNA1 or CNA2 - NOT total corneal opacification).
Secondary CCO may be due to Kerato-irido-lenticular dysgenesis (e.g.
iridocorneal adhesions, keratolenticular adhesions i.e. Peters anomaly),
irido-goniodysgenesis (e.g. infantile glaucoma, aniridia etc.) or acquired e.g.
trauma, infection or metabolic (e.g. mucopolysaccharidosis). Primary CCO that
need penetrating keratoplasty do better than secondary CCO unless the cause
is treated first (e.g. infantile glaucoma). The exception to this is type 1 Peters
anomaly (where there is only iridocorneal adhesion).
1. Mataftsi A, Islam L, Kelberman D, Sowden JC, Nischal KK.
Chromosomeabnormalities and the genetics of congenital corneal opacification.
Mol Vis.2011; 17:1624-40.
2. Nischal KK, Naor J, Jay V, MacKeen LD, Rootman DS.
Clinicopathologicalcorrelation of congenital corneal opacification using
ultrasound biomicroscopy. Br J Ophthalmol. 2002 Jan; 86 (1):62-9.
3. Nischal KK Congenital corneal opacities - a surgical approach to
nomenclature and classification. Eye. 2007 Oct; 21(10):1326-37.
IS-PED-TH 45 (2)
Pseudo-Peters Anomaly in PHPV/PFV
De Faber Jan-Tjeerd
1. The Rotterdam Eye Hospital
In some cases of PHPV/PFV the entire iris and lens complex can be pushed
anterior towards the cornea with adhesions to the endothelium simulating
the clinical picture of a Peters anomaly, presenting with raised intra-ocular
pressure and elongated axial length compared with the fellow eye. Careful
blunt dissection with visco-elastic devices of iris and lens from the endothelium
can be performed. The area of adhesion will be cloudy after surgery for a few
weeks due to endothelial cell loss, however due to endothelial cell re-shuffling
this will clear.
IS-PED-TH 45 (3)
Systemic Conditions Associated with Congenital Malformations of
the Anterior Segment
Traboulsi Elias
1. Cleveland Clinic
Anterior segment malformations result from abnormalities in development of
the cornea, iris and sometimes the lens. Mutations in transcription factors that
regulate neural crest-derived structures are often responsible, and result in
not only ocular, but also systemic malformations of organs derived from neural
crest. This presentation will review the genetics and clinical manifestations of
several syndromes associated with anterior segment dysgenesis with a focus
on the spectrum of the Axenfeld-Rieger syndrome and its underlying genetic
defects.
68
IS-PED-TH 45 (4)
Persistent Pupillary Membranes
Pollock Travis
1. Sheikh Khalifa Medical City
Persistent papillary membranes are congenital anomalies where failure of
regression of the anterior tunica vasculosa lentis occurs. These membranes
have been associated with refractive error, amblyopia, and other anterior
segment anomalies. Most cases can be managed conservatively. When there
is interference with the visual axis, and the risk of amblyopia is high, they
can be removed surgically (The use of visco-elastic devices, micro-forceps,
vitrectome and YAG laser have all been described). Clinical examples and
surgical techniques will be demonstrated.
IS-PED-TH 45 (5)
Aniridia
Kekunnaya Ramesh
1. L V Prasad Eye Institute
Aniridia is a severe, congenital ocular malformation inherited in an autosomaldominant
fashion with high penetrance and variable expression. Genetic,
clinical and management aspects, in brief will be discussed.
IS-PED-TH 45 (6)
Intracorneal Cyst an Absolute Indication for Corneal Transplant Even
in Unilateral Cases
Moore William
1. Clinical and Academic Department of Ophthalmology, Great Ormond Street
Hospital
In this session, Intra-corneal cysts, a sub-set of Anterior Segment
Developmental Anomaly (ASDA), will be discussed. The pathophysiology
will be explained and the clinical appearance, ultrasound biomicroscopic
appearance and histology will be demonstrated. Management options will be
illustrated and explained with the recommendation that corneal transplantation
is the treatment of choice, even in unilateral cases. Attendees will gain a fuller
understanding of ASDA and the rationale for and confidence to recommend
corneal transplantation for intra-corneal cysts associated with ASDA.
IS-PED-TH 45 (7)
Congenital Malformations of the Angle and Glaucoma
Khan Arif (1,2)
1. King Khaled Eye Specialist Hospital
2. King Faisal Specialist Hospital and Research Center
Primary congenital/infantile glaucoma is the most common congenital angle
malformation on the Arabian Peninsula and is usually caused by CYP1B1
mutations. Other forms and causes of childhood glaucoma also exist; in
addition, there are also conditions that resemble the disease and thus can be
mistaken as glaucoma. From this presentation, the attendee will understand
important forms, causes, and potential mimickers of early childhood glaucoma.
Eye for an Eye
Thu 16 Feb 16:30 - 18:00 Hall 9
IS-OCP-TH 46 (1)
Evisceration in the Modern Age
McCulley Timothy
1. Johns Hopkins School of Medicine
From a technical standpoint, evisceration and enucleation are both good
procedures. Either can be used in most clinical scenarios. Exceptions include
evisceration for intraocular tumors and enucleation for endophthalmitis.
Previously, fears of sypmpathetic ophthalmia (SO) reduced the utilization of
evisceration. However, with critical review of the literature, it is clear that SO
occurs very rarely, if ever, as a consequence of evisceration. Accordingly, due
to its simplicity, efficiency and good cosmetic results evisceration has once
again been gaining popularity.