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Slide Seminar
Contemporary ophthalmic pathology

September 1st, 14.15-16.15 Room 5B
Chairpersons: Sarah Coupland (UK) and Rui Proenca (Portugal)
Case 1
Presented by: Tero Kivelä, Helsinki, Finland

While watching television, a 24-year-old man aspiring to become an air steward noticed a sudden cloud in his visual field, left eye. The next day, visual acuity was 1.0 (20/20), right eye, and 0.1 (20/200), left eye. The fundus showed preretinal and vitreous haemorrhage, a congested optic disc, retinal microinfarctions (cotton-wool spots), venous beading, multiple dot-and-blot haemorrhages in all quadrants, and myriad tiny glistening white spots all over the retina, left eye. The fundus on the right eye was similar, except that no vitreous bleeding was present. Fluorescein angiography showed an enlarged foveal avascular zone, intraretinal microvascular abnormalities (IRMA), a wide peripheral field of total capillary occlusion with only some larger retinal arterioles and venules remaining, and multiple retinal neovascular vessels bordering the non-perfused retina.

Bilateral panretinal photocoagulation was initiated. After two further months, vision worsened to 0.2 (20/100), right eye, because of vitreous haemorrhage. Right eye was scheduled for vitrectomy, and the left eye received a further 2000 laser spots. Within two weeks, vision dropped to 0.4 (20/50) in the left eye and a fibroproliferative reaction was rapidly developing. Vitrectomy proved difficult. The membranes were tightly adherent and bled profusely.

After one month, the patient suddenly lost light perception from the right eye and a central retinal arterial occlusion was diagnosed. Vision of the left eye had improved to 0.4 (20/50). Progressive vitreoretinal traction led to second vitrectomy. Soon thereafter, the patient died at home. Autopsy revealed pneumoconiosis, hepatic cirrhosis and splenomegaly.
Slide 1Slide 2Clinical Right eye initialClinical Left eye initialClinical Right eye follow-upMacro Right eyeMacro Left eye
HE RetinaHE Retina partially polarized lightHE Choroid partially polarized light
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Case 2
Presented by: Rob verdijk, Rotterdam, Netherlands

65 year old female with a papillary lesion of the right caruncle.
Slide 1
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Case 3
Presented by: Paul Vandervalk, The Netherlands

The patient was a 56 year-old woman who presented with yellow plaques in the palpebral conjunctiva of both eyes. There were no other complaints.
Slide 1
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Case 4
Presented by: Sarah Coupland, Liverpool, United Kingdom

In March 2011, a 53 year-old male presented to his GP with a history of decreasing vision and photopsia over a four-week period. He was referred to the Liverpool Ocular Oncology Centre in May 2011. On examination, his best corrected visual acuity (BCVA) of the left eye was 6/6, and 6/38 in the right eye. On fundoscopy, there was a large infero-nasal collar stud tumour with an associated exudative retinal detachment. There was a mild increase in the IOP of the same eye with patchy discolouration of the anterior surface of the iris. Treatment options were discussed with the patient, and he chose enucleation
Slide 1
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Case 5
Presented by: Luciane Irion, Manchester/Liverpool, United Kingdom

A 2-year-old boy presented with a right lateral canthal lesion growing for 3 weeks. On palpation, the lesion was firm and reasonably mobile however partial lateral attachment to periosteum could not be excluded clinically. He kept full range of eye movements and the anterior segment was unremarkable. He had no visual problems. There was no past history of local trauma. His medical history included asthma which was successfully controlled with inhalers.

With a clinical diagnosis of a possible dermoid cyst, surgical excision was scheduled without radiological imaging of the orbits as this would require general anaesthetics given the restlessness of the little boy.

The specimen excised was a pale, firm nodular tissue, measuring 30 x 23 x 18 mm. The cut surface was greyish with homogeneous appearance.
DesminSMA 1S100Clinical presentation
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Case 6
Presented by: Stefan Seregard, Stockholm, Sweden

A 39-year old woman had originally migrated from Thailand 2.5 years earlier and then had a history of human immunedeficiency virus (HIV) infection and active hepatitis B. Some 5 months after arrival in Sweden, she developed a disseminated cryptococcal infection including cryptococcal meningitis. Some ten months later she had ophthalmological examination because of blurred vision and was found to have 2 mm central infiltrate of the left eye corneal stroma. She received therapy for a presumed fungal infection, but repeat cultures and polymerase chain reaction (PCR) DNA sequencing intended to detect a range of infectious causes were negative. Some slight regression was noted after one month, but two small corneal biopsies were negative. The patient was then referred for a deeper (about one third of corneal thickness) biopsy.

The removed, approximately 1 x 0.5 x 0.3 mm, specimen was submitted in formaldehyde and routinely processed. Paraffin-embedded sections were stained with a range of histochemical stains using by standard protocols. The tissue was lined by a slightly thinned but regular corneal epithelium. In some areas, the lining epithelium and the sub-epithelial Bowman’s membrane were missing. The superficial corneal stroma was partially necrotic and abundant small (2-3mm in diameter) microorganisms were present in the stroma. Special stains for fungus (Grocott) were negative, but organisms stained with the PAS and Giemsa and trichrome stains. Deparaffinized tissue was used for transmission electron microscopy and sent to the Centre for Disease Control (CDC), Atlanta, GA.
Slide 1GiemsaMicrospor Giemsa 3Microspor trichromBild1
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Case 7
Presented by: Rui Proenca, Coimbra, Portugal

A 23-months-old Caucasian boy from Madeira Island was referred to the Ophthalmology Department with a clinical picture of leukocoria and strabismus. He was otherwise healthy after a normal delivery from unrelated parents, and there was no history of genetic disturbances in the family. Ophthalmoscopy under general anesthesia revealed a localized retinal detachment and an elevated whitish mass with diffuse limits occupying the posterior pole of the left eye. Ultrasonography revealed a heterogeneously echogenic mass measuring 3mm in height, as well as highly reflective signals consistent with intra retinal calcifications. Computed tomographic (CT) confirmed the presence of foci of calcification.
Slide 1. Clinical presentationSlide 2. UltrasonographySlide 3. MRISlide 4 (H.E.)Slide 5 (H.E.)Slide 6 (H.E.)Slide 7 (H.E.)
Slide 8 (H.E.)Slide 9 (H.E.)Slide 10 (H.E.)
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Case 8
Presented by: Lina Carvalho; Cristina Fonseca; Rui Proença, 3000 Coimbra, Portugal

We report the case of a 5-year-old boy referred to the ophthalmology department with asymptomatic decreased visual acuity in the left eye, incidentally discovered during a routine medical examination. There was no family history of any ophthalmological conditions. On the ophthalmological examination, left eye best corrected visual acuity was hand motion perception and an asymmetrical pupillary red reflex was found in the left eye, with no leukocoria. Ocular motility and alignment were preserved and no strabismus was documented. Dilated indirect ophthalmoscopy showed a vitreous hemorrhage, multifocal tumoral lesions and total exsudative retinal detachment. An examination under anesthesia was performed in order to permit a complete assessment of the extent of the ocular disease. A large exophytic, chalky white mass, with focal calcifications was identified beneath the retina, in the nasal superior quadrant, associated to serous retinal detachment and multiple smaller tumoral foci in the posterior pole and inferior quadrants. Vitreous hemorrhage and seeding was also present. The ultrasonography performed confirmed the clinical findings and demonstrated typical calcifications within the tumor. Gadolinium-enhanced MRI excluded orbital and intracranial extension. Before these clinical and imagiologic findings, the diagnosis of retinoblastoma was made and this case staged in group Vb, according to Reese-Ellsworth Classification of Retinoblastoma for Eye Preservation. The patient underwent 9 cycles of systemic chemotherapy to reduce tumoral volume and was subsequently enucleated. Close follow-up has been maintained since, according to the adequate protocol, as well as, orbital cavity rehabilitation performed with external prosthesis adaptation. The patient is currently an 11-year-old boy who is annually examined with dilated indirect ophthalmoscopy of the contralateral eye, with no signs of recurrence.
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