CASE REPORT

Sickle cell-hemoglobin C retinopathy: transient obstructionof retinal and choroidal circulations and transient dryingout of retinal neovessels

Alessandro Mantovani Æ Innocente Figini

Received: 16 January 2008 / Accepted: 22 January 2008 / Published online: 23 February 2008

� Springer Science+Business Media B.V. 2008

Abstract We present a case of sickle cell-hemo-

globin C disease that presented acute retinal and

choroidal peripheral non-perfusion on the base of

chronic microvascular obstruction, which transiently

closed retinal neovessels.

Keywords Fluorescein angiography �Indocyanine green (ICG) angiography �Sickle C retinopathy � Staurenghi scanning

laser ophthalmoscope (SLO) retinal lens

Case report

A 40-year-old African man presented with vitreous

hemorrhage in his left eye. In his right eye, fundus

examination showed mid-peripheral and peripheral

retinal vascular occlusion.

Fluorescein fundus angiography (FFA) performed

with the scanning laser ophthalmoscope (SLO) Sta-

urenghi retinal lens, giving panoramic images, showed

abrupt vascular stops (Fig. 1), shown in more detail

with 55 field images (Figs. 2 and 3). Indocyanine green

angiography (ICGA) performed with the SLO

Staurenghi retinal lens showed additionally patchy

areas of hypofluorescence corresponding to chorio-

capillaris non-perfusion in the superior hemi-fundus

(Fig. 4).

FFA and ICGA were repeated after 1 week, with the

same Heidelberg HRA 2 camera, and FFA showed

reperfusion of the acutely closed vessels (Figs. 5 and

6). However, pre-existing zones of retinal capillary

non-perfusion were present in the equatorial zone.

More interestingly, the image showed two areas of

retinal neovessels (Fig. 7) that had not been present

when the preceding FFA had been performed 1 week

earlier during acute vascular closure because the feeder

vesssels of the retinal neovascular nets were not

perfused (Fig. 3). Wide-angle ICGA performed

1 week later showed quasi-complete resolution of the

choriocapillaris non-perfusion (Fig. 8). In the mean-

time, hemoglobin electrophoresis had been performed

and showed sickle cell hemoglobin C disease.

Comment

Fluorescein angiographic findings occurring in sickle

cell retinopathy include peripheral retinal vascular

occlusion with peripheral anastomoses, capillary

dilatation and sea-fan retinal neovascularization

along the posterior border of the zones of perfused

and non-perfused retina [1, 2, 3].

Another finding is evidence of occlusion of one or

more paracentral arterioles [4].

A. Mantovani (&) � I. Figini

Department of Ophthalmology, Valduce Hospital, Como,

Italy

e-mail: oculistica@valduce.it

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Int Ophthalmol (2008) 28:135–137

DOI 10.1007/s10792-008-9199-1

In our case all these characteristics were present,

but the peculiarity was that retinal neovessels were

transiently undetected on the FFA because they were

transiently not perfused due to acute non-perfusion in

addition to chronic non-perfused areas. When the

acute vascular closure resolved, retinal neovessels

were again able to leak. Another interesting finding

was the presence of choriocapillaris non-perfusion,

shown by ICGA, which also resolved after the acute

transient occlusive episode.

Fig. 2 Posterior pole. Several vascular segments are not

perfused, and there is macular ischemia

Fig. 3 Temporal mid-periphery vascular occlusion, with drying

out of retinal neovessels

Fig. 4 ICGA picture taken with the Staurenghi retinal lens.

Numerous patchy areas of hypofluorescence (choriocapillaris

non-perfusion) are seen in the superior hemi-fundus

Fig. 1 Picture taken with the Staurenghi retinal lens. Com-

plete vascular occlusion of the mid-peripheral and peripheral

retinal vasculature is seen

136 Int Ophthalmol (2008) 28:135–137

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References

1. Gass JDM (1997) Stereoscopic atlas of macular diseases:

diagnosis and treatment, 4th edn. Mosby, St. Louis,

pp 530–534

2. Hingorani M et al (1996) Retinopathy in hemoglobin C trait.

Eye 10(Pt 3):338–342

3. Galinos SO et al (1975) Spontaneous remodeling of the

peripheral retinal vasculature in sickling disorders. Am

J Ophthalmol 79(5):853–870

4. Asdourian GK et al (1976) Macular and perimacular

vascular remodelling in sickling hemoglobinopathies. Br J

Ophthalmol 60:431–453

Fig. 5 Picture taken with the Staurenghi retinal lens showing

retinal re-perfusion in the posterior pole, with capillary non-

perfusion in the equatorial zone corresponding to chronically

non-perfused areas

Fig. 8 ICGA picture taken with the Staurenghi retinal lens

shows a few residual patchy areas of hypofluorescence in the

superior hemi-fundus and peripheral hypofluorescence over

360�, corresponding to the chronically non-perfused zones

Fig. 7 Temporal periphery, showing equatorial ischemia with

dilatated capillaries at the junction of the perfused and non-

perfused retina and retinal neovessels

Fig. 6 Normal perfusion of retinal circulation in the posterior

pole

Int Ophthalmol (2008) 28:135–137 137

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