PMC8329363_01
The main clinical manifestation of the patient before surgery. (A) shows the obvious cataract in the right eye of the patient. (B) shows the exudative retinal detachment in the inferior part of the retina, accompanied by naevus-like multifocal reddish patches in the superior part of the fundus. Total retinal detachment was observed in the posterior pole of the retina when the patient was in the supine position (C). (D-F) show the similar clinical manifestation of the left eye.
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F1
A slit-lamp examination of the anterior segment of the eyes showed obviously tortuous and dilated episcleral vessels (Supplementary Figure 1), significantly decreased depth of anterior chamber, iris bombe, slight eversion of iris pigment at the pupillary margin and prominent cortical opacity of the lens (Figure 1). There were no signs of synechia, neovascular membranes in the iris, or active inflammation (Figure 1). Color fundus retinal photography revealed typical exudative retinal detachment in both eyes (Figure 1), accompanied by naevus-like multifocal reddish patches under RPE in the superior part of the fundus.
Pathological examination of biopsies collected from the choroid during surgery. (A) shows hematoxylin-eosin (HE) staining of the biopsies, which revealed a predominant spindle-shaped melanocytic cell mass. Immunohistochemical staining showed that the tissue was positive for antibodies against S-100 (B) and negative for antibodies against Ki67 (C).
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F2
Haematoxylin-eosin (HE) staining for biopsies from the choroid suggested predominantly spindle-shaped melanocytic cell masses, while immunohistochemistry showed that the samples were positive for antibodies against S-100, and negative for antibodies against Ki67, LCA, PAX-5, and SOX-10 (Figure 2).
The main clinical manifestation of the patient after surgery. (A) shows a mass of giraffe-like multifocal reddish patches (arrow) in the retina. These patches were consistent with increased autofluorescence (B, arrow), decreased fluorescence in early phase of FFA (D), and with multiple hyperreflective elevated lesions of the retinal pigment epithelium (C, asterisk), as revealed via ocular coherence tomography. It shows significantly deepening of the anterior chamber after surgery (E,F).
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F3
The depth of the anterior chamber had increased and the iris bombe had disappeared (Figure 3). A mass of multifocal reddish patches (Figure 3) was found all around the retina, especially in the mid-peripheral sections. These patches were consistent with the elevated hyper-reflective mass under RPE (Figure 3) in the OCT data, and also with the hyperfluorescence in the autofluorescence images and hypofluorescence in the early FA data.
The UBM images showing the thickness variation during the surgery. (A) shows the thickened ciliary body and choroid before the surgery. (B-D) show the thickness variation of ciliary body and choroid 1, 9, 18 months (6 months after rectectomy) after the surgery, respectively.
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F4
Six months after the rectectomy, the subretinal fluid had completely disappeared and the thickness of the ciliary body and choroid had significantly decreased (Figure 4).