Clinicopathologic correlation of the multiple recurrent serosanguineous retinal pigment epithelial detachments syndrome.
OBJECTIVE
Ocular clinicopathologic correlation in a patient with bilateral multiple recurrent hemorrhagic detachments of the sensory retina and retinal pigment epithelium (RPE), which eventually led to rubeosis and loss of useful vision in one eye, is described. The clinical appearance was most consistent with multiple recurrent serosanguineous RPE detachments syndrome (which may be similar to posterior uveal bleeding, or PUB, syndrome).


METHODS
Light microscopic examination of 450 serial sections through a 3.7-mm area including the macula, optic nerve head, and superior and inferior peripapillary region of the eye that underwent diagnostic enucleation was performed. One fresh cap was used for immunohistochemical staining. Tissue from the posterior aspect of the other cap was prepared for electron microscopy.


RESULTS
Microscopic examination disclosed extensive fibrovascular proliferation in the subretinal space and within Bruch's membrane, and the presence of at least 23 choroidal blood vessels that traversed defects in Bruch's membrane. Immunohistochemical studies of inflammatory infiltrates within the choroid and fibrovascular tissue demonstrated both a B- and T-cell response.


CONCLUSIONS
In this case, recurrent serosanguineous detachments of the sensory retina and RPE were secondary to choroidal neovascularization with extensive fibrovascular proliferation in the subretinal space and within Bruch's membrane. Marked lymphocytic infiltration was present throughout the choroid and the fibrovascular tissue. Clinical improvement in the fellow eye after immunosuppressive therapy suggested that an immunologic component might be a causative factor, although the waxing and waning course in this condition precludes any definitive evaluation for its use in multiple recurrent serosanguineous RPE detachments syndrome.
MacCumber
M W
MW
Eye Pathology Laboratory, Johns Hopkins Medical Institutions, Baltimore, Maryland.
Dastgheib
K
K
Bressler
N M
NM
Chan
C C
CC
Harris
M
M
Fine
S
S
Green
W R
WR
D016570:Bruch Membrane / Q000648:ultrastructure; D002829:Choroid / Q000648:ultrastructure; D015353:Eye Enucleation; D005355:Fibrosis; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D006801:Humans; D008297:Male; D008875:Middle Aged; D010857:Pigment Epithelium of Eye / Q000473:pathology*; D012008:Recurrence; D012163:Retinal Detachment / Q000473:pathology* / Q000601:surgery; D012166:Retinal Hemorrhage / Q000473:pathology; D013577:Syndrome