Retinopathy of Prematurity in Anencephaly:Pathological and Immunohistochemical Studies of Six Eyes from Three Female Stillborn Holoanencephalic Infants

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Kim, Mikyung; Chi, Je G.
Issue Date
Seoul National University College of Medicine
Seoul J Med 1992;33(4):253-258
AnencephalyRetinaVasculogenesisSpindle cellAngioblastic cellS-lOO proteinLamininRetinopathy of prematurityROP
Retinopathy of prematurity (ROP), a disease of developing blood vessels,
is seen predominantly in premature infants requiring oxygen administration. We have
noted various degrees of ROP in 6 eyes from 3 female stillborn holoanencephalic
babies. Their gestational ages ranged from 30 to 35 weeks. All 6 eyes showed marked
hypoplasia of the ganglion cell layer, the nerve fiber layer, and the optic nerve head. At
the junction of vascularized and avascular anterior retina, proliferation of vanguard
spindle cells (in 4 eyes from 2 cases) and of rear guard angioblastic cells (in all 6 eyes)
were noted. Intravitreal vascular proliferation (in 3 eyes from 2 cases) and partial retinal
detachment with vitreous hemorrhage (in 1 eye) were also seen. Immunohistochemical
studies revealed positive staining for 5-100 protein and negative staining for
laminin & Ulex aeropaeus in spindle cells (2/2 cases); the reversed pattern in
angioblastic cells (3/3 cases). The reduced mass of retina generally lacking intact
ganglion cells and their axons in our cases may induce local vasoconstriction in response
to tissue hyperoxia, which result in the retinal hypoxia and consequently, provide
the impetus for the establishment of neovascularization. The immunohistochemical
findings lend support to the neuroglial origin of the vanguard spindle cells.
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College of Medicine/School of Medicine (의과대학/대학원)Dept. of Medicine (의학과)The Seoul Journal of MedicineThe Seoul Journal of Medicine Vol. 33 No.4 (1992)
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