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M. Neudorfer, S. Harel, C. Stolovitch, D. Goldenberg, T. Wygnanski, A. Kessler; The Use of Ultrasound Color Doppler Imaging in the Diagnosis of PHPV . Invest. Ophthalmol. Vis. Sci. 2006;47(13):3303.
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Persistent hyperplastic primary vitreous (PHPV) is a rare congenital, nonhereditary malformation of the eye which results from persistence of the fetal fibrovascular primitive hyaloid system. The classic presentation is that of unilateral leukocoria and microphthalmos. The diagnosis is usually based on clinical examination, and the most important differential diagnosis is retinoblastoma. Since coexistence of PHPV with cataract or retinal detachment can cause opaque media, the use of adjunctive diagnostic procedures is recommended. Computerized tomography, magnetic resonance imaging and conventional ultrasonography can be helpful, but the results are not always conclusive. The need for rapid and precise diagnosis justifies the effort to find more efficacious diagnostic tools. Color Doppler ultrasound imaging is a noninvasive technique which combines the display of color–coded blood flow (depicting direction and velocity) with a conventional gray–scale ultrasound image (depicting the structure).
The purpose of this study was to evaluate the role of color Doppler ultrasound imaging in diagnosing PHPV.
12 eyes of 10 patients (3 boys 7 girls, aged 3 months – 12 years) with PHPV were studied by means of color Doppler ultrasound imaging (ATL–HDI 5000; Philips Medical Systems, Bothell, WA). The diagnosis was confirmed in all cases by histology, surgical evidence or long–term follow–up.
Gray–scale ultrasound showed a membranoid structure crossing the eye from the optic nerve to the retrolental capsule in all cases (PHPV). Color Doppler demonstrated blood flow in all these membranoid structures. Ultrasonography demonstrated calcifications in three cases and large abnormal lens with pathological blood flow in the margins in three other cases.
Color Doppler examination can be a supplementary noninvasive means to assist in the diagnosis of PHPV.
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