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T. M. Ranchod, N. Hathaway, M. T. Trese; Microphthalmia, Posterior Lenticonus, Persistent Fetal Vasculature and Coloboma (MPPC): A New Syndrome. Invest. Ophthalmol. Vis. Sci. 2009;50(13):4815.
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© ARVO (1962-2015); The Authors (2016-present)
To report a newly identified syndrome of bilateral microphthalmia, posterior lenticonus, persistent fetal vasculature, and chorioretinal coloboma (MPPC).
Eight patients with MPPC were identified. Clinical data collected retrospectively includes visual acuity, findings on office examination as well as examination under anesthesia, and in some cases fluorescein angiography. Post-operative visual acuity and clinical results were recorded when surgical intervention was performed.
All patients were found to have grossly microphthalmic eyes with corneal diameters less than 8mm. In all cases, the crystalline lens was found to be retrodisplaced with severe posterior lenticonus, and the ciliary processes were frequently drawn across the visual axis to the lens capsule. A stalk of persistent fetal vascular tissue extended from the posterior pole of the lens to the optic disc. Posterior chorioretinal coloboma was present. Some cases also exhibited grossly dysplastic retina. Presentation was frequently asymmetric. Five patients underwent lensectomy, vitrectomy, membrane peeling and fluid-healon exchange in at least one eye, with improved visual function in at least two of the five.
Bilateral microphthalmia, posterior lenticonus, persistent fetal vasculature and chorioretinal coloboma (MPPC) is a distinct syndrome previously unreported.
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