Eyeworld

EW INTERNATIONAL 94 February 2017 by Stefanie Petrou Binder, MD, EyeWorld Contributing Writer As simple as it sounds, real life scenarios can present challenging disease constellations and often pan out differently. Dr. Ram discussed some specific cases he faced treating children with PHPV. Limited anterior PHPV with classic stalk One case involved a 3-year-old child who presented with limited anterior PHPV that obscured the visual axis. Indirect ophthalmoscopy revealed a normal retina and a classic stalk at- tached to the lens. The axial length of the eye was 18.3 mm. Following a CCC and cortical extraction, Dr. Ram performed a primary posterior capsulotomy around the attachment of the stalk. The hyaloid vessels in the stalk were cauterized, followed by limited anterior vitrectomy. In view of the large posterior capsu- lotomy, the IOL was implanted in the ciliary sulcus with capture of the optic in the rhexis margins. The viscoelastic material was aspirated and the ports sutured. back to the optic nerve. This form is commonly associated with cataract. In posterior PHPV, the remnant stalk arises off the optic nerve but does not reach the lens, and therefore is generally not associated with cataract. The most commonly seen clinical presentation of PHPV is the combined form. Cataract surgery in these pa- tients begins like standard cataract surgery. After making side and main port incisions, Dr. Ram performs a continuous curvilinear capsulorhexis (CCC) followed by irrigation and as- piration of the soft cortical material. He begins to cauterize the persistent fetal vessels in a circular fashion to obtain an avascular cleavage plane. He cuts the membrane along this cleavage plane with vertical micros- cissors, creating a posterior capsulo- tomy, and then cauterizes and cuts the stalk with the hyaloid vessels. Finally, he performs a limited ante- rior vitrectomy and can then safely implant an IOL in the capsular bag. lens with varying degrees of opacity, including total white cataract, which can be progressive and worsen with age. There is a characteristic traction of the ciliary processes behind the iris, a shallow anterior chamber, and microphthalmia. "Cataract removal in individu- als with PHPV is a necessary step in cases associated with opacifications along the visual axis," Dr. Ram said in a video presentation at the 2016 American Academy of Ophthalmol- ogy annual meeting. "However, cata- ract associated with PHPV is a chal- lenging surgical situation. In cases of anterior PHPV with visual axis obscuration, surgical outcomes have been dismal. However, by following these basic surgical principles, a fa- vorable outcome can be achieved in a majority of afflicted children." PHPV is usually divided into three types: anterior, posterior, and a combination of the two forms. Anterior PHPV occurs when the remnant stalk is attached to the back of the lens but no longer extends Indian surgeons share expertise for management of persistent hyperplastic primary vitreous P ersistent hyperplastic primary vitreous (PHPV) or persistent fetal vasculature (PFV) is a rare congenital developmental anomaly of the eye resulting from the failed regression of embryological, primary vitreous, and hyaloid vasculature that typically forms a membrane at the back of the lens. Treatment goals include saving the eye from the complications of untreated PHPV, mainly glaucoma and phthisis bulbi (a shrunken, non-functioning eye). Removing the PHPV membrane and reducing the tractional forces on the ciliary body allows surgeons to reduce the chances of phthisis. Preventive lens extraction is an im- portant aspect of treatment in very young patients, to prevent second- ary glaucoma, and to allow visual restoration and rehabilitation in eyes where cataract is significant and the visual axis is obscured. The dense, vascularized mem- brane typically seen behind the lens in these patients may seriously com- promise cataract surgery, however, causing retinal detachment and in- traoperative bleeding that are some- times severe enough to force the surgeon to abandon surgery. Jagat Ram, MD, professor and chairman, Department of Ophthalmology, Ad- vanced Eye Center, and colleagues from the Post Graduate Institute of Medical Education and Research, Chandigarh, India, described a sur- gical technique for the management of PHPV that prevents bleeding and other complications and results in favorable visual outcomes. PHPV: Facts and surgical principles PHPV typically presents unilaterally, with iridohyaloidal vessels seen over the anterior and posterior iris surfac- es and a white papillary reflex due to a dense retrolenticular membrane and/or the existence of cataract. Cataracts range from small opacities to a widespread vascularized plaque, or membrane, on the back of the Surgical approach to PHPV Presentation spotlight An eye with combined persistent hyperplastic primary vitreous (PHPV) with cataract in a 3-month-old child showing vascularized posterior capsule and stretched ciliary processes. Source: Jagat Ram, MD

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