Surgical approach to PHPV

International: Presentation spotlight
February 2017

by Stefanie Petrou Binder, MD
EyeWorld Contributing Writer

Indian surgeons share expertise for management of persistent hyperplastic primary vitreous

Persistent 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 important aspect of treatment in very young patients, to prevent secondary glaucoma, and to allow visual restoration and rehabilitation in eyes where cataract is significant and the visual axis is obscured.

The dense, vascularized membrane typically seen behind the lens in these patients may seriously compromise cataract surgery, however, causing retinal detachment and intraoperative bleeding that are sometimes severe enough to force the surgeon to abandon surgery. Jagat Ram, MD, professor and chairman, Department of Ophthalmology, Advanced Eye Center, and colleagues from the Post Graduate Institute of Medical Education and Research, Chandigarh, India, described a surgical technique for the management of PHPV that prevents bleeding and other complications and results in favorable visual outcomes.

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

PHPV: Facts and surgical principles

PHPV typically presents unilaterally, with iridohyaloidal vessels seen over the anterior and posterior iris surfaces 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 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 individuals 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 Ophthalmology annual meeting. “However, cataract associated with PHPV is a challenging surgical situation. In cases of anterior PHPV with visual axis obscuration, surgical outcomes have been dismal. However, by following these basic surgical principles, a favorable 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 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 patients begins like standard cataract surgery. After making side and main port incisions, Dr. Ram performs a continuous curvilinear capsulorhexis (CCC) followed by irrigation and aspiration 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 microscissors, creating a posterior capsulotomy, and then cauterizes and cuts the stalk with the hyaloid vessels. Finally, he performs a limited anterior vitrectomy and can then safely implant an IOL in the capsular bag.

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 attached 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 capsulotomy, 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.

Diffuse white cataract with PHPV

Dr. Ram described a second scenario involving a 6-month-old infant with a diffuse white cataract. Ultrasonography revealed the presence of a stalk extending between the optic disc and the lens, suggesting the diagnosis of combined PHPV. The axial length was 17 mm. After the removal of the cortical lens material, a densely vascularized membrane with a stalk was visible on retro illumination. The persistent fetal vessels on the membrane were cauterized with the help of endocautery to create an avascular plane. Using vertical microscissors, the membrane was cut 360 degrees, thus creating a posterior capsulotomy. The hyaloid vessels of the stalk were endocauterized and the remnants of the membrane taken out, followed by an anterior vitrectomy to release vitreous traction. Corresponding anterior and posterior capsulorhexis allowed the implantation of a single- piece IOL into the capsular bag. Dr. Ram explained that since PHPV was mostly unilateral, IOL placement was highly desirable to avoid amblyopia arising from unilateral aphakia.

Densely vascularized membrane with prominent ciliary processes and thin stalk

A third scenario was that of a 2-year-old child presenting with a densely vascularized membrane with large prominent ciliary processes. Cutting the CCC is these cases can be challenging because of the tension and size of the capsule. The persistent fetal vessels in the membrane were cauterized in a confluent pattern to create an avascular cleavage zone, and a posterior capsulotomy was created. Dr. Ram was careful at this point not to cut through the tense stalk of fetal vessels. After completing the posterior capsulotomy, the fetal vessels in the stalk were cauterized and the anterior vitrectomy completed to obtain a clear visual axis. An IOL was then implanted into the capsular bag.

“Postoperatively, these eyes should be managed on a higher frequency of topical steroids, along with cycloplegics and antibiotics. Residual refractive error, if any, should be corrected and intensive amblyopia therapy should be initiated. Children with PHPV require a long-term follow-up to monitor for visual axis opacification, glaucoma, and posterior segment complications,” Dr. Ram said.


Editors’ note

Dr. Ram has no financial interests related to his comments.

Contact information

Ram: drjagatram@gmail.com