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Author: Steve Charles, MD, Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine

Steve Charles is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Macula Society, and Retina Society

Editors: Russell P Jayne, MD, Consulting Vitreoretinal Surgeon, The Retina Center at Las Vegas; Simon K Law, MD, PharmD, Assistant Professor of Ophthalmology, Jules Stein Eye Institute; Chief of Section of Ophthalmology Surgical Services, Department of Veterans Affairs Healthcare Center, West Los Angeles; Lance L Brown, OD, MD, Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri; Hampton Roy Sr, MD, Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Author and Editor Disclosure

Synonyms and related keywords: massive vitreous retraction, MVR, massive periretinal proliferation, MPP, proliferative vitreoretinopathy, PVR, vitreoretinal surgery

Background

Proliferative vitreoretinopathy (PVR) is the most common cause of failure in retinal detachment surgery. It can occur in untreated eyes or present after pneumatic retinopexy, cryotherapy, laser retinopexy, scleral buckling, or vitrectomy. Because excessive retinopexy and operating on inflamed eyes can cause PVR, some cases should be considered iatrogenic. Epimacular membranes occurring after retinal detachment surgery can be thought of as limited PVR. It can occur from glial or RPE proliferation in diabetic traction retinal detachment cases with retinal breaks. Penetrating or blunt trauma may also result in PVR.

Pathophysiology

PVR is a reparative process initiated by full- or partial-thickness retinal breaks, retinopexy, and other types of retinal damage. Loss of contact inhibition causes the surrounding glial or retinal pigment epithelial (RPE) cells to migrate to both surfaces of the retina. Although the cells exhibit modest mitotic activity, it is largely a hypocellular keloid–like process. Glial or RPE cells migrate further and cover the posterior surface of the detached posterior hyaloid face. Fibronectin-lined coated pits serve as attachments of RPE or glial cells to collagen fibers and other components of the extracellular matrix. The migration/contraction mechanism causes tangential force on the retina via multiple star folds and fixed folds. The collagen of anterior and posterior vitreous cortex contracts because of a similar hypocellular contraction process.

Frequency

United States

Of all retinal detachment surgery cases, 5-10% result in PVR.

International

Worldwide incidence is the same as in the United States.

Mortality/Morbidity

PVR has no associated mortality. Morbidity is limited to blindness in the affected eye if not treated successfully. If several operations are required to repair PVR, poor vision may result even with a successfully attached retina.

Race

No known racial predilection exists.

Sex

No known sexual predilection exists.

Age

Although PVR can occur at all ages, some observers believe that proliferative tissue may develop more rapidly in children.



History

  • Visual loss in the part of the visual field corresponding to the initial area of retinal detachment (chief complaint)
  • Field loss
    • Shadows
    • Loss of portions of the visual field
  • Light flashes

Physical

  • Retinal examination using indirect ophthalmoscopy and/or slit lamp biomicroscopy
    • Retinal detachment
    • Combination of star folds, fixed folds, subretinal proliferation, and vitreous contraction - In some patients, the vitreous contraction component predominates, whereas in other patients, the periretinal proliferation is more apparent. Subretinal proliferation can be annular (napkin ring), placoid, or dendritic (strands). Hypocellular vitreous contraction can involve circumferential fibers, radial preequatorial fibers, and contracted anterior and posterior vitreous cortex.

Causes

  • Retinal breaks directly or indirectly cause most cases of PVR.
    • Direct causation is a result of the loss of contact inhibition of retinal glial cells and RPE cells.
    • PVR may also result from cryopexy, diathermy, and laser retinopexy, especially if excessive.
  • Gas and silicone may contribute to PVR by sequestrating RPE, inflammatory, and glial cells; fibronectin; various cytokines; and fibrin at the retinal surface.



Retinal Detachment, Exudative
Retinal Detachment, Postoperative
Retinal Detachment, Rhegmatogenous
Retinal Detachment, Tractional


Imaging Studies

  • Diagnostic ultrasound is only necessary if opacities of the cornea, the anterior chamber, or the lens; pupillary membranes; retro–intraocular lens (IOL) membranes; or vitreous opacities prevent optical visualization of the retina.

Procedures

  • Indirect ophthalmoscopy and slit lamp biomicroscopy, using a 90-diopter lens, are performed for diagnostic purposes. Contact lens examination can be used with slit lamp biomicroscopy for higher magnification and better resolution.
  • B-scan diagnostic ultrasound is performed if the retina cannot be seen because of corneal disease, cataract, posterior capsular opacification, vitritis, or vitreous hemorrhage.

Histologic Findings

Biopsy is never indicated, and histologic samples should not be obtained during surgery. Minimal mitotic activity with significant interaction between RPE and glial cells and extracellular matrix has been reported in pathologic specimens.



Medical Care

  • Topical and subconjunctival or retrobulbar repository steroids should be used at the time of surgery for all patients who are not steroid glaucoma responders. Using topical and subconjunctival steroids may avoid the adverse effects associated with oral corticosteroids.
  • Intraocular steroids, 5-fluorouracil (5-FU), daunomycin, and low-dose radiation are not effective in this disorder.

Surgical Care

The surgical objective is to enable retinal conformation to the RPE. In cases of moderate star folds without static vitreous traction, scleral buckling without vitreous surgery is indicated. Minimal retinopexy to the breaks should be used to avoid both inflammation and further proliferation. To reduce the incidence of recurrent PVR and inflammation, retreatment of the RPE and excessive retinopexy should be avoided. Post-reattachment retinopexy helps reduce proliferation and overtreatment of the RPE, but it is applicable only to vitrectomy and pneumatic retinopexy settings. Laser probably causes less PVR than cryotherapy, but it is impractical during scleral buckling surgery.

  • Use a broad, moderately high, encircling buckle with a smooth contour for moderate PVR not requiring vitrectomy.
    • Scleral buckling is best achieved with a silicone exoplant and 2-3 mattress sutures per quadrant. The broad buckle extends back to the thicker, stronger, untreated retina and to the ora to prevent anterior leakage. Extrusion and infection rates may be higher with sponges.
    • Posterior scleral bites should be single, long, and circumferential, and as posterior as possible to avoid damage to the vortex veins.
    • The anterior bites should be parallel to the limbus and placed in the scleral condensations at the muscle ring, representing the external landmark of the ora.
    • Because they tie without an assistant and do not slip, 5-0 monofilament nylon sutures are preferred to Mersilene. The ends must be cut flush with the knot to avoid protrusion through the conjunctiva.
    • To achieve reattachment and to create space for the large buckle, complete or near complete drainage of the subretinal fluid (SRF), preferably with the direct needle drainage method, is required.
  • Vitrectomy in aphakic eyes or anterior chamber paracentesis in phakic or pseudophakic eyes may be necessary to achieve volume requirements of the large buckle without increased intraocular pressure (IOP).
    • Air, or preferably expandable gas injection, seals the retinal breaks via a surface tension effect and allows restoration of a pressure gradient and better drainage of the SRF.
    • Air (gas) injection causes lateral displacement of the retina. To avoid retinal incarceration in the drainage site, perform transscleral drainage of the SRF posteriorly, after injection.
  • Perform vitreous surgery when it is anticipated that scleral buckling alone cannot sufficiently compensate for vitreous traction and periretinal membrane contraction to reattach the retina. It is often necessary to remove the lens using trans pars plana lensectomy to permit better removal of the anterior traction and to enable decompartmentalization, which may reduce recurrent PVR. The method of choice is endocapsular lensectomy with the aspirating phacofragmenter and linear (proportional) suction. Experienced surgeons often use phacoemulsification and IOL implantation to contain silicone oil in the vitreous cavity.
    • The anterior and posterior vitreous cortices are usually in contact, resulting in a frontal plane configuration.
    • First, remove the frontal plane component, preferably with the vitreous cutter and minimal linear suction.
    • Resect the radial component (anterior loop traction) of anterior PVR with the suction cutter if sufficient distance exists between the anterior attachment at the pars plana and the posterior attachment of this former peripheral cortical vitreous to the retina at the equator. In some instances, fine curved scissors are required to resect this anterior loop component. The circumferential component can be removed with the vitreous cutter or fine, curved scissors.
  • Epiretinal membrane can be peeled away from the retina when it is minimally adherent. Use end-opening forceps for inside-out peeling of epiretinal membranes; picks, bent needles, microvitreoretinal (MVR) blades, and over/under or side-opening forceps probably cause more trauma to the retina and are not recommended.
  • If the membrane is dense and well developed, it can be delaminated from the retinal surface using both scissor blades between the retina and the membrane.
    • Use segmentation and/or delamination with a fine curved scissors with blades parallel to the retina.
    • Segmentation of the epiretinal membrane in the center of a star fold and between each fold releases the tangential traction. The goal is to release sufficient tangential traction to allow retinal conformation to the RPE with minimal damage to the retina, rather than removing all membranes.
  • If they are creating sufficient contour change in the retina to prevent reattachment, subretinal membranes can be segmented with scissors or removed with forceps. This can be accomplished through a preexisting retinal break, or closed forceps can be used to punch through the retina to create a retinotomy. Subretinal surgery does not require large retinotomies, which create unnecessary damage to the retina and exposure of the RPE.
  • Internal drainage of the SRF should precede fluid-air exchange to enable removal of all posterior SRF through preexisting peripheral retinal breaks.
    • Drainage retinotomies are often helpful.
    • Liquid perfluorocarbons can stabilize the retina during membrane peeling as well as help remove all subretinal fluid.
    • Use of a soft-tip cannula placed through a convenient retinal break or a posterior drainage retinotomy and held near the RPE allows a feasibility test for intraoperative retinal attachment.
    • Appearance of subretinal air indicates the failure to release all tangential forces on the retina and the need for further segmentation, delamination, retinectomy, scleral buckling, or resection. It may also indicate inoperability.
  • Retinectomy
    • If internal drainage of the SRF and fluid-air exchange with continued internal drainage of the SRF results in subretinal air, incremental retinectomy with the vitreous cutter can be effective to release tangential forces on the retina.
    • Retinectomy is indicated when the retina is incarcerated in a wound or previous drain site and when dense membranes are strongly adherent over broad areas of atrophic retina.
    • Perform air-gas exchange or air-silicone exchange after internal drainage of the SRF, fluid-air exchange, and laser endophotocoagulation.
    • Perform confluent moderate intensity endolaser around breaks.
    • Avoid excessive retinopexy and panretinal photocoagulation to reduce tissue damage and reproliferation.
    • Laser indirect ophthalmoscope is not necessary in vitreous surgery and may cause corneal damage and light scatter-mediated macular damage.
  • Gas or silicone oil surface tension management is required in all cases requiring vitrectomy.
    • Internal drainage of subretinal fluid should precede fluid-air exchange and continue as the exchange is completed.
    • Subsequent air-gas exchange allows creation of a complete fill of the vitreous space without hypotony or multiple small bubbles.
    • Using an 18% concentration of C3F8 has been shown to produce better outcomes than a 25% concentration of SF6 because the bubble lasts longer (ie, 3 wk instead of 1 wk). Do not use concentrations greater than these because expansion causes an elevation of IOP with a total fill.
    • The gas mixture is injected through the infusion cannula.
    • Fluid egress is accomplished through a soft-tip cannula positioned through the retinal break, near the RPE, and managed by a foot-controlled linear (proportional) suction system.
  • Surface tension management with highly purified silicone oil (1000-5000 centistokes) is preferable to gas for most advanced or recurrent PVR cases.
    • If a posterior chamber lens is in place, it maintains the silicone posteriorly, avoiding corneal contact and subacute angle-closure glaucoma without an inferior peripheral iridectomy.
    • If the eye is phakic, use phacoemulsification and a posterior chamber lens because cataract formation universally follows extended use of silicone oil.
    • Use an inferior iridectomy in all aphakic eyes to prevent silicone pupillary block.
    • Remove any capsule present with the end-opening forceps to avoid fibrosis at the iridectomy site.
    • If possible, avoid viscoelastics, blood, and inflammation, which increase emulsification of the silicone oil.
    • Patients who are aphakic and have silicone oil are instructed to avoid prolonged supine positioning to reduce subacute angle-closure glaucoma and corneal contact.
    • In most cases, silicone oil is not removed. Performing multiple procedures just to enable silicone removal is not indicated, especially in older patients and in patients who are pseudophakic.
    • Silicone is used for rhegmatogenous confinement and facilitates stabilization of recurrent inferior and peripheral retinal detachments.
    • Remove silicone only if all breaks are sealed by retinopexy.

Consultations

General ophthalmologists should refer PVR cases to a vitreoretinal surgeon.

Activity

  • The ultimate strength of retinopexy lesions is reached 7-10 days after application.
  • Gas and silicone bubbles float in aqueous humor; therefore, head positioning is crucial to developing adherence in the 10-day period after surgery.
  • C3F8 has been shown to be more effective than SF6 in management of PVR. Apparently, it is because the bubble duration is typically 3 weeks with C3F8, while it is only 1 week with SF6.
  • Bed rest is not required.
  • Patients with inferior retinal breaks must be prone for 7-10 days after surgery using either silicone or gas.



Subconjunctival triamcinolone is indicated at the end of surgery, unless the patient is a steroid glaucoma responder. Topical prednisolone acetate is the topical drug of choice and should be used at least 4 times per day.

Drug Category: Corticosteroids

Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.

Drug NameTriamcinolone (Aristocort)
DescriptionDecreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Longer duration creates additional steroid glaucoma risk in patients who are steroid responders.
Adult Dose20 mg subconjunctival
Pediatric Dose10 mg subconjunctival
ContraindicationsDocumented hypersensitivity; fungal, viral, and bacterial skin infections; glaucoma; some cases of herpetic keratitis; any evidence of infection
InteractionsNone reported
PregnancyB - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
PrecautionsIf infection develops and is not responsive to antibiotic treatment, discontinue until infection is under control

Drug NameBetamethasone (Diprolene, Betatrex)
DescriptionFor inflammatory dermatosis responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability.
Adult Dose6 mg subconjunctival
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; paronychia; cellulitis; impetigo; angular cheilitis; erythrasma; erysipelas; rosacea; perioral dermatitis; acne
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsIf infection develops and is not responsive to antibiotic treatment, discontinue until infection is under control

Drug NamePrednisolone acetate (Pred Forte, AK-Pred)
DescriptionUsed to suppress inflammation. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reducing capillary permeability.
Adult DoseSolution: Instill 1-2 gtt into conjunctival sac q1h during day and q2h noct; once desired response is obtained, use 1 gtt q4h; may reduce to 1 gtt tid/qid to control symptoms
Suspension: Shake well before using and instill 1-2 gtt into conjunctival sac 2-4 times/d; if necessary, may increase dosing frequency during initial 24-48 h
Pediatric DoseAdminister as in adults
ContraindicationsDocumented hypersensitivity; viral, fungal, or tubercular infections
InteractionsNone reported
PregnancyC - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
PrecautionsCaution in hypertension; known to cause cataract formation with long-term use



Further Inpatient Care

  • Outpatient surgery for vitreoretinal surgery is the criterion standard. Concomitant medical conditions should determine the need for an inpatient approach.

Further Outpatient Care

  • All patients must be examined on the first postoperative day to determine if increased IOP, flat chamber, incorrect patient positioning, or endophthalmitis (a rare finding) is present. Most patients are then examined in 1-3 weeks.

In/Out Patient Meds

  • Antibiotics: Topical fourth-generation fluoroquinolones (Vigamox) are used 4 times/day for approximately 1 week after surgery. All patients receive subconjunctival cefazolin (vancomycin if allergic to penicillins) and subconjunctival ceftriaxone at the end of surgery. Systemic antibiotics are not indicated.
  • Cycloplegics: Topical cycloplegics, such as Cyclogyl 1%, are used 2-3 times/day for 2-3 weeks after surgery.
  • Subconjunctival steroids, such as Kenalog (triamcinolone acetonide), are used in all patients except those who are steroid responders. The author never uses systemic steroids in these patients.
  • Topical steroids are used in all patients who are not steroid responders. The drops are administered 4 times/day. Prednisolone acetate 1% is the preferred agent.

Transfer

  • General ophthalmologists should transfer patients with PVR to vitreoretinal surgeons.

Deterrence/Prevention

  • Excessive retinopexy (especially cryopexy), operating on inflamed eyes, bleeding, iris trauma, excessive operating times, retained lens material, viscoelastics, and excessive operative trauma contribute to recurrent PVR.

Complications

  • Recurrent PVR is the most common complication, occurring at a frequency of 25-50%.
  • Cataracts may occur from prolonged gas or silicone oil contact with the lens.
  • Uveitis may occur from excessive retinopexy, lengthy surgery, iris trauma, or retained lens material.
  • Intravitreal, anterior chamber, subretinal, or suprachoroidal hemorrhage may occur.
  • Glaucoma secondary to uveitis, excessive gas bubbles, pupillary block, silicone oil emulsification, surgical trauma to vortex veins or aqueous veins, or steroid glaucoma may occur.
  • Ocular or periocular manifestations
    • Retina - Epiretinal membranes, fixed folds, star folds, and subretinal placoid or dendritic proliferation
    • Vitreous - Condensation, contraction, pigmentation, and posterior vitreous detachment
    • Other - Visual loss

Prognosis

  • The anatomical success rate is dependent on the patient mix, the technique used, unknown patient factors, and the surgeon's skill. The success rate varies from 50-90%.
  • The visual prognosis is dependent on duration and height of the detachment, media clarity, epimacular membranes, and other unknown factors.

Patient Education

  • Inform the patients about positioning, activity, visual prognosis, complications, medications, anesthesia risk factors, and anatomical and visual success rates.



Medical/Legal Pitfalls

  • The patient and the family should be informed about the relatively high recurrence rates, poor visual prognosis, complications, and treatment.
  • Refractive error changes are common after scleral buckling surgery, lens removal, and long-term use of silicone oil.



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Retinal Detachment, Proliferative excerpt

Article Last Updated: Aug 20, 2007