Rhegmatogenous Retinal Detachment
an Ophthalmologic Emergency
Background: Rhegmatogenous retinal detachment is the most common retinological emergency threatening vision, with an incidence of 1 in 10 000 persons per year, corresponding to about 8000 new cases in Germany annually. Without treatment, blindness in the affected eye may result.
Method: Selective review of the literature.
Results: Rhegmatogenous retinal detachment typically presents with the perception of light flashes, floaters, or a “dark curtain.” In most cases, the retinal tear is a consequence of degeneration of the vitreous body. Epidemiologic studies have identified myopia and prior cataract surgery as the main risk factors. Persons in the sixth and seventh decades of life are most commonly affected. Rhegmatogenous retinal detachment is an emergency, and all patients should be seen by an ophthalmologist on the same day that symptoms arise. The treatment consists of scleral buckle, removal of the vitreous body (vitrectomy), or a combination of the two. Anatomical success rates are in the range of 85% to 90%. Vitrectomy is followed by lens opacification in more than 70% of cases. The earlier the patient is seen by an ophthalmologist, the greater the chance that the macula is still attached, so that visual acuity can be preserved.
Conclusion: Rhegmatogenous retinal detachment is among the main emergency indications in ophthalmology. In all such cases, an ophthalmologist must be consulted at once.
Retinal detachment is the term used to describe detachment of the neurosensory retina from the underlying membrane, the retinal pigment epithelium. The separation of the two layers takes place within the fissure formed by the invagination of the optic cup (e1).
Three forms of retinal detachment are distinguished:
- The most frequent is the rhegmatogenous form of detachment, in which a retinal tear allows liquefied vitreous humor to penetrate under the retina (Figure 1).
- In the far less common tractional form, the retina is pulled away from the substrate by cord-like scars, e.g., fibrosing proliferation membranes in diabetic retinopathy.
- Much less frequent again is exudative retinal detachment; where the underlying cause is a barrier dysfunction, for example in the case of intraocular tumors or exudative vascular diseases.
The most common cause of rhegmatogenous retinal detachment is degeneration of the vitreous body. The vitreous is made up almost entirely (98%) of water and is stabilized by collagen fibrils that extend into the superficial (internal) layers of the retina (1, e2). Physiological degeneration of this vitreous scaffold has been demonstrated as early as the first few years of life (e3, e4). In the course of time the collagen fibrils harden, sometimes leading to perception of the mobile dots and threads known as muscae volitantes or “floaters” (e1). The progressive loss of elasticity eventually results in separation of the vitreous from the retina (Figure 2a). This process is described as “posterior vitreous detachment.” In this context, the risk that a tear will arise in the retina is most acute when the vitreous body is still attached to the retina at one or more points and its weight exerts traction (Figure 2b). Because the vitreous usually begins to separate from the retina at the posterior pole of the eye, extending to the so-called equator, the tension on the retina is particularly strong in this region. The equator marks the transition of the central to the peripheral retina (Figures 1, 2a, 3) and is the point where the retina is at its thinnest (0.18 mm versus 0.23 mm at the center) (e5), which explains the predilection for tension-related holes in the retina (Figures 1, 3, 4). Every fifth patient with posterior vitreous detachment develops a retinal hole (e6).
The incidence of rhegmatogenous retinal detachment in the general population in Europe is ca. 1 in 10 000, corresponding to around 8000 new cases each year in Germany (2, e7, e8). The danger is greatest in the age range 55 to 70 years. The risk of retinal detachment in the second eye is between 3.5% and 5.8% in the first year and 9% to 10% within 4 years; existing detachment in one eye is therefore the most frequent risk factor (2). There are typical risk factors that increase the danger of rhegmatogenous retinal detachment, principal among them shortsightedness, cataract surgery, and trauma. The higher incidence of retinal detachment in patients with these risk factors is attributed to points of particularly strong adhesion between the vitreous body and the retina (2).
After reading this article, the reader should be able to:
- Interpret the possible symptoms of retinal detachment
- Name the treatment options
- Observe the rules of aftercare and recognize the typical postoperative features.
We searched PubMed, Embase and the Cochrane Registry using the terms “retinal detachment,” “rhegmatogenous retinal detachment,” “scleral buckling,” “vitrectomy,” and “risk factors” and then made a representative (in our view) selection of the publications identified.
Shortsightedness of up to –3 diopters (D) quadruples the risk of retinal detachment, and myopia of more than –3 D increases the danger of detachment tenfold. Myopia also leads to earlier vitreous liquefaction, which explains why retinal detachment generally occurs earlier in shortsighted patients than in those without refraction defects (3, e8–e11). In various study groups, around 50% of all patients with rhegmatogenous retinal detachment were myopic (e12, e13). Myopia is a particularly relevant risk factor because it is increasingly more common among children (4, e14); every third European adult is now shortsighted (e14).
Another risk factor for rhegmatogenous retinal detachment is operative insertion of an artificial lens. Cataract surgery accelerates liquefaction of the vitreous humor, explaining the higher incidence of detachment. Six years after cataract surgery the risk of detachment is sevenfold, and the danger grows as the postoperative interval increases (e15). The risk of suffering rhegmatogenous retinal detachment after an uncomplicated cataract operation is approximately 1/1000 (2). Around 30% of patients with retinal detachment have a history of cataract surgery (3, 5–7, e16, e17). Some 650 000 cataract operations are carried out each year in Germany (8). Recent findings suggest that demographic developments will lead to an increase in the proportion of those with such surgery among patients with retinal detachment (8). However, the considerable technical advances in cataract surgery in the past few years make it difficult to predict future effects. The increased risk of retinal detachment should be explained to cataract patients before operation, but it should not be a reason for abandoning surgery that is otherwise indicated.
The sudden acceleration of the vitreous body in blunt ocular trauma may lead to extensive tearing of the retina around the base of the vitreous far out in the periphery; alternatively, small holes may arise in the fundus of the eye. The rate of traumatic retinal detachment is comparatively low, at 0.2/10 000 (2).
Ophthalmologists are often asked whether pregnant women with myopia or retinal detachment can be advised to give birth naturally or whether a cesarean section would be preferable. There is now a clear answer to this question: Provided the retina is currently attached, neither shortsightedness nor a history of rhegmatogenous retinal detachment speaks against natural childbirth (9, e18).
A subject of ongoing investigation is whether oral intake of fluoroquinolones (particularly ciprofloxacin) leads to increased incidence of retinal detachment. In a Canadian database study, the rate of detachment during drug intake was 5 times higher than in a control group (10). Over the course of the 8-year observation period (from 2000 to 2007), a cohort of almost a million persons was evaluated. A total of 4384 experienced a retinal detachment during this time. The proportion of persons who had taken fluoroquinolones was 3.3% in the detachment group versus 0.6% in the control group (n = 43 840). This possible effect is explained by accelerated vitreous liquefaction with subsequent retinal tearing. No prospective studies on this topic have been published. To date, the data do not justify a recommendation to avoid taking fluoroquinolones, even in patients with recognized risk factors for retinal detachment; however, patients should be advised of the potential dangers.
Clinical findings and examination
Most patients report abnormal visual phenomena before the actual detachment of the retina. These can take the form of newly occurring opacities described as cobwebs or threads, sometimes as a swarm of midges. Occasionally the patient perceives flashes of light that can be provoked by changing the direction of gaze. Sometimes the patient has difficulty determining which eye is affected. If the retina then becomes detached, the patient perceives a light to dark gray shadow; in rare cases the shadow is completely black. In contrast to vitreous opacity, this shadow does not move when the direction of gaze changes. If the retinal detachment extends to the optic fovea or the visual axis is occluded, considerable worsening of vision ensues. Occasionally vascular tears result in vitreous hemorrhages, again leading to impairment of vision. Examination of the background of the eye (funduscopy) takes in the entire retina from the posterior pole to the ora serrata. A detachment is recognized by the dune-like appearance and mobility of the retina, and the hole responsible for the detachment can often be discerned (Figures 3, 4). The hole may be more difficult to find, however, particularly after cataract operations; in ca. 5% to 20% of patients with retinal detachment following cataract surgery the very small and peripherally located holes are overlooked preoperatively (e19, e20).
Attentive patients usually notice the visual symptoms very quickly, but do not always recognize their importance or attach much urgency to them. Most patients present with a detached macula and therefore have an unfavorable prognosis from the outset (11–13, e21). It has been estimated that between 50% and 70% of patients present too late because they did not recognize the typical symptoms of detachment; this is independent of educational level (e22, e23). It is therefore especially important to ensure that high-risk patients are informed accordingly.
Typically, retinal detachment is treated by mechanical and scar-induced sealing of all holes in the retina. Jules Gonin was the first to recognize that hole closure forms an essential part of the treatment of retinal detachment (e24–e27). Several different procedures are now available and can be used singly or in combination: laser coagulation or cryocoagulation for scar induction and scleral buckling or vitrectomy to close the holes.
In laser coagulation the laser light enters the eye via the pupil. The laser energy is absorbed in the retinal pigment epithelium, leading to heat (ca. 60 °C) and coagulation necrosis (e28, e29). Cryocoagulation involves freezing of the eyeball all the way from the outside to the retina by application of a cryo probe (ca. –80 °C). Both procedures are followed after a few days by formation of a scar, but only if the retina is in contact with the underlying retinal pigment epithelium. Therefore, scar induction by either laser coagulation or cryocoagulation is effective only for prevention of detachment in a still-attached retina; both forms of coagulation are pointless if detachment has already occurred.
The procedures employed for surgical management of retinal detachment are scleral buckling and vitrectomy. Here too laser coagulation or cryocoagulation is used for hole closure, but only after surgery to repair the detachment. Data for both of these surgical options are available from recent prospective randomized clinical trials.
After precise localization of all retinal breaks and marking of the sclera, the holes are treated with cryopexy for scar induction. The traction exerted on the holes by the vitreous body is then reduced by a foam sponge sutured to the sclera (14) (Figure 5).
In certain configurations of retinal holes or in the presence of multiple breaks, a silicone band can be placed around the whole eyeball; this is known as encircling band. When the buckling has abolished the traction effect on the holes, the retinal pigment epithelium absorbs the subretinal fluid and the retina becomes reattached in the space of a few days. Depending on the situation, a single scleral buckling procedure achieves reattachment rates of ca. 85% to 90% (11, 13, 15–17, e30–e32). A frequent complication of scleral buckling procedures is deformation of the eyeball with changes in refraction. In practice this is a problem only with cerclage, hardly ever occurring with a sponge (e33, e34). Double vision and eye movement problems are each reported in around 15% of cases early after operation (18). Occasionally the sponge becomes infected (0.3% [e35]) or migrates into the eyeball (<0.01% ). In the vast majority of cases the symptoms recede after a few days or weeks, so that neither sponges nor cerclages are removed.
Vitrectomy begins with the removal of the vitreous humor causing the retinal detachment, followed by displacement of the subretinal fluid by means of a heavy tamponade (perfluorodecalin or perfluorocarbon) and scarring of the retina by laser coagulation or cryocoagulation. The vitreous is then replaced by a tamponade (Figure 6), which holds the retina against the underlying retinal pigment epithelium until a firm scar has formed around the retinal hole. A mixture of air and gas or a silicone oil tamponade can be chosen for this purpose. The air/gas mixture is usually chosen in simpler situations (e.g., when the hole is at the top of the eyeball). The advantage of the air/gas tamponade is that it is absorbed and thus does not require removal. The disadvantage is that the mixture expands postoperatively (due to warming and uptake of nitrogen from the blood), with the danger of pressure decompensation, so the patient should avoid changes in altitude—not only flights but also mountain crossings. Furthermore, air/gas mixtures result in a massive change in refraction of ca. –50 D (e36). The gases most frequently used are sulfur hexafluoride (SF6), perfluoroethane (C2F6), and perfluoropropane (C3F8). How long the gas remains in the eye depends on which gas is chosen, how much of it is injected, and on the intraocular pressure. On average the gases remain in the eye for between 14 days (SF6) and 2 months (C3F8) (19, e37).
In complicated situations silicone oil can be used as tamponade. The advantage of oil is that the tamponade is stable, without expansion, while the disadvantage is the necessity for surgical removal. Moreover, oil causes a change in refraction of around +6 D, leading to blurred vision. Reattachment rates of 85% to 90% are also reported for vitrectomy (1, 5, 11, 13, 16, 17, 20, e30–e32). The frequent complications are cataract in the first year after surgery (77% ) and unintentional creation of retinal holes during surgery (up to 17% ). Rare complications include bleeding into the vitreous humor, in around 1% of cases (22), and inflammation of the inner eye, even endophthalmitis, although the latter is very rare indeed (<0.01%) (22). The technical advances in minimally invasive trocar-guided vitrectomy (Figure 6) have had no effect on the endophthalmitis rate (e38–e40), but have reduced the rate of iatrogenic retinal holes by a factor of 4 (21, e41). Nevertheless, the classic method continues to be used in parallel with the minimally invasive technique because no firm conclusions can yet be drawn with regard to benefits and drawbacks.
The operations can be performed with the patient under retrobulbar local anesthesia or general anesthesia, although the latter is preferable particularly for scleral buckling. It is important to note that nitrous oxide anesthesia must be avoided if an air/gas mixture is being used for internal tamponade, as otherwise the intraocular pressure rises so high that blindness can result (19, e42, e43).
Aftercare and postoperative features
There is no rigid scheme for postoperative care that can be applied to every patient. In the first few weeks after operation the patient is examined by an ophthalmologist at short intervals, the frequency depending on the findings. The most important period is the first 6 weeks, during which most complications occur. One significant complication after surgical interventions is scarring of the retina. In proliferative vitreoretinopathy (PVR) fibrotic membranes form on, under, or within the retina, leading to hardening and mechanical shortening of the retina and elevation from the sclera (e1). Regardless of the procedure used, PVR occurs in around 15% of cases and is more pronounced in younger patients and in those with more advanced disease (with literature reports varying from 7% to 55%) (Table) (13, e44, e45).
In a PVR reaction the typical symptoms of “flashes of light and smoke signals,” the correlate of vertical vitreous traction on the retina, are absent. Should the PVR reaction detach the retina close to the fovea, however the patient again describes a renewed shadow and loss of vision (e46).
In the postoperative phase patients are restricted in their activities by local symptoms (swelling, reddening, pain), impaired visual acuity with tamponade, and by the necessity of using eye drops. The transitory (tamponade) or persisting (injury of the macula or optic nerve) loss of spatial vision leads to problems for many patients in the first few weeks, particularly with near work. This should be considered and discussed during the rehabilitation period.
Stage-appropriate treatment and study findings
Changes or rhegmatogenous retinal hole without detachment
An incidentally discovered retinal hole without detachment does not always require treatment. There is no consensus regarding interpretation of the available data on peripheral retinal degeneration, which has traditionally been seen as a risk factor for detachment. The principal representative of this group of changes is lattice degeneration, which is found in around 7% of the normal population but in up to 46% of patients with retinal detachment (2, 23, 24, e47). The likelihood that detachment will develop from asymptomatic lattice degeneration is less than 1%, however, so general prophylactic laser coagulation is currently not recommended—except in the presence of risk factors that favor detachment (status post trauma, detachment in the other eye, family history of detachment) (25). Nevertheless, a Cochrane Review published in 2012 underlined the low evidence level of the available data and the difficulty of formulating reliable recommendations (e48).
In contrast, holes found in a symptomatic patient whose retina is still attached but who is at increased risk of detachment should be treated by laser coagulation according to the published recommendations (25, e49, e50).
Rhegmatogenous retinal hole with detachment
The surgical management of retinal detachment has changed considerably in recent years. While most patients used to be treated by scleral buckling, vitrectomy now predominates (26, 27, e51–e53). Comparative studies have shown that both methods remain valid and each has clear indications, but also that they can be carried out simultaneously or successively (13, 16, 26, 28–30, e52). Apart from the surgeon’s personal experience with the two types of operation, the choice of procedure depends principally on the precise findings in the individual patient (24). In the following simple situation, buckling is preferable to vitrectomy:
- Eye with native lens (phakic), no previous surgery (14–17, 31–33, e54)
- Hole clearly discernible, not too large (12, 34)
- No or only slight PVR reaction (12, 16, 34)
- Good view of site.
This is the case in almost half of all patients with retinal detachment (5). One quarter of patients, however, exhibit complicating factors at the time of presentation (5); in such cases vitrectomy is superior to buckling procedures (e55). Thanks to a prospective randomized European trial (SPR Study), we now have robust data to resolve the question of the best treatment for the remaining patients following cataract surgery: In most patients with retinal detachment after intraocular lens insertion (so-called pseudophakic detachment), vitrectomy is superior to scleral buckling or cerclage (13, 35). In eyes with the native lens, however, buckling procedures obtained better results with regard to the rate of reoperation (e31). Therefore, the lens status influences the choice of operation. The Table shows the most important anatomical and functional parameters of the prospective randomized trials published to date, divided by lens status (31).
An important question—and a common reason for litigation—is the timing of surgical intervention. This is always critical in patients with retinal detachment, because the longer the photoreceptors are separated from the retinal pigment epithelium, the greater the structural alterations in the retina and the potential functional impairments. The mean final visual acuity of patients whose macula was still attached at the time of operation corresponds approximately to the preoperative value, but those with macular detachment attain a mean acuity of only 0.1 to 0.2 (39). This is too low to read normal newspaper text (which requires acuity of ca. 0.5). Therefore, progression of detachment to the macula must be prevented. The available literature offers little information from which conclusions can be drawn regarding the speed of progression of retinal detachment (e56, e57). Many different parameters play a part: A detachment in the upper half of the eye with a large U-shaped hole typically behaves more aggressively than a detachment in the lower hemisphere with small holes and a largely attached vitreous, as is often found, for example, in young shortsighted patients. Recent studies indicate that the surgical management of retinal detachment can be planned according to the individual situation (e.g., anticoagulation), considering that emergency management is associated with a higher rate of complications (39, 40, e57). In many cases flattening of the detached retina can be achieved by strict positioning of the patient on the side of the hole responsible for the detachment, thus facilitating surgical intervention (e57). If the macula is already detached, an operation in the next few days can be arranged (40).
With the aim of further improving the operative management of retinal detachment, an ongoing multicenter prospective randomized trial at German retinal surgery centers, supported by a competence network for clinical studies in retinology (retina.net; in German), is investigating whether a combination of scleral buckling procedures and vitrectomy can yield a better outcome than vitrectomy alone in the difficult group of patients with retinal detachment following cataract surgery. The first results are expected in 2014.
Conflict of interest statement
The authors declare that no conflict of interest exists.
Manuscript received on 3 June 2013, revised version accepted on
9 September 2013.
Translated from the original German by David Roseveare.
Prof. Dr. med. Nicolas Feltgen
37075 Göttingen, Germany
@For eReferences please refer to:
Department of Ophthalmology, RWTH Aachen: Prof. Dr. med. Walter
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