In eyes with advanced diabetic retinopathy, cataract surgery may lead to progression and worsening of retinopathy, which can have detrimental effects on vision (Dowler et al, 1992). In eyes with minimal diabetic changes, cataract surgery is not as likely to cause progression of retinopathy (Wagner et al, 1996; Krepler et al, 2002; Flesner et al, 2002; Kim et al, 2007). Therefore, performing cataract surgery at an earlier stage may be beneficial for diabetic patients because it is associated with fewer complications and better postoperative recovery of vision. Postoperatively, topical steroids and nonsteroidal antiinflammatory drugs are prescribed because they control inflammation and may play a role in the prevention and treatment of macular edema. Macular thickness can be evaluated at serial postoperative visits via optical coherence tomography before the topical medications are stopped. Development of posterior capsular opacification and persistent postoperative inflammation may be more common in diabetics. Consideration should be given to the use of a larger diameter optic in

conjunction with a larger capsulotomy for patients with diabetes (Kato et al, 2001).

funduscopic examinations and referred to retinal colleagues as needed.

**2.3 Retinal vein occlusion**

**2.4 Epiretinal membrane**

and to recognize any cystoid macular edema risk.

Despite an uneventful cataract surgery, diabetic retinopathy can become exacerbated in the postoperative period, so patients should be monitored closely with serial dilated

Retinal vein occlusion is the second most common retinal vascular disease of the eye after diabetic retinopathy. Many of the patients are elderly and have cataracts, and a common treatment of retinal vein occlusion, an intravitreal steroid injection, increases the risk of cataract formation or progression. Unfortunately, small or resolved branch retinal vein occlusions may go unnoticed and only manifest as unexplained visual loss. A careful preoperative evaluation with close attention to unexplained visual loss (out of proportion to the degree of cataract) is critical to detect deficits that will not improve with cataract surgery

Retinal vein occlusions may lower the threshold for blood retina barrier breakdown. Both central retinal vein occlusion and branch retinal vein occlusion are risk factors. Patients with retinal vein occlusion who undergo cataract surgery have an increased risk of postoperative cystoid macular edema. In a large study by Henderson et al conducted between 2001 and 2006, the risk of postoperative cystoid macular edema in uncomplicated cataract surgery was 30 times higher if the operated eye had a history of retinal vein occlusion (Henderson et al, 2007). This risk persisted even in eyes without preoperative macular edema. Intravitreal triamcinolone acetonide and vascular endothelial growth factor inhibiting medications seem to be an effective primary treatment option for macular edema due to retinal vein occlusions and may be given at the time of or before cataract surgery (Jonas et al, 2005, Rensch et al, 2009). Topical nonsteroidal anti-inflammatory drops may be used to prevent the development of

cystoid macular edema in patients with retinal vein occlusion (Henderson et al, 2007).

Behçet's syndrome, sarcoidosis, necrotizing angiitis, multiple sclerosis).

Rare retinal vascular diseases may also increase the risk of cystoid macular edema in cataract patients. These include retinal telangiectasis (Coats' disease, radiation retinopathy, and idiopathic retinal telangiectasia) and several forms of retinal vasculitis (Eales' disease,

It is well known that idiopathic epiretinal membrane occurs typically after posterior vitreous detachment, and gradually progresses with aging. A previous retrospective study suggested that extracapsular cataract extraction is the most common surgical cause of epiretinal membrane (Appiah et al, 1988). A more recent prospective study also showed that the prevalence of idiopathic epiretinal membrane increased from 14.8% preoperatively to 25.3% at 6 months after extracapsular cataract extraction (Jahn et al, 2001). The risk of post– cataract surgery cystoid macular edema is also increased in patients with epiretinal membrane (Henderson et al, 2007).

Idiopathic epiretinal membrane surgery has been reported to improve visual acuity in between 67% and 82% of cases (Margherio et al, 1985). However, subsequent development of progressive nuclear sclerosis occurs in 12.5% to 63% of post vitrectomy patients (Margherio et al, 1985). Ando and associates compared visual outcomes of the combined vitrectomy and cataract procedure to simple vitrectomy in idiopathic epiretinal membrane cases (Ando et al, 1998). Preoperative visual acuities and other patient characteristics were similar in the two study groups. Although more postoperative complications were noted in the combined group with two cases of fibrin formation, one case of macular edema, and four cases of anterior chamber inflammation, the visual outcomes were similar. Post-operatively, both groups showed initial visual improvement; 73% of combined procedure group compared to 88% of the simple vitrectomy group. However, within two years, cataracts formed in 70% of the simple vitrectomy group. The authors recommend the combined procedure for phakic patients older than 55 years undergoing vitrectomy for epiretinal membrane. Alexandrakis et al. described the surgical outcomes of combined cataract surgery and pars plana vitrectomy in eight cases of idiopathic epiretinal membrane formation (Alexandrakis et al, 1999). No intraoperative complications were observed, and at a mean follow-up visit of 22 months, visual acuity had improved in seven patients (88%. Median pre-operative and post-operative visual acuity were 20/200 and 20/50, respectively. Other studies that use the combined procedure to remove epiretinal membranes have reported favorable results as well (Koenig et al, 1992; Demetriades et al, 2003). Cataract surgery seems to be essential in phakic eyes to achieve long-term improvement in visual acuities in eyes with epiretinal membranes and good preoperative acuities.

In a study, the progression of idiopathic epiretinal membrane is not accelerated by smallincision phacoemulsification cataract surgery. Furthermore, visual acuity is not impaired markedly at least for the following year (Hayashi and Hayashi, 2009). However, it is still unclear whether or not a secondary epiretinal membrane progresses after cataract surgery in eyes with other retinal morbidity such as retinal detachment surgery or retinal pathology.

#### **2.5 Peripheral retinal breaks and retinal detachment**

The preoperative treatment of the retinal breaks and retinal degenerations has traditionally come into consideration as a possible means of preventing retinal detachments after cataract extraction, especially in myopic eyes. Specifically, flap retinal tears (even when asymptomatic) are usually treated. Round retinal holes, lattice degeneration, white without pressure, and other peripheral retinal abnormalities are typically observed.

There is an increasing tendency to support the concept that retinal detachments generally are associated with recent retinal breaks. Reports supporting the prophylactic treatment of preexisting retinal breaks prior to cataract surgery is lacking. Most of the eyes with lattice degeneration do not detach after small incision cataract extraction even when YAG laser capsulotomy is performed later. Those that do develop a retinal detachment frequently do not detach from retinal breaks adjacent to or within the lattice lesions, but from unrelated

Cataract Surgery in Retina Patients 377

to prevent posterior capsule opacification and post-operative vision loss, the authors included a posterior capsulotomy as part of the procedure. Post-operatively, 61 patients (65%) had improved to 20/40 or better. Closure of the macular hole after the initial surgery occurred in 80 patients (89%). Four holes closed with an additional operation. After nine months or more, three patients experienced reopening of the macular hole, which was successfully managed with repeat vitrectomy. Reported complications included eight (9%) post-operative cases of cystoid macular edema, all of which were resolved by topical and sub-Tenon's steroid application. Another eight patients (9%) developed small, segmental synechiae of the anterior capsule iris. Post-operative retinal detachments occurred in only three patients (3%). The combined procedure also allows for a more complete vitrectomy that includes removal of the anterior vitreous without the risk of lens injury. Thus, a better gas fill can be achieved which may provide longer tamponade and an increased closure rate

Simcock and Scalia reported the results of combined phacoemulsification cataract removal and vitrectomy in 13 consecutive eyes with full thickness macular holes. Mild preoperative lens opacity was present in all 13 patients. Each eye underwent phacoemulsification followed by pars plana vitrectomy and finally intraocular lens implantation. Twelve of the 13 patients had visual improvement in the postoperative period. None of the eyes developed cystoid macular edema (Simcock and Scalia, 2000). There have been concerns regarding the incidence of cystoid macular edema following combined procedure for macular hole. Sheidow and Gonder reported a 43% incidence of both clinical and angiographical evidence of cystoid macular edema in a study of seven eyes undergoing combined procedure for macular hole (Sheidow and Gonder, 1998). However, other studies have not confirmed this observation and macular hole appears to be an acceptable

In combined surgeries, intraoperative aphakia provided maximum visibility for posterior vitreous peeling and peripheral visualization. Scleral ports could be placed more anteriorly reducing the risk of a retinal tear. The risk of vitrectomy induced cataract was eliminated and a more complete vitrectomy could be performed leading to greater gas fill and therefore

Cataract development is more frequent in patients with high myopia than in the general population. Reports have shown that the mean age for cataract surgery in patients with high myopia is 65 years. However, in eyes with an axial length greater than 29mm, the incidence is significant at age 50 years. Nuclear cataract is most typical in high myopia and in its earliest stage, increases the optical power of the lens and thus the optical power of the myopic eye. In these patients, nuclear cataract may be difficult to recognize, because in some cases nuclear sclerosis is the initial step in nuclear cataract development. Middle-aged patients who developed cataract might have had early nuclear sclerosis without evidence of cataract. The earliest manifestation may be an increase in the dioptric correction of myopia (Metge and Pichot de Champfleury, 1994; Wong et al, 2001; Leske et al, 2002; Younan et al,

Myopic eyes have a higher risk of retinal complications compared with emmetropic eyes. During the preoperative evaluation, a careful examination should be done for any retinal breaks, holes or degenerations, as well as any macular pathology. The highly myopic

for macular holes (Thompson et al, 1996).

indication for combined procedure.

a better postoperative tamponade.

**2.7 High myopia**

2002).

areas which previously looked clinically normal. If a patient has a history of retinal detachment in one eye and lattice degeneration with retinal holes in the other eye, cryosurgery or laser surgery is needed to close the holes in the second eye. Usually cryosurgery is required because the cataract may preclude the use of laser. The type of tear present and other factors including the location of the tear and the existence of high myopia would influence the ophthalmologist's judgment in deciding when to treat. Since seven to eight percent of the population has lattice degeneration, it is obvious that not all patients with lattice degeneration should be treated. Regardless of whether the patient is treated prior to cataract surgery, those patients should be followed closely with careful examination of the peripheral retina postoperatively following cataract removal.

Girard and Saade reported a 3.5% incidence of simultaneous primary rhegmatogenous retinal detachment and visually significant cataract (Girard and Sade, 1997). In such cases, they advocate a combined procedure including phacoemulsification, intraocular lens implantation and scleral buckling surgery. Cataract formation is a common occurrence after retinal detachment repair, especially when gas tamponade is employed. For this reason, recurrent retinal detachment may be found in eyes with cataracts, which may make repair more difficult. Options available to the retinal surgeon include cataract surgery followed by pars plana vitrectomy or combined cataract surgery and vitrectomy.

Another study described experiences using the combined procedure to treat 16 cases of recurrent retinal detachments (Chaudhry et al, 2000). Eyes were selected for inclusion in the study based on presence of a dense cataract and a recurrent rhegmatogenous retinal detachment with mild proliferative vitreoretinopathy following primary surgical repair using encircling scleral buckle. In nine eyes (56%), visual acuity improved to 20/200 or better post-operatively. In 13 eyes (81%), the initial reoperation was successful in retinal reattachment. Two additional eyes achieved retinal reattachment with a second pars plana vitrectomy, increasing the anatomic success rate to 94%. The study had a selection bias in that the eyes with more severe proliferative vitreoretinopathy were not found suitable for the combined procedure. However for primary rhegmatogenous retinal detachment and recurrent retinal detachment with mild proliferative vitreoretinopathy, the combined procedure appears to be well tolerated.

#### **2.6 Macular hole**

Macular holes are commonly found in older patients. Some eyes have concurrent macular hole and cataract, making internal limiting membrane peeling more difficult during vitrectomy because of blurred media. The incidence of cataract development following macular hole surgery is also extremely high. Thompson et al. reported visually significant cataract formation in 76% of the study eyes following vitrectomy for macular hole (Thompson et al, 1995). Consecutive and combined surgeries for macular hole and cataract extraction are both effective procedures.

Various studies found that the functional and anatomic results of combined surgery were equivalent to consecutive procedures (Theocharis et al, 2005; Simcock and Scalia, 2001; Kotecha et al, 2000; Muselier et al, 2010). Nevertheless, combining cataract surgery with vitrectomy may prevent a second operation to correct post-vitrectomy cataract formation. In the largest series, Lahey and associates described combined procedure to treat 89 cases of macular holes (Lahey et al, 2002). These patients received combined phacoemulsification, implantation of posterior chamber intraocular lens and pars plana vitrectomy. Additionally,

areas which previously looked clinically normal. If a patient has a history of retinal detachment in one eye and lattice degeneration with retinal holes in the other eye, cryosurgery or laser surgery is needed to close the holes in the second eye. Usually cryosurgery is required because the cataract may preclude the use of laser. The type of tear present and other factors including the location of the tear and the existence of high myopia would influence the ophthalmologist's judgment in deciding when to treat. Since seven to eight percent of the population has lattice degeneration, it is obvious that not all patients with lattice degeneration should be treated. Regardless of whether the patient is treated prior to cataract surgery, those patients should be followed closely with careful examination

Girard and Saade reported a 3.5% incidence of simultaneous primary rhegmatogenous retinal detachment and visually significant cataract (Girard and Sade, 1997). In such cases, they advocate a combined procedure including phacoemulsification, intraocular lens implantation and scleral buckling surgery. Cataract formation is a common occurrence after retinal detachment repair, especially when gas tamponade is employed. For this reason, recurrent retinal detachment may be found in eyes with cataracts, which may make repair more difficult. Options available to the retinal surgeon include cataract surgery followed by

Another study described experiences using the combined procedure to treat 16 cases of recurrent retinal detachments (Chaudhry et al, 2000). Eyes were selected for inclusion in the study based on presence of a dense cataract and a recurrent rhegmatogenous retinal detachment with mild proliferative vitreoretinopathy following primary surgical repair using encircling scleral buckle. In nine eyes (56%), visual acuity improved to 20/200 or better post-operatively. In 13 eyes (81%), the initial reoperation was successful in retinal reattachment. Two additional eyes achieved retinal reattachment with a second pars plana vitrectomy, increasing the anatomic success rate to 94%. The study had a selection bias in that the eyes with more severe proliferative vitreoretinopathy were not found suitable for the combined procedure. However for primary rhegmatogenous retinal detachment and recurrent retinal detachment with mild proliferative vitreoretinopathy, the combined

Macular holes are commonly found in older patients. Some eyes have concurrent macular hole and cataract, making internal limiting membrane peeling more difficult during vitrectomy because of blurred media. The incidence of cataract development following macular hole surgery is also extremely high. Thompson et al. reported visually significant cataract formation in 76% of the study eyes following vitrectomy for macular hole (Thompson et al, 1995). Consecutive and combined surgeries for macular hole and cataract

Various studies found that the functional and anatomic results of combined surgery were equivalent to consecutive procedures (Theocharis et al, 2005; Simcock and Scalia, 2001; Kotecha et al, 2000; Muselier et al, 2010). Nevertheless, combining cataract surgery with vitrectomy may prevent a second operation to correct post-vitrectomy cataract formation. In the largest series, Lahey and associates described combined procedure to treat 89 cases of macular holes (Lahey et al, 2002). These patients received combined phacoemulsification, implantation of posterior chamber intraocular lens and pars plana vitrectomy. Additionally,

of the peripheral retina postoperatively following cataract removal.

pars plana vitrectomy or combined cataract surgery and vitrectomy.

procedure appears to be well tolerated.

extraction are both effective procedures.

**2.6 Macular hole**

to prevent posterior capsule opacification and post-operative vision loss, the authors included a posterior capsulotomy as part of the procedure. Post-operatively, 61 patients (65%) had improved to 20/40 or better. Closure of the macular hole after the initial surgery occurred in 80 patients (89%). Four holes closed with an additional operation. After nine months or more, three patients experienced reopening of the macular hole, which was successfully managed with repeat vitrectomy. Reported complications included eight (9%) post-operative cases of cystoid macular edema, all of which were resolved by topical and sub-Tenon's steroid application. Another eight patients (9%) developed small, segmental synechiae of the anterior capsule iris. Post-operative retinal detachments occurred in only three patients (3%). The combined procedure also allows for a more complete vitrectomy that includes removal of the anterior vitreous without the risk of lens injury. Thus, a better gas fill can be achieved which may provide longer tamponade and an increased closure rate for macular holes (Thompson et al, 1996).

Simcock and Scalia reported the results of combined phacoemulsification cataract removal and vitrectomy in 13 consecutive eyes with full thickness macular holes. Mild preoperative lens opacity was present in all 13 patients. Each eye underwent phacoemulsification followed by pars plana vitrectomy and finally intraocular lens implantation. Twelve of the 13 patients had visual improvement in the postoperative period. None of the eyes developed cystoid macular edema (Simcock and Scalia, 2000). There have been concerns regarding the incidence of cystoid macular edema following combined procedure for macular hole. Sheidow and Gonder reported a 43% incidence of both clinical and angiographical evidence of cystoid macular edema in a study of seven eyes undergoing combined procedure for macular hole (Sheidow and Gonder, 1998). However, other studies have not confirmed this observation and macular hole appears to be an acceptable indication for combined procedure.

In combined surgeries, intraoperative aphakia provided maximum visibility for posterior vitreous peeling and peripheral visualization. Scleral ports could be placed more anteriorly reducing the risk of a retinal tear. The risk of vitrectomy induced cataract was eliminated and a more complete vitrectomy could be performed leading to greater gas fill and therefore a better postoperative tamponade.

#### **2.7 High myopia**

Cataract development is more frequent in patients with high myopia than in the general population. Reports have shown that the mean age for cataract surgery in patients with high myopia is 65 years. However, in eyes with an axial length greater than 29mm, the incidence is significant at age 50 years. Nuclear cataract is most typical in high myopia and in its earliest stage, increases the optical power of the lens and thus the optical power of the myopic eye. In these patients, nuclear cataract may be difficult to recognize, because in some cases nuclear sclerosis is the initial step in nuclear cataract development. Middle-aged patients who developed cataract might have had early nuclear sclerosis without evidence of cataract. The earliest manifestation may be an increase in the dioptric correction of myopia (Metge and Pichot de Champfleury, 1994; Wong et al, 2001; Leske et al, 2002; Younan et al, 2002).

Myopic eyes have a higher risk of retinal complications compared with emmetropic eyes. During the preoperative evaluation, a careful examination should be done for any retinal breaks, holes or degenerations, as well as any macular pathology. The highly myopic

2009).

**2.9 Retinitis pigmentosa**

et al, 1985).

capsulotomy is required (Jackson et al, 2001).

**2.10 Retinopathy of prematurity**

Cataract Surgery in Retina Patients 379

that limbal approach was not associated with tumor recurrence or metastasis (Payne et al,

Management of the posterior capsule is controversial in the setting of previous treatment for retinoblastoma. Theoretically, the posterior capsule may act as a barrier to tumor spread if viable tumor cells are present in the eye, therefore, the posterior capsule should be kept intact whenever possible. Nevertheless, it is frequently necessary to perform a primary posterior capsulotomy and anterior vitrectomy in pediatric cataracts, even in the setting of prior treatment for retinoblastoma. Since posterior capsular opacity is common after external beam radiotherapy, it is sometimes necessary to remove the posterior capsule to clear the visual axis. The risks and benefits of primary posterior capsulotomy and anterior vitrectomy should be considered on a case-by-case basis, taking into account the location of the cataract, the age of the patient, the availability of the YAG laser, the length of the

Cataract is a well-recognised complication of all types of retinitis pigmentosa. When compared with patients with age related cataract, patients with retinitis pigmentosa develop lens opacities earlier (Pruett, 1983; Fishman et al, 1985; Heckenlively, 1982). In addition, a relatively minor lens opacity may cause significant functional symptoms in these patients. Apart from the general risks of cataract surgery, there are specific additional factors that may result in a poor visual outcome after cataract extraction in the presence of retinitis pigmentosa. These include, retinal atrophy at the macula, macular edema occurring in approximately 10–15% of patients and phototoxic retinal damage in normal patients undergoing cataract extraction (Grover et al, 1997; Spalton et al, 1978; Newsome, 1986; Lee and Sternberg, 1993). The threshold for light damage is probably lower in retinitis pigmentosa, which could adversely affect visual outcome. Posterior capsular opacification and anterior capsular contraction is more aggressive in the presence of retinitis pigmentosa, (Nishi and Nishi, 1993; Hayashi et al, 1998). The reason for the increased cellular proliferation on the capsular remnant in retinal dystrophies is unknown, although the cellular nature of the posterior capsule in retinal dystrophies may account for this (Fishman

Patients with retinitis pigmentosa benefit from early cataract surgery, and that the vast majority have a subjective improvement in their symptoms of glare. The benefit of surgery for patients with a poor preoperative visual acuity is less marked, usually because of preexisting macular disease, but postoperative macular edema was less common than expected. Patients with retinitis pigmentosa appear to be susceptible to anterior capsule contraction and therefore a small capsulorhexis should be avoided. It would appear to be sensible to avoid silicone intraocular lenses because of the risk of their dislocation if an early

Cataracts occur more commonly in retinopathy of prematurity patients compared to general population. Low birth weight and prematurity are risk factors for both retinopathy of prematurity and cataracts (San Giovanni et al, 2002; Repka et al, 1998). Cataracts develop at a greater frequency over time, as current treatment modalities have preserved vision in eyes that would have otherwise been lost. Transpupillary laser photocoagulation is now the standard

quiescent period, and the location and stage of the tumor (Payne et al, 2009).

patients may also have myopic macular degeneration, epiretinal membranes or other significant changes. These may limit the postoperative visual acuity and may influence the development of postoperative complications such as cystoid macular edema. If any posterior segment issues are detected, referral to a vitreoretinal colleague for treatment is recommended before cataract surgery. In addition to the typical cataract evaluation, care must be taken to accurately assess the retinal status and measure the axial length of the eye. Highly myopic eyes often have a posterior staphyloma, which can give an erroneously long axial length when measured with the standard A-scan ultrasound. Using an optical method for measurement tends to be more accurate, as it measures directly at the fovea. The intraocular lens calculation formulae are less accurate at the extremes, and this is particularly true for highly myopic eyes. Of the two-variable formulae, the SRK/T tends to perform particularly well, as do more complex formulae such as the Haigis and Holladay 2 (Wang et al, 2008). The selection of the intraocular lens depends on each patient's ocular status and needs.

The advantage of cataract surgery in myopic patients is the larger anterior chamber depth, which allows more working room during phacoemulsification. However, the infusion pressure from the phaco handpiece can cause over-inflation of the anterior chamber and a tendency to push the entire lens-iris diaphragm posteriorly. To overcome this, the infusion pressure can be decreased by lowering the bottle height; however, this will result in less inflow of fluid and a higher tendency for surge. Another solution is to break the reverse pupillary block by making sure that there is fluid flow under the iris to equalize the anterior and posterior chamber pressures. By neutralizing this pressure gradient, the cataract will not be pushed so deeply within the eye, and adequate infusion pressure can be used. The postoperative refraction in myopes can take time to stabilize due to the variation in effective lens position as the capsular bag shrink-wraps around the intraocular lens. During this period, inflammation can be controlled using topical steroids and nonsteroidal anti inflammatory drugs. During the postoperative period, repeat dilated fundus examinations are mandatory in order to search for possible retinal breaks that may have been created during surgery (Alio et al, 2000; Tosi et al, 2003; Güell et al, 2003).

#### **2.8 Retinoblastoma**

Cataract formation is one of the most common ocular complications of external beam radiotherapy for retinoblastoma, which typically occurs in three years following treatment (Schipper et al, 1985; Miller et al 2005). Studies have shown that cataract surgery in patients who have previously received radiation therapy for retinoblastoma is generally not associated with tumor recurrence or spread (Brooks et al, 1990; Portellos and Buckley, 1998). Controversies in cataract management include the surgical approach, the management of the posterior capsule and anterior vitreous. In the setting of prior treatment for retinoblastoma, these decisions take on even greater importance with the added concern for reactivation or metastasis of the tumor. Both clear corneal and pars plana approaches have been used with success in children undergoing cataract surgery following treatment for retinoblastoma. Although Brooks et al advised against pars plana approachs based on their experience of tumor recurrence, other series have not reported tumor recurrences with pars plana insicions (Brooks et al, 1990). Miller and associates reported a series of 16 eyes, all of which underwent a combined pars plana vitrectomy and cataract extraction, and showed no evidence of tumor recurrence in their series (Miller et al 2005). Payne et al have also shown

patients may also have myopic macular degeneration, epiretinal membranes or other significant changes. These may limit the postoperative visual acuity and may influence the development of postoperative complications such as cystoid macular edema. If any posterior segment issues are detected, referral to a vitreoretinal colleague for treatment is recommended before cataract surgery. In addition to the typical cataract evaluation, care must be taken to accurately assess the retinal status and measure the axial length of the eye. Highly myopic eyes often have a posterior staphyloma, which can give an erroneously long axial length when measured with the standard A-scan ultrasound. Using an optical method for measurement tends to be more accurate, as it measures directly at the fovea. The intraocular lens calculation formulae are less accurate at the extremes, and this is particularly true for highly myopic eyes. Of the two-variable formulae, the SRK/T tends to perform particularly well, as do more complex formulae such as the Haigis and Holladay 2 (Wang et al, 2008). The selection of the intraocular lens depends on each patient's ocular

The advantage of cataract surgery in myopic patients is the larger anterior chamber depth, which allows more working room during phacoemulsification. However, the infusion pressure from the phaco handpiece can cause over-inflation of the anterior chamber and a tendency to push the entire lens-iris diaphragm posteriorly. To overcome this, the infusion pressure can be decreased by lowering the bottle height; however, this will result in less inflow of fluid and a higher tendency for surge. Another solution is to break the reverse pupillary block by making sure that there is fluid flow under the iris to equalize the anterior and posterior chamber pressures. By neutralizing this pressure gradient, the cataract will not be pushed so deeply within the eye, and adequate infusion pressure can be used. The postoperative refraction in myopes can take time to stabilize due to the variation in effective lens position as the capsular bag shrink-wraps around the intraocular lens. During this period, inflammation can be controlled using topical steroids and nonsteroidal anti inflammatory drugs. During the postoperative period, repeat dilated fundus examinations are mandatory in order to search for possible retinal breaks that may have been created

Cataract formation is one of the most common ocular complications of external beam radiotherapy for retinoblastoma, which typically occurs in three years following treatment (Schipper et al, 1985; Miller et al 2005). Studies have shown that cataract surgery in patients who have previously received radiation therapy for retinoblastoma is generally not associated with tumor recurrence or spread (Brooks et al, 1990; Portellos and Buckley, 1998). Controversies in cataract management include the surgical approach, the management of the posterior capsule and anterior vitreous. In the setting of prior treatment for retinoblastoma, these decisions take on even greater importance with the added concern for reactivation or metastasis of the tumor. Both clear corneal and pars plana approaches have been used with success in children undergoing cataract surgery following treatment for retinoblastoma. Although Brooks et al advised against pars plana approachs based on their experience of tumor recurrence, other series have not reported tumor recurrences with pars plana insicions (Brooks et al, 1990). Miller and associates reported a series of 16 eyes, all of which underwent a combined pars plana vitrectomy and cataract extraction, and showed no evidence of tumor recurrence in their series (Miller et al 2005). Payne et al have also shown

during surgery (Alio et al, 2000; Tosi et al, 2003; Güell et al, 2003).

status and needs.

**2.8 Retinoblastoma**

that limbal approach was not associated with tumor recurrence or metastasis (Payne et al, 2009).

Management of the posterior capsule is controversial in the setting of previous treatment for retinoblastoma. Theoretically, the posterior capsule may act as a barrier to tumor spread if viable tumor cells are present in the eye, therefore, the posterior capsule should be kept intact whenever possible. Nevertheless, it is frequently necessary to perform a primary posterior capsulotomy and anterior vitrectomy in pediatric cataracts, even in the setting of prior treatment for retinoblastoma. Since posterior capsular opacity is common after external beam radiotherapy, it is sometimes necessary to remove the posterior capsule to clear the visual axis. The risks and benefits of primary posterior capsulotomy and anterior vitrectomy should be considered on a case-by-case basis, taking into account the location of the cataract, the age of the patient, the availability of the YAG laser, the length of the quiescent period, and the location and stage of the tumor (Payne et al, 2009).

#### **2.9 Retinitis pigmentosa**

Cataract is a well-recognised complication of all types of retinitis pigmentosa. When compared with patients with age related cataract, patients with retinitis pigmentosa develop lens opacities earlier (Pruett, 1983; Fishman et al, 1985; Heckenlively, 1982). In addition, a relatively minor lens opacity may cause significant functional symptoms in these patients. Apart from the general risks of cataract surgery, there are specific additional factors that may result in a poor visual outcome after cataract extraction in the presence of retinitis pigmentosa. These include, retinal atrophy at the macula, macular edema occurring in approximately 10–15% of patients and phototoxic retinal damage in normal patients undergoing cataract extraction (Grover et al, 1997; Spalton et al, 1978; Newsome, 1986; Lee and Sternberg, 1993). The threshold for light damage is probably lower in retinitis pigmentosa, which could adversely affect visual outcome. Posterior capsular opacification and anterior capsular contraction is more aggressive in the presence of retinitis pigmentosa, (Nishi and Nishi, 1993; Hayashi et al, 1998). The reason for the increased cellular proliferation on the capsular remnant in retinal dystrophies is unknown, although the cellular nature of the posterior capsule in retinal dystrophies may account for this (Fishman et al, 1985).

Patients with retinitis pigmentosa benefit from early cataract surgery, and that the vast majority have a subjective improvement in their symptoms of glare. The benefit of surgery for patients with a poor preoperative visual acuity is less marked, usually because of preexisting macular disease, but postoperative macular edema was less common than expected. Patients with retinitis pigmentosa appear to be susceptible to anterior capsule contraction and therefore a small capsulorhexis should be avoided. It would appear to be sensible to avoid silicone intraocular lenses because of the risk of their dislocation if an early capsulotomy is required (Jackson et al, 2001).

#### **2.10 Retinopathy of prematurity**

Cataracts occur more commonly in retinopathy of prematurity patients compared to general population. Low birth weight and prematurity are risk factors for both retinopathy of prematurity and cataracts (San Giovanni et al, 2002; Repka et al, 1998). Cataracts develop at a greater frequency over time, as current treatment modalities have preserved vision in eyes that would have otherwise been lost. Transpupillary laser photocoagulation is now the standard

Cataract Surgery in Retina Patients 381

The patient's history is particularly important to determine the onset of symptoms and the development of the cataract. After most pars plana vitrectomy surgeries, cataracts develop slowly, over the course of months or years after retinal surgery, in the form of increased nuclear sclerosis and often posterior subcapsular opacities. The use of intraocular gas or silicone oil as a retinal tamponade may induce cataract changes at a somewhat more rapid rate, but it is still typically months before the patient notices a visual decline. If the patient reports a history of quickly developing a cataract, such as a white cataract, days or weeks after the vitrectomy, then iatrogenic damage to the lens capsule should be suspected. While it is uncommon, it is possible for the pars plana vitrectomy instruments to damage the posterior lens capsule, which can rupture and then cause the lens to opacify very quickly. Clinical examination should include careful evaluation of the posterior capsule by either slit lamp or ultrasound, if direct visualization is not possible. If the ultrasound shows an abnormally large lens thickness or an out-pouching of the posterior lens surface, a defect in the posterior lens capsule likely exists. Intraocular lens calculations may be somewhat less accurate due to difficulty in estimating the postop effective lens position. The absence of vitreous and possible prior damage to zonules may cause the intraocular lens to sit more posterior than predicted, resulting in a hyperopic surprise. This is why aiming for a mild degree of postop myopia by using a slightly higher-powered intraocular lens tends to give better results. Three-piece monofocal acrylic intraocular lenses in these eyes may have more options for lens fixation, such as in-the-bag, in-the-sulcus and sulcus placement of the haptics with optic capture through the capsulorrhexis. In addition, the acrylic material minimizes condensation on the optic and adhesion to silicone oil if a repeat vitrectomy is

Mastering in surgical techniques for cataract extraction and improvements in intraocular lens technology have increased the indications for cataract surgery. Additionally, pars plana vitrectomy is now performed for a wide variety of vitreoretinal diseases. It is widely accepted today that the most effective procedure for lens extraction is sutureless clear corneal phacoemulsification. The common approach for pars plana vitrectomy is transconjunctival small incision sutureless vitrectomy, also known as minimally invasive

Combined phacoemulsification and vitrectomy is indicated if the opacified lens interferes with the visualization of the retina, hindering the operation. However, if the cataract allows for good visualization of the posterior pole, the surgeon must decide on the best approach; a combined procedure, clear cornea phacoemulsification and then pars plana vitrectomy, both performed at the same surgical session, or a two-step procedure, pars plana vitrectomy is performed first, and then clear cornea phacoemulsification performed as a secondary procedure during a second surgical

A combined approach with minimally invasive vitreoretinal surgery has been rising in popularity among vitreoretinal surgeons, mainly because it has several advantages when compared with the two-step procedure. These include faster visual recovery and patient satisfaction, no suture-related astigmatism, less postoperative inflammation, less

needed in the future.

session (Pollack et al, 2004).

**3.1.1 Combined procedure**

**3.1 Cataract surgery and pars plana vitrectomy**

vitreoretinal surgery (Fujii et al, 2002; Eckardt, 2005).

treatment for threshold retinopathy of prematurity. However, laser-treated eyes have a higher incidence of secondary cataracts than cryo-treated eyes (Christiansen and Bradford, 1995; Christiansen and Bradford, 1997; Kaiser and Trese, 1995). Lens opacities associated with retinopathy of prematurity appears in three types. First, focal punctuate or vacuolated opacities may occur at the subcapsular level. These are usually transient and visually insignificant. Second, progressive lens opacities may occur in patients without retinal detachment. Most of these eyes have had transpupillary laser treatment or "lens sparing" vitrectomy. These cataracts may progress rapidly or much more slowly, but they almost always eventually obstruct the entire visual axis and require surgery (Alden et al, 1973; Drack et al, 1992).

A visually significant cataract after laser treatment or vitrectomy for retinopathy of prematurity is approached much like childhood cataracts in children without retinopathy of prematurity (Wilson et al, 2005). At times the anterior capsule can be fibrotic, but a vitrectorhexis can still be easily performed. Intraocular lens calculations can be performed using an immersion A-scan ultrasound unit and a portable keratometer in the operating room, after the child is under general anesthesia for cataract surgery. Intraocular lenses are implanted routinely, unless the child is in the early months of life and has microphthalmia. Most commonly, a single-piece hydrophobic acrylic intraocular lens is implanted in children. In anticipation of myopic shift of refraction, the intraocular lens power for a child undergoing cataract surgery should be customized based on many characteristics – especially age, laterality (one eye or both), amblyopia status (mild or severe), likely compliance with glasses, and family history of myopia. For a child with retinopathy of prematurity and cataract, slightly higher hypermetropia may be considered in anticipation of developing more myopia, especially if treated with cryotherapy (Trivedi et al, 2007). A primary posterior capsulectomy and anterior vitrectomy is performed for children who are younger than 6 years ofage. If previous vitrectomy has been performed as part of the retinopathy of prematurity treatment, the surgeon must be aware that the posterior capsule may have been violated during the previous surgery.

The two surgical approaches in stage V retinopathy of prematurity are pars plana lensectomy versus lensectomy via the limbal approach. A pars plana lensectomy can be combined with an attempt to repair retinal pathology. The limbal approach is easier and more consistent, as the pars plana entry may be difficult in these immature eyes with retinal detachment. Even when the anterior chamber is extremely shallow, an anterior corneal entry can usually be made with the assistance of a viscous ophthalmic viscosurgical device.

Although cataract extraction in eyes with regressed retinopathy of prematurity may present challenges, such as high myopia, monocularity, glaucoma, and previous ocular surgery, phacoemulsification in these eyes proved to be relatively safe as well as visually rehabilitating. The surgeon should be aware of the special considerations in this population, alert to potential zonular weakness intraoperatively, and careful of increased postoperative risks, including retinal detachment (Farr et al, 2001).

#### **3. Cataract surgery and retinal surgery**

Cataract and vitreoretinal diseases often occur simultaneously. The surgical management of patients with vitreoretinal diseases and cataract has always been a unique situation for vitreoretinal surgeons. The major difficulty is not only visual interference created by lens opacification, but also deciding on a patient-by patient basis whether cataract extraction should be combined or approached as a two-step procedure.

treatment for threshold retinopathy of prematurity. However, laser-treated eyes have a higher incidence of secondary cataracts than cryo-treated eyes (Christiansen and Bradford, 1995; Christiansen and Bradford, 1997; Kaiser and Trese, 1995). Lens opacities associated with retinopathy of prematurity appears in three types. First, focal punctuate or vacuolated opacities may occur at the subcapsular level. These are usually transient and visually insignificant. Second, progressive lens opacities may occur in patients without retinal detachment. Most of these eyes have had transpupillary laser treatment or "lens sparing" vitrectomy. These cataracts may progress rapidly or much more slowly, but they almost always eventually obstruct the

A visually significant cataract after laser treatment or vitrectomy for retinopathy of prematurity is approached much like childhood cataracts in children without retinopathy of prematurity (Wilson et al, 2005). At times the anterior capsule can be fibrotic, but a vitrectorhexis can still be easily performed. Intraocular lens calculations can be performed using an immersion A-scan ultrasound unit and a portable keratometer in the operating room, after the child is under general anesthesia for cataract surgery. Intraocular lenses are implanted routinely, unless the child is in the early months of life and has microphthalmia. Most commonly, a single-piece hydrophobic acrylic intraocular lens is implanted in children. In anticipation of myopic shift of refraction, the intraocular lens power for a child undergoing cataract surgery should be customized based on many characteristics – especially age, laterality (one eye or both), amblyopia status (mild or severe), likely compliance with glasses, and family history of myopia. For a child with retinopathy of prematurity and cataract, slightly higher hypermetropia may be considered in anticipation of developing more myopia, especially if treated with cryotherapy (Trivedi et al, 2007). A primary posterior capsulectomy and anterior vitrectomy is performed for children who are younger than 6 years ofage. If previous vitrectomy has been performed as part of the retinopathy of prematurity treatment, the surgeon must be aware that the posterior capsule

The two surgical approaches in stage V retinopathy of prematurity are pars plana lensectomy versus lensectomy via the limbal approach. A pars plana lensectomy can be combined with an attempt to repair retinal pathology. The limbal approach is easier and more consistent, as the pars plana entry may be difficult in these immature eyes with retinal detachment. Even when the anterior chamber is extremely shallow, an anterior corneal entry can usually be made with the assistance of a viscous ophthalmic viscosurgical device. Although cataract extraction in eyes with regressed retinopathy of prematurity may present challenges, such as high myopia, monocularity, glaucoma, and previous ocular surgery, phacoemulsification in these eyes proved to be relatively safe as well as visually rehabilitating. The surgeon should be aware of the special considerations in this population, alert to potential zonular weakness intraoperatively, and careful of increased postoperative

Cataract and vitreoretinal diseases often occur simultaneously. The surgical management of patients with vitreoretinal diseases and cataract has always been a unique situation for vitreoretinal surgeons. The major difficulty is not only visual interference created by lens opacification, but also deciding on a patient-by patient basis whether cataract extraction

entire visual axis and require surgery (Alden et al, 1973; Drack et al, 1992).

may have been violated during the previous surgery.

risks, including retinal detachment (Farr et al, 2001).

should be combined or approached as a two-step procedure.

**3. Cataract surgery and retinal surgery**

The patient's history is particularly important to determine the onset of symptoms and the development of the cataract. After most pars plana vitrectomy surgeries, cataracts develop slowly, over the course of months or years after retinal surgery, in the form of increased nuclear sclerosis and often posterior subcapsular opacities. The use of intraocular gas or silicone oil as a retinal tamponade may induce cataract changes at a somewhat more rapid rate, but it is still typically months before the patient notices a visual decline. If the patient reports a history of quickly developing a cataract, such as a white cataract, days or weeks after the vitrectomy, then iatrogenic damage to the lens capsule should be suspected. While it is uncommon, it is possible for the pars plana vitrectomy instruments to damage the posterior lens capsule, which can rupture and then cause the lens to opacify very quickly. Clinical examination should include careful evaluation of the posterior capsule by either slit lamp or ultrasound, if direct visualization is not possible. If the ultrasound shows an abnormally large lens thickness or an out-pouching of the posterior lens surface, a defect in the posterior lens capsule likely exists. Intraocular lens calculations may be somewhat less accurate due to difficulty in estimating the postop effective lens position. The absence of vitreous and possible prior damage to zonules may cause the intraocular lens to sit more posterior than predicted, resulting in a hyperopic surprise. This is why aiming for a mild

degree of postop myopia by using a slightly higher-powered intraocular lens tends to give better results. Three-piece monofocal acrylic intraocular lenses in these eyes may have more options for lens fixation, such as in-the-bag, in-the-sulcus and sulcus placement of the haptics with optic capture through the capsulorrhexis. In addition, the acrylic material minimizes condensation on the optic and adhesion to silicone oil if a repeat vitrectomy is needed in the future.

#### **3.1 Cataract surgery and pars plana vitrectomy**

Mastering in surgical techniques for cataract extraction and improvements in intraocular lens technology have increased the indications for cataract surgery. Additionally, pars plana vitrectomy is now performed for a wide variety of vitreoretinal diseases. It is widely accepted today that the most effective procedure for lens extraction is sutureless clear corneal phacoemulsification. The common approach for pars plana vitrectomy is transconjunctival small incision sutureless vitrectomy, also known as minimally invasive vitreoretinal surgery (Fujii et al, 2002; Eckardt, 2005).

Combined phacoemulsification and vitrectomy is indicated if the opacified lens interferes with the visualization of the retina, hindering the operation. However, if the cataract allows for good visualization of the posterior pole, the surgeon must decide on the best approach; a combined procedure, clear cornea phacoemulsification and then pars plana vitrectomy, both performed at the same surgical session, or a two-step procedure, pars plana vitrectomy is performed first, and then clear cornea phacoemulsification performed as a secondary procedure during a second surgical session (Pollack et al, 2004).

#### **3.1.1 Combined procedure**

A combined approach with minimally invasive vitreoretinal surgery has been rising in popularity among vitreoretinal surgeons, mainly because it has several advantages when compared with the two-step procedure. These include faster visual recovery and patient satisfaction, no suture-related astigmatism, less postoperative inflammation, less

Cataract Surgery in Retina Patients 383

approach, patients who undergo sequential surgeries may experience increasing discomfort. Another disadvantage is cost; two surgeries cost more than the combined procedure

There are advantages and disadvantages to each approach, but both are safe and effective. Combined surgery requires a shorter postoperative recovery time, anterior vitreous structures can be removed without risk of touching the lens, visualization of the posterior pole is good during vitrectomy, and it involves only one surgical session, which may reduce patient discomfort and decrease risks and costs. Also, patients with retinal vascular diseases frequently undergo panretinal photocoagulation during the operation, decreasing the risk of

However, there are potential disadvantages to combined surgery, such as increased operating time and stress on the surgeon, difficulty visualizing the capsulorrhexis because of an absent or reduced red reflex, cataract wound dehiscence caused by globe manipulation during subsequent vitreous surgery, and intraoperative miosis after cataract extraction. Other disadvantages include bleeding from anterior structures, loss of corneal transparency from corneal edema and Descemet's folds, inadvertent exchange of anterior segment fluids with posterior segment tamponading agents, intraocular lens decentration and iris capture in eyes with gas-air or silicone oil tamponade and prismatic effects and undesirable light reflexes during vitreoretinal surgery caused by intraocular lens before posterior segment

Postsurgical complications are similar in both approaches. In the two-step procedure, it should be kept in mind that the surgeon is facing complications associated with phacoemulsification and pars plana vitrectomy, just as in the combined procedure, but during separate surgical sessions (Koenig et al, 1990). The most common intraoperative complications associated with phacoemulsification include tears during anterior capsulorrhexis, rupture of the posterior capsule with the phaco tip, and dislocation of

Scleral buckling surgery is associated with a lower rate of cataract formation than pars plana vitrectomy or combined vitrectomy and buckle surgeries. Therefore, scleral buckling may be considered as the primary surgical option in the treatment of uncomplicated rhegmatogenous retinal detachments where the crystalline lens is

Haller and Kerrison reported that eyes which have undergone scleral buckling surgery have good visual outcomes after cataract surgery and a low risk of recurrent retinal detachment. The same study showed more intraoperative complications during extracapsular cataract surgery in patients who had undergone vitrectomy for retinal detachment but a low rate of intraoperative complications in patients with previous scleral buckling (Haller and Kerrison, 1997). Smiddy and associates reported no recurrent retinal tears or detachments in patients who underwent extracapsular cataract surgery after previous scleral buckling with an average follow-up period of 24 months (Smiddy et al, 1988). Ruiz and Saatci reported a favorable outcome for extracapsular cataract surgery with intraocular lens implantation in eyes that had undergone successful scleral buckling. In this study however, 3.4% of eyes developed recurrent retinal redetachment 15 months after cataract surgery (Ruiz and Saatçi, 1991) . Eshete et al

(Grusha et al, 1998; Chang et al, 2002; Ahfat et al, 2003).

developing retinal and iris neovascularization.

nuclear fragments into the vitreous cavity.

**3.2 Cataract surgery and scleral buckling surgery**

procedures.

sufficiently clear.

conjunctival fibrosis, easier vitreous shaving, better access to the vitreous base, and more effective postoperative tamponade (Koenig et al, 1990; Pollack et al, 2004; Axer-Siegel et al, 2006; Mochizuki et al, 2006; Treumer et al, 2006; Demetriades et al, 2003; Wensheng et al, 2009).

There are three ways to start this procedure. One option is to introduce the vitrectomy trocars, then perform phacoemulsification, complete the vitrectomy via pars plana, and leave intraocular lens implantation as the last step. A second option is to start by performing phacoemulsification and, once this is completed, introduce the vitrectomy transconjunctival trocars. Perform the vitrectomy via pars plana and, once again, leave intraocular lens implantation for the last step. A third option is to perform phacoemulsification with intraocular lens implantation first, and then perform vitreoretinal surgery. After phacoemulsification and intraocular lens implantation, a prophylactic 10-0 nylon suture is placed to avoid anterior chamber collapse, and iris prolapse. It is recommended to leave viscoelastic material in the anterior chamber during the vitrectomy procedure to maintain anterior chamber depth.

#### **3.1.2 Two-step procedure**

The vitreous body is semi-solid, thick and viscous in a healthy eye. These properties allow it to help support the cataract during surgery when the patient is supine. This results in a normal anterior chamber depth and a more routine cataract surgery. In an eye that has undergone a prior vitrectomy, saline and aqueous have replaced the vitreous, resulting in a fluid-filled eye that does not provide additional support of the cataract during phacoemulsification. This causes the anterior chamber to be overly deep during cataract surgery. To address this, the infusion pressure can be decreased by lowering the bottle height on the phacoemulsification machine. To compensate for lower infusion, the aspiration flow rate should also be dropped.

Additionally, posterior support can be increased by giving a retrobulbar block because the anesthetic bolus will tend to provide pressure to the back of the eye. If there is reverse pupillary block, caused when the iris makes a tight seal on the anterior lens capsule, this can be solved by tenting up the iris with a second instrument or even by placing a single iris hook. These eyes may also have zonular damage or laxity, which can lead to difficulties during cataract surgery. If there is a posterior capsule rupture, either from the vitrectomy or cataract surgery, the lens nucleus should be brought forward, out of the capsular bag, and viscoelastic should be placed behind it to support it. If any cataract pieces are displaced into the posterior segment, they will rapidly descend onto the retina due to the lack of vitreous. These pieces are best removed by the vitreoretinal surgeon using a pars plana approach.

Patients who undergo cataract surgery after a prior retinal surgery are at higher risk for some postoperative complications. Patients with prior macular surgery are more prone to cystoid macular edema, even after an uncomplicated cataract surgery. These patients should be treated with anti-inflammatory medications for a prolonged period, and their macular status should be monitored at postoperative visits. Patients with prior retinal detachment surgery are at a higher risk for a recurrent detachment after cataract surgery, so their retinal periphery should be checked carefully. In addition, it may take longer for these patients to heal after surgery and to achieve a stable postoperative refraction. Although total intraoperative time is shorter for a two step procedure compared with a combined

conjunctival fibrosis, easier vitreous shaving, better access to the vitreous base, and more effective postoperative tamponade (Koenig et al, 1990; Pollack et al, 2004; Axer-Siegel et al, 2006; Mochizuki et al, 2006; Treumer et al, 2006; Demetriades et al, 2003; Wensheng et al,

There are three ways to start this procedure. One option is to introduce the vitrectomy trocars, then perform phacoemulsification, complete the vitrectomy via pars plana, and leave intraocular lens implantation as the last step. A second option is to start by performing phacoemulsification and, once this is completed, introduce the vitrectomy transconjunctival trocars. Perform the vitrectomy via pars plana and, once again, leave intraocular lens implantation for the last step. A third option is to perform phacoemulsification with intraocular lens implantation first, and then perform vitreoretinal surgery. After phacoemulsification and intraocular lens implantation, a prophylactic 10-0 nylon suture is placed to avoid anterior chamber collapse, and iris prolapse. It is recommended to leave viscoelastic material in the anterior chamber during the vitrectomy procedure to maintain

The vitreous body is semi-solid, thick and viscous in a healthy eye. These properties allow it to help support the cataract during surgery when the patient is supine. This results in a normal anterior chamber depth and a more routine cataract surgery. In an eye that has undergone a prior vitrectomy, saline and aqueous have replaced the vitreous, resulting in a fluid-filled eye that does not provide additional support of the cataract during phacoemulsification. This causes the anterior chamber to be overly deep during cataract surgery. To address this, the infusion pressure can be decreased by lowering the bottle height on the phacoemulsification machine. To compensate for lower infusion, the

Additionally, posterior support can be increased by giving a retrobulbar block because the anesthetic bolus will tend to provide pressure to the back of the eye. If there is reverse pupillary block, caused when the iris makes a tight seal on the anterior lens capsule, this can be solved by tenting up the iris with a second instrument or even by placing a single iris hook. These eyes may also have zonular damage or laxity, which can lead to difficulties during cataract surgery. If there is a posterior capsule rupture, either from the vitrectomy or cataract surgery, the lens nucleus should be brought forward, out of the capsular bag, and viscoelastic should be placed behind it to support it. If any cataract pieces are displaced into the posterior segment, they will rapidly descend onto the retina due to the lack of vitreous. These pieces are best removed by the vitreoretinal surgeon

Patients who undergo cataract surgery after a prior retinal surgery are at higher risk for some postoperative complications. Patients with prior macular surgery are more prone to cystoid macular edema, even after an uncomplicated cataract surgery. These patients should be treated with anti-inflammatory medications for a prolonged period, and their macular status should be monitored at postoperative visits. Patients with prior retinal detachment surgery are at a higher risk for a recurrent detachment after cataract surgery, so their retinal periphery should be checked carefully. In addition, it may take longer for these patients to heal after surgery and to achieve a stable postoperative refraction. Although total intraoperative time is shorter for a two step procedure compared with a combined

2009).

anterior chamber depth.

**3.1.2 Two-step procedure**

using a pars plana approach.

aspiration flow rate should also be dropped.

approach, patients who undergo sequential surgeries may experience increasing discomfort. Another disadvantage is cost; two surgeries cost more than the combined procedure (Grusha et al, 1998; Chang et al, 2002; Ahfat et al, 2003).

There are advantages and disadvantages to each approach, but both are safe and effective. Combined surgery requires a shorter postoperative recovery time, anterior vitreous structures can be removed without risk of touching the lens, visualization of the posterior pole is good during vitrectomy, and it involves only one surgical session, which may reduce patient discomfort and decrease risks and costs. Also, patients with retinal vascular diseases frequently undergo panretinal photocoagulation during the operation, decreasing the risk of developing retinal and iris neovascularization.

However, there are potential disadvantages to combined surgery, such as increased operating time and stress on the surgeon, difficulty visualizing the capsulorrhexis because of an absent or reduced red reflex, cataract wound dehiscence caused by globe manipulation during subsequent vitreous surgery, and intraoperative miosis after cataract extraction. Other disadvantages include bleeding from anterior structures, loss of corneal transparency from corneal edema and Descemet's folds, inadvertent exchange of anterior segment fluids with posterior segment tamponading agents, intraocular lens decentration and iris capture in eyes with gas-air or silicone oil tamponade and prismatic effects and undesirable light reflexes during vitreoretinal surgery caused by intraocular lens before posterior segment procedures.

Postsurgical complications are similar in both approaches. In the two-step procedure, it should be kept in mind that the surgeon is facing complications associated with phacoemulsification and pars plana vitrectomy, just as in the combined procedure, but during separate surgical sessions (Koenig et al, 1990). The most common intraoperative complications associated with phacoemulsification include tears during anterior capsulorrhexis, rupture of the posterior capsule with the phaco tip, and dislocation of nuclear fragments into the vitreous cavity.

#### **3.2 Cataract surgery and scleral buckling surgery**

Scleral buckling surgery is associated with a lower rate of cataract formation than pars plana vitrectomy or combined vitrectomy and buckle surgeries. Therefore, scleral buckling may be considered as the primary surgical option in the treatment of uncomplicated rhegmatogenous retinal detachments where the crystalline lens is sufficiently clear.

Haller and Kerrison reported that eyes which have undergone scleral buckling surgery have good visual outcomes after cataract surgery and a low risk of recurrent retinal detachment. The same study showed more intraoperative complications during extracapsular cataract surgery in patients who had undergone vitrectomy for retinal detachment but a low rate of intraoperative complications in patients with previous scleral buckling (Haller and Kerrison, 1997). Smiddy and associates reported no recurrent retinal tears or detachments in patients who underwent extracapsular cataract surgery after previous scleral buckling with an average follow-up period of 24 months (Smiddy et al, 1988). Ruiz and Saatci reported a favorable outcome for extracapsular cataract surgery with intraocular lens implantation in eyes that had undergone successful scleral buckling. In this study however, 3.4% of eyes developed recurrent retinal redetachment 15 months after cataract surgery (Ruiz and Saatçi, 1991) . Eshete et al

Cataract Surgery in Retina Patients 385

Ahfat FG, Yuen CHW & Groenewald CP (2003). Phacoemulsification and intraocular lens

Alden ER, Kalina RE & Hodson WA (1973). Transient cataracts in low birth weight infants. *J*

Alexandrakis G, Chaudhry NA, Flynn HW Jr & Murray TG (1999). Combined cataract

Alió JL, de la Hoz F, Ruiz-Moreno JM & Salem TF (2000). Cataract surgery in highly myopic

Ando A, Nishimura T & Uyama M (1998). Surgical outcome on combined procedures of lens

Appiah AP, Hirose T & Kado M (1988). A review of 324 cases of idiopathic preremacular

Axer-Siegel R, Dotan G, Rosenblatt I, Benjamini Y & Weinberger D (2006). Combined pars

Brooks HL Jr, Meyer D, Shields JA, Balas AG, Nelson LB & Fontanesi J (1990). Removal of

Chang MA, Parides MK, Chang S & Braunstein RE (2002). Outcome of phacoemulsification

Chaudhry NA, Flynn HW Jr, Murray TG, Belfort A & Mello M Jr (2000). Combined cataract surgery and vitrectomy for recurrent retinal detachment. *Retina.* 2000;20: 257–261. Cheema RA, Al-Mubarak MM, Amin YM & Cheema M (2009). Role of combined cataract

Christiansen SP & Bradford JD (1995). Cataract in infants treated with argon laser

Christiansen SP & Bradford JD (1997). Cataracts following diode laser photoablation for

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Dong LM, Stark WJ, Jefferys JL, Al-Hazzaa S, Bressler SB, Solomon SD & Bressler NM

epiretinal membrane. *Ophthalmic Surg Lasers*. 1998; 29: 974–979.

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(2009). *Arch Ophthalmol* 2009; 127(11):1412–1419.

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surgery, intraocular lens insertion, and vitrectomy in eyes with pars plana vitrectomy and lens management 141 idiopathic epiretinal membrane. *Ophthalmic*

eyes corrected by phakic anterior chamber angle-supported lenses. *J Cataract Refract*

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demonstrated that phacoemulsification and intraocular lens implantation can be performed safely after scleral buckling surgery and excellent best-corrected visual acuity results can be attained in most eyes without any modification in surgical technique. No eye had retinal redetachment in their study (Eshete et al, 2000).

Combining phacoemulsification with scleral buckling rather than vitrectomy may be a more optimal surgical decision in selected cases. However, proper case selection (fresh rhegmatogenous retinal detachment with minimal proliferative vitreoretinopathy, etc) and familiarity with the surgical techniques is mandatory for achieving higher success rates. Conventional large incision cataract surgeries cannot be combined with scleral buckling because of the instability of the wound and inability to maintain the eyeball contour because of fluctuation in intraocular pressure (Garder et al, 1993). Also, there is an increased risk of vitreous loss and aggravation of retinal pathology. Phacoemulsification provides a better stability of the wound during the procedure because of the small incision size.

Combined scleral buckling for retinal detachment and phacoemulsification was first reported by Lazar and Bracha (Lazar and Bracha, 1977). Girard and Saade reported a case series of 15 patients who underwent a triple procedure; phacoemulsification, intraocular lens implantation and scleral buckling for recurrent rhegmatogenous retinal detachment with a significant cataract (Girard and Sade, 1997). They noted high postoperative intraocular pressure in only one of their cases, which was attributed to the use of viscoelastic. Overall anatomical success rate was 87% and functional success was 54%. They concluded that combined phacoemulsification and scleral buckling was a safe and effective procedure. Tsai and Wu confirmed the effectiveness of cataract surgery together with scleral buckling, with no significant complications. The authors believed that combined cataract surgery and scleral buckling can improve visualization for detection of peripheral retinal holes and can improve the results of the operation (Tsai and Wu, 2004).

#### **3.3 Cataract surgery and pneumatic retinopexy**

Pneumatic retinopexy is an alternative to scleral buckling for the surgical repair of selected retinal detachments. A gas bubble is injected into the vitreous cavity, and the patient is positioned so that the bubble closes the retinal break, allowing absorption of the subretinal fluid. Cryotherapy or laser photocoagulation is applied around the retinal break to form a permanent seal. Temporary gas tamponade for pneumatic retinopexy is not associated with permanent changes in lens transparency (Mougharbel et al, 1994).

#### **4. Conclusion**

Cataract surgery improves vision in patients with preexisting retinal disease and is necessary for the physician to monitor and treat the underlying pathology. However, surgeons must be cautious about certain retinal diseases and previous retinal surgeries which can make a patient more prone to complications following cataract surgery. Understanding the risk factors and applying certain methods of preventative treatment can minimize both intraoperative and postoperative effects. In addition, working closely with retina specialists in the management of patients whose cataract surgery is complicated by retinal issues may help the cataract surgeon to bring these cases to a more successful outcome.

#### **5. References**

384 Cataract Surgery

demonstrated that phacoemulsification and intraocular lens implantation can be performed safely after scleral buckling surgery and excellent best-corrected visual acuity results can be attained in most eyes without any modification in surgical technique. No eye had retinal

Combining phacoemulsification with scleral buckling rather than vitrectomy may be a more optimal surgical decision in selected cases. However, proper case selection (fresh rhegmatogenous retinal detachment with minimal proliferative vitreoretinopathy, etc) and familiarity with the surgical techniques is mandatory for achieving higher success rates. Conventional large incision cataract surgeries cannot be combined with scleral buckling because of the instability of the wound and inability to maintain the eyeball contour because of fluctuation in intraocular pressure (Garder et al, 1993). Also, there is an increased risk of vitreous loss and aggravation of retinal pathology. Phacoemulsification provides a better stability of the wound during the procedure

Combined scleral buckling for retinal detachment and phacoemulsification was first reported by Lazar and Bracha (Lazar and Bracha, 1977). Girard and Saade reported a case series of 15 patients who underwent a triple procedure; phacoemulsification, intraocular lens implantation and scleral buckling for recurrent rhegmatogenous retinal detachment with a significant cataract (Girard and Sade, 1997). They noted high postoperative intraocular pressure in only one of their cases, which was attributed to the use of viscoelastic. Overall anatomical success rate was 87% and functional success was 54%. They concluded that combined phacoemulsification and scleral buckling was a safe and effective procedure. Tsai and Wu confirmed the effectiveness of cataract surgery together with scleral buckling, with no significant complications. The authors believed that combined cataract surgery and scleral buckling can improve visualization for detection of peripheral retinal holes and can improve the results of the operation (Tsai

Pneumatic retinopexy is an alternative to scleral buckling for the surgical repair of selected retinal detachments. A gas bubble is injected into the vitreous cavity, and the patient is positioned so that the bubble closes the retinal break, allowing absorption of the subretinal fluid. Cryotherapy or laser photocoagulation is applied around the retinal break to form a permanent seal. Temporary gas tamponade for pneumatic retinopexy is not associated with

Cataract surgery improves vision in patients with preexisting retinal disease and is necessary for the physician to monitor and treat the underlying pathology. However, surgeons must be cautious about certain retinal diseases and previous retinal surgeries which can make a patient more prone to complications following cataract surgery. Understanding the risk factors and applying certain methods of preventative treatment can minimize both intraoperative and postoperative effects. In addition, working closely with retina specialists in the management of patients whose cataract surgery is complicated by retinal issues may help the cataract surgeon

redetachment in their study (Eshete et al, 2000).

because of the small incision size.

**3.3 Cataract surgery and pneumatic retinopexy**

to bring these cases to a more successful outcome.

permanent changes in lens transparency (Mougharbel et al, 1994).

and Wu, 2004).

**4. Conclusion**

Cataract Surgery in Retina Patients 387

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**26**

*USA*

**Cataract Surgery in Patients with a History of**

Both pediatric and adult malignant intraocular ocular tumors are often treated with external beam radiation or brachytherapy. Because the lens is the most radiosensitive ocular structure, cataracts are a common occurrence after such treatments. Some studies report up to an 87% occurrence of cataracts within 3 years in retinoblastoma patients treated with external beam radiation (Blach et al., 1996; Fontanesi et al., 1996). In the Collaborative Ocular Melanoma Study, the largest multicenter prospective randomized trial ever performed for uveal melanoma patients, 83% of patients had developed cataracts by 5 years post-treatment (Collaborative Ocular Melanoma Study Group, 2007). Because many patients treated for intraocular tumors survive for extended periods and have the capacity for excellent vision, cataract surgery is an important part of the treatment algorithm for these patients. However, this group of patients poses a unique set of surgical challenges not present in standard cataract patients. In this chapter, we will review the incidence, surgical techniques, visual and anatomic outcomes, and complications for this unique group of

Cataract is a well-known complication after radiation to the eye after all forms of radiation. Many studies have indicated that after external beam radiation therapy, cataracts can develop after doses as low as 2 Gy (Dynlacht, 2006; Gordon, 1995). For over 100 years, external beam radiation has been used to treat retinoblastoma because in certain cases, it can achieve tumor control and enable retention of vision when other treatment modalities have failed (Chodick et al., 2009). Although other treatment modalities such as chemotherapy and laser are more commonly used for retinoblastoma patients at the present time, external beam radiation is still used as salvage therapy in advanced cases. Furthermore, many patients who received external beam radiation in the past are still under the care of ophthalmic oncologists and are currently in need of or will need cataract surgery in the future. Chodick et al. examined the records of a series of 753 patients who were diagnosed with retinoblastoma from 1914 to 1984. Only one patient who had not had radiotherapy had

**1. Introduction**

patients.

**2.1 Epidemiology 2.1.1 Incidence**

**2. Cataracts in retinoblastoma patients**

**Retinoblastoma and Melanoma**

*Bascom Palmer Eye Institute, Department of Ophthalmology,*

A.C. Schefler and T.G. Murray

*University of Miami Miller School of Medicine*

### **Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma**

A.C. Schefler and T.G. Murray *Bascom Palmer Eye Institute, Department of Ophthalmology, University of Miami Miller School of Medicine USA*

#### **1. Introduction**

390 Cataract Surgery

Wong TY, Klein BE, Klein R, Tomany SC & Lee KE (2001) Refractive errors and incident cataracts: the Beaver Dam eye study. *Invest. Ophthalmol. Vis. Sci*. 42, 1449–1454. Van de Schaft TL, Mooy CM, Mulder PGH, Pameyer JH & de Jong PT (1994). Increased

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*Sc.* 43, 3625–3632.

prevalence of disciform macular degeneration after cataract extraction with

cataract and cataract surgery: the blue mountains eye study. *Inves. Ophthalmol Vis*

Both pediatric and adult malignant intraocular ocular tumors are often treated with external beam radiation or brachytherapy. Because the lens is the most radiosensitive ocular structure, cataracts are a common occurrence after such treatments. Some studies report up to an 87% occurrence of cataracts within 3 years in retinoblastoma patients treated with external beam radiation (Blach et al., 1996; Fontanesi et al., 1996). In the Collaborative Ocular Melanoma Study, the largest multicenter prospective randomized trial ever performed for uveal melanoma patients, 83% of patients had developed cataracts by 5 years post-treatment (Collaborative Ocular Melanoma Study Group, 2007). Because many patients treated for intraocular tumors survive for extended periods and have the capacity for excellent vision, cataract surgery is an important part of the treatment algorithm for these patients. However, this group of patients poses a unique set of surgical challenges not present in standard cataract patients. In this chapter, we will review the incidence, surgical techniques, visual and anatomic outcomes, and complications for this unique group of patients.

#### **2. Cataracts in retinoblastoma patients**

#### **2.1 Epidemiology**

#### **2.1.1 Incidence**

Cataract is a well-known complication after radiation to the eye after all forms of radiation. Many studies have indicated that after external beam radiation therapy, cataracts can develop after doses as low as 2 Gy (Dynlacht, 2006; Gordon, 1995). For over 100 years, external beam radiation has been used to treat retinoblastoma because in certain cases, it can achieve tumor control and enable retention of vision when other treatment modalities have failed (Chodick et al., 2009). Although other treatment modalities such as chemotherapy and laser are more commonly used for retinoblastoma patients at the present time, external beam radiation is still used as salvage therapy in advanced cases. Furthermore, many patients who received external beam radiation in the past are still under the care of ophthalmic oncologists and are currently in need of or will need cataract surgery in the future. Chodick et al. examined the records of a series of 753 patients who were diagnosed with retinoblastoma from 1914 to 1984. Only one patient who had not had radiotherapy had

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 393

cataract, we strongly recommend enucleation rather than performing cataract surgery with its associated risk of tumor recurrence which may or may not be able to be adequately treated.

> **Procedure (No. of eyes)**

ECCE + caps+Ant Vtx + PCIOL

Phaco+caps+

ECCE+PPV+P

ICCE; ECCE;

172 mos) NA Mean 184

ECCE ± caps ± Ant Vtx ± PCIOL

ECCE or Asp ± caps ± Ant Vtx ± IOL

Table 1. Reports of cataract surgery in patients with a history of retinoblastoma. Modified and adapted from Payne et al. (2009). NA, Data not reported; Ant Vtx, anterior vitrectomy; ECCE, extracapsular cataract extraction; ICCE, intracapsular cataract extraction; IOL, posterior chamber intraocular lens; Phaco, phacoemulsification; PPL, pars plana lensectomy;

Ant Vtx+PCIOL

CIOL

**Median follow up, mos (range)**

144) Asp or PPL 72 (6-178) 3 20/20-20/50 (19)

Mean 20

mos) Asp (60-189) 0 20/60 (2); HM (1)

PCIOL 144 0 20/30

mos Asp (24-42) 0 "No improvement"

PPL 72 (12-360) 5 >20/200 (12)

72 (13-148) 0

(19-105) <sup>0</sup>

Mean 90 ± <sup>69</sup><sup>3</sup>

Mean 66

mos) Asp ± PPV (24-78) 0 6/18 (2)

**No. of RB recurences**

(6-39 0 20/20-20/30 (6)

NA 0 >6/9 (4) at 6 wks

(30-94) <sup>0</sup>20/20-20/40 (11)

(60-339) <sup>1</sup>20/30; CF; HM;

Enuc

CF (2) HM (2)

20/20 (4) 20/20-20/200 (9) <20/200 (2) NLP (3) NA (3)

**Visual outcome (No. of eyes)**

20/80-CF (12)

20/50-20/250 (5)

20/200-20/400 (5)

20/20-20/60 (6) 20/70-20/200 (2)

20/20-20/60 (3) 20/70-20/200 (4) 20/400 (4) CF (5) HM (1) F+F (1) Enuc (1)

**Author, Year No. of**

Monge et al.,

Brooks et al.,

Portellos &

Madreperla et

Bhattacharjee et

Shanmugam et

Honovar et al.,

Moshfeghi et al.,

Hoehn et al.,

Osman et al.,

**eyes**

1986 2 30 mos (72

1990 42 29 mos (17-

Buckley, 1998 11 55 mos (16-

al., 2000 3 34 mos (9-40

al., 2004 5 Minimum 24

Sinha et al., 2004 9 Minimum 12

Miller et al., 2005 16 Minimum 18

2005 34 16 mos (3-54

2005 4 89 mos (12-

Payne et al., 2008 12 35 mos (17-

2011 21 21.5 ms (3-

88)

al., 2003 1 84 mos ECCE+Caps+

mos

mos

mos)

240)

2010 19 NA Asp + IOL Mean 58

164 mos)

NLP, no light perception; HM, hand motion; CF, count fingers.

**Median Quiescent interval (Range)**

cataract surgery while 51 cataract surgeries were performed in patients who had had radiation. Overall, Kaplan-Meier analysis demonstrated that after 40 years of follow-up, 77% of eyes with 2 or 3 radiotherapy treatments underwent cataract surgery. The estimated survival time to cataract surgery in eyes that did not undergo radiation was 71.7 years compared to 50.8 years and 31.6 years for eyes treated with one and 2 or 3 radiotherapy treatments, respectively. Of note, many patients in this study underwent older forms of treatment with more radiation scatter such as orthovoltage radiation and cobalt plaques. Nonetheless, the incidence of cataracts requiring cataract surgery in retinoblastoma survivors was high and underscored the necessity for ongoing ophthalmic follow-up in these patients.

#### **2.1.2 Risk factors**

In the study by Chodick et al., the clinical risk factors that contributed to cataract development requiring cataract surgery were: increased number of external beam radiation treatments, diagnosis of retinoblastoma after the first year of life (versus earlier), and radiation dose. No other studies published up until this point have had extended follow-up or enough power to assess the risk factors for cataract development adequately.

#### **2.2 Visual and anatomic results**

#### **2.2.1 Visual results**

There have been several studies examining the visual outcomes in retinoblastoma survivors who have undergone cataract surgery (Table 1).

The data from Table 1 suggests that visual outcomes in these patients can be surprisingly excellent. In particular, the studies by Miller et al. (2005), Payne et al. (2008), and Hoehn et al. (2010) reported good visual results and no major complications. Patients in this population have many other reasons to have poor visual outcomes including tumor involvement of the macula, radiation retinopathy, progressive retinal scarring from laser or cryotherapy treatments, and amblyopia.

#### **2.3 Surgical approach and complications**

Controversies in pediatric cataract management include the surgical approach, management of the posterior capsule and anterior vitreous, and IOL implantation. Table 1 demonstrates that there has been a wide range of approaches to these controversies in retinoblastoma patients. Our group, Miller et al. (2005) reported good visual and anatomic results in 16 patients after ECCE/PPV/PCIOL. Payne et al. (2008) also reported good results with an ECCE approach with a capsulotomy, anterior vitrectomy, and IOL placed only in some patients. In contrast, Hoehn et al. (2010) reported outstanding results with only a lens aspiration and IOL with no posterior capsulotomy, deferring this procedure to a later EUA when needed.

The major concern for patients undergoing any intraocular procedure who have a history of retinoblastoma is tumor recurrence or the development of metastatic disease as a result of the surgery. Recurrences have been reported in series by Brooks et al. (1990), Honovar et al. (2005), Moshfeghi et al. (2005), and Osman et al. (2011). All of these series included patients who had short quiescent intervals from the time of attainment of local control of the retinoblastoma and cataract surgery, sometimes as short as 3 months. It seems that a longer quiescent interval is the only identifiable risk factor for this devastating complication. In patients with a short quiescent interval whose fundus cannot be visualized due to a dense

cataract surgery while 51 cataract surgeries were performed in patients who had had radiation. Overall, Kaplan-Meier analysis demonstrated that after 40 years of follow-up, 77% of eyes with 2 or 3 radiotherapy treatments underwent cataract surgery. The estimated survival time to cataract surgery in eyes that did not undergo radiation was 71.7 years compared to 50.8 years and 31.6 years for eyes treated with one and 2 or 3 radiotherapy treatments, respectively. Of note, many patients in this study underwent older forms of treatment with more radiation scatter such as orthovoltage radiation and cobalt plaques. Nonetheless, the incidence of cataracts requiring cataract surgery in retinoblastoma survivors was high and underscored the necessity for ongoing ophthalmic follow-up in

In the study by Chodick et al., the clinical risk factors that contributed to cataract development requiring cataract surgery were: increased number of external beam radiation treatments, diagnosis of retinoblastoma after the first year of life (versus earlier), and radiation dose. No other studies published up until this point have had extended follow-up

There have been several studies examining the visual outcomes in retinoblastoma survivors

The data from Table 1 suggests that visual outcomes in these patients can be surprisingly excellent. In particular, the studies by Miller et al. (2005), Payne et al. (2008), and Hoehn et al. (2010) reported good visual results and no major complications. Patients in this population have many other reasons to have poor visual outcomes including tumor involvement of the macula, radiation retinopathy, progressive retinal scarring from laser or

Controversies in pediatric cataract management include the surgical approach, management of the posterior capsule and anterior vitreous, and IOL implantation. Table 1 demonstrates that there has been a wide range of approaches to these controversies in retinoblastoma patients. Our group, Miller et al. (2005) reported good visual and anatomic results in 16 patients after ECCE/PPV/PCIOL. Payne et al. (2008) also reported good results with an ECCE approach with a capsulotomy, anterior vitrectomy, and IOL placed only in some patients. In contrast, Hoehn et al. (2010) reported outstanding results with only a lens aspiration and IOL with no

The major concern for patients undergoing any intraocular procedure who have a history of retinoblastoma is tumor recurrence or the development of metastatic disease as a result of the surgery. Recurrences have been reported in series by Brooks et al. (1990), Honovar et al. (2005), Moshfeghi et al. (2005), and Osman et al. (2011). All of these series included patients who had short quiescent intervals from the time of attainment of local control of the retinoblastoma and cataract surgery, sometimes as short as 3 months. It seems that a longer quiescent interval is the only identifiable risk factor for this devastating complication. In patients with a short quiescent interval whose fundus cannot be visualized due to a dense

posterior capsulotomy, deferring this procedure to a later EUA when needed.

or enough power to assess the risk factors for cataract development adequately.

these patients.

**2.1.2 Risk factors**

**2.2.1 Visual results**

**2.2 Visual and anatomic results**

who have undergone cataract surgery (Table 1).

cryotherapy treatments, and amblyopia.

**2.3 Surgical approach and complications**

cataract, we strongly recommend enucleation rather than performing cataract surgery with its associated risk of tumor recurrence which may or may not be able to be adequately treated.

Table 1. Reports of cataract surgery in patients with a history of retinoblastoma. Modified and adapted from Payne et al. (2009). NA, Data not reported; Ant Vtx, anterior vitrectomy; ECCE, extracapsular cataract extraction; ICCE, intracapsular cataract extraction; IOL, posterior chamber intraocular lens; Phaco, phacoemulsification; PPL, pars plana lensectomy; NLP, no light perception; HM, hand motion; CF, count fingers.

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 395

The number of studies reporting risk factors for cataract development after Iodine 125 brachytherapy is limited. Overall, a review of the literature indicates that cataractogenesis is likely a function of tumor size, tumor location, radiation dose, and patient age. There appears to be no obvious advantage to any particular form of radiation (i.e. Cobalt 60, Ruthenium 106, proton beam therapy) (Collaborative Ocular Melanoma Study, 2007).

Several studies have reported on visual acuity results after cataract surgery. In the Collaborative Ocular Melanoma Study, in patients with medium-sized tumors undergoing cataract surgery, the median best corrected visual acuity at baseline was 20/25 (Collaborative Ocular Melanoma Study Group, 2007). In the group that underwent cataract surgery, the median visual acuity before surgery was 20/125 (range 20/25< 20/1600) and median visual acuity at the study visit immediately after cataract surgery was 20/50 (range 20/20-20/1600). In the study, 32 of the 48 eyes (66%) had improved vision of greater than or equal to 2 lines, 13 eyes (27%) remained stable, and 3 (6%) had decreased vision of greater than or equal to 2 lines at final follow-up. Figure 1 demonstrates the change in visual acuity in the 3 months after cataract surgery in patients in the Collaborative Ocular Melanoma

Fig. 1. Patients' visual acuity before and in the 3 visits after cataract surgery in the Collaborative Ocular Melanoma Study. The dot represents the mean of the box and the range of the box extends from the 25th percentile to the 75th percentile. The whiskers extend

up to 1.5 times with interquartile range from each end of the box. Reproduced with

Our group investigated visual acuity outcomes in patients undergoing cataract surgery with injection of intravitreal triamcinolone injection (Cebulla et al., 2008). In this study, 51 eyes of 49 patients with a history of choroidal nevi or uveal melanoma underwent cataract surgery with injection of intravitreal triamcinolone (IVTA) during surgery. There were 30 patients

permission from Collaborative Ocular Melanoma Study (2007).

**3.1.2 Risk factors**

**3.2.1 Visual results**

Study.

**3.2 Visual and anatomic results**

#### **3. Cataracts in uveal melanoma patients**

#### **3.1 Epidemiology**

#### **3.1.1 Incidence**

There have been several studies examining the incidence and risk factors for the development of cataracts after Iodine 125 brachytherapy, the most commonly employed treatment for uveal melanoma in the United States (Table 1). Taking these studies as a group, approximately 40% of all patients have been reported to develop cataracts with most studies having a follow-up period of 5 years. Among these studies, however, the Collaborative Ocular Melanoma Study trial was the only prospective, multicenter trial and the rate of cataract development in this study was much higher, 83% by 5 years.

Table 2. Rates of Cataract Development after Cataract from Iodine 125 Brachytherapy for Uveal Melanoma. Updated and Adapted from Collaborative Ocular Melanoma Study Group (2007). NA, Data not reported.

#### **3.1.2 Risk factors**

394 Cataract Surgery

There have been several studies examining the incidence and risk factors for the development of cataracts after Iodine 125 brachytherapy, the most commonly employed treatment for uveal melanoma in the United States (Table 1). Taking these studies as a group, approximately 40% of all patients have been reported to develop cataracts with most studies having a follow-up period of 5 years. Among these studies, however, the Collaborative Ocular Melanoma Study trial was the only prospective, multicenter trial and

> **Follow-up time Median (Range)**

5 yrs NA NA NA

**Time to cataract development Median (Range)**

( 2 yrs) NA NA

(23-112) NA NA

(20-57) NA NA

10.4) <1-8 yrs NA

yrs)

by 5 yrs NA NA Gender, age,

NA (data not provided in this format)

90) 3-53 mos Tumor location

2.5 yrs (6 mos-5

**Risk factors for cataract development**

age, diabetes

diameter

Age, Baseline tumor size, Dose to lens

the rate of cataract development in this study was much higher, 83% by 5 years.

**Rate of cataract development**

et al., 1992 53/4 NA NA NA NA

et al., 1993 239/33 25-50% by 5 yrs NA, (2-10 yrs) 9-81 mos NA

et al., 2002 152/5 28% 33 mos NA NA

et al., 2005 92/9 11% 31 mos (4-86) NA NA

Meta-analysis 1543/614 40% – – –

Table 2. Rates of Cataract Development after Cataract from Iodine 125 Brachytherapy for Uveal Melanoma. Updated and Adapted from Collaborative Ocular Melanoma Study

et al., 2004 96/65 2% by 2 yrs NA Median 18 mos Dose to lens,

et al., 1987 26/3 NA Mean 45 mos

et al., 1990 56/20 NA Mean 39 mos

et al., 1993 144/43 NA 46 mos (25-

et al., 2004 89/57 69% by 3 yrs 3.5 yrs (0.3-

NA 21% by 2 yrs, 50%

et al., 1988 58/13 NA Mean 48.7

**3. Cataracts in uveal melanoma patients**

**No. of patients at risk/No. of cataracts**

et al., 2002 270/NA 2% at 2 yrs, 6% at

COMS, 2007 538/362 83% by 5 yrs

Group (2007). NA, Data not reported.

**3.1 Epidemiology 3.1.1 Incidence**

**Author, Year**

Garretson

Bosworth

Mameghan

Quivey

Shields

Bechrakis

Fontanesi

Puusari

Puusari

Stack

Lumbroso-Le Rouic et al., 2004

Lean

The number of studies reporting risk factors for cataract development after Iodine 125 brachytherapy is limited. Overall, a review of the literature indicates that cataractogenesis is likely a function of tumor size, tumor location, radiation dose, and patient age. There appears to be no obvious advantage to any particular form of radiation (i.e. Cobalt 60, Ruthenium 106, proton beam therapy) (Collaborative Ocular Melanoma Study, 2007).

#### **3.2 Visual and anatomic results**

#### **3.2.1 Visual results**

Several studies have reported on visual acuity results after cataract surgery. In the Collaborative Ocular Melanoma Study, in patients with medium-sized tumors undergoing cataract surgery, the median best corrected visual acuity at baseline was 20/25 (Collaborative Ocular Melanoma Study Group, 2007). In the group that underwent cataract surgery, the median visual acuity before surgery was 20/125 (range 20/25< 20/1600) and median visual acuity at the study visit immediately after cataract surgery was 20/50 (range 20/20-20/1600). In the study, 32 of the 48 eyes (66%) had improved vision of greater than or equal to 2 lines, 13 eyes (27%) remained stable, and 3 (6%) had decreased vision of greater than or equal to 2 lines at final follow-up. Figure 1 demonstrates the change in visual acuity in the 3 months after cataract surgery in patients in the Collaborative Ocular Melanoma Study.

Fig. 1. Patients' visual acuity before and in the 3 visits after cataract surgery in the Collaborative Ocular Melanoma Study. The dot represents the mean of the box and the range of the box extends from the 25th percentile to the 75th percentile. The whiskers extend up to 1.5 times with interquartile range from each end of the box. Reproduced with permission from Collaborative Ocular Melanoma Study (2007).

Our group investigated visual acuity outcomes in patients undergoing cataract surgery with injection of intravitreal triamcinolone injection (Cebulla et al., 2008). In this study, 51 eyes of 49 patients with a history of choroidal nevi or uveal melanoma underwent cataract surgery with injection of intravitreal triamcinolone (IVTA) during surgery. There were 30 patients

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 397

Fig. 2B. Photo of tumor apex of patient in Figure 2A at diagnosis.

who had had a history of melanoma treated with Iodine-125 brachytherapy in the cohort. Among the melanoma patients, the visual acuity at 6 months after surgery was worse in 2 patients (11%), stable in 2 patients (11%), and better in 14 patients (78%). At the final followup, vision was worse in 3 patients (10%), stable in 7 patients (24%), and better in 19 patients (66%). The mean number of Snellen lines gained after surgery was 3.9 3.8 (SD) at 6 months and 3.1 3.8 at last follow up (both p<0.001). This data compares favorably to the data outlined above and from Gragoudas et al. (1992), the largest study published to date in patients undergoing proton beam radiotherapy (Figures 2A-2G).

Fig. 2A. Patient with uveal melanoma at the time of diagnosis. Visual acuity was 20/40.

who had had a history of melanoma treated with Iodine-125 brachytherapy in the cohort. Among the melanoma patients, the visual acuity at 6 months after surgery was worse in 2 patients (11%), stable in 2 patients (11%), and better in 14 patients (78%). At the final followup, vision was worse in 3 patients (10%), stable in 7 patients (24%), and better in 19 patients (66%). The mean number of Snellen lines gained after surgery was 3.9 3.8 (SD) at 6 months and 3.1 3.8 at last follow up (both p<0.001). This data compares favorably to the data outlined above and from Gragoudas et al. (1992), the largest study published to date in

Fig. 2A. Patient with uveal melanoma at the time of diagnosis. Visual acuity was 20/40.

patients undergoing proton beam radiotherapy (Figures 2A-2G).

Fig. 2B. Photo of tumor apex of patient in Figure 2A at diagnosis.

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 399

Fig. 2D. Patient in Figure 2C after cataract surgery. Note the increased ability to visualize the macula and optic nerve. The patient's best corrected vision improved to 20/60.

Fig. 2C. Patient in Figure 2A, 21 months after diagnosis. Note the yellow hue of the photograph and inability to visual the macula and optic nerve well to assess radiation retinopathy. The vision was 20/80. The patient subsequently underwent cataract surgery with intravitreal triamcinolone acetonide.

Fig. 2C. Patient in Figure 2A, 21 months after diagnosis. Note the yellow hue of the photograph and inability to visual the macula and optic nerve well to assess radiation retinopathy. The vision was 20/80. The patient subsequently underwent cataract surgery

with intravitreal triamcinolone acetonide.

Fig. 2D. Patient in Figure 2C after cataract surgery. Note the increased ability to visualize the macula and optic nerve. The patient's best corrected vision improved to 20/60.

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 401

Fig. 2F. B-scan ultrasound of the tumor in Figure 2E after plaque surgery just before cataract

surgery with an apical height of 5.8 mm.

Fig. 2E. B-scan ultrasound of the tumor in Figure 2A at diagnosis with an apical height of 7.5 mm.

Fig. 2E. B-scan ultrasound of the tumor in Figure 2A at diagnosis with an apical height of

7.5 mm.

Fig. 2F. B-scan ultrasound of the tumor in Figure 2E after plaque surgery just before cataract surgery with an apical height of 5.8 mm.

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 403

The complications reported in large studies of patients undergoing cataract surgery after

0 (0%) 8 (27%) 1 (3%)

13 (27%) 6 (12%) 1 (2%)

7 (10%)

1 (1.4%) 8 (11%) 4 (6%) 2 (3%)

> 2 (3%) 2 (3%) 1 (1%) 1 (1%) 3 (4%) 5 (6%)

**Author Complication Incidence**

Posterior capsular opacity Cystoid macular edema

Enucleation required for tumor recurrence, scleral necrosis, or pain

Enucleation for retinal detachment, neovascular glaucoma, pain, tumor

Table 3. Complications observed after cataract surgery in patients with a history of uveal

Complication profiles seem to have improved over the past 20 years, likely due to the modernization of phacoemulsification techniques such as small incisions, and of the more judicious use of cataract surgery, reserving the procedure only for patients with preoperative visual potential and no pre-existing neovascular glaucoma secondary to

In summary, cataract surgery has been safely performed in patients with a history of both retinoblastoma and melanoma. Controversy still exists regarding the best technique in patients with a history of retinoblastoma, but we have had success with no tumor recurrences in a series of patients with long-term follow-up using a ECCE/PPV/IOL approach. Tumor recurrence is a known risk of this surgery, and patients should demonstrate a quiescent interval of at least 18 months before surgery. Visual results are largely dependent on side effects from the primary tumor. In uveal melanoma patients, cataracts are common after plaque therapy and occur on average 2.5 years after initial treatment. Most patients regain vision and vision is typically limited by radiation retinopathy, cystoid macular edema, and neovascular glaucoma. Surgical adjuncts such as IVTA may help in decreasing the inflammatory nature of these tumors and

Posterior capsular opacity Vitreous hemorrhage Retinal detachment

Cystoid macular edema Neovascular glaucoma Retinal detachment

Tumor recurrence

Dislocated IOL

Tumor recurrence Retinal detachment Vitreous hemorrhage Chronic wound leak Neovascular glaucoma

recurrence

melanoma after plaque brachytherapy or proton beam radiotherapy.

contribute to tumor regression. Further prospective studies are needed.

plaque therapy for uveal melanoma are listed in Table 3.

**3.3 Complications**

Cebulla et al., 2008

COMS et al., 2007

Wachtlin et al., 2000

Gragoudas et al.,

radiation vasculopathy.

**4. Conclusion**

1992

Fig. 2G. B-scan ultrasound of the tumor in Figure 2E eight months after cataract surgery. The tumor's apical height has decreased further to 5.6 mm.

#### **3.2.2 Anatomic outcomes**

The study by our group published in 2008 is the only study to our knowledge which has addressed the issue of anatomic changes induced in the treated melanoma as a result of cataract surgery (Cebulla et al., 2008). In this study, cataract surgery was combined with intravitreal triamcinolone (IVTA) at the time of surgery. Triamcinolone was chosen because it has been demonstrated to inhibit angiogenesis and to have direct antineoplastic activity (DiSorbo et al., 1986; Ebrahem et al., 2006). Furthermore, triamcinolone acetonide has been used to decrease radiation retinopathy and optic neuropathy in eyes with uveal melanoma treated with plaque therapy (Shields et al., 2006). In the study, echographic measurements of patients' tumors were followed in melanoma patients after cataract surgery-IVTA. This analysis demonstrated that 12 (46%) of 26 melanomas diminished in size over a median follow-up period of 11.8 months (range, 1-26 months). Regression analysis demonstrated a shallow but statistically significant (p=0.039) negative slope in tumor height after cataract surgery-IVTA. Tumor growth did not occur in any patients. It was difficult to determine if this effect was due to a continuing effect from previous brachytherapy, although the median interval between plaque therapy and cataract surgery was 34 months (range 7-114 months), and the majority of the decrease in tumor height typically occurs within the first 2.5 years of tumor treatment. Ultimately, in long-term follow-up, the rate of tumor recurrence in this population may be lower than the published rates of 2.0% to 19.4% (Gragoudas et al., 1992; Wachtlin et al., 2000) due to the additional antitumoral effect from the IVTA approach. Further prospective studies are needed to address this question.

#### **3.3 Complications**

402 Cataract Surgery

Fig. 2G. B-scan ultrasound of the tumor in Figure 2E eight months after cataract surgery.

The study by our group published in 2008 is the only study to our knowledge which has addressed the issue of anatomic changes induced in the treated melanoma as a result of cataract surgery (Cebulla et al., 2008). In this study, cataract surgery was combined with intravitreal triamcinolone (IVTA) at the time of surgery. Triamcinolone was chosen because it has been demonstrated to inhibit angiogenesis and to have direct antineoplastic activity (DiSorbo et al., 1986; Ebrahem et al., 2006). Furthermore, triamcinolone acetonide has been used to decrease radiation retinopathy and optic neuropathy in eyes with uveal melanoma treated with plaque therapy (Shields et al., 2006). In the study, echographic measurements of patients' tumors were followed in melanoma patients after cataract surgery-IVTA. This analysis demonstrated that 12 (46%) of 26 melanomas diminished in size over a median follow-up period of 11.8 months (range, 1-26 months). Regression analysis demonstrated a shallow but statistically significant (p=0.039) negative slope in tumor height after cataract surgery-IVTA. Tumor growth did not occur in any patients. It was difficult to determine if this effect was due to a continuing effect from previous brachytherapy, although the median interval between plaque therapy and cataract surgery was 34 months (range 7-114 months), and the majority of the decrease in tumor height typically occurs within the first 2.5 years of tumor treatment. Ultimately, in long-term follow-up, the rate of tumor recurrence in this population may be lower than the published rates of 2.0% to 19.4% (Gragoudas et al., 1992; Wachtlin et al., 2000) due to the additional antitumoral effect from the IVTA

approach. Further prospective studies are needed to address this question.

The tumor's apical height has decreased further to 5.6 mm.

**3.2.2 Anatomic outcomes**

The complications reported in large studies of patients undergoing cataract surgery after plaque therapy for uveal melanoma are listed in Table 3.

Table 3. Complications observed after cataract surgery in patients with a history of uveal melanoma after plaque brachytherapy or proton beam radiotherapy.

Complication profiles seem to have improved over the past 20 years, likely due to the modernization of phacoemulsification techniques such as small incisions, and of the more judicious use of cataract surgery, reserving the procedure only for patients with preoperative visual potential and no pre-existing neovascular glaucoma secondary to radiation vasculopathy.

### **4. Conclusion**

In summary, cataract surgery has been safely performed in patients with a history of both retinoblastoma and melanoma. Controversy still exists regarding the best technique in patients with a history of retinoblastoma, but we have had success with no tumor recurrences in a series of patients with long-term follow-up using a ECCE/PPV/IOL approach. Tumor recurrence is a known risk of this surgery, and patients should demonstrate a quiescent interval of at least 18 months before surgery. Visual results are largely dependent on side effects from the primary tumor. In uveal melanoma patients, cataracts are common after plaque therapy and occur on average 2.5 years after initial treatment. Most patients regain vision and vision is typically limited by radiation retinopathy, cystoid macular edema, and neovascular glaucoma. Surgical adjuncts such as IVTA may help in decreasing the inflammatory nature of these tumors and contribute to tumor regression. Further prospective studies are needed.

Cataract Surgery in Patients with a History of Retinoblastoma and Melanoma 405

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