Dr. Sonam Yangzes, Y16805, Dr. Jagat Ram, Dr. Natasha Gautam, Dr. Ramandeep Singh
Abstract
Background: To evaluate the long-term outcome of children with uveitic cataract undergoing cataract surgery with or without intraocular lens implantation
Methods:
Data were obtained from medical records of uveitic patients less than 16 years of age, operated between January 2000 and January 2014. Out of the 57 eyes of 39 patients ( 26 girls and 13 boys), 19 patients had bilateral involvement while 19 patients had unilateral involvement (median 10), The main outcome measures were visual acuity and post surgical complications.
Results: The average age of the uveitic children undergoing surgery was 11.5 years SD (range 4-16 years). The underlying etiology was JIA in 15 patients (38.46%) followed by idiopathic uveitis in 11 patients (28.20%); 6 cases of TB in 6 cases( 15.38%), Behcet disease in 2 (5.12%), VKH ( 5.12% ) , HLA B-27 positive chronic anterior uveitis (2.56%) in 1, toxocariasis (2.56%) in 1and CMV in 1full (2.56%). Phaco-aspiration with intraocular lens implantation was done in 26 eyes ( 48.14%) while 27 eyes (50%) underwent pars plana lensectomy and vitrectomy. Phaco-aspiration without IOL implantation was done in one eye (1.85%). Postoperatively, visual acuity of 20/40 was achieved in 20 eyes (37.03%) in pseudophakic group and 13 patients (24.07%) in aphakic group. Perioperative systemic steroids with immunosuppressive therapy was given in 34 cases (62.97%) while only systemic steroids were given in 20 cases (37.03%).Visually significant posterior capsular opacification was noted in 10 eyes (18.51%). One eye underwent glaucoma drainage device surgery post phaco-aspiration. Cystoid macular edema was noted in 4 eyes (7.4%) .The average follow up was 4.7 years. SD
Conclusion: Cataract surgery has a good outcome in majority of patients with uveitis. IOL implantation does not increase rate of complications. With meticulous surgical techniques and immunosuppressive therapy, one can achieve good post-operative visual outcomes.
INTRODUCTION:
Cataract development is a frequent complication in patients withchronic uveitis either as a result of chronic inflammation or secondary to steroid use.1,2Cataract surgery in children with uveitis remains a surgical challenge. Implantation of intraocular lens in children with uveitis is still controversial. Recent studies have revealed good outcomes in children with uveitis after posterior chamber intraocular lens (PCIOL) implantation.3,4 While PCIOL implantation is ideal in uveitis cases, pars planavitrectomy with lensectomy is a preferred modality in cases with presence of cyclitic membrane or vitritis.5Long term outcome of these two group of patients is essential to ascertain the need for IOL implantation and to know whether in the modern era of advanced surgical technique, children with uveitis could be visually rehabilitated to a better extend. Hence, we report long term outcomes of cataract surgery in children with uveitis in both aphakic and pseudophakic children.
Material and methods:
Medical records of paediatric uveitis patients, who had undergone cataract surgery with primary IOL implantation or pars planavitrectomy with lensectomy between January 2000 to January 2014 at Advanced Eye Centre, Post Graduate Institute Institute of Medical Education and Research, Chandigarh, India, were reviewed. Inclusion criteria were, age <16years, chronic inflammation under control for atleast 3 consecutive months before surgery, corrected distance visual acuity (CDVA) < 20/50 or cataract precluding direct fundus evaluation, media opacity (vitritis) and aminimum follow up of 6 months. All cataracts were graded according to Lens Opacities Classification System III. [5]
Surgeries were performed under general anaesthesia by a two experienced surgeons (JR and AG ). Two groups of children were identified: Group A: Phacoemulsification with primary IOL implant group
Group B: Pars planalensectomy with vitrectomy group
Standard steps of phacoemulsification with IOL implantation were followed in Group A. IOL implanted was either hydrophobic acrylic or Poly methyl metha acrylate (PMMA). Similarly, standard steps of parsplanavitrectomy with lensectomy were performed in Group B. Children in group B were left aphakic. Aetiology of uveitis was noted in all cases.
All children received preoperative steroids and were tapered off according to the clinical response. Outcome measures included post-operative visual acuity, need for re-surgery and related complications. Pre-operative visual acuity and visual acuity at last follow up were also noted.
Student t test was used to analyse various outcomes and P value of 0.05 or less was considered statistically significant.
RESULTS:
Sixty oneeyes of 40 patients (27 girls and 13 boys) were included. Tables 1 and 2 show the etiological diagnosis and baseline clinical characteristics. The age of children ranged from 5 to 16 years (9.5 years mean SD;8.5 years median). We divided the cases in two groups: a) Group A (PCIOL group); 20 patients (30 eyes;47.61%) and b) Group B (Aphakic group); 20 patients (33 eyes; 52.38%). Twenty one (53.65%) cases had bilateral involvement and 19 (46.34%) had unilateral involvement. Anatomical diagnosis of the cases included, 31 eyes (49.20%) with anterior uveitis, 10 eyes (15.87%) with intermediate uveitis, 4eyes(9.52%) with posterior uveitis and 16 eyes (25.32%) with panuveitis. Overall, Juvenile Idiopathic Arthritis (JIA) was the most common diagnosis overall with 18 cases (43.90%). Idiopathic uveitis comprised of 6 patients (14.63}%). Other causes included 8cases of TB ( 15.38%), 2Behcet disease (5.12%) 2 VKH ( 5.12% ) , 1 HLA B 27 positive chronic anterior uveitis(2.56%), 1 case of Fuch’s uveitis,1 case of toxocariasis(2.56%) and 1 case of CMV retinitis. All patients received peri-operative corticosteroids. Posterior synechiae was seen in 40 eyes, band shaped keratopathy was seen in 26 eyes. The mean follow up was 4.51years SD(range [0.5-13 years )
Peri-operative systemic steroids with immunosuppressive therapy were given in 27cases (67.5%) while only systemic steroids were given in 20 cases (32.5%). Five patients were on methotrexate (12.5%), 7 on azathioprine(17.5%) and 10 patients were on both methotrexate and azathioprine (25.0%). Three patients (4 eyes) received intravitreal dexamethasone implant at the time of cataract surgery in Group A (pseodophakic). Mean follow up was 4.7 years SD.
The preoperative CDVA could not be assessed in 5 preverbal children. Their visual acuity was assessed by the CSM method. Pre and post operative visual acuity was possible in 12 eyes in Group B aphakic group and eyes in 21 eyes in Group A (pseudophakic group). The mean pre-operative visual acuity in the PCIOL group was 0.52 logMAR and 0.58 logMAR in Aphakic group . All the patients had improvement in visual acuity except for 2 patients (3 eyes) out of which 2 eyes had preexisting macular pathology (CNVM) and 1 eye developed retinal detachment. Postoperatively, visual acuity of >=20/40 was achieved in 21eyes(70.0%) in pseudophakic group and 12 patients (38.70%) in the aphakic group. Table 2 shows the distribution of visual acuity at presentation and on last follow up. Table 3 and 4 show the pre and post operative visual acuity of the patients in both groups.
The IOL material was hydrophobic acrylic in all cases (96.66%) except for one (3.22%) case where PMMA was used.
Posterior capsular opacification or membrane formation was the most common complication seenmore in the PCIOL group compared to Aphakic group. Among the eyes undergoing phacoemusification, two eyes (6.6%) underwent phacoemulsification with trabeculectomy 4 eyes(13.33%) were implanted with intravitreal dexamethasone implant. IOL explantation was required in one eye (3.33%).
Postoperatively, 4 eyes fom the IOL group (13.33%) underwent NdYagcapsulotomy while 6 patients (20%) underwent surgical capsulotomy for visually significant posterior capsular opacification. Raised intraocular pressure was noted in 23 eyes (37.7%), 10 in aphakic group and 13 in pseudophakic group. Two patients (6.6%) underwent phacoaspiration with trabeculotomy in same sitting , 4 eyes underwent trabeculotomy (7.01%) while 4 eyes (7.01%) underwent implantation of glaucoma drainage device. In twenty eyes (35.08%), topical anti glaucoma medications were required to control IOP. We noted that IOP related complications were more in pseudophakic than in aphakic group. Cystoid macular edema was noted in 4 eyes (7.01%), which resolved over an average period of 3. 4SD weeks. Hypotonic maculopathy occurred in one eye (1.04%) following pars planalensectomy and vitrectomy. IOL explantation was required in 1 eye in view of IOL decentration. Retinal detachment occurred following cataract surgery in one eye with CMV retinitis. The same eye had undergone retinal detachment surgery prior to cataract surgery. Cystoid macular edema was seen in 5 eyes in each group.
Glaucoma surgery was needed in 8 cases, with 1 case of TB, 1 case with Behcetdisease , 5 cases of JIA and 1 idiopathic uveitis. The pseudophakic group attained better post-operative visual acuity on last follow up as compared to aphakic group (P0.003). Surgical iridectomy was performed in in 12 eyes in pseudophakic group.
In conclusion, PCIOL implantation seems to be associated with favourable outcomes. Use of hydrophobic acrylic IOLs and meticulous immunosuppression therapy is highly advantageous in the present era. Parsplanavitrectomy with lensectomy is associated with better visual outcomes in certain situations such as cyclitic membrane and pre-operative hypotony.Additional procedures required in IOL implantation group, commonly consists of capsulotomy. Hence, IOL implantation after optimum control of inflammation is no longer a contraindication in pediatric uveitis.
DISCUSSION:
Cataract formation is one of the most common complication in uveitis. Evidence on outcomes of cataract surgery with IOL implantation in children with JIA associated uveitis is limited. Hooper et al have reported good visual outcomes in cataract surgery in Fuch’s uveitis.6Cataract surgery in Fuchs heterochromatic uveitis has variable surgical outcomes. 7,8
Peri-operative inflammation control is essential in uveitis cases as it determines the post-operative success.9 All the cases had optimal control of inflammation for atleast 3 consecutive months before surgery. Peri-operative immunosuppressive therapy was given in all cases in the form of oral and topical steroids or oral immunosuppressive therapy. In our study two groups of patients were analysed, one with primary IOL implantation and the other without IOL implantation. Nemat et al10 compared visual outcomes of primary intraocular lens implantation in children with JIA associated uveitis with non-JIA associated uveitis. They concluded, even though the JIA associated group had more severe complications in the early post-operative period compared to non- JIA group, ultimate visual outcome was favourable in both.Use of foldable hydrophobic acrylic IOLs and PMMA IOLs has been considered safe and effective in several studies.10,11Adan et al12have reported two cases of JIA associated uveitis, both requiring IOL explantation due to persistent uveitis, CME and hypotony. In our study, hypotony was noted in one case of idiopathic panuveitis following PPL and PPV. In our study also, the gain in visual acuity was better in pseudophakic group compared to aphakic group. Primary posterior capsulotomy with anterior vitrectomy was performed in all children less than 8 years old (2 patients in our case (11.53%). even though 16 eyes out of 26 had visually significant cataract (61.53%), surgical capsulotomy was done in 6 eyes while Nd YAG capsulotomy was done in 3 eyes. Average duration of inception of capsular opacification was 10.20 months. Nematet al10reported 85% incidence of anterior and posterior capsular opacification. BenEzra and Cohen5 reported that in young children with uveitis, IOL implantation seems preferable to correction with contact lenses in those needing unilateral cataract surgery.
JIA associated uveitis was the most common aetiology followed by idiopathic uveitis. Among the infective causes, presumed tuberculosis was the leading cause of uveitis (15.38%) in their study.
Increase IOP spikes was seen in 24 eyes (39.34%) in the immediatepost operative period out of which 15 eyes (62.5%) had JIA. Four patients were steroid responders.. JIA is known to have increased post-operative inflammation compared to non-JIA counterparts. Nemat et al10 andEdelsten et al 13reported favourable surgical outcomes in a small series of children with JIA associated uveitis after cataract surgery with posterior chamber IOLs even when combined with trabeculotomy. In our case, combined phacoemusification with trabeculotomy was done in 2 eyes. One of those eyes require glaucoma drainage device due to pupillary block glaucoma. In our study, both groups had significant improvement in visual acuity and good control of post-operative inflammation was achieved in both groups. Quinones et al 14 reported 92% improvement in visual outcomes in eyes with PMMA IOLs placed in the bag, There was no difference in post-operative inflammation between patients who received an IOL and those who did not. Palsson et al 15 advocated combined phacoemulsification, IOL and vitrectomy in cases with vitreous pathologies and reported favourable visual outcomes.
Studies conducted on the use of intravitreal steroid implants e.g. Ozurdex (dexamethasone) have reported to have favourable outcomes. In our study, intra-vitreal dexamethasone implant (ozurdex) was injected intraoperatively in 3 eyes in pseudophakic group.
In conclusion, PCIOL implantation seems to be associated with favourable outcomes. Use of hydrophobic acrylic IOLs and meticulous immunosuppression therapy is highly advantageous in the present era. Parsplanavitrectomy with lensectomy is associated with better visual outcomes in certain situations such as cyclitic membrane and pre-operative hypotony.Additional procedures required in IOL implantation group, commonly consists of capsulotomy. Hence, IOL implantation after optimum control of inflammation is no longer a contraindication in pediatric uveitis.
References:
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- Kanski JJ, Shun-Shin GA. Sysyemic uveitis syndromes in children: an analysis of 340 cases. Ophthalmology 1984; 91: 1247-51; discussion by E S Perkins, 1251-52.
- Tugal- Tutkun I, Havrlikova K, Power WJ, et al. Changing patterns in uveitis of childhood. Ophthalmology 1996;103:375-83
- Lundvall A, Zetterstorm C. Cataract extraction and intraocular lens implantation in children with uveitis. Br J Ophthalmol 2000;84:791-3.
- BenEzra D, Cohen E. Cataract surgery in children with chronic uveitis. Ophthalmology 2000;107; 797-800
- Terrada C, Julian K, Sachs D, et al. Cataract surgery with primary intraocular lens implantation in children with uveitis. J Cataract Refract Surg 2011;37: 1977-
- Ram J, Jain S, Pandav SS, Mangat GS. Post operative complications in intraocular lens implantation in patients with Fuch’s heterochromatic uveitis. J Cataract Refract Surg 1995;21:548-551
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| Etiology | Aphakic group | Pseudophakic group |
| JIA | 12 | 6 |
| Idiopathic | 3 | 3 |
| TB | 7 | 1 |
| Behcet’s disease | 1 | 1 |
| VKH | 1 | 1 |
| HLA B27 related | 0 | 1 |
| Fuch’s uveitis | 0 | 1 |
| Toxocara | 1 | 0 |
| CMV retinitis | 0 | 1 |
Table 1: Etiology of cases in both groups. VKH: Vogt Koyanagi Harada, CMV: Cytomegalovirus.
| Complication | Pseudophakic group | Aphakic group |
| PCO | 10 | 4 |
| CME | 3 | 6 |
| Increased IOP | 8 | 6 |
| Iris bombe | 3 | 2 |
| ERM | 2 | 3 |
| Membrane over IOL | 14 | Not applicable |
| IOL decentration | 1 | Not applicable |
| Retinal detachment | 1 | 2 |
| Hypotony | 0 | 2 |
Table 2: Complications related to each group post surgery.
| Patient no./age/sex/eye | Pre-operative | Last follow-up |
| 1/8/M/OD | CF | 20/20 |
| 2/8/M/OS | CF | 20/20 |
| 3/12/F/OS | 20/40 | 20/20 |
| 4/9/F/OD | 20/40 | 20/40 |
| 5/8/F/OS | 20/40 | 20/20 |
| 6/8/F/OS | 20/120 | 20/30 |
| 7/15/M/OD | 20/40 | 20/40 |
| 8/14/M/OD | 20/200 | HM* |
| 9/7/M/0D | 20/40 | 20/30 |
| 10/7/M/OS | 20/40 | 20/30 |
| 11/14/F/OD | CF | CF |
| 12/14/F/OS | CF | CF |
| 13/10/F/OS | CF | CF |
| 14/10/F/OS | 20/80 | 20/80 |
| 15/11/F/OD | 20/200 | 20/20 |
| 16/11/F/OS | 20/200 | 20/30 |
| 17/4/F/OD | CF | 20/40 |
| 18/4/F/OS | 20/80 | 20/40 |
| 19/11/F/OD | 20/200 | NO PL |
| 20/14/M/OD | 20/80 | 20/60 |
| 21/16/F/OD | 20/40 | 20/20 |
| 22/16/F/OS | 20/40 | 20/20 |
| 23/11/F/OD | 20/40 | 20/20 |
| 24/11/F/OS | 20/40 | 20/30 |
| 25/12/M/OD | 20/80 | 20/20 |
| 26/6/M/OD | CF | 20/120 |
| 27/6/M/OS | CF | 20/100 |
| 28/12/M/OD | 20/120 | 20/20 |
| 29/12/M/OS | 20/80 | 20/20 |
| 30/15/F/OD | 20/60 | 20/20 |
Table 3: visual acuity in pre-operative and on last follow up
| Patient no./age/sex/eye | Pre-operative | Last follow up |
| 1/11/F/OS | HM | PL |
| 2/6/F/OD | HM | 20/200 |
| 3/15/F/OD | HM | CF |
| 4/15/F/OS | HM | CF |
| 5/9/F/OD | 20/120 | CF |
| 6/9/F/OD | CF | 20/30 |
| 7/9/F/OS | PL | 20/30 |
| 8/3/F/OD | – | 20/120 |
| 9/3/F/OS | – | 20/120 |
| 10/5/F/OD | – | CF |
| 11/6/M/OD | – | 20/100 |
| 12/6/F/OD | – | 20/40 |
| 13/6/F/OS | – | 20/20 |
| 14/13/M/OD | CF | CF |
| 15/13/M/OS | 20/120 | 20/30 |
| 16/5/M/OD | 20/120 | 20/20 |
| 17/10/M/OD | HM | CF |
| 18/10/M/OS | HM | CF |
| 19/7/F/OS | – | 20/200 |
| 20/6/M/OD | – | HM |
| 21/6/M/OS | – | 20/400 |
| 22/8/M/OD | 20/100 | 20/30 |
| 23/8/F/OD | 20/120 | 20/100 |
| 24/8/F/OS | 20/40 | 20/20 |
| 25/7/F/OD | 20/100 | 20/30 |
| 26/7/F/OS | 20/100 | 20/30 |
| 27/6/F/OD | 20/100 | 20/20 |
| 28/6/F/OS | 20/100 | 20/20 |
| 29/7/F/OD | 20/100 | 20/80 |
| 30/7/F/OS | 20/100 | 20/40 |
| 31/6/F/OD | – | CF |
Table 4: visual acuity in aphakic group: pre-operatively and on last follow up