Journal of Pediatric Ophthalmology and Strabismus

Original Article 

Clinical Characteristics and Treatment of Secondary Glaucoma After Pediatric Congenital Cataract Surgery in a Tertiary Referral Hospital in Spain

Karina Spiess, MD; Jesús Peralta Calvo, MD, PhD

Abstract

Purpose:

To analyze clinical characteristics, treatment, and long-term outcomes of pediatric patients with glaucoma after congenital cataract surgery at a single tertiary care hospital.

Methods:

Medical records of pediatric patients diagnosed as having glaucoma secondary to congenital cataract surgery between 1996 and 2016 were reviewed retrospectively.

Results:

A total of 58 eyes of 42 patients were included with a median follow-up time of 55 months (interquartile range [IQR]: 27 to 128) after glaucoma diagnosis. Mean time of glaucoma onset after cataract surgery was 35 months (IQR: 5 to 96). At diagnosis, 81% of the eyes were aphakic and the majority presented with an open angle (86%). Multivariate analysis demonstrated that glaucoma diagnosis was made earlier in eyes with persistent fetal vasculature (β = −0.334, P = .006) and aphakic eyes (β = 0.404, P = .001). Two-thirds of eyes required surgical treatment for glaucoma. Seventy percent had an Ahmed glaucoma valve (New World Medical, Inc) implantation as their primary procedure, followed by trabeculectomy (24%) and synechiolysis with peripheral iridotomy (6%). All medically treated eyes and 78% of the surgically treated eyes achieved intraocular pressure (IOP) control at the final visit.

Conclusions:

Diagnosis of glaucoma after congenital cataract surgery seems to follow a bimodal distribution (years 1 and 5 after cataract surgery). Two-thirds of the eyes required surgical hypotensive treatment to achieve IOP control. Ahmed glaucoma valve implantation is a safe and effective surgical option to be considered as both first- and second-line treatment. Functional outcome was more favorable in those eyes with medically controlled glaucoma.

[J Pediatr Ophthalmol Strabismus. 2020;57(5):292–300.]

Abstract

Purpose:

To analyze clinical characteristics, treatment, and long-term outcomes of pediatric patients with glaucoma after congenital cataract surgery at a single tertiary care hospital.

Methods:

Medical records of pediatric patients diagnosed as having glaucoma secondary to congenital cataract surgery between 1996 and 2016 were reviewed retrospectively.

Results:

A total of 58 eyes of 42 patients were included with a median follow-up time of 55 months (interquartile range [IQR]: 27 to 128) after glaucoma diagnosis. Mean time of glaucoma onset after cataract surgery was 35 months (IQR: 5 to 96). At diagnosis, 81% of the eyes were aphakic and the majority presented with an open angle (86%). Multivariate analysis demonstrated that glaucoma diagnosis was made earlier in eyes with persistent fetal vasculature (β = −0.334, P = .006) and aphakic eyes (β = 0.404, P = .001). Two-thirds of eyes required surgical treatment for glaucoma. Seventy percent had an Ahmed glaucoma valve (New World Medical, Inc) implantation as their primary procedure, followed by trabeculectomy (24%) and synechiolysis with peripheral iridotomy (6%). All medically treated eyes and 78% of the surgically treated eyes achieved intraocular pressure (IOP) control at the final visit.

Conclusions:

Diagnosis of glaucoma after congenital cataract surgery seems to follow a bimodal distribution (years 1 and 5 after cataract surgery). Two-thirds of the eyes required surgical hypotensive treatment to achieve IOP control. Ahmed glaucoma valve implantation is a safe and effective surgical option to be considered as both first- and second-line treatment. Functional outcome was more favorable in those eyes with medically controlled glaucoma.

[J Pediatr Ophthalmol Strabismus. 2020;57(5):292–300.]

Introduction

Glaucoma is a potentially sight-threatening complication following congenital cataract surgery. An incidence of up to 41% has been reported,1 but it varies depending on diagnostic criteria for glaucoma, length of follow-up, and clinical characteristics of the sample. Pathogenesis is likely to be multifactorial. Several factors have been proposed, such as postoperative inflammation2,3 chemical factors from the vitreous or lens remnants,1,4–6 mechanical collapse of the trabecular meshwork,4,7,8 or syndromic etiology.3,9

Controversy exists as to whether persistent fetal vasculature (PFV) is associated with an increased risk of glaucoma following lensectomy. PFV frequently presents with microcornea, microphthalmia, and anterior segment dysgenesis, and these have been widely described as risk factors for glaucoma after congenital cataract surgery.

Secondary glaucoma after congenital cataract surgery typically has an insidious onset with subtle clinical signs that often lead to a delayed diagnosis. Initially, most cases are managed with topical hypotensive medication; however, a high proportion (up to 50%) of the cases require surgical intervention.10–13

Several surgical procedures have been described, ranging from angle surgery (goniotomy or trabeculotomy), filtering surgery (trabeculectomy), and tube shunts to cyclodestructive procedures. Visual outcome is usually poor and related to glaucoma neuropathy, refractive and deprivation amblyopia, concomitant ocular pathology, and complications related to surgery.

We present a retrospective review of patients with glaucoma following congenital cataract surgery, with particular emphasis on clinical presentation, treatment, and long-term outcomes at a tertiary referral center.

Patients and Methods

This was a retrospective case series review of pediatric patients with secondary glaucoma following congenital cataract surgery in a tertiary referral center from 1996 to 2016. Patients with acquired cataracts, trauma, aniridia, or cataracts associated with Lowe syndrome, an age of 2 years and older with congenital cataract surgery, and previous/concomitant ocular hypertension or primary intraocular lens (IOL) implantation were excluded. This study was approved by the ethics committee of our hospital.

Glaucoma following cataract surgery was defined according to the consensus established by The Childhood Glaucoma Research Network (World Glaucoma Association in 2018) as intraocular pressure (IOP) greater than 21 mm Hg with associated anatomical optic disc changes or other signs of progressive myopia.14

All congenital cataract surgeries were performed with the same technique by surgeons of the same department. All surgeries involved anterior vitrectomy.

Epidemiological and clinical data (sex, etiology of cataract, age at the time of cataract surgery, intraocular lens (IOL) implantation, laterality, type of glaucoma, glaucoma medications, surgical treatment, early and late postoperative complications, IOP [Perkins applanation tonometer], cup-to-disc ratio, and best corrected visual acuity at diagnosis and final examination) were reviewed from patients' records.

Both trabeculectomy and Ahmed glaucoma valve (New World Medical, Inc) implantation for glaucoma are accepted treatment options for glaucoma following congenital cataract surgery. Because this was a retrospective review, there were no established criteria for selection of the surgical technique. Trabeculectomy was avoided in patients with thin sclera (as is the case in buphthalmic eyes).

Success was defined as IOP of less than 21 mm Hg with (qualified) or without (complete) medication on the last two follow-up examinations and without devastating complications, which included phthisis/evisceration, endophthalmitis, chronic retinal detachment, or vision of no light perception.

Visual acuity at final visit was converted to logarithm of the minimum angle of resolution (logMAR) scale. In patients with low vision, the semiquantitative scale of counting fingers, hand movements, and perception of light was quantified as 1.9, 2.3, 2.7, and 3.0 logMAR, respectively.15

Statistical analysis was performed using SPSS statistical software version 20 for Windows (SPSS, Inc). For statistical analysis of the surgical results, all unilateral cases and only the first operated eye of bilateral cases were included. When both eyes had simultaneous glaucoma surgery, both eyes were included. We used the stepwise method for linear regression analyses.

Results

A total of 58 eyes of 42 patients (59% males) with glaucoma following congenital cataract surgery were included in this study. Median follow-up time since glaucoma diagnosis was 55 months (range: 265 months; interquartile range [IQR]: 27 to 128).

The causes of the congenital cataracts were 43% persistent fetal vasculature (PFV) (of these, 48% had an anterior form of PFV and 52% were mixed anterior-posterior), followed by 33% with hereditary congenital cataract, 17% sporadic, 5% chromosomopathy-related, and 2% secondary to congenital infectious disease (rubella). Median age at cataract surgery was 52 days (range: 379 days; IQR: 33 to 83) and 81% of the eyes underwent surgery in the first 3 months of life. Most patients with bilateral congenital cataract had simultaneous surgery for both eyes (94%).

Eleven eyes (19%) had secondary IOL implantation prior to diagnosis of glaucoma. These patients had a median age at cataract surgery of 67 days (range: 373; IQR: 12 to 105) and a median age at secondary IOL implantation of 29 months (range: 88; IQR: 12 to 61). A total of 55% of eyes with secondary IOL implantation had PFV, 27% were sporadic, and 18% had hereditary congenital cataracts.

Glaucoma was diagnosed after a median elapsed time of 35 months (range: 178; IQR: 5 to 96) after cataract surgery. Most cases were diagnosed within the first year after congenital cataract surgery (20 cases, 35%), with a second peak of incidence at 5 years (7 cases) (Figure 1). Bilateral glaucoma was diagnosed in 16 patients and only one eye was affected in 26 patients, of whom 11 underwent bilateral congenital cataract surgery. In 62.5% of the patients with bilateral glaucoma, diagnosis was made simultaneously in both eyes. At diagnosis, 81% of the eyes were aphakic (47 eyes) and the majority presented an open angle configuration (86%).

Bar chart showing number of cases diagnosed as having glaucoma in each year after congenital cataract surgery.

Figure 1.

Bar chart showing number of cases diagnosed as having glaucoma in each year after congenital cataract surgery.

Eyes diagnosed as having PFV developed glaucoma at a median time of 15 months after congenital cataract surgery (range: 120; IQR: 4 to 63) and eyes without PFV at 50 months (range: 178; IQR: 7 to 107) (P = .084).

Glaucoma diagnosis in aphakic eyes was made earlier than in pseudophakic eyes, at a median time of 18 months (range: 178; IQR: 4 to 60) and 70 months (range: 108; IQR: 45 to 123), respectively (P = .002). Multivariate analysis was performed; the best adjusted model (R2 = 21.7) demonstrated that eyes with PFV (β = −0.334, P = .006) and aphakia (β = 0.404, P = .001) were significantly associated with earlier glaucoma diagnosis. However, age at cataract surgery was not a significant predictor (P = 227) (Table 1).

Multivariate Analysis of Time Elapsed Between Congenital Cataract Surgery and Glaucoma Diagnosis

Table 1:

Multivariate Analysis of Time Elapsed Between Congenital Cataract Surgery and Glaucoma Diagnosis

Mean IOP at glaucoma diagnosis was 29.1 ± 5.6 mm Hg, with a median cup-to-disc ratio of 0.8 (IQR: 0.4 to 0.8).

Medical treatment was started in 85% (49 eyes) at the moment of diagnosis and the remaining 15% underwent immediate surgical treatment, most of them with an angle closure component.

Most of the eyes (98%) were treated with topical hypotensive agents, whereas one patient was treated with additional oral acetazolamide. Approximately 60% of the patients had topical treatment with combination therapy of two active drugs and the remaining 40% had monotherapy. The most frequently prescribed drugs were beta-blockers (82%), followed by carbonic anhydrase inhibitors, prostaglandins, and alpha-2 adrenergic agonists (Figure 2).

Bar chart representing the main hypotensive agents prescribed at diagnosis of glaucoma (number of eyes). Bbl = beta-blockers; tCAI = topical carbonic anhydrase inhibitors, a-adr = alpha-2 adrenergic agonists; PGl = prostaglandins and prostamides; sCAI = systemic carbonic anhydrase inhibitors

Figure 2.

Bar chart representing the main hypotensive agents prescribed at diagnosis of glaucoma (number of eyes). Bbl = beta-blockers; tCAI = topical carbonic anhydrase inhibitors, a-adr = alpha-2 adrenergic agonists; PGl = prostaglandins and prostamides; sCAI = systemic carbonic anhydrase inhibitors

Satisfactory IOP control with only topical hypotensive treatment was achieved in 41% of the eyes in which medical treatment was started (20 eyes). The remaining 59% required subsequent surgical treatment, with a median elapsed time of 3 months (range: 114; IQR: 0.5 to 29) between the start of medical treatment and surgery.

Overall, a total of 38 eyes required surgical intervention for glaucoma (66%). Mean IOP prior to surgery was 31.5 ± 6.1 mm Hg (range: 24) and median cup-to-disc ratio was 0.8 (range: 0.7; IQR: 0.7 to 0.9).

Most patients with bilateral glaucoma requiring surgery underwent simultaneous surgery for both eyes. Only 1 patient had subsequent glaucoma surgery for both eyes, and therefore the second eye was not included in the following analyses. The chosen primary surgical technique in unilateral cases and simultaneous bilateral cases was tube implantation in 70% of the eyes (Ahmed glaucoma valve), followed by trabeculectomy without mitomycin C (24%) and synechiolysis with peripheral iridotomy (6%). One single glaucoma surgical procedure was performed in 23 eyes (62 %), whereas 14 eyes required more than one surgical procedure to achieve IOP control.

The two main causes of unsatisfactory IOP control after the first surgery were insufficient IOP reduction with only one functional Ahmed glaucoma valve or trabeculectomy (38%), followed by Tenon's cyst (21%) (Figure 3).

Causes of failure after first surgical glaucoma procedure. AVG = Ahmed glaucoma valve (New World Medical, Inc)

Figure 3.

Causes of failure after first surgical glaucoma procedure. AVG = Ahmed glaucoma valve (New World Medical, Inc)

In total, 61 surgical procedures were performed, including primary and subsequent surgeries, of which 72% were Ahmed glaucoma valve implantations (64% model FP7, 27% model S2, and 9% model FP8), 18% trabeculectomies (without mitomycin C), 5% decapsulations of Tenon' cyst, and 5% synechiolysis with peripheral iridotomies.

Overall, the most common complications in the early postoperative period, defined as the first week after surgery, were severe hypotony (≤ 5 mm Hg IOP) in 31% of the cases, associated with flat or shallow anterior chamber in 22% of the cases, followed by choroidal detachment (21%) (Figure 4). Most of the complications resolved spontaneously. Early surgical reintervention was required in only 7% of the cases: vitrectomy (3 eyes), choroidal detachment drainage (2 eyes), and anterior chamber washout (1 eye). There was no significant difference in the prevalence of early postoperative complications in eyes with trabeculectomy or Ahmed glaucoma valve implantation (Table 2).

Bar graph representation of early postoperative complications observed (first 7 days after surgery). VH = vitreous hemorrhage; CD = choroidal detachment; RD = retinal detachment

Figure 4.

Bar graph representation of early postoperative complications observed (first 7 days after surgery). VH = vitreous hemorrhage; CD = choroidal detachment; RD = retinal detachment

Prevalence of Early Postoperative Complications in Eyes With Trabeculectomy and Ahmed Glaucoma Valve Implantation

Table 2:

Prevalence of Early Postoperative Complications in Eyes With Trabeculectomy and Ahmed Glaucoma Valve Implantation

Median follow-up time after first glaucoma surgery was 89 months (range: 240 months; IQR: 23 to 141).

Devastating late complications in surgically treated eyes were mainly related to tractional retinal detachment (8.1%, 3 cases), endophthalmitis (5.4%, 1 case), and chronic hypotony with combined effusive retinal and choroidal detachment (5.4%, 1 case).

Ahmed glaucoma valve implantation was used as the first- or second-line procedure in 30 eyes. Of these, IOP control was achieved in 23 eyes (77%), 8 without adjuvant topical therapy (complete success), and 15 with additional topical hypotensive treatment (qualified success).

Overall, 10.8% (4 eyes) developed phthisis bulbi and 5.4% (2 eyes) underwent evisceration (Table 3). Median number of glaucoma surgeries per eye was 1.00 (range: 4; IQR: 1.00 to 2.25) (Figure 5).

Surgical Procedures and Outcome

Table 3:

Surgical Procedures and Outcome

Number of glaucoma procedures per eye.

Figure 5.

Number of glaucoma procedures per eye.

The median survival time of the first surgical procedure was 107.0 ± 45.5 months (95% CI: 17.8 to 196.2) for Ahmed glaucoma valve and 15 months for trabeculectomy (log Rank; P = .009). Cumulative survival rates were 71.2%, 66.1%, and 60.1%, respectively, for the first Ahmed glaucoma valve implantation at months 12, 24, and 48 and 66.7% at month 12 and 0% at month 24 for the trabeculectomy (Figure 6).

Survival curve for Ahmed glaucoma valve (AVG) (New World Medical, Inc) implantation and trabeculectomy.

Figure 6.

Survival curve for Ahmed glaucoma valve (AVG) (New World Medical, Inc) implantation and trabeculectomy.

Overall, medically and surgically treated eyes had a mean IOP of 16.75 ± 4.25 mm Hg with a median cup-to-disc ratio of 0.6 (range: 0.9; IQR: 0.3 to 0.8) at their last follow-up (Table 4).

Outcome at Final Visit, Excluding Eviscerated Eyes and Phthisis Bulbi

Table 4:

Outcome at Final Visit, Excluding Eviscerated Eyes and Phthisis Bulbi

At final examination, 10.3% of the eyes had no light perception. Overall, median visual acuity at the final visit was 0.7 logMAR (range: 2.7; IQR: 0.12 to 1.9), with 29.7% of the eyes having a best corrected visual acuity of better than 20/40 and 35.1% worse than 20/200. Excluding eyes with no light perception, median best corrected visual acuity was significantly better in medically treated (0.4 logMAR; IQR: 0.1 to 1.3) compared to surgically treated (0.75 logMAR; IQR: 0.4 to 1.9) eyes. Multivariate analysis showed that treatment modality (medical vs surgical) was significantly associated with better final visual outcome (β = 0.433, P = .017) (Table 5).

Linear Regression Multivariate Analyses of BCVA at Final Visit

Table 5:

Linear Regression Multivariate Analyses of BCVA at Final Visit

Discussion

Early congenital cataract surgery is a known risk factor for development of glaucoma. Chak et al16 reported that an age 10-fold higher at the moment of lensectomy (eg, 50 days instead of 5 days) reduces the risk of secondary aphakic glaucoma by 60%. Several authors recommend deferring surgery after the first month of life to reduce the risk of glaucoma.11,17–20 The Infant Aphakia Treatment Study showed a risk reduction of 50% when postponing surgery for unilateral congenital cataract surgery from 4 to 8 weeks of age.21

The majority of cases diagnosed as having glaucoma following cataract surgery have an open angle. It has been suggested that early surgery alters the maturation process of the trabecular meshwork due to structural damage of the angle, lack of cytokines segregated by the lens, and postoperative inflammation.22 Closed angle glaucoma and mixed angle mechanisms have also been described.

In our study, the median age at cataract surgery was 52 days, which is comparable to the results of Sahin et al (2.58 months),23 Ambroz et al (2 months),24 and Baris et al (3.31 months).25 Mean age varies widely among publications because this variable is highly dependent on the inclusion criteria. However, delayed surgery for congenital cataract is also related to a higher risk of deprivation amblyopia and absence of binocular vision.11 Several studies have shown that there is less risk of amblyopia when performing congenital cataract surgery before 6 weeks in unilateral cases and before 10 weeks of age in bilateral cataracts.26–29

Trivedi et al30 observed a mean elapsed time of 117.9 months between congenital cataract surgery and glaucoma diagnosis in aphakic patients. Michaelides et al31 and Vishwanath et al18 observed a bimodal distribution of glaucoma diagnosis, with a first peak at the first year after congenital cataract surgery and the second peak at years 4 and 5, which parallels our results of a higher number of diagnosed cases during these years.

In the study of Egbert et al,32 60% of the patients were diagnosed as having glaucoma in the first 5 years after congenital cataract surgery, showing certain similarities with our results, whereas 67% of the eyes were diagnosed during this period of time. On the contrary, Magnusson et al33 observed that 60% of patients were diagnosed as having glaucoma during the first year after congenital cataract surgery. However, they included different surgical techniques for congenital cataract with variable degrees of anterior vitrectomy, which probably explains the high number of cases in the first year.

We have observed that eyes with PFV develop glaucoma earlier than eyes without PFV. This is likely due to associated anatomical alterations that predispose to an earlier development of glaucoma such as microcornea, microphthalmia, and angle dysgenesis. PFV has also been linked to microcoria, which makes cataract surgery more challenging, with a higher likelihood of lens remnants.8,11,34–36

Only a few studies included PFV in their inclusion criteria. Johnson and Keech7 and Trivedi et al31 also described an earlier development of glaucoma in eyes with PFV. Swamy et al37 observed that PFV was a significant predictor of progression to secondary glaucoma in the univariate model.

Several studies observed a later onset of glaucoma in pseudophakic eyes compared to aphakic eyes, with certain parallels to our results. The protective role of the IOL has been widely discussed; however, a selection bias may explain this observation because the decision for IOL implantation is usually made in older children with low risk features (without microcornea or microphthalmia).2,31,37 Other authors suggest that an IOL acts as a physical barrier for toxic molecules from the vitreous and, at the same time, it helps to mechanically stabilize the angle, avoiding its collapse.9,38,39

First-line treatment diagnosis of open-angle glaucoma secondary to lensectomy for congenital cataract is mainly pharmacological, usually topical. The most commonly prescribed drugs are beta-blockers and prostaglandins.11,40 However, a considerable proportion of patients usually require surgical management for long-term IOP control. In our series, two-thirds of the eyes underwent surgery. In published articles, the proportion of surgical intervention ranges from 27% to 85% depending on inclusion criteria and length of study follow-up.16,17,32,35,41,42

Several surgical techniques have been described for glaucoma following cataract surgery, mainly trabeculectomy and drainage devices, but also less invasive techniques such as laser, goniotomy, trabeculotomy, and ciliary body ablation (cryotherapy or laser).

In recent publications, we can observe a tendency toward an increased use of glaucoma drainage devices.43–45 In our case series, Ahmed glaucoma valve implantation was the main primary surgical technique (70%) and it was also the treatment of choice in those cases with subsequent failure of trabeculectomy. We observed that the main complications after Ahmed glaucoma valve implantation were encapsulation (Tenon's cyst) and tube-related incidents such as obstruction or retraction that require further surgical procedures. Despite the need for multiple surgical procedures, we observed a successful outcome in terms of IOP control in approximately three-quarters of the eyes (with and without adjunctive topical therapy) that had first- or second-line Ahmed glaucoma valve implants.

Commonly, several hypotensive surgical interventions are required to achieve IOP control in glaucoma following cataract surgery if topical treatment is insufficient; 31% to 75% of the eyes require two or more hypotensive procedures according to the published data.11,23,25,45–47 These results support our findings, with 38% of surgically treated eyes requiring two or more interventions. This more positive result compared to other studies can be explained by a more aggressive first-line treatment such as trabeculectomy or Ahmed glaucoma valve implantation. Several other authors opted for less invasive first-line treatments such as goniotomies, trabeculotomies, or repetitive sessions of cycloablation, which are usually less effective in the long term.46,47

Both trabeculectomy and glaucoma devices seem to become less effective over time.48,49 The main challenges of trabeculectomies in children and their failure over time are related to the thick Tenon's capsule, the excessive scarring response, and the elastic and thinned sclera, especially in buphthalmic eyes. Ahmed glaucoma valve implantation has been described as a safe and efficient technique in eyes with extensive subconjunctival scarring secondary to multiple previous surgeries.50 The most common late complication related to Ahmed glaucoma valve implantation is Tenon's cyst formation, which results in a reduced efficacy of the device. The fibrotic tissue surrounding the plate can be surgically removed, restoring valve function. Nevertheless, after decapsulation the long-term survival rate of the device will be significantly reduced. Al-Omairi et al51 described a case series of decapsulation with success rates of 100%, 50%, and 16% after 1 month, 1 year, and 2 years, respectively. Shah et al52 suggested better outcomes in terms of IOP control with a secondary surgical procedure than with Tenon's cyst excision.

Devastating complications associated with surgical management of glaucoma following cataract surgery are tractional retinal detachment, endophthalmitis, and exudative retinal and/or choroidal detachment secondary to hypotony. The risk of developing complications is highly dependent on the duration of the study and therefore difficult to compare with previous publications.

Visual outcomes for patients with glaucoma following cataract surgery are usually poor. This silent condition usually results in a late diagnosis with a certain degree of already established neuropathy, amblyopia risk factors (strabismus, loss of corneal transparency, lens reproliferation, nystagmus, and concomitant ocular pathology), and also the need for several surgical procedures and its complications.53,54

Our median visual acuity at final examination was 20/100 (Snellen scale), with a visual acuity of 20/40 or better in 30% of the eyes and worse than 20/200 in 35% of the eyes. Chen et al11 observed a mean final visual acuity of 20/400, with more encouraging results published by Bhola et al46 (54% VA 20/40 or better, 11% worse than 20/200) and Lundvall and Kugelberg20 (62.5% 20/50 or better and 12.5% 20/200 or worse).

Our results indicate that glaucoma following cataract surgery can be diagnosed at any time after lensectomy, observing a bimodal distribution with two peaks of diagnosis: at the first year and at year 5 after congenital cataract surgery. In almost two-thirds of the eyes, IOP could not be achieved with topical treatment alone. Ahmed glaucoma valve implantation has been our treatment of choice for surgical management with similar early postoperative complications as in trabeculectomies and which are commonly self-limiting. The limitations of our study are its small sample size and its retrospective design. A randomized clinical trial with head-to-head comparison of different therapeutic options would be an optimal design, taking into consideration that the length of follow-up is of great importance to assess long-term outcomes.

We believe that Ahmed glaucoma valve implantation should be considered an effective option for treatment of glaucoma after congenital cataract surgery because it offers satisfactory long-term control. However, reoperations for late-onset complications might be necessary, such as Tenon's cyst formation, insufficient control with only one device, or tube retraction. More positive functional outcomes could be observed in eyes without PFV and those cases with pharmacologically controlled glaucoma.

References

  1. Simon JW, Mehta N, Simmons ST, Catalano RA, Lininger LL. Glaucoma after pediatric lensectomy/vitrectomy. Ophthalmology. 1991;98(5):670–674. doi:10.1016/S0161-6420(91)32235-8 [CrossRef]
  2. Al-Dahmash S, Khan AO. Pediatric pseudophakic glaucoma following surgery for isolated childhood cataract. Ophthalmic Surg Lasers Imaging. 2010;41(4):463–466. doi:10.3928/15428877-20100525-01. [CrossRef]
  3. Phelps CD, Arafat NI. Open-angle glaucoma following surgery for congenital cataracts. Arch Ophthalmol. 1977;95(11):1985–1987. doi:10.1001/archopht.1977.04450110079005 [CrossRef]
  4. Mills MD, Robb RM. Glaucoma following childhood cataract surgery. J Pediatr Ophthalmol Strabismus. 1994;31(6):355–360.
  5. Gimbel HV, Basti S, Ferensowicz M, DeBroff BM. Results of bilateral cataract extraction with posterior chamber intraocular lens implantation in children. Ophthalmology. 1997;104(11):1737–1743. doi:10.1016/S0161-6420(97)30033-5 [CrossRef]
  6. Michael I, Shmoish M, Walton DS, Levenberg S. Interactions between trabecular meshwork cells and lens epithelial cells: a possible mechanism in infantile aphakic glaucoma. Invest Ophthalmol Vis Sci. 2008;49(9):3981–3987. doi:10.1167/iovs.08-1674 [CrossRef]
  7. Johnson CP, Keech RV. Prevalence of glaucoma after surgery for PHPV and infantile cataracts. J Pediatr Ophthalmol Strabismus. 1996;33(1):14–17.
  8. Walton DS. Pediatric aphakic glaucoma: a study of 65 patients. Trans Am Ophthalmol Soc. 1995;93:403–413.
  9. Asrani S, Freedman S, Hasselblad V, et al. Does primary intraocular lens implantation prevent “aphakic” glaucoma in children?J AAPOS. 2000;4(1):33–39. doi:10.1016/S1091-8531(00)90009-0 [CrossRef]
  10. Kirwan C, O'Keefe M, Lanigan B, Mahmood U. Ahmed valve drainage implant surgery in the management of paediatric aphakic glaucoma. Br J Ophthalmol. 2005;89(7):855–858. doi:10.1136/bjo.2004.056143 [CrossRef]
  11. Chen TC, Bhatia LS, Halpern EF, Walton DS, Bhatia LS. Risk factors for the development of aphakic glaucoma after congenital cataract surgery. Trans Am Ophthalmol Soc. 2006;104(5):241–251. doi:10.3928/01913913-20060901-01 [CrossRef]
  12. Yi K, Chen TC. Aphakic glaucoma after congenital cataract surgery. Int Ophthalmol Clin. 2008;48(2):87–94. doi:10.1097/IIO.0b013e3181692d7a [CrossRef]
  13. Asrani SG, Wilensky JT. Glaucoma after congenital cataract surgery. Ophthalmology. 1995;102(6):863–867. doi:10.1016/S0161-6420(95)30942-6 [CrossRef]
  14. Beck A, Chang TC FS. Section 1: definition, classification, differential diagnosis. In: Weinreb RN, Grajewski A, Papadopoulos M, Grigg J, Freedman S, eds. World Glaucoma Association Consensus Series-9: Childhood Glaucoma.Kugler Publications; 2013:3–10.
  15. Lange C, Feltgen N, Junker B, Schulze-Bonsel K, Bach M. Resolving the clinical acuity categories “hand motion” and “counting fingers” using the Freiburg Visual Acuity Test (FrACT). Graefes Arch Clin Exp Ophthalmol. 2009;247(1):137–142. doi:10.1007/s00417-008-0926-0 [CrossRef]
  16. Chak M, Rahi JSBritish Congenital Cataract Interest Group. Incidence of and factors associated with glaucoma after surgery for congenital cataract: findings from the British Congenital Cataract Study. Ophthalmology. 2008;115(6):1013–1018.e2. doi:10.1016/j.ophtha.2007.09.002 [CrossRef]
  17. Rabiah PK. Frequency and predictors of glaucoma after pediatric cataract surgery. Am J Ophthalmol. 2004;137(1):30–37. doi:10.1016/S0002-9394(03)00871-7 [CrossRef]
  18. Vishwanath M, Cheong-Leen R, Taylor D, Russell-Eggitt I, Rahi J. Is early surgery for congenital cataract a risk factor for glaucoma?Br J Ophthalmol. 2004;88(7):905–910. doi:10.1136/bjo.2003.040378 [CrossRef]
  19. Parks MM, Johnson DA, Reed GW. Long-term visual results and complications in children with aphakia: a function of cataract type. Ophthalmology. 1993;100(6):826–840. doi:10.1016/S0161-6420(93)31566-6 [CrossRef]
  20. Lundvall A, Kugelberg U. Outcome after treatment of congenital bilateral cataract. Acta Ophthalmol Scand. 2002;80(6):593–597. doi:10.1034/j.1600-0420.2002.800607.x [CrossRef]
  21. Beck AD, Freedman SF, Lynn MJ, Bothun E, Neely DE, Lambert SRInfant Aphakia Treatment Study Group. Glaucoma-related adverse events in the Infant Aphakia Treatment Study: 1-year results. Arch Ophthalmol. 2012;130(3):300–305. doi:10.1001/archophthalmol.2011.347. [CrossRef]
  22. Lambert SR. The timing of surgery for congenital cataracts: minimizing the risk of glaucoma following cataract surgery while optimizing the visual outcome. J AAPOS. 2016;20(3):191–192. doi:10.1016/j.jaapos.2016.04.003 [CrossRef]
  23. Sahin A, Caça I, Cingü AK, et al. Secondary glaucoma after pediatric cataract surgery. Int J Ophthalmol. 2013;6(2):216–220. doi:10.3980/j.issn.2222-3959.2013.02.21 [CrossRef]
  24. Ambroz SC, Töteberg-Harms M, Hanson JVM, Funk J, Barthelmes D, Gerth-Kahlert C. Outcome of pediatric cataract surgeries in a Tertiary Center in Switzerland. J Ophthalmol. 2018;2018:3230489. doi:10.1155/2018/3230489 [CrossRef]
  25. Baris M, Biler ED, Yilmaz SG, Ates H, Uretmen O, Kose S. Treatment results in aphakic patients with glaucoma following congenital cataract surgery. Int Ophthalmol. 2014;2017:1–9. doi:10.1007/s10792-017-0777-y [CrossRef]
  26. Birch EE, Stager DR. The critical period for surgical treatment of dense congenital unilateral cataract. Invest Ophthalmol Vis Sci. 1996;37(8):1532–1538. doi:10.1016/j.jaapos.2008.07.010 [CrossRef]
  27. Birch EE, Cheng C, Stager DR Jr, Weakley DR Jr, Stager DR Sr, . The critical period for surgical treatment of dense congenital bilateral cataracts. J AAPOS. 2009;13(1):67–71. doi:10.1016/j.jaapos.2008.07.010 [CrossRef]
  28. Lambert SR, Lynn MJ, Reeves R, Plager DA, Buckley EG, Wilson ME. Is there a latent period for the surgical treatment of children with dense bilateral congenital cataracts?J AAPOS. 2006;10(1):30–36. doi:10.1016/j.jaapos.2005.10.002 [CrossRef]
  29. Lloyd IC, Ashworth J, Biswas S, Abadi RV. Advances in the management of congenital and infantile cataract. Eye (Lond). 2007;21(10):1301–1309. doi:10.1038/sj.eye.6702845 [CrossRef]
  30. Trivedi RH, Wilson ME Jr, Golub RL. Incidence and risk factors for glaucoma after pediatric cataract surgery with and without intraocular lens implantation. J AAPOS. 2006;10(2):117–123. doi:10.1016/j.jaapos.2006.01.003 [CrossRef]
  31. Michaelides M, Bunce C, Adams GGW. Glaucoma following congenital cataract surgery—the role of early surgery and posterior capsulotomy. BMC Ophthalmol. 2007;7(1):13. doi:10.1186/1471-2415-7-13 [CrossRef]
  32. Egbert JE, Christiansen SP, Wright MM, Young TL, Summers CG. The natural history of glaucoma and ocular hypertension after pediatric cataract surgery. J AAPOS. 2006;10(1):54–57. doi:10.1016/j.jaapos.2005.07.002 [CrossRef]
  33. Magnusson G, Abrahamsson M, Sjöstrand J. Glaucoma following congenital cataract surgery: an 18-year longitudinal follow-up. Acta Ophthalmol Scand. 2000;78(1):65–70. doi:10.1034/j.1600-0420.2000.078001065.x [CrossRef]
  34. Parks MM, Johnson DARG, Reed GW. Long-term visual results and complications in children with aphakia: a function of cataract type. Ophthalmology. 1993;100(6):826–840. doi:10.1016/S0161-6420(93)31566-6 [CrossRef]
  35. Freedman SF, Lynn MJ, Beck AD, Bothun ED, Örge FH, Lambert SRInfant Aphakia Treatment Study Group. Glaucoma-related adverse events in the first 5 years after unilateral cataract removal in the Infant Aphakia Treatment Study. JAMA Ophthalmol. 2015;133(8):907–914. doi:10.1001/jamaophthalmol.2015.1329 [CrossRef]
  36. Wallace DK, Plager DA. Corneal diameter in childhood aphakic glaucoma. J Pediatr Ophthalmol Strabismus. 1996;33(5):230–234.
  37. Swamy BN, Billson F, Martin F, et al. Secondary glaucoma after paediatric cataract surgery. Br J Ophthalmol. 2007;91(12):1627–1630. doi:10.1136/bjo.2007.117887 [CrossRef]
  38. Mataftsi A, Haidich A-B, Kokkali S, et al. Postoperative glaucoma following infantile cataract surgery: an individual patient data meta-analysis. JAMA Ophthalmol. 2014;132(9):1059–1067. doi:10.1001/jamaophthalmol.2014.1042 [CrossRef]
  39. Ahmadieh H, Javadi MA. Intra-ocular lens implantation in children. Curr Opin Ophthalmol. 2001;12(1):30–34. doi:10.1097/00055735-200102000-00006 [CrossRef]
  40. Maeda-Chubachi T, Chi-Burris K, Simons BD, et al. A6111137 Study Group. Comparison of latanoprost and timolol in pediatric glaucoma: a phase 3, 12-week, randomized, double-masked multicenter study. Ophthalmology. 2011;118(10):2014–2021. doi:10.1016/j.ophtha.2011.03.010 [CrossRef]
  41. Kass MA, Heuer DK, Higginbotham EJ, et al. The Ocular Hypertension Treatment Study: a randomized trial determines that topical ocular hypotensive medication delays or prevents the onset of primary open-angle glaucoma. Arch Ophthalmol. 2002;120(6):701–713, 829–830. doi:10.1001/archopht.120.6.701 [CrossRef].
  42. Al-Dahmash S, Khan AO. Aphakic glaucoma after cataract surgery for isolated nontraumatic pediatric cataract. Eye Contact Lens. 2010;36(3):177–180. doi:10.1097/ICL.0b013e3181dc1923 [CrossRef]
  43. Neustein RF, Bruce BB, Beck AD. Primary congenital glaucoma versus glaucoma following congenital cataract surgery: comparative clinical features and long-term outcomes. Am J Ophthalmol. 2016;170:214–222. doi:10.1016/j.ajo.2016.08.012 [CrossRef]
  44. Lambert SR, Purohit A, Superak HM, Lynn MJ, Beck AD. Long-term risk of glaucoma after congenital cataract surgery. Am J Ophthalmol. 2013;156(2):355–361.e2. doi:10.1016/j.ajo.2013.03.013 [CrossRef]
  45. Zetterberg M, Nyström A, Kalaboukhova L, Magnusson G. Outcome of surgical treatment of primary and secondary glaucoma in young children. Acta Ophthalmol. 2015;93(3):269–275. doi:10.1111/aos.12566 [CrossRef]
  46. Bhola R, Keech RV, Olson RJ, Petersen DB. Long-term outcome of pediatric aphakic glaucoma. J AAPOS. 2006;10(3):243–248. doi:10.1016/j.jaapos.2006.01.005 [CrossRef]
  47. Comer RM, Kim P, Cline R, Lyons CJ. Cataract surgery in the first year of life: aphakic glaucoma and visual outcomes. Can J Ophthalmol. 2011;46(2):148–152. doi:10.3129/i11-006 [CrossRef]
  48. Beck AD, Freedman S, Kammer J, Jin J. Aqueous shunt devices compared with trabeculectomy with mitomycin-C for children in the first two years of life. Am J Ophthalmol. 2003;136(6):994–1000. doi:10.1016/S0002-9394(03)00714-1 [CrossRef]
  49. Yang HK, Park KH. Clinical outcomes after Ahmed valve implantation in refractory paediatric glaucoma. Eye (Lond). 2009;23(6):1427–1435. doi:10.1038/eye.2008.261 [CrossRef]
  50. Al-Haddad C, Al-Salem K, Ismail K, Noureddin B.Long-term outcomes of Ahmed tube implantation in pediatric glaucoma after multiple surgeries. Int Ophthalmol. 2017;1–4. doi:10.1007/s10792-017-0743-8 [CrossRef]
  51. Al-Omairi AM, Al Ameri AH, Al-Shahwan S, et al. Outcomes of Ahmed glaucoma valve revision in pediatric glaucoma. Am J Ophthalmol. 2017;183:141–146. doi:10.1016/j.ajo.2017.09.015 [CrossRef]
  52. Shah AA, WuDunn D, Cantor LB. Shunt revision versus additional tube shunt implantation after failed tube shunt surgery in refractory glaucoma. Am J Ophthalmol. 2000;129(4):455–460. doi:10.1016/S0002-9394(99)00410-9 [CrossRef]
  53. Keech RV, Tongue AC, Scott WE. Complications after surgery for congenital and infantile cataracts. Am J Ophthalmol. 1989;108(2):136–141. doi:10.1016/0002-9394(89)90007-X [CrossRef]
  54. Chrousos GA, Parks MM, O'Neill JF. Incidence of chronic glaucoma, retinal detachment and secondary membrane surgery in pediatric aphakic patients. Ophthalmology. 1984;91(10):1238–1241. doi:10.1016/S0161-6420(84)34161-6 [CrossRef]

Multivariate Analysis of Time Elapsed Between Congenital Cataract Surgery and Glaucoma Diagnosis

VariableStandardized β Coefficient95% CIP
Age at cataract surgery (days)0.149.227
Persistent fetal vasculature yes/no−0.334−56.308 to −9.637.006
Intraocular lens yes/no0.40420.969 to 79.925.001

Prevalence of Early Postoperative Complications in Eyes With Trabeculectomy and Ahmed Glaucoma Valve Implantation

ComplicationTrabeculectomyAhmed Glucoma Valve ImplantationP
Hypotony45%32%.485
Choroidal detachment27%23%.709
Exudative retinal detachment27%20%.689
Vitreous hemorrhage27%27%1.000

Surgical Procedures and Outcome

ProcedureNo. of Functional Trabeculectomies/AVGQualified SuccessComplete SuccessFailure, IOP at Final Visit > 22 mm HgEvisceration/Phthisis Bulbi
Trabeculectomy only (5 eyes)One (4 eyes)1 eye3 eyes
Two (1 eye)1 eye
AVG only (25 eyes)One (17 eyes)8 eyes6 eyes2 eyes phthisisbulbi, 1 eye evisceration
Two (8 eyes)5 eyes2 eyes1 eye phthisis bulbi
AVG failed, then trabeculectomy (1 eye)One trabeculectomy (1 eye)1 eye
Trabeculectomy not functional, then AVG (3 eyes)One AVG (2 eyes)1 eye phthisis bulbi, 1 eye evisceration
Two AVG (1 eye)1 eye
Trabeculectomy not sufficient, additional AVG (1 eye)One trabeculectomy + one AVG1 eye
Synechiolisis with peripheral iridotomy (2 eyes)1 eye1 eye

Outcome at Final Visit, Excluding Eviscerated Eyes and Phthisis Bulbi

VariableMedically Treated EyesSurgically Treated EyesP
Mean IOP at final visit (mm Hg)16.9 ± 3.816.8 ± 4.5.959
Median cup-to-disc ratio at final visit0.4 (range: 0.7; IQR: 0.2 to 0.6)0.7 (range: 0.9; IQR: 0.4 to 0.8).052
Median number of active principles (topical hypotensive medication)2.5 (range: 3; IQR: 2 to 3)1.5 (range: 4; IQR: 0 to 4).001
BCVA (logMAR)0.4 (range: 1.9; IQR: 0.1 to 1.3)0.75 (range: 2.7; IQR: 0.4 to 1.9).097

Linear Regression Multivariate Analyses of BCVA at Final Visit

VariableStandardized β Coefficient95% CIP
Treatment (medical vs surgical)0.4330.064 to 0.589.017
PFV yes/no0.199.252
IOL yes/no0.119.495
Age at cataract surgery (days)−0.100.574
Unilateral or bilateral congenital cataract−0.017.923
Authors

From Hospital Universitario La Paz, Madrid, Spain (KS, JPC); and Universidad Autónoma de Madrid, Madrid, Spain (JPC).

The authors have no financial or proprietary interest in the materials presented herein.

The authors thank Mrs. Rosario Madero Jarabo for her support with statistical data analysis.

Correspondence: Karina Spiess, MD, Hospital Universitario La Paz, Paseo de la Castellana 261, 28046 Madrid, Spain. Email: karinaspiess@gmx.net

Received: December 15, 2019
Accepted: April 24, 2020

10.3928/01913913-20200707-01

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