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Home , Buphthalmos, Childhood cataract

CLINICAL SCIENCES Aphakic After Congenital Surgery

Teresa C. Chen, MD; David S. Walton, MD; Lini S. Bhatia, MD

Objective: To describe the largest series of patients who (22.5%). For the lensectomies, 103 eyes underwent mod- developed aphakic glaucoma after lensectomy for con- ern vitrectomy techniques, and 10 underwent older tech- genital . niques. Lensectomies were performed in 80.6% of eyes be- fore age 1 year. Onset of glaucoma after lensectomy was Methods: A retrospective review was performed of all by 1 year in 37.1% of eyes, by 6 years in 75.9%, and by 33 patients seen by a pediatric glaucoma specialist between years in 100%. Of eyes that had gonioscopy, 93.9% had October 1, 1970, and November 30, 2002. Patients with open angles. Glaucoma surgery was needed in 57.1% of intraocular pressures greater than 25 mm Hg after - eyes. The median final visual acuity was 20/400. ectomy were studied. Patients with either conditions in- dependently associated with glaucoma or any signs of Conclusions: Most cases of aphakic glaucoma are of the glaucoma before lensectomy were excluded. open-angle type. Various risk factors are suggested, and the prognosis is guarded. Lifelong follow-up is needed Results: We studied 170 eyes of 117 patients. The to screen for glaucoma. mean±SD follow-up period was 8.6±7.6 years. The most common cataract types were complete (40.8%) and nuclear Arch Ophthalmol. 2004;122:1819-1825

LAUCOMA CONTINUES TO accounting for 75.0% to 93.8%5,19,20 of glau- be one of the most com- coma cases after lensectomy. mon complications of We describe the largest series in the lit- sur- erature of patients with aphakic glau- gery and ranges from 0% coma to better define the risk factors, clini- toG 32%.1-14 The incidence of aphakic glau- cal course, and prognosis of this disease. coma also seems to increase with longer follow-up.1-5 In patients who receive fol- METHODS low-up for longer than 5 years, the inci- dence of glaucoma has been reported to A retrospective medical record review was per- be as high as 41%.3 formed for all patients seen by a pediatric glau- Although it was commonly believed that coma specialist (D.S.W.) between October 1, newer automated techniques would de- 1970, and November 30, 2002. Patients were crease the incidence of glaucoma,1,3,6,15-18 the included if they had repeated intraocular pres- incidence after automated lensectomy and sures (IOPs) greater than 25 mm Hg after con- genital cataract surgery. Goldmann or Per- vitrectomy is still relatively high and un- 2,6,19 1,3,4,8,9,11,16,17 kins applanation tonometry was used. Cataracts changed at 0% to 32%, which were defined as congenital if they were iden- is similar to the incidence of 5.5% to 27.0% tified in the first 6 months of life, were domi- in patients who underwent older needling nantly inherited, or were of the lamellar type. and aspiration procedures.6-8,10,12 How- Patients were included if they underwent lens- ever, with the introduction of vitrectomy ectomy before age 20 years. Author Affiliations: Glaucoma instruments for childhood cataract sur- Inclusion criteria consisted of having at Service, Department of gery in 1975,9 angle-closure glaucoma with least 1 month of follow-up after aphakic glau- , Harvard coma was diagnosed. Patients were excluded Medical School and pupillary block after lensectomy has be- if they had a history of trauma, intraocular 1-3,6,7,16-20 Massachusetts Eye and Ear come less common. Now, most neoplasm, radiation therapy, anterior , Infirmary, Boston. cases of glaucoma after pediatric lensec- anterior segment dysgenesis, Stickler syn- Financial Disclosure: None. tomy are of the later-onset open-angle type, drome, Lowe syndrome, maternal rubella

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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Table 1. Types of Cataracts in 71 Eyes Table 2. Complications in 85 Eyes With Aphakic Glaucoma After Lensectomy

Type of Cataract Eyes, No. (%) Complication Eyes, No. (%)* Complete 29 (40.8) Pupillary membrane 49 (57.6) Nuclear 16 (22.5) Residual lens material 15 (17.6) Persistent hyperplastic primary vitreous 9 (12.7) Posterior capsule opacification 12 (14.1) Lamellar 6 (8.5) Pupillary block 9 (10.6) Membranous 4 (5.6) Posterior synechiae 3 (3.5) Cortical 3 (4.2) Vitreous hemorrhage 3 (3.5) Anterior polar 2 (2.8) 2 (2.4) Posterior subcapsular 1 (1.4) Malignant glaucoma 2 (2.4) Posterior cortical 1 (1.4) Severe inflammation 2 (2.4) 1 (1.2) Nonhealing wound 1 (1.2)

60 *Some eyes had more than 1 complication.

50 Table 3. Associated Ocular Anomalies in 58 Eyes 40 With Aphakic Glaucoma

30 Ocular Anomaly Eyes, No. (%)* Eyes, % 20 Microphthalmos 24 (14.1) 11 (6.5) 10 Persistent hyperplastic primary vitreous 9 (5.3) Nasolacrimal duct obstruction 9 (5.3) 0 o o o o o o o o Mixed tropias 7 (4.1) >6 y 0-2 m 3-6 m 7-12 m 13-24 m 25-36 m 37-48 m 49-60 m 61-72 m 5 (2.9) Age *Some eyes had more than 1 anomaly. Figure 1. Distribution of patient age at lensectomy.

syndrome, or trisomy 13. Patients with a history of corticoste- the operative techniques used in 57 eyes were unknown. roid use before lensectomy or a history of maternal corticoste- Fifty-five (53.4%) of 103 eyes underwent lensectomy with roid use, those with signs of congenital glaucoma before primary posterior capsulotomy and anterior vitrectomy. lensectomy, and those with retinal detachment or vitreous One patient underwent extracapsular cataract extraction hemorrhage before lensectomy were also excluded. with a posterior chamber intraocular lens at age 1 month. Information included a complete history and eye examina- tion. Also recorded were the intraoperative and postoperative The mean±SD age at the time of lensectomy was 0.7±1.4 courses. Results are expressed as mean±SD. years (range, 1 week to 10 years). One hundred thirty- seven (80.6%) of 170 eyes underwent surgery before the patient was aged 1 year (Figure 1). RESULTS No intraoperative complications were recorded in any of the eyes. Eighty-five (50.0%) of 170 eyes had compli- One hundred seventy eyes of 117 patients with aphakic cations after lensectomy (Table 2). glaucoma met the inclusion and exclusion criteria. There Forty-eight (28.2%) of 170 eyes had a family history were 55 males and 62 females. Mean±SD follow-up time of congenital cataracts in first-degree relatives. Thirty- was 8.6±7.6 years (range, 1 month to 28 years). Patient eight (79.2%) of these 48 eyes were from 19 patients with ages at the last follow-up visit ranged from 5 months to bilateral congenital cataracts. 63 years. Associated ocular conditions were present in 58 (34.1%) Fifty-three patients had bilateral aphakic glaucoma, of 170 eyes (Table 3). Associated systemic anomalies were 30 had bilateral lensectomy but aphakic glaucoma in seen in 39 (33.3%) of 117 patients (Table 4). The only 1 eye, and 34 had unilateral lensectomy with sub- mean±SD IOP before lensectomy was 15.2±3.5 mm Hg sequent aphakic glaucoma. No patient with unilateral (range, 5-22 mm Hg) and after the development of apha- lensectomy developed glaucoma in the other eye. In kic glaucoma was 34.0±7.1 mm Hg (range, 25-50 mm Hg). patients in whom cataract type was recorded (71 eyes), The mean±SD corneal diameter before lensectomy was the most common type was the complete cataract 9.9±1.2 mm (range, 7-13 mm). The mean±SD axial length (Table 1). by ultrasonography (A scan) before lensectomy was Thirty-nine (22.9%) of 170 eyes were operated on by 15.6±2.3 mm (range, 12-21 mm). The mean±SD birth D.S.W., and 131 eyes (77.1%) were operated on by other weight was 3.2±0.8 kg (range, 0.6-4.5 kg). surgeons. Modern vitrectomy instruments were used in Aphakic glaucoma developed after lensectomy in 63 103 eyes (91.2%), whereas older techniques (ie, nee- (37.1%) of 170 eyes by age 1 year and in 129 (75.9%) by dling, aspiration, or linear extraction) were used in 10 eyes; age 6 years (Table 5). The mean±SD interval that glau-

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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 Table 4. Associated Systemic Abnormalities in 39 Patients Table 5. Interval Between Lensectomy and Aphakic With Aphakic Glaucoma Glaucoma Diagnosis in 170 Eyes

Systemic Abnormality Patients, No.* Patient Age Eyes, No. (%) Developmental delay 9 0-2 mo 26 (15.3) Cardiac anomaly 6 3-6 mo 21 (12.4) Prematurity 5 7-12 mo 16 (9.4) Intrauterine infections 13-24 mo 17 (10.0) Human immunodeficiency virus 2 25-36 mo 16 (9.4) Unknown viral syndrome 1 37-48 mo 20 (11.8) Renal anomaly 2 49-60 mo 6 (3.5) Metabolic diseases 61-72 mo 7 (4.1) Glucose-6-phosphate dehydrogenase deficiency 1 Ͼ6y 41 (24.1) Hypoglycemia 2 Hyperbilirubinemia 3 Atopy Asthma 4 Table 6. Cup-Disc Ratio and Appearance Allergies 3 at the Time of Diagnosis of Aphakic Glaucoma in 169 Eyes Hematologic disorders Acute lymphocytic leukemia 1 Leukopenia 2 Characteristic Eyes, No. (%) Anemia 1 Cup-disc ratio Thrombocytopenia 1 0.2-0.3 88 (52.1) Peroxisomal disorders 0.4-0.5 27 (16.0) Rhizomelic chondrodysplasia punctata 1 0.6-0.7 9 (5.3) Marden Walker syndrome 1 0.8-0.9 11 (6.5) Central nervous system disorders 0.99 2 (1.2) Cranial nerve palsies 1 Optic nerve appearance Seizures 1 Vertical elongation 15 (8.9) Gastrointestinal disorders Good red reflex 12 (7.1) Malrotation of intestines 1 Poor red reflex 3 (1.8) Gastroesophageal reflux disease 1 Abnormal appearance due to PHPV 2 (1.2) Pyloric stenosis 1 Umbilical hernia 1 Abbreviation: PHPV, persistent hyperplastic primary vitreous. Imperforate anus 1 Maternal conditions in utero Gestational diabetes mellitus 1 Multiple drug use 1 Table 7. Surgical Procedures Performed for Postoperative Other Complications in 70 Eyes With Aphakic Glaucoma Exchange transfusion at birth 1 Cleft palate 1 Surgical Procedure Eyes, No. (%)* Pupillary membranectomy 39 (55.7) *Some patients had more than 1 abnormality. Residual lens material 9 (12.9) Posterior capsule opacification 10 (14.3) Iridectomy 13 (18.6) coma was diagnosed after lensectomy was 4.0±4.6 years. Some surgery for pupillary block 8 (11.4) Retinal detachment surgery 2 (2.9) Open-angle glaucoma was the most common type of Anterior vitrectomy 16 (22.9) aphakic glaucoma. Gonioscopy showed open angles in 139 (93.9%) of the 148 eyes examined. In 74 (50.0%) *Some eyes underwent more than 1 surgical procedure. of the 148 eyes, there was some degree of peripheral anterior synechiae. Some eyes also showed increased pigmentation and a glazed appearance of the trabecular (56%) of 70 eyes (Table 7). Surgical procedures unre- meshwork. Nine (6.1%) of 148 eyes had closed angles. lated to postoperative complications were performed in Four eyes had corneal haze, which precluded gonios- 28 (16.5%) of the 170 eyes. surgery was per- copy. The gonioscopy findings of 18 eyes were not formed in 22 (12.9%) of 170 eyes. In 8 of these 22 eyes, known. strabismus was present before lensectomy. In the After the development of glaucoma, the posterior remaining 14 eyes, it is unknown whether the onset of pole could be seen in 169 eyes. Ninety-four (55.6%) of strabismus was before or after cataract surgery. Second- the 169 eyes showed good vascularization of the optic ary intraocular lens surgery was performed in 4 eyes, disc, whereas 58 (34.3%) showed varying degrees of nasolacrimal duct probing in 1 eye, and refractive cor- pallor. After the development of aphakic glaucoma, the neal surgery in 1 eye. mean±SD cup-disc ratio was 0.29±0.13 (range, 0.2- Topical glaucoma medications were applied to 156 0.99) (Table 6). (91.8%) of 170 eyes after the onset of glaucoma (Table 8). Seventy (41.2%) of 170 eyes were operated on for The initial treatments in the other 14 eyes were either postoperative complications, with the most common unknown or consisted of systemic medications or sur- operation being pupillary membrane removal in 39 gery. The most common single agent used was a ␤-blocker

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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 30 Table 8. Number of Topical Glaucoma Medications Used 27.9 After the Initial Diagnosis of Aphakic Glaucoma in 170 Eyes 25

Topical Glaucoma 20 Medications, No. Eyes, No. (%) 16.3 0 14 (8.2) 15

1 75 (44.1) Eyes, % 10.6 9.6 9.6 2 53 (31.2) 10 3 20 (11.8) 6.7 4.8 4 8 (4.7) 3.8 3.8 5 3.8 2.9

0 ents 20/200 20/400 ovem 20/25-20/4020/50-20/6020/70-20/100 Table 9. Types of Topical Glaucoma Medications Used After CountingHand Fingers M Light Perception No Light Perception Fixation and Following the Initial Diagnosis of Aphakic Glaucoma in 170 Eyes No Fixation and Following Visual Acuity Eyes, No. Figure 2. Visual acuity at the last follow-up visit in 170 eyes. Topical Glaucoma Single-Drug Multidrug Medication Regimen Regimen Total ␤-Blockers 84 72 156 further surgery). Of the 61 (62.9%) of 97 eyes that un- Cholinergic agents 5 41 46 derwent trabeculectomy, only 1 was without antime- Adrenergic agonists 0 39 39 Carbonic anhydrase inhibitors 1 38 39 tabolites. Forty-three eyes used mitomycin, and 17 eyes Prostaglandin analogues 0 10 10 used fluorouracil. Fifteen (24.6%) of these 61 eyes had Hyperosmotic agents 0 0 0 successful outcomes (ie, IOP Յ21 mm Hg, with or with- out medications, and no need for further surgery). Thirty-four (35.1%) of 97 eyes had tube surgery (ie, 32 Ahmed valves [New World Medical, Inc, Rancho Cu- Table 10. Surgical Procedures Performed camonga, Calif] and 2 Molteno valves [Molteno Oph- for Aphakic Glaucoma in 170 Eyes thalmic Limited, Dunedin, New Zealand]), with a suc- cess rate of 44.1% (ie, IOP Յ21 mm Hg, with or without Surgical Procedure Eyes, No. medications, and no need for further surgery). None 73 Cyclodestructive procedures were performed in 21 Goniotomy or trabeculotomy only 9 (21.6%) of 97 eyes and included cyclocryotherapy, di- Trabeculectomy with or without antimetabolites 32 ode laser transscleral cyclophotocoagulation, and con- Tube implantation only 15 tact Nd:YAG laser cyclotherapy. The success rate for cy- Cycloablation only 11 clodestructive procedures was 14.3% (ie, IOP Յ21 Goniotomy + trabeculectomy 7 with or without antimetabolites mm Hg, with or without medications, and no need for Goniotomy + tube implantation 1 further surgery). Trabeculectomy with or 9 At the last follow-up visit, median and mean visual without antimetabolites + tube implantation acuities were 20/400 and 20/515, respectively. Visual acu- Trabeculectomy with or 1 ities ranged from 20/25 to no light perception. Figure 2 without antimetabolites + cycloablation Goniotomy + trabeculectomy with or 3 shows that 10.6% of eyes had visual acuity of 20/25 to without antimetabolites + tube implantation 20/40, 23.0% of 20/50 to 20/200, and 66.2% of 20/400 Trabeculectomy with or without 5 or worse at the last follow-up visit. antimetabolites + tube implantation + cycloablation Goniotomy + trabeculectomy with or without 3 antimetabolites + cycloablation COMMENT Goniotomy + trabeculectomy with or 1 without antimetabolites + tube implantation + cycloablation With modern lensectomy techniques, most cases of aphakic glaucoma are of the open-angle type. Various risk factors are associated with aphakic glaucoma, which has a guarded prognosis. Because glaucoma may in 84 (49.4%) of 170 eyes (Table 9). Sixty (35.3%) of not develop for years after lensectomy, lifelong fol- 170 eyes needed systemic carbonic anhydrase inhibitor low-up is needed. therapy. In this study, most cases of aphakic glaucoma after lens- Glaucoma surgery was performed in 97 (57.1%) of 170 ectomy for congenital cataracts were of the open-angle eyes (Table 10). Sixty-seven (69.1%) of 97 eyes un- type (139 [93.9%] of 148 eyes). This is consistent with derwent a single procedure, and 30 eyes (30.9%) needed the literature,5,19,20 which describes 75.0% to 93.8% of pa- multiple surgical procedures to control IOP. Gonioto- tients with open angles. Although most patients with apha- mies or trabeculotomies were performed in 24 (24.7%) kic glaucoma have open angles, acquired angle changes of 97 eyes, with a success rate of 16.0% (ie, IOP Յ21 can be seen in 96% of eyes and are characterized by cir- mm Hg, with or without medications, and no need for cumferential forward positioning of the to the pos-

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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 terior or middle trabecular meshwork.19 The residual sistent hyperplastic primary vitreous (PHPV)23 have been exposed trabecular meshwork is sometimes noted to suggested as risk factors for the development of glau- have scattered pigment deposits,5,15,19,21 white crystal- coma. In the 170 eyes in our study, microphthalmos line deposits suggesting residual lens tissue caught (14.1%) and PHPV (5.3%) were 2 of the more common in the trabecular meshwork,19 or peripheral anterior ocular abnormalities. Other researchers5,6,8,14,17 have also synechiae.2,3,19,22 reported microphthalmos in 8.3% to 26.6% of eyes. Most cases of aphakic glaucoma develop years after Johnson and Keech4 did not believe that PHPV was a risk lensectomy. The mean±SD time after lensectomy that factor and noted that patients with and without PHPV glaucoma developed in our patients was 4.0±4.6 years have a 32% incidence of aphakic glaucoma after pediat- (Table 5), compared with other reports of 5.3 to 12.2 ric lensectomy. Wallace and Plager24 pointed out that the years.2,3,5,7,20,23-25 Therefore, buphthalmos, which usu- underlying risk factor for these conditions may primar- ally occurs in the first 2 to 3 years of life, is generally not ily be the smaller . present. The latest time after lensectomy that glaucoma Our study is consistent with others in that an in- developed in our study was 33 years, compared with the creased risk of aphakic glaucoma may be related to cer- literature’s citing of 4515 to 6526 years. Because it is also tain cataract types, such as complete,2 nuclear,2,15,23 and difficult to predict which eyes will develop aphakic glau- PHPV23 (Table 1). Although 1 study3 suggested that cata- coma,25 lifetime surveillance for glaucoma after congen- ract type was not associated with subsequent glaucoma, ital cataract surgery is necessary.15,19 that series of 8 eyes may have been too small to accu- Even after 5 years of follow-up,27 children with con- rately assess this. These congenital cataract types often genital cataracts rarely, if ever, develop glaucoma if they necessitate early surgery for better visual rehabilitation. do not undergo lensectomy.16 This finding is also sup- Also, nuclear and PHPV cataracts are more commonly ported by other researchers who have noted that no associated with smaller corneal diameters.23,24 patient with bilateral developed unilateral It has been suggested that in some patients cataracts glaucoma3,25 and that no patient with unilateral aphakia and glaucoma may be signs of a yet undescribed con- had bilateral glaucoma.3,25 Our study, however, had genital ocular syndrome. This possibility is supported by 30 patients with bilateral lensectomy but unilateral the bilateral lensectomy cases with atypical angle abnor- glaucoma. malities in some but is less likely because of the late on- The etiology of aphakic glaucoma is still unclear.28,29 set of the glaucoma and the normal angle appearance in Undergoing lensectomy at a young age,1,2,4,7,8,28 espe- others.15,25 Although such an ocular syndrome may not cially in the first year of life,2,19,23 may be a risk factor for necessarily be inherited,1,15 48 (28.2%) of 170 eyes in our the development of aphakic glaucoma. Only 1 study3 sug- study involved a family history of congenital cataracts in gests that it may not be a risk factor. Most of our pa- first-degree relatives. Thirty-eight (79.2%) of these 48 tients with aphakic glaucoma (80.6%) underwent lens- eyes belonged to 19 patients with bilateral cataracts. All ectomy in the first year of life (Figure 1). This is consistent of these patients with bilateral cataracts developed bilat- with the 77.0% to 92.9% reported in the literature.2,4,19 eral glaucoma. It has been suggested that the immaturity of the devel- It has also been suggested that the aphakic state per oping infant’s angle leads to increased susceptibility to se may increase the risk of glaucoma. In our study, 169 secondary surgical trauma.2 Additional contributing fac- of the 170 eyes were left aphakic at the time of initial cata- tors seen in younger patients undergoing surgery in- ract surgery. One theory as to why aphakia causes glau- clude increased postoperative inflammation, associated coma was suggested by Paul Kaufman, MD, in response congenital ocular anomalies, and increased technical dif- to Walton’s19 American Ophthalmological Society pre- ficulties with their associated increased postoperative com- sentation. Taking out the lens during the first year of life plications.2,6,15,16,30 Despite the increased risk of aphakic without putting anything in its place may prevent nor- glaucoma with early surgery, it seems inadvisable to de- mal meshwork development, which may require cer- lay surgery solely for fear of aphakic glaucoma.2 The in- tain normal structural interactions between the native lens, creased risks of early lensectomy must be balanced against zonules, , and trabecular meshwork.19 At that the need to decrease the period of visual deprivation.1 same American Ophthalmological Society meeting, Al- Corneal diameters less than 10 mm have also been as- bert W. Biglan, MD, observed that patients with corneal sociated with 88.5% to 94.0%23,24 of patients with apha- diameters less than 10 mm are usually left aphakic.19 The kic glaucoma and may be another risk factor.2,7,23 One aphakic state may allow blockage of the angle by vitre- study3 with only 8 eyes found no association. Our pa- ous5 or may allow vitreous factors to alter trabecular mesh- tients had a mean±SD corneal diameter of 9.9±1.2 mm. work structure and maturation.25,29 Parks et al23 noted that aphakic glaucoma developed in Conversely, it has been suggested that primary pseu- only 2.9% of patients with normal corneal diameters com- dophakia decreases the incidence of glaucoma.25 Only 1 pared with 31.9% of eyes with small corneal diameters. of the 170 eyes in our study had primary lens implanta- A smaller cornea may reflect an abnormal anterior seg- tion at the time of initial cataract surgery. Asrani et al25 ment and subtle filtration angle defects, which increase noted that of 377 eyes with primary lens implantation the risk of aphakic glaucoma.19,24 for congenital cataracts, only 1 eye developed glau- In our study, one third of the eyes had associated ocu- coma, with 3.9 years of follow-up, which is less than the lar anomalies (Table 3). Other researchers6,31 have re- mean time of aphakic glaucoma development of 5.3 to ported that 50% of patients with aphakic glaucoma have 12.2 years.3,20,23-25 Unlike our study, Asrani et al25 ex- additional eye abnormalities. Microphthalmos8,29 and per- cluded patients with risk factors for aphakic glaucoma

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©2004 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/25/2021 (ie, associated ocular anomalies and corneal diameters patients with aphakia and may not be recognized if vi- Ͻ10 mm). Also, only 19.4% of patients underwent lens- sion is reduced from other causes.3,15 Asrani et al25 noted ectomy at age 1 year or younger. Another study32 noted that 63.6% of patients were treated with medications alone. that only 3 (6.7%) of 45 eyes developed glaucoma after Glaucoma surgery was performed in 97 (57.1%) of 170 pediatric lensectomy and primary lens implantation; how- eyes (Table 10) compared with 36.4% in the literature.5 ever, this study also had a shorter follow-up period of Success rates for all types of glaucoma surgery (primary 23 months and excluded certain high-risk patients (ie, and secondary) are guarded and range from 14.3% to patients with corneal diameters Ͻ10 mm and patients 44.1% (ie, IOP Յ21 mm Hg, with or without glaucoma Ͻ1 year). Perhaps if certain high-risk patients had not medications, and no need for further surgery). Sixty- been excluded from these studies and if there were longer seven (69.1%) of the 97 eyes underwent a single proce- follow-up times, the incidence of glaucoma in these stud- dure. Asrani and Wilensky5 noted that 78.6% of pa- ies would have been higher. tients achieved successful IOP control with 1 operation Retained degenerating lens proteins may be toxic to without using glaucoma medications. the trabecular meshwork15,18,19 and may increase the risk Systemic abnormalities were seen in 33.3% of our pa- of aphakic glaucoma. Cytokines released by residual lens tients. As in our study (Table 4), Peyman et al33 noted epithelial cells may also be responsible.16 In our study, that developmental delay (4 of 25 patients) and cardiac 15.0% of eyes had retained lens material. Other stud- abnormalities (5 of 25 patients) were most common. ies6,19 have reported 41.6% to 78.0% of patients with re- The visual prognosis is also guarded. In our study, vi- sidual cortex or lens material. Perhaps modern phaco- sual acuity at the last follow-up visit was 20/400 or worse emulsification techniques with complete cortical removal in 66.2% of eyes (Figure 2). The literature5,15,18 notes that may benefit patients with congenital cataracts.19,33 The 11.4% to 100% of patients achieve a final visual acuity continuous tear capsulorrhexis may also eliminate re- of less than 20/200 or 20/400 in the affected eye. The mean sidual capsular tags to which the iris can adhere and that visual acuity in our patients was 20/515, which is simi- may cause chronic inflammation.19 lar to the 20/510 in the study by Simon et al.3 Once apha- Poor pupillary dilation can make the surgery techni- kic glaucoma was diagnosed, Asrani and Wilensky5 noted cally more difficult19 and has been associated with an in- stable vision in 68.8% of patients. They also found that creased risk of glaucoma.2,29 This may increase the rate poor vision in most patients was partly due to delay in of complications and the chance for residual cortex. diagnosis and treatment. Poor visual acuity in patients A corticosteroid-induced mechanism seems unlikely with aphakic glaucoma is attributable to secondary mem- because postoperative corticosteroids are usually used for branes, , strabismic or deprivational ambly- only 1 to 2 months.25 This is inconsistent with the usual opia, and glaucomatous optic nerve damage.1,6 later onset of aphakic glaucoma. However, perhaps the Ophthalmoscopy of eyes in our study after the devel- use of high-dose corticosteroids34 in infancy may alter the opment of aphakic glaucoma revealed a mean±SD cup- still-developing microstructure of the trabecular mesh- disc ratio of 0.29±0.13 (Table 6). Simon et al3 reported work.29 None of our patients had a history of prolonged a mean cup-disc ratio of 0.54 (range, 0.15-0.80) in glau- postoperative corticosteroid use. comatous eyes. The increased postoperative inflammation seen in pe- Despite the introduction of automated surgical tech- diatric patients does not seem to be a substantial risk fac- niques in the 1970s, the incidence of aphakic glaucoma tor. Only 2 of our patients were noted to have severe post- remains high. Open-angle glaucoma is now the most com- operative inflammation. Although the occurrence of mon type of glaucoma after congenital cataract surgery, chronic inflammation was not specifically assessed in the and it occurs at a mean±SD duration of 4.0±4.6 years study by Walton,19 he noted that the anterior segments after lensectomy. Various risk factors have been sug- of these patients were not characterized by cells, flare, gested. Treatment may be difficult, with 57.1% of pa- and and, in this way, were different from tients needing glaucoma surgery. Prognosis is guarded, those of children with chronic anterior uveitis who de- with about two thirds of our patients having a final vi- velop glaucoma.19 sual acuity of 20/400 or worse. Close lifelong follow-up A history of secondary surgery is seen more often in is needed in these patients to detect this condition in its children who develop aphakic glaucoma.6,16 Seventy earliest stages and prevent loss of vision. (41.2%) of 170 eyes in our study had surgery for post- operative complications, most commonly for pupillary membrane removal (Table 7). Other researchers6,19 have Submitted for Publication: October 10, 2003; final re- similarly reported that 58.6% to 75.0% of eyes with glau- vision received April 12, 2004; accepted August 12, 2004. coma undergo 1 or more secondary membrane proce- Correspondence: Teresa C. Chen, MD, Glaucoma Service, dures. Retained lens protein and repeated surgery for sec- Department of Ophthalmology, Harvard Medical School ondary membrane may cause increased postoperative and Massachusetts Eye and Ear Infirmary, 243 Charles St, inflammation and trabecular meshwork damage.6,35 Boston, MA 02114 ([email protected]). Once glaucoma develops, medical treatment in chil- Funding/Support: This study was supported in part by dren may be difficult (Tables 8 and 9). Miotics may re- the Miles Pediatric Research Fund, Boston, Mass. duce visual acuity when dense capsular opacifications are Previous Presentation: This study was presented in part present. In one study,15 2 (11.1%) of 18 patients experi- at the Annual Meeting of the Association for Research enced retinal detachments related to miotic treatment. Epi- in Vision and Ophthalmology; May 4, 2003; Fort Lau- nephrine-induced is also a concern in these derdale, Fla.

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