Risk Factors for Complications After Congenital Cataract Surgery without Intraocular Lens Implantation in the First 18 Months of Life
Article Outline
Purpose
To determine individual risk factors for the development of postoperative complications after pediatric cataract surgery in the first 18 months of life.
Design
Interventional, consecutive case series.
Methods
We retrospectively reviewed the records of 71 eyes of 46 children who underwent surgery for congenital cataract within the first 18 months of life. A limbal approach bimanual lens aspiration, posterior capsulorrhexis, and anterior vitrectomy without intraocular lens implantation was performed in all children. We examined the interrelationships of operative and postoperative complications with other variables such as patient age, family history, or ocular abnormalities. The mean follow-up period was 39 months.
Results
The most frequent postoperative complications were late-onset open-angle glaucoma (10.8%) and vitreous hemorrhage (10.8%), whereas early-onset glaucoma (4.6%) was less common. Secondary cataract was observed in seven eyes (9.2%). We determined a family history of aphakic glaucoma in first-degree relatives (P = .007) as well as cataract surgery in the first three months of life (P = .039) and nuclear cataracts (P = .0009) to be strong predictors of late-onset glaucoma. Secondary cataract formation was associated strongly with lensectomy in the first five months of life. The diagnosis of postoperative hemorrhages was associated significantly with the presence of persistent fetal vasculature (P < .0001).
Conclusions
Patients with preoperative predictors at presentation such as young age at the time of surgery, a family history of aphakic glaucoma, nuclear cataract, or persistent fetal vasculature syndrome offer a clear target for extensive postoperative care after congenital cataract surgery.
Advances in microsurgical techniques and amblyopia management have improved the safety and effectiveness of pediatric cataract treatment.1, 2, 3, 4, 5 However, management of congenital cataracts remains a challenge and postoperative complications are still common.6, 7, 8 Over the past years, a number of retrospective studies have reported varying prevalences of several postoperative complications after pediatric lensectomy. However, most of these studies have estimated the risk of secondary cataract formation or aphakic glaucoma separately, and there are only few reports including various complications.9, 10, 11, 12, 13, 14
It is imperative that children with visually significant congenital cataracts receive prompt surgery, correction of aphakia, and occlusion therapy to improve visual acuity and to prevent further amblyopia. Several studies have reported a strong positive correlation between earlier surgery for monocular and bilateral congenital cataract and better visual outcome.2, 3, 4, 5 There is, however, increasing evidence that earlier surgery is associated with a higher prevalence of postoperative complications. Just recently, it has been demonstrated that lensectomy for bilateral infantile cataract during the first month of life is associated with an increased risk of subsequent aphakic glaucoma compared with surgery performed later.10 The discovery of glaucoma and secondary cataract formation occurring years after removal of the lens has been documented by many reports.7, 9, 16, 17, 18 However, because the natural history of pediatric aphakic glaucoma is still unknown, whether there is a direct relationship between the time of cataract surgery or the necessity of after-cataract removal and the development of aphakic glaucoma remain a matter of controversy. Moreover, little is known about the incidence and management of retinal complications such as retinal detachment (RD) or vitreous hemorrhage after congenital cataract surgery.
The aim of the present investigation was to obtain longitudinal data on postoperative complications after pediatric cataract surgery without intraocular lens (IOL) implantation. Moreover, this study aimed to examine the interrelationships of these complications with other variables such as patient age, family history, or morphologic abnormalities to determine individual risk factors for the development of postoperative difficulties.
Methods
Participants
A total of 43 consecutive children aged 18 months and younger (65 eyes) with unilateral or bilateral congenital cataract, treated and followed up at our institution between August 1, 1996 and June 30, 2006, were included in this retrospective study. Exclusion criteria were ocular trauma, infection, congenital glaucoma, anterior segment dysgenesis, Lowe syndrome, maternal rubella syndrome, trisomy 13, optic nerve or other fundus abnormalities, and prematurity.
Treatment Administration
The surgical technique, performed by one surgeon (T.K.), included pupil dilatation with a combination of cyclopentolate 1%, tropicamide 1%, and phenylephrine 2.5%. A three-step entrance to the anterior chamber was created by a limbal conjunctival incision, a scleral incision (12 o'clock), and finally a tunnel extending 1.5 mm to clear cornea. Initial entry into the anterior chamber was made using two paracenteses at 10 and 2 o'clock. An anterior continuous curvilinear capsulotomy of 5 to 5.5 mm was performed with a forceps under high-viscosity viscoelastic substance (Healon GV; Pharmacia, Uppsala, Sweden). After hydrodissection, the lens was aspirated using a small-port bimanual system (Koch/Kohnen bimanual A/I system, G-22100/1; Geuder, Heidelberg, Germany), a posterior curvilinear capsulorrhexis of 4 to 4.5 mm and an anterior vitrectomy were performed.
At the end of the surgery, viscoelastic was replaced by balanced salt solution (Alcon Surgical Inc, Fort Worth, Texas, USA), and the scleral (10-0 nylon; Ethicon, Hamburg, Germany) and conjunctival (7-0 Vicryl; Alcon Surgical, Inc) wounds were closed with running sutures. Topical antibiotics three times daily, alternating with 1% prednisolone acetate drops for a total of five times daily and topical nonsteroidal anti-inflammatory agents four times daily, were given initially. Aphakic rehabilitation was treated as soon as possible with contact lenses. Patching for amblyopia was instituted when indicated within one to two weeks after surgery one-half of waking time by adhesive eye patches.
Preoperative and Follow-up Examinations
In all patients, before surgery, complete bilateral ocular examinations, including best-corrected visual acuity (BCVA), keratometry, cycloplegic retinoscopy, and A-scan and B-scan ultrasonography (10 MHz), were conducted. BCVA was assessed using the Teller Acuity Cards Test (Vistech Consultants, Inc, Dayton, Ohio, USA) or the Lea Test (Precision Vision, La Salle, Illinois, USA), depending on the age and with one eye occluded. All refraction readings were obtained after instillation of combination of cyclopentolate 1%, tropicamide 1%, and phenylephrine 2.5%.
Diagnosis of Aphakic Glaucoma
Most subjects were able to cooperate with a comprehensive glaucoma examination including cycloplegic refraction, axial length and corneal diameter measurement, anterior segment and optic nerve examination, fundus photography, and determination of intraocular pressure (IOP) without requiring sedation. The IOP was considered valid if the child was able to cooperate without duress or squeezing of the eyelids. In younger children, examination in general anesthesia was performed if there was any suspicion of aphakic glaucoma.
Diagnosis of glaucoma was made if IOP was more than 25 mm Hg,12, 13, 14 combined with at least one of the following criteria: corneal edema or enlargement, optic nerve cupping (asymmetry > 0.2 or cup-to-disk ratio > 0.4), or abnormal asymmetric axial length elongation. Goldmann or Perkins applanation tonometry was used.
All eyes with the diagnosis of glaucoma underwent gonioscopic examination. Glaucomatous eyes were subdivided into two subtypes: early angle-closure glaucoma presenting within the first year after lensectomy associated with angle abnormalities in the gonioscopic examination or late open-angle glaucoma usually presenting up to sometimes years after uneventful lensectomy without angle abnormalities.16, 19
Diagnosis of Persistent Hyperplastic Primary Vitreous
Persistent hyperplastic primary vitreous (PHPV) included a spectrum of diseases associated with prominent hyaloid vessel remnants. Before surgery, all cataract eyes underwent ultrasound examination. Based on B-scan sonography, diagnosis of persistent fetal vasculature (PFV) was made when one or more of the following clinical features was associated with congenital cataract: an elevated vitreous membrane or stalk from the optic nerve, retrolental fibrovascular membrane, or elongated ciliary processes.
Data Analysis and Statistics
Statistical analysis of the prevalence of several postoperative complications was performed by the Fisher exact test. All tests were two-tailed, and acceptable significance was recorded when P values were less than .05. Analyses were conducted using the BIAS statistical software package (BIAS for Windows Software, version 7.07; Epsilon-Verlag GbR, Hofheim, Germany).
Results
The records of 46 consecutive children younger than 18 months of age (71 eyes) who underwent cataract extraction without IOL implantation between August 1, 1996 and June 30, 2006 were reviewed. One patient had bilateral atrophic optic nerves associated with Marshall syndrome and two patients had Lowe syndrome. According to the exclusion criteria, all three patients were excluded from the analysis. Forty-three patients and 65 eyes were included for further analysis of the complications. The mean age at the time of the initial examination was 4.2 months (range, one week to 14 months). The diagnosis of unilateral cataract was made in 21 of these patients, whereas bilateral cataract was found in 22 children. The patients ranged in age from two weeks to 15 months at the time of surgery (mean, 4.5 months). Twenty patients were male and 23 were female. The mean follow-up period was 39.0 months (range, six to 108 months).
Six (14%) of 43 patients had a family history of congenital cataracts in first-degree relatives. The morphologic types of cataract were nuclear (20 eyes), complete (25 eyes), lamellar (eight eyes), and posterior (12 eyes). Other preoperative ocular abnormalities included microphthalmos in 12 eyes. Moreover, preoperative ultrasound revealed unilateral PFV of the posterior segment in 12 eyes. Two children with bilateral cataract had Down syndrome.
The overall prevalence of postoperative complications including aphakic glaucoma, posterior capsule opacification (PCO), RD, vitreous and anterior segment hemorrhage, and fibrinous reaction was 35.4% (Table). No intraoperative complications were recorded in any of the eyes. Additional surgery for postoperative complications was necessary in 17 eyes (26.2%).
TABLE. Association of Preoperative Risk Factors and Postoperative Complications after Congenital Cataract Surgery without Intraocular Lens Implantation
| Type of Complication | Prevalence, No. of Patients (%) | Association with Preoperative Risk Factor | |
|---|---|---|---|
| Vitreous hemorrhage | 7 (10.8%) | PFV | P<.0001 |
| Retinal detachment | 2(3.1%) | PFV | P=.032 |
| Aphakic glaucoma | 10(15.4%) | Age at time of surgery < three mos | P=.039 |
| Nuclear cataract | P=.0009 | ||
| After cataract removal | P=.23,n.s. | ||
| Early onset angle closure | 3(4.6%) | ||
| Late-onset open-angle glaucoma | 7(10.8%) | Family history of aphakic glaucoma | P=.007 |
| Secondary cataract | 6(9.2%) | Age at time of surgery < five mos | P=.037 |
| Fibrinous reaction | 3(4.6%) | Microphthalmos | P=.46,n.s. |
| Anterior chamber hemorrhage | 2(3.1%) | ||
Postoperative vitreous hemorrhage occurred in seven (10.8%) of 65 eyes, ranging from mild (which did not prevent retinal examination) in two eyes to moderate or severe (such that an ultrasound was necessary to determine the status of the retina) in five eyes. Further analysis revealed that this complication was significantly associated with PHPV or PFV. Twelve (18.5%) of 65 eyes had PHPV or PFV and seven (58.3%) of these 12 eyes were found to have developed postoperative vitreous bleeding complications (P < .0001; Figure).
FIGURE.
(Top) Intraoperative photograph showing persistent fetal vasculature (PFV) and opacity of the posterior capsule. (Bottom) Preoperative B-scan ultrasound (10 MHz) of the same eye revealing posterior PFV.
Moreover, RD was highly associated with PHPV (P = .032). This complication was detected in only two eyes with this ocular abnormality. In one eye, rhegmatogenous RD developed three weeks after surgery, and the other eye had tractional RD four years after cataract surgery. Both eyes underwent pars plana vitrectomy performed by one surgeon (L.-O.H.). The vitreous cavity hemorrhage cleared significantly in each case within three weeks. Additional surgery to remove clotted blood was not required.
After surgery, in this series two eyes showed anterior hemorrhages, significantly clearing within five days. In one case, anterior hemorrhage was associated with posterior hemorrhage and PFV.
Postoperative fibrinous reaction was present in three eyes (4.6%). In the subgroup of patients with microphthalmos, there was a tendency to have a higher frequency of fibrinous reaction (8.3% vs 3.8%). However, this difference did not reach statistical significance (P = .46). Only one eye (1.5%) required pupillary membranectomy.
In our series, aphakic glaucoma was the most frequent postoperative complication. The overall prevalence of glaucoma was 15.4% (10 eyes) during the mean follow-up period of three years and three months. Three patients had bilateral aphakic glaucoma, three had bilateral cataract surgery but aphakic glaucoma in only one eye, and one had unilateral cataract treatment with subsequent aphakic glaucoma. In no patient with unilateral cataract surgery did glaucoma develop in the other eye.
We found that most cases of glaucoma (four patients; seven eyes) were of the later-onset open-angle type, which developed at a mean of 33.9 months after surgery (range, 13 to 48 months). Unilateral early-onset angle-closure glaucoma was present in three children (mean time of onset, 4.3 months after surgery; range, three to five months). Of these cases, two eyes responded to anterior vitrectomy and did not require ocular hypotensive medication afterward. One patient required multiple surgery for glaucoma control. Two eyes with open-angle glaucoma responded to ocular hypotensive medication, whereas the other five eyes required surgical treatment.
The presence of late-onset glaucoma was associated strongly with a family history of aphakic glaucoma. Subgroup analysis revealed that two children had a family history of aphakic glaucoma after congenital cataract surgery in first-degree relatives. In both children, aphakic glaucoma developed, which was statistically significant (P = .007).
Interestingly, all three children presenting with bilateral glaucoma were of the later-onset open-angle type. Moreover, in the current study there was a strong relationship between age at the time of surgery and the development of aphakic glaucoma: 28.5% of the children in whom aphakic glaucoma developed underwent cataract surgery when they were younger than two months of age and 71.4% of them underwent surgery during the first three months of life. All children in whom aphakic glaucoma developed underwent cataract surgery in the first six months of life. We found that eight (25.8%) of 31 eyes undergoing cataract surgery when the children were three months or younger developed glaucoma compared with only two (5.9%) of 34 eyes undergoing surgery thereafter (P = .039).
There was a tendency for cataract eyes associated with other ocular abnormalities to have a higher frequency of developing aphakic glaucoma: microphthalmos associated with microcornea was present in three eyes (30%) and PHPV was found in one eye. However, this was not statistically significant (P = .37 and P = .67).
Twenty eyes had nuclear cataract, which was strongly associated with the development of aphakic glaucoma (P = .0009). Secondary cataract was detected in six eyes (9.2%) and developed after surgery at a mean of 2.67 months (range, one to five months). Interestingly, there was a strong relationship between time of surgery and development of secondary cataract: all six eyes underwent cataract surgery within the first five months and developed secondary cataract within five months after surgery. This was statistically significant (P = .037).
However, further analysis failed to prove a significant association between the necessity of after-cataract removal and the development of aphakic glaucoma. Two eyes with after-cataract removal developed aphakic glaucoma (P = .23). Moreover, there was no relationship between other ocular abnormalities like PFV or microphthalmos and the development of secondary cataract.
Discussion
In the present study, we sought to investigate the overall incidence of early and late postoperative complications after cataract surgery without IOL implantation in children younger than 18 months of age. Moreover, the purpose of our study was to determine systematically preoperative risk factors associated with postoperative complications to identify which patients are at greatest risk of developing complications.
The current study revealed an overall prevalence of postoperative complications of 35.4% after congenital cataract surgery in the first 18 months of life. The most frequent postoperative complication was aphakic glaucoma (15.4%). Compared with other studies with a longer follow-up, this incidence in our series may increase. The reported prevalence of aphakic glaucoma in children after cataract surgery varies between 6% and 59%.9, 10, 12, 13, 14, 15, 16, 17, 18, 20 This variability has been linked to differences in the patient population, the type of cataract, the age at surgical correction, the definition of glaucoma, and the length of follow-up.
The cause of aphakic glaucoma is still poorly understood. It has been suggested that immaturity of the developing infant's angle leads to increased susceptibility to surgical trauma. Moreover, the combination of difficult surgery, retained lens matter, and poor pupil dilatation may contribute to an increased postoperative inflammation leading to early-onset acute glaucoma. It seems plausible that with an improvement of surgical techniques, including extensive removal of lens matter and anterior vitrectomy, this subtype of aphakic glaucoma is becoming less common.
Further analysis of our patients with aphakic glaucoma revealed that 70% of all cases were of the late-onset open-angle type. This compares favorably with previous findings.21, 22 To date, the pathogenesis of late-onset open-angle aphakic glaucoma remains undetermined. Some authors have suggested that these eyes have congenital anterior segment abnormalities that make glaucoma more likely to develop. Interestingly, we found a trend toward the development of bilateral late-onset open-angle glaucoma in contrast to unilateral glaucoma, suggesting that these eyes had some predisposition to this complication. Moreover, in this series there was a strong association between the development of open-angle glaucoma and a family history of aphakic glaucoma in first-degree relatives. Both observations compare favorably with the hypothesis that both cataract and glaucoma may be signs of an as-yet poorly understood and undescribed congenital ocular syndrome.7, 16
The high risk of glaucoma in children with a family history of aphakic glaucoma raises the question of whether a more aggressive approach to glaucoma monitoring is justified. However, it is a clinical observation that the immaturity of an infant's cardiovascular, pulmonary, and gastrointestinal systems, as well as their liver, kidneys, and thermoregulation, puts them at greater risk from the effects of general anesthesia.23 Hence, we do not recommend routine examinations under anesthesia in children who undergo surgery early in life and who have a family history of aphakic glaucoma. In light of the high risk in these children of developing aphakic glaucoma, we would perform extensive monitoring of the anterior segment, optic nerve, and IOP whenever possible and without sedation throughout the child's life.
Postoperative inflammation is unlikely to be the direct cause of glaucoma in eyes treated up to several years after lensectomy. However, some authors have suggested that early cataract surgery may interfere with the maturation of the trabecular meshwork ultrastructure, leading to increased lifetime risk of open-angle glaucoma.19, 24 This could be mediated by factors such as exposure of the immature trabecular meshwork to lens material or the vitreous and mechanical surgical trauma. In our study, there was a strong association between having cataract surgery before the age of three months and the development of open-angle aphakic glaucoma.
The finding that young age at time of surgery is an important determinant of subsequent risk of developing glaucoma is consistent with the results of other studies.1, 8, 10, 13, 25, 26 In contrast, Watts and associates reported a reduced incidence of glaucoma in children who underwent congenital cataract surgery before two weeks of age.15 However in their study, the mean follow-up of the 12 infants who underwent surgery at this young age was only 2.3 years. In a recent retrospective study, Chen and associates concluded that the risk of aphakic glaucoma is the same, regardless of when cataract surgery is performed during the first year of life.20 However, we have to take into consideration that during the study period of 30 years, there have been substantial improvements in surgical techniques for the treatment of pediatric cataracts.
It is well established that the pediatric cornea reaches adult thickness at between two and four years of age.27 There is increasing evidence that central corneal thickness (CCT) has a clinically significant effect on IOP measurements in patients with pediatric glaucoma. However, the question of precisely how much to adjust the measured value remains controversial. In our institution, pachymetric examination routinely has been performed since 2003 in children with congenital cataract (Ultrasound pachymeter Tomey SP 3000; Tomey, Erlangen, Germany). Because this evaluation was not present uniformly in all study records, this information was not used for the definition of glaucoma. Therefore, the present study cannot rule out the possibility that the IOP was overestimated in some eyes. However, because the diagnosis of glaucoma required an increased IOP associated with at least one additional obvious sign of glaucoma, we believe that all 10 eyes considered to have aphakic glaucoma truly had this disease. Further prospective data regarding the long-term ocular course of children with thinner or thicker corneas, and comparison among larger numbers of children of various diagnostic groups, may provide additional insight regarding the diagnostic importance of CCT in children.
In the current series, vitreous hemorrhage and RD were strongly associated with persistent fetal vasculature syndrome. The prevalence of RD was similar to that found in other studies.13 To date, there is little data on the prevalence of postoperative hemorrhage after pediatric cataract surgery. Chen and associates reported a very low prevalence of 0.5%.20 However, it remains unclear whether in their study patients with PHPV underwent posterior capsulorrhexis and anterior vitrectomy. The importance of rapid clearing of a dense vitreous hemorrhage in pediatric patients is apparent. To date, there are no standard indications for surgical intervention of nonclearing vitreous hemorrhages in children. Because, in our study, vitreous hemorrhages cleared significantly in each case within three weeks, we recommend conservative management in children for the first postoperative weeks. B-scan ultrasound examinations are indicated to assess the status of retinal attachment.
Preoperative ultrasound may aid in determining the presence of PHPV when ophthalmoscopy is not possible. The identification of PHPV may improve patient counseling, planning of different surgical procedures, and postoperative expectation. Indications for surgical intervention of PHPV have changed over the years as knowledge of the disease and surgical instrumentation have advanced. Our finding of a strong association of vitreous hemorrhage and PFV raises the question of whether children with congenital cataract associated with PFV should undergo intraocular diathermy. It seems plausible that this surgical technique may reduce postoperative bleeding complications. However, further studies are required to determine the true benefit of this approach.
In our study, all children underwent posterior capsulorrhexis and anterior vitrectomy. We observed secondary cataract formation in six eyes (9.2 %). This is consistent with the results of Hosal and Biglan, who have shown an association of posterior capsulorrhexis and anterior vitrectomy with a decreased risk of PCO in children.11 However, there is still no consensus about the management of the posterior capsule during cataract removal in a child.28, 29, 30, 31, 32, 33 According to our results, we would recommend performing primary posterior capsulorrhexis with anterior vitrectomy in young children without primary IOL implantation.
Moreover, we determined young age (< five months) at the time of surgery to be a strong risk factor for the development of PCO. This is also consistent with a study by Hosal and Biglan, who concluded the younger the child at cataract surgery, the greater the risk of secondary membrane formation.11
Although several authors11, 34 suggest that the presence of an IOL increases the risk of secondary membrane formation, there is increasing evidence that a well-placed IOL (e.g., optic capture) can reduce the incidence of secondary cataract in children.31, 32, 35 However, this is still a matter of controversy, and there are few data on children younger than 18 months of age.
Twenty-seven percent of all eyes required additional surgery for the treatment of complications. Compared with that of a previous study by Lundvall and Zetterström published in 1999, this prevalence is lower.8 In their retrospective study, 54% of 83 aphakic eyes required additional surgery, and the most frequent complications were after-cataract glaucoma (38.6%) and early-onset aphakic glaucoma (13%).
The surgical treatment of pediatric cataracts is constantly changing. It may be hypothesized that improved surgical techniques have contributed to a lower incidence of postoperative complications. Further research will be required to investigate the ideal timing in pediatric cataract surgery.
The authors indicate no financial support or financial conflict of interest. Involved in design of study (C.K.-H., L.-O.H.); conduct of study (C.K.-H., M.L., T.K., L.-O.H.); collection of the data (C.K.-H., M.L.); management, analysis, and interpretation of the data (C.K.-H.); preparation of the manuscript (C.K.-H., L.-O.H.); and review and approval of the manuscript (C.K.-H., L.-O.H., M.L., T.K.). The study was approved by the Institutional Review Board of the Johann Wolfgang Goethe-University Hospital, Frankfurt am Main, Germany; this study adheres to the tenets of the Declaration of Helsinki.
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Claudia Kuhli-Hattenbach, MD, graduated from Johann Wolfgang Goethe-University in Frankfurt am Main, Germany and underwent ophthalmology training at the Frankfurt University Eye Hospital. Dr Kuhli-Hattenbach completed her residency and is currently specializing in Pediatric Ophthalmology and Strabismus. Her main research interests include pediatric eye diseases and coagulation diasorders in ocular vascular occlusive diseases.
PII: S0002-9394(08)00150-5
doi:10.1016/j.ajo.2008.02.014
© 2008 Elsevier Inc. All rights reserved.
