The correction of unilateral aphakia in children treated for orbital rhabdomyosarcoma

The survival rate of children with localized orbital rhabdomyosarcoma is now greater than 90% 3 years after diagnosis as a result of advances in radiation and chemotherapy. Ninety percent of these children develop cataracts within 1 to 4 years after the completion of radiotherapy. The correction of aphakia in these children is complicated by the concurrent keratoconjunctivitis and dryness associated with radiotherapy. Three patients with a diagnosis of orbital rhabdomyosarcoma underwent treatment for uniocular cataract. Two of the patients were unable to use extended wear contact lenses. Both underwent epikeratophakia with poor results. One patient who had clinically significant ocular drying prior to cataract extraction underwent successful implantation of an intraocular lens as a primary procedure, with excellent visual results. Clinicians should be aware of the difficulties associated with contact lens wear and epikeratophakia tissue lenses in children who have had high doses of radiation for orbital rhabdomyosarcoma. Such patients are probably best served by primary intraocular lens implantation or by preservation of the posterior capsule at the time of cataract extraction to allow secondary lens implantation if contact lens wear is unsuccessful.     

Traumatic cataract surgery with intraocular lens implantation in children.

We evaluated our experience of intraocular lens implantation in 22 children with uniocular traumatic cataract. Good visual acuity was achieved in a large proportion of the children despite associated corneal opacities and several intraoperative and postoperative complications. Our study suggests that intraocular lens implantation may have a major role in management of monocular cataracts in children.     

Changes in astigmatism after congenital cataract surgery and foldable intraocular lens implantation

PURPOSE: To evaluate the changes in astigmatism after cataract extraction and implantation of a foldable intraocular lens (IOL) in children. Only eyes with astigmatism of 3.0 D or more were included in the study. METHODS: The charts of children who had undergone surgery for nontraumatic cataract using a foldable IOL were retrospectively reviewed. In 13 eyes with astigmatism of 3.0 D or more, the refraction was tested and recorded at 1 week, 3 months, and 5 months postoperatively. A paired t test was used to compare the variables. RESULTS: Mean astigmatism 1 week postoperatively was 4.7 +/- 1.9 D (range, 3.0-10.0 D). Thereafter, the astigmatic component of the refractive error underwent a spontaneous steady decline, reaching a mean value of 0.9 +/- 0.9 D (range, 0-2.25 D) 5 months after surgery. The difference between the mean values at 1 week and 5 months was statistically significant (P < .0001). CONCLUSION: Children who underwent congenital cataract surgery and IOL implantation showed a significant spontaneous reduction in astigmatism postoperatively.     

Intraocular lens implantation in children and youth.

PURPOSE: To evaluate the safety and the effectiveness posterior chamber intraocular lenses (PCIOLs) in children and youth with cataract. METHODS: Retrospective study of operative and postoperative results was based on 146 eyes in 125 children ages 6 to 18 years (mean: 11.7 years) who underwent extracapsular cataract extraction with IOL implantation in the period from 1986 to 1996. Follow up was 1 to 10 years. OUTCOME MEASURE: Best corrected distance and near visual acuity, operative complications, and early and late postoperative complications. Exclusion criteria were uveitis, retinal detachment, prior glaucoma, and systemic diseases. RESULTS: Early postoperative complications were observed in 12.3% of cases including iritis in 7.5%, and transient corneal edema in 3.4% of eyes. Posterior capsule opacification requiring capsulotomy was needed in 81.5% of eyes after 8.5 years. YAG laser capsulotomy was performed in 68.1% and surgical intervention in 26.8% of cases. In 5.1% of the cases, both techniques were used. In 74.7% of patients, best corrected visual acuity of 0.5 or better was achieved. The mean postoperative spherical equivalent was +0.35 diopters (D, SD 1.35). Spectacle-corrected near visual acuity of 0.5 on the Snellen chart was obtained in 82.8% of eyes. CONCLUSION: IOL implantation in children older than 6 years appears to be an efficacious, satisfactory, and safe procedure in the management of pediatric cataract.     

Educational paper

Congenital cataract is the commonest worldwide cause of lifelong visual loss in children. Although congenital cataracts have a diverse aetiology, in many children, a cause is not identified; however, autosomal dominant inheritance is commonly seen. Early diagnosis either on the post-natal ward or in the community is important because appropriate intervention can result in good levels of visual function. However, visual outcome is largely dependent on the timing of surgery when dense cataracts are present. Good outcomes have been reported in children undergoing surgery before 6 weeks of age in children with unilateral cataract and before 10 weeks of age in bilateral cases. Placement of an artificial intraocular lens implant after removal of the cataract has become established practice in children over 2 years of age. There remains debate over the safety and predictability of intraocular lens implantation in infants. Despite early surgery and aggressive optical rehabilitation, children may still develop deprivation amblyopia, nystagmus, strabismus, and glaucoma. The diagnosis and management of congenital cataracts has improved substantially over the past 30 years with a concurrent improvement in outcomes for affected children. Many aspects of the pre-, intra-, and postoperative management of these patients continue to be refined, highlighting the need for good quality data and prospective collaborative studies in this field.     

Long-term rates of PCO following small incision foldable acrylic intraocular lens implantation in children

PURPOSE: To evaluate the long-term incidence of postoperative posterior capsular opacification (PCO) in children undergoing small incision foldable acrylic lens implantation with at least 2 years of follow up. METHODS: In 18 children, 26 eyes underwent small incision cataract extraction with posterior chamber foldable acrylic lens implantation. The posterior capsule was left intact in all patients at the time of surgery. RESULTS: With a mean follow up of 2.75 years and a mean age at surgery of 8.25 years, 13 of 26 eyes (50%) developed visually significant PCO requiring intervention. In the group of children under 4 years of age, 5 of 5 eyes (100%) developed visually significant PCO, while 8 of 21 eyes (38%) in the group of children over 4 years of age developed opacification. Four of 26 eyes (15%) required two procedures (either repeat Nd:YAG laser capsulotomy or pars plana secondary membrane removal) to clear the visual axis. CONCLUSION: In this study, the incidence of PCO following small incision acrylic lens implantation in children over 4 years of age is lower than those rates reported by conventional large incision rigid lens techniques with a minimum of 2 years follow up. This technique has advantages over conventional techniques in older children because it offers less surgical intervention, a lower cost to patients, and less risk of vitreous and retinal complications.     

Sensory deviations subsequent to senile cataract

PURPOSE: To evaluate the effect of extracapsular cataract extraction with posterior chamber intraocular lens (IOL) implantation in patients with sensory deviations subsequent to senile cataract. METHODS: Twenty patients with dense cataract and associated sensory deviations underwent follow-up between April 1996 and April 1998 after extracapsular cataract extraction with posterior chamber IOL implantation in the deviating eye. All patients underwent follow-up for a minimum period of 6 months. RESULTS: Preoperatively, all patients had a visual acuity of PL positive (perception of light present) and PR (projection of rays) accurate in the deviating eye. Two patients had an esodeviation, and 18 patients had an exodeviation. Postoperatively, all patients had a corrected visual acuity of 20/40 or better in the operated eye at 12 weeks. Nineteen patients had ocular alignment within +/-8 prism diopters of orthophoria at 12 weeks. CONCLUSION: Sensory deviations subsequent to senile cataract usually resolve spontaneously after cataract surgery if visual gains are 20/40 or better. The prognosis for binocular vision is good.     

Retrospective comparison of surgical techniques to prevent secondary opacification in pediatric cataracts

PURPOSE: To evaluate the effect of different surgical methods for management of the posterior capsule and anterior vitreous on the rate of posterior capsule opacification in pediatric cataracts. METHODS: Charts of 34 children (47 eyes) aged 40 days to 18 years (mean: 8.5 years) who had primary cataract surgery with or without posterior chamber intraocular lens (IOL) implantation during the past 5 years were reviewed. In 26 eyes, cataracts were managed with a posterior continuous curvilinear capsulorhexis, and in 21 eyes, the posterior capsule was left intact. Follow-up averaged 10 months (range: 6.5 months to 5 years). RESULTS: Visually significant secondary cataract developed in nine eyes with intact posterior capsules, and seven eyes required Nd:YAG laser posterior capsulotomy. The average time for YAG capsulotomy postcataract removal in the second group was 4 months. The visual axis remained clear in all eyes that had posterior continuous curvilinear capsulorhexis with or without posterior chamber IOL. Complications such as fibrinoid membrane, stromal edema, posterior synechiae, updrawn pupil, and transient glaucoma occurred in both groups at a similar rate. CONCLUSION: Primary posterior continuous curvilinear capsulorhexis is an effective method for preventing secondary cataract formation in pediatric cataracts.     

Pregnancy-exaggerated galactosemia and congenital cataracts

One child In a family and two children in another family had galactosemia and congenital cataract. Two of them had total soft cataracts while in one, cataract was less soft. In addition, they had mild lactosuria. The mothers of the affected children had significant lactosuria and mild galactosuria without cataracts. Fathers did not have galactosuria or lactosuria. Clinically unaffected siblings in one family had mild galactosuria and lactosuria. Pregnancy-exaggerated galactosemia was suspected in these two mothers who gave birth to children with congenital cataract. As an extension of this work, 5001 pregnant women were screened for galactose in urine just before the delivery of babies. Mild galactosuria was present in 54 (1.08%). Three children had congenital cataract and one had changes in posterior pole and cornea. Restriction of lactose by reducing intake of milk and milk products during pregnancy by mothers with galactosuria is recommended to avoid the birth of children with congenital cataract.     

Cataract and ocular hypertension in children on inhaled corticosteroid therapy

PURPOSE: To ascertain the incidence of posterior subcapsular cataract and ocular hypertension in a cohort of children < or = 12 years on inhaled steroid therapy. PATIENTS AND METHODS: In this prospective study, a detailed history regarding corticosteroid therapy was obtained for children attending an asthma clinic. The presence and type of lens changes (cataract) was recorded and intraocular pressure (IOP) was measured. The children underwent another eye examination 2 years later. RESULTS: Ninety-five patients were enrolled in the study. Mean patient age was 7 +/- 3 years, and mean duration of inhaled steroid therapy was 2 +/- 1 years. Thirty-six percent of patients received inhaled steroids exclusively, 61% received inhaled steroids with a short course of oral steroids, and 3% received inhaled steroids with a long course of oral steroids. Only 3 (3%) patients had cortical changes that were not visually significant, and none had posterior subcapsular or nuclear cataract. There was no significant differences between children with cataract and those without cataract with respect to age; duration of asthma; and duration, average daily dose, and cumulative dose of inhaled steroids. IOP ranged from 11 to 20 mm Hg (mean, 16 +/- 3 mm Hg). None of the children had ocular hypertension or glaucoma. Ninety patients underwent eye examination 2 years later; none was found to develop posterior subcapsular cataract or increased IOP. CONCLUSION: This study indicates the use of inhaled steroids in children with asthma is probably safe as far as not inducing posterior subcapsular cataract or ocular hypertension.     

The use of two-stage Molteno implants in developmental glaucoma

Developmental glaucoma, which cannot be controlled by conventional techniques, poses a difficult problem. Twenty-three eyes of 18 patients with developmental glaucoma underwent a two-stage implantation of the double template Molteno valve with follow-up between 12 and 84 months. The final pressure with medication was less than 21 mm Hg in 78% of the cases. These results were obtained in four out of four eyes with primary congenital glaucoma, six out of eight eyes with secondary congenital glaucoma, and eight of 11 eyes in which glaucoma followed surgery for congenital cataract. We feel that two-stage implantation of Molteno valves has a place in the management of difficult developmental glaucomas and is a reasonable early option when glaucoma occurs following surgery for congenital cataract.     

Surgical technique, visual outcome, and complications of pediatric intraocular lens implantation.

PURPOSE: To evaluate retrospectively the surgical technique, visual outcome, and complications of pediatric cataract extraction (CE) and intraocular lens (IOL) implantation. METHODS: Forty-three patients ages 2 to 12 underwent CE with IOL implantation with a minimum follow up of 1 month. RESULTS: All IOLs were implanted in the posterior chamber with 17 (40%) in the bag, 25 (58%) sulcus fixated, and one (2%) partially in the bag (one haptic in the bag, one in the sulcus). Primary posterior capsulectomy was performed in 12 (28%) cases. A final visual acuity of at least 20/40 was achieved in 26 (60%) and at least 20/80 in 32 (74%). Posterior capsule opacification developed in 18 (42%) and pupillary capture in 7 (16%). Seventeen (40%) patients had postoperative visual acuity worse than 20/40. Of these, nine (53%) had this visual outcome as a result of presumed amblyopia. CONCLUSIONS: Posterior chamber IOL implantation affords a safe and effective method of visual rehabilitation for cataractous children 2 years of age and older. Amblyopia and antecedent posterior segment trauma, rather than IOL-related or surgical complications, are the limiting factors in final visual outcome.     

Update on the surgical management of pediatric cataracts

The treatment of pediatric cataracts is rapidly evolving, and the visual prognosis for infants and children with cataracts is improving, due in part to earlier surgery, increased frequency of intraocular lens (IOL) implantation, more effective amblyopia therapy following surgery, and better forms of optical correction. A surgeon treating a child with a cataract is faced with An assortment of therapeutic dilemmas such as whether an IOL should be implanted, whether foldable lenses are appropriate, and whether the posterior capsule should be opened during surgery. Although the surgery can be technically difficult in small and unforgiving eyes, it is often the easy part as treatment decisions and results have lifelong implications. The visual system, which is immature at birth, has a latent period of approximately 6 weeks before it becomes sensitive to deprivation, and binocular vision first appears at approximately 3 months of age. It is therefore imperative that infants with visually significant congenital cataracts receive prompt treatment during the sensitive periods to decrease the risk of developing amblyopia or binocular abnormalities. An ideal time for early surgical intervention resulting in few complications and maximal visual outcome has not been identified with certainty. Perhaps the greatest overall change in the approach to the surgical treatment of pediatric cataracts has been the adaptation of techniques used in adult cataract surgery. This article focuses on the methods currently used in the management of pediatric cataracts.     

Cataract surgery in children with uveitis

PURPOSE: To report the technique and postoperative results of cataract surgery in children with uveitis. METHOD: Between 1988 and 1998, nine children (age range: 2.5-11 years) who developed secondary uveitic cataract and underwent cataract extraction were studied retrospectively. Seven children had juvenile rheumatoid arthritis and two had chronic anterior uveitis of unknown etiology. The surgical technique was lensectomy and wide anterior vitrectomy with limbal approach, lysis of anterior synechiae and in some cases, peripheral iridectomy. Postoperative aphakia was corrected with soft contact lenses in all patients. Follow-up ranged from 6 months to 6 years. RESULTS: Postoperatively, visual acuity in all patients improved and final visual acuity ranged from 20/70 to 20/25. Significant intraoperative complications did not occcur in any patient. One boy with juvenile rheumatoid arthritis developed cystoid macular edema 1 month postoperatively, which was successfully managed. He also developed hypertonia 1 year later, which was also successfully managed. Seven of the nine children had fewer and milder relapses of uveitis after surgery. CONCLUSION: Cataract surgery, using the lensectomy-vitrectomy technique in children with uveitis, is a safe technique with a relatively small percentage of postoperative complications and good functional results.     

Cataracts after autologous bone marrow transplantation in children

We recorded the incidence and degree of posterior subcapsular cataract (PSC) in 29 children who had undergone autologous (n = 28) or syngeneic (n = 1) bone marrow transplantation (BMT) due to haematologic or lymphoid malignancy. Conditioning prior to transplantation consisted either of a combination of chemotherapy and total body irradiation (TBI) (n = 21) or of chemotherapy only (n = 8). TBI was given in one fraction of 7.5 Gy. Nine patients had received previous cranial irradiation. The patients were followed for 4-10y (median 8 y) after transplantation. Of 29 patients, 22 developed PSC, all within 4 y after BMT. With the exception of one patient who developed unilateral PSC, all had received TBI. Conversely, 100% of those who received TBI developed PSC. In this group (+TBI), eight patients (38%) developed significant PSC, defined as best corrected visual acuity <0.8 in either eye. Six patients (10 eyes) have since needed surgical repair consisting of extracapsular cataract extraction and intraocular lens implantation. There was no clear relationship between previous cranial irradiation and cataract development, nor any other obvious baseline differences between those in the +TBI group who developed significant PSC and those who did not. Although effects of previous therapy cannot be ruled out, TBI appears to be the main cause of PSC in this group of patients. Twelve patients in the +TBI group had well-preserved visual acuity throughout the study, reflecting a slow progression of PSC. This compares favourably with previous reports of allogeneic BMT, possibly owing to less need for corticosteroids after autologous BMT. We conclude that the incidence of PSC was high after autologous BMT where the conditioning regimen included total body irradiation.     

Clinical symptoms and visual outcome in patients with presumed congenital cataract

PURPOSE: To investigate clinical symptoms and visual outcome in patients who had presumed congenital cataract with visual acuity > or =20/200. METHODS: Twenty-nine patients (50 eyes) ranging in age from 5-28 years were included in this study. Congenital cataract was diagnosed by patients' past history, previous medical records, and typical findings of congenital cataract. Inclusion criteria were patients with visual acuity measured by Snellen chart, best corrected visual acuity > or =20/200, and no eye or systemic diseases influencing visual acuity. Seven patients had unilateral cataracts and 22 patients had bilateral cataracts. Cataracts were nuclear in 29 eyes, anterior or posterior subcapsular in 12 eyes, and polar in 7 eyes. All patients underwent lens aspiration or phacoemulsification combined with posterior chamber intraocular lens (PC-IOL) implantation without performing intentional posterior capsulotomy and anterior vitrectomy. RESULTS: The most common clinical symptom was visual disturbance followed by blurred vision, glare and knitting the brow, strabismus, and leukocoria. Comparison of calculated PC-IOL power showed a more significant myopic shift in unilateral cataract than in bilateral cataract. Postoperative best corrected visual acuity >20/25 was attained in 28 (65.1%) eyes with bilateral cataracts and 1 (14.3%) eye with a unilateral cataract. CONCLUSIONS: Cataract surgery is effective not only in improving visual acuity but also in eliminating clinical symptoms. Delaying cataract surgery in early infancy should be considered to avoid intentional operative procedures in patients with bilateral congenital cataract preserving visual acuity >20/200.     

原发性肾病综合征患儿应用糖皮质激素致眼部不良反应的防治

原发性肾病综合征是儿童常见的肾小球疾病之一,糖皮质激素是治疗肾病综合征的首选药物,且使用的周期较长。长期使用糖皮质激素可出现库欣貌、高血压、免疫功能降低、生长发育迟缓、眼部不良反应等,其中糖皮质激素眼部不良反应以青光眼和白内障较为常见。糖皮质激素性青光眼和白内障常常发生隐蔽,患儿往往不能准确地表达其不适,同时部分临床医...     

Onset of cataract in early infancy associated with a 32G-->C transition in the iron responsive element of L-ferritin

We describe the onset of cataract in early infancy in a family with hereditary hyperferritinaemia-cataract syndrome. The two probands presented with isolated hyperferritinaemia and had developed cataracts at the age of 18 months. Two members of their family with high ferritin levels (1270-1450 microg/l) had suffered from cataract since childhood. The mutation responsible was a 32G-->C change in the lateral bulge of the stem structure of the iron responsive element of the L-ferritin subunit gene. Mutations at this level cause particularly high ferritin levels, whereas the age of cataract onset and its severity are controversial subjects. In our family, early ophthalmic examination ruled out the possibility that cataract was due to age-related persistence of high ferritin levels in the lens and suggested that other factors may modulate the phenotype. CONCLUSION: cataract may appear early in hereditary hyperferritinaemia-cataract syndrome and this syndrome should be suspected and ferritin levels measured in all cases of cataract in children, even when the onset is in early infancy.     

Corneal keloid in Lowe syndrome

We describe a 3-year-old boy with Lowe syndrome who previously underwent bilateral cataract surgery with intraocular lens implantation and strabismus surgery and developed an inferior corneal keloid. The lesion was resected. We report the results of immunohistochemistry analysis of the specimen, as well as alternative treatment modalities for this rare condition.     

Cataract in children

Of the estimated 1.4 million children world-wide who are blind, cataract is responsible for an estimated 190 000 (14%). The incidence varies from 1 to 3/10 000 live births or 10 per million of the total population in low-income countries. Early diagnosis, referral and surgery are important in improving results. A recent study from Sweden indicates that examination of babies in maternity wards results in earlier referral compared with well-baby clinics or no formal screening.

Conclusion: It is important that standardized protocols be developed and implemented to screen children for ocular anomalies, especially cataract, before discharge from maternity units.