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Two children with bilateral congenital cataracts were operated for cataract surgery of one eye each. The first was a 6 months old (preterm delivery at 7 months gestation) with no other apparent ocular pathology ([figure 1A–C][1]); the second was a 10 months old case of Peter’s anomaly with

To assess the intraocular pressure and visual acuity before and after pediatric congenital cataract surgery performed at a relatively older age. A retrospective analysis of all consecutive pediatric patients diagnosed and operated for bilateral congenital cataracts during a seven-year period (2012–2018) in rural southern Ethiopia. Non-ambulatory vision was defined as hand motion or worse. The main outcome measures were intraocular pressure (IOP) and visual acuity. Thirty-two children were included, 17 females (53.1%), with a mean age of 11 years (± 2.83) [range, 7–18]. A total of 59 eyes were operated on. The mean follow-up was 4.8 ± 1.8 years (range, 2–8). VA improved from 20/1400 preoperatively to 20/440 postoperatively OD and 20/540 OS (p 21 mmHg) preoperatively, of which three remained high after surgery. Positive correlations were found between IOP, axial length and cup-to-disc ratio. None of the patients required pressure-lowering procedures. Bilateral congenital cataracts may be associated with ocular hypertension, with possible progression to glaucoma if left untreated. Surgery at a relatively older age often was associated with a significant improvement in intraocular pressure and ambulatory vision.

PURPOSE To evaluate changes in central corneal thickness (CCT) and intraocular pressure (IOP) in children after surgery for congenital cataracts and to investigate the association between CCT and IOP. METHODS For this prospective observational cohort study, we recruited patients undergoing surgery for unilateral or bilateral congenital cataracts. CCT and IOP were measured before surgery and 1, 3, 6, 12, and 24 months after surgery. RESULTS Seventy-six children (152 eyes) were enrolled; 33 eyes were unaffected by cataracts, 77 were aphakic, and 42 were pseudophakic. In aphakic eyes, mean CCT increased by 31.14 ± 44.32 μm at 12 months postoperation and 33.09 ± 35.42 μm at 24 months postoperation; this increase was significantly higher than that in pseudophakic eyes 12 months after surgery (8.36 ± 19.91 μm; P < 0.001) and 24 months after surgery (0.31 ± 14.19 μm; P = 0.024). However, no significant differences in IOP were found between the different phakic states at 12 and 24 months postoperation (P = 0.672 and P = 0.080, respectively). There were also no significant differences in CCT and IOP before and after surgery in the unaffected eyes. CONCLUSIONS Mean CCT peaked at 12 months, and the mean IOP remained normal in both the aphakic and pseudophakic eyes during this study. CCT and IOP were positively correlated, regardless of the phakic status or age, a relationship which suggests that both parameters should be monitored closely in postsurgical patients for up to 12 months and in this time, may impact the ability to diagnose glaucoma.

Patients treated for bilateral congenital cataracts provide a unique model to test the role of early visual input in shaping the development of the human cortex. Previous studies showed that brief early visual deprivation triggers long-lasting changes in the human visual cortex. However, it remains unknown if such changes interact with the development of other parts of the cortex. With high-resolution structural and resting-state fMRI images, we found changes in cortical thickness within, but not limited to, the visual cortex in adult patients, who experienced transient visual deprivation early in life as a result of congenital cataracts. Importantly, the covariation of cortical thickness across regions was also altered in the patients. The areas with altered cortical thickness in patients also showed differences in functional connectivity between patients and normally sighted controls. Together, the current findings suggest an impact of early visual deprivation on the interactive development of the human cortex.

AIM: To investigate the behavioral and psychological disorders and the prevalence of parent ratings of attention deficit hyperactivity disorder (ADHD) symptoms among children with bilateral congenital cataracts (CCs). METHODS: This cross-sectional study investigated children with bilateral CC aged 3-8y (CC group) using Conners' Parent Rating Scale-48 (CPRS-48) from July to December 2016. The abnormal rates of psychological symptoms in CC children and normal vision (NV) children were compared using the Chi-square test. The scores of CC children were compared with those of NV children and the Chinese urban norm using the independent samples t-test and one-sample t-test, respectively. RESULTS: A total of 262 valid questionnaires were collected. The ratio of CC children to NV children was 119:143. The overall rate of psychological symptoms in CC children was 2.28 times higher than that in NV children (46.22% vs 20.28%, Pearson's χ(2)=20.062; P

Visual deprivation in childhood can lead to lifelong impairments in multisensory processing. Here, the Size-Weight Illusion (SWI) was used to test whether visuo-haptic integration recovers after early visual deprivation. Normally sighted individuals perceive larger objects to be lighter than smaller objects of the same weight. In Experiment 1, individuals treated for dense bilateral congenital cataracts (who had no patterned visual experience at birth), individuals treated for developmental cataracts (who had patterned visual experience at birth, but were visually impaired), congenitally blind individuals and normally sighted individuals had to rate the weight of manually explored cubes that differed in size (Small, Medium, Large) across two possible weights (350 g, 700 g). In Experiment 2, individuals treated for dense bilateral congenital cataracts were compared to sighted individuals in a similar task using a string set-up, which removed haptic size cues. In both experiments, indistinguishable SWI effects were observed across all groups. These results provide evidence that early aberrant vision does not interfere with the development of the SWI, and suggest a recovery of the integration of size and weight cues provided by the visual and haptic modality.

WOS:000640755100001 PMID: 33864186 Purpose To detect crystallin gene mutations in Turkish children with congenital cataracts. Methods The present study included 56 children (38 males and 18 females) who were diagnosed with congenital cataract in our ophthalmology clinic. The patients' blood samples were collected and sent to the medical genetics laboratory. The samples were assessed using the sequence analysis method, which covered all exons of CRYAA, CRYAB, CRYBB1, CRYBB2, CRYBB3, CRYGC and CRYGD. Results In total, 56 patients with congenital cataracts were included in the present study. Of these, 68% were male and 32% were female. The age range of the patients was 2 months to 5 years. The mean age of onset was 21.08 +/- 15.15 months. All the patients had bilateral congenital cataracts. The female-to-male ratio was 1:2.1. Mutation analysis was performed to detect possible mutations in CRYAA, CRYAB, CRYBB1, CRYBB2, CRYBB3, CRYGC and CRYGD. Of the four mutations detected, one was novel (c.383A > T in CRYGD) and three were known (c.592C > T in CRYBB2, c.164A > G in CRYGC and c.592C > T in CRYBB2). Two of these three mutations were detected in the same gene (CRYBB2). Crystallin gene mutations were detected in 7% of patients with congenital cataracts (four out of 56 patients) in the present study. Conclusions We think that mutations in crystallin genes are responsible for 7% of congenital cataract cases in our country. The detection of these mutations may help in the molecular diagnosis of congenital cataracts.

Congenital cataract is the leading cause of preventable childhood blindness. The purpose of this review is to discuss a systematic approach to the evaluation of bilateral congenital cataracts, both hereditary and non-hereditary. Children with bilateral cataracts require a detailed history and ophthalmic and systemic examination to guide further genetic and systemic evaluation. It is important to communicate with the pediatrician as well during this process. With advances in genetic testing, it is possible to identify causes for sporadic cataracts, as well as assess phenotype-genotype correlation of cataracts.

A term, growth-restricted newborn presented with a sepsis-like picture, persistent pulmonary hypertension and bilateral cataracts. Initial review of prenatal and family history, as well as microbiological investigations were noncontributory. Following lenticular extraction, viral examination of the lenses confirmed the presence of rubella. Congenital rubella syndrome (CRS) presents with a constellation of signs and symptoms that overlap with many other conditions. The presence of bilateral cataracts in a newborn is a rare finding and this case is used to review the broad etiology of congenital cataracts. We propose a structured diagnostic approach for clinicians, remembering that CRS is a rare but possible etiology. The early diagnosis of CRS in this case, allowed us to initiate appropriate management and preventive measurements.

Nance-Horan syndrome is a rare X-linked recessive inherited disease with clinical features including severe bilateral congenital cataracts, characteristic facial and dental abnormalities. Data from Chinese Nance-Horan syndrome patients are limited. We assessed the clinical manifestations of a Chinese Nance-Horan syndrome pedigree and identified the genetic defect. Genetic analysis showed that 3 affected males carried a novel small deletion in NHS gene, c.263_266delCGTC (p.Ala89TrpfsTer106), and 2 female carriers were heterozygous for the same variant. All 3 affected males presented with typical Nance-Horan syndrome features. One female carrier displayed lens opacities centered on the posterior Y-suture in both eyes, as well as mild dental abnormalities. We recorded the clinical features of a Chinese Nance-Horan syndrome family and broadened the spectrum of mutations in the NHS gene.

AIM: To evaluate the prevalence, clinical features, and the factors affecting onset of strabismus and nystagmus in patients with bilateral congenital cataracts. METHODS: This study evaluated 116 eyes of 58 patients who underwent lens removal for the treatment of bilateral congenital cataracts between January 1999 and January 2011. The presence and type of strabismus and nystagmus were determined before and after surgery. Type of strabismus and final visual acuity were compared in patients with and without nystagmus. Patients were divided into three groups (orthotropia/orthotropia, orthotropia/strabismus, and strabismus/strabismus) according to their preoperative and postoperative ocular alignment. Age at cataract surgery and associations of nystagmus and primary intraocular lens (IOL) implantation with strabismus were analyzed. RESULTS: Six patients (10.3%) had strabismus preoperatively and an additional 11 (19.0%) developed postoperative strabismus. Exotropia was more common than esotropia both preoperatively and postoperatively. Eighteen patients (31.0%) had postoperative nystagmus, with sensory nystagmus being the most common type. Of the 18 patients with nystagmus, 10 had strabismus, with exotropia being more common than esotropia. Postoperative visual acuity was poor in patients with nystagmus. Age at cataract surgery and rate of primary IOL implantation were significantly lower, and postoperative nystagmus was more common, in the orthotropia/strabismus group than in the other two groups. CONCLUSION: Exotropia and sensory nystagmus are common in patients with bilateral congenital cataracts. Age at cataract surgery and rate of IOL implantation are lower and nystagmus more common in patients with postoperative onset of strabismus. Nystagmus is associated with poor visual prognosis.

Describe an unusual case of bilateral pigment dispersion syndrome (PDS) following years of uninterrupted treatment with atropine 1% for bilateral congenital cataracts, speculate on potential mechanisms leading to this condition.This is a case report.A 45-year-old white patient on long-term treatment with atropine 1% ointment since his infancy for bilateral congenital cataracts developed PDS with secondary ocular hypertension.The patient showed all the hallmarks of PDS with secondary ocular hypertension. An anterior segment Swept-Source optical coherence tomography was obtained to review the iris profile. The patient showed good pressure response to topical prostaglandin therapy.This is the second case report of PDS in a patient with chronic use of topical atropine. The proposed mechanisms for pigment dispersion are discussed and the possibility raised of dispersion being a potential side effect of the drug.

Warburg syndrome is a rare disorder characterized by microcephaly, microcornea, congenital cataract, developmental delay, and hypogonadism. Here, we report two siblings from India who presented with developmental delay, microcornea, microphthalmia, and bilateral congenital cataracts, born to the third-degree consanguineously married couple. Both children had hypoplasia of corpus callosum. In this report, we aim to highlight and compare clinical features of these two cases with previously reported cases.

Patients treated for bilateral congenital cataract are later impaired on several hallmarks of adults' expertise with upright faces but report no problem with remembering faces. Here, we provide the first formal data on their face memory. We compared 12 adults with a history of visual deprivation from bilateral congenital cataracts to 24 age-matched controls with normal vision on their ability to recognize famous and recently learned faces, and on their subjective impression of their face memory. Bilateral congenital cataract patients demonstrated a prosopagnosic-like deficit, being slower and less accurate in recognizing both famous faces and recently learned faces, despite not differing on most questions about their impression of their face memory. Patients' results on three perceptual tasks (the composite face effect, the Benton test of recognizing faces through a change in point of view, and the Jane test of sensitivity to feature spacing) were also not correlated with their face memory deficits. These results suggest that early visual input is necessary not only for perceptual expertise in differentiating among unfamiliar upright faces, but also for normal accuracy in remembering the identity of individual faces. © 2012 Wiley Periodicals, Inc. Dev Psychobiol 56: 96-108, 2014.

People treated for bilateral congenital cataracts offer a model to study the influence of visual deprivation in early infancy on visual and multisensory development. We investigated cross-modal integration capabilities in cataract patients using a simple detection task that provided redundant information to two different senses. In both patients and controls, redundancy gains were consistent with coactivation models, indicating an integrated processing of modality-specific information. This finding is in contrast with recent studies showing impaired higher-level multisensory interactions in cataract patients. The present results suggest that basic cross-modal integrative processes for simple short stimuli do not depend on visual and/or crossmodal input since birth.

Amblyopia is a condition involving reduced acuity caused by abnormal visual input during a critical period beginning shortly after birth. Amblyopia is typically considered to be irreversible during adulthood. Here we provide the first demonstration that video game training can improve at least some aspects of the vision of adults with bilateral deprivation amblyopia caused by a history of bilateral congenital cataracts. Specifically, after 40 h of training over one month with an action video game, most patients showed improvement in one or both eyes on a wide variety of tasks including acuity, spatial contrast sensitivity, and sensitivity to global motion. As well, there was evidence of improvement in at least some patients for temporal contrast sensitivity, single letter acuity, crowding, and feature spacing in faces, but not for useful field of view. The results indicate that, long after the end of the critical period for damage, there is enough residual plasticity in the adult visual system to effect improvements, even in cases of deep amblyopia caused by early bilateral deprivation.

Background Many cases of unilateral congenital cataract have an associated posterior capsular plaque. Observations such as this have led to the hypothesis that posterior capsular plaque is caused by persistent fetal vasculature, generally a unilateral condition, even if no obvious hyaloid remnant is visible. We investigated the incidence of posterior capsular plaque associated with bilateral congenital cataracts. Methods Children with bilateral congenital cataracts were identified from three surgical practices. Children were included if lensectomies were performed prior to age 7 months. Surgical videos were evaluated for cataract morphology. Results Fourteen children with bilateral cataracts were identified. Surgical videos were available for 24 eyes. Five eyes (21%) had evidence of persistent hyaloid remnants. Of the remaining 19 eyes, cataracts were categorized as nuclear in 10 cases, nuclear with cortical extension in 6 cases, and posterior cortical in 3 cases. Evidence of posterior capsular plaque was seen in seven cases (37%) on video review. Conclusions Video review revealed a much lower incidence of posterior capsular plaque in eyes with bilateral congenital cataracts compared to unilateral congenital cataracts, lending presumptive evidence to the hypothesis that most unilateral lens opacities arise from persistent fetal vasculature.

We used opposing figural aftereffects to investigate whether there are at least partially separable representations of upright and inverted faces in patients who missed early visual experience because of bilateral congenital cataracts (mean age at test 19.5 years). Visually normal adults and 10-year-olds were tested for comparison. Adults showed the expected opposing aftereffects for upright and inverted faces. Ten-year-olds showed an adultlike aftereffect for upright faces but, unlike the adult group, no aftereffect for inverted faces. Patients failed to show an aftereffect for either upright or inverted faces. Overall, the results suggest that early visual input is necessary for the later development of (at least partially) separable representations of upright and inverted faces, a developmental process that takes many years to reach an adult-like refinement.

Early visual deprivation caused by bilateral congenital cataracts produces deficits in discriminating faces that differ in the spacing of features, but not in feature shape (Le Grand et al. (2001) Nature 410: 810). We investigated whether these deficits are specific to human faces by testing patients' ability to discriminate between stimuli differing only in feature spacing in human and monkey faces (Experiment 1) and in houses (Experiment 2). Patients, as a group, showed deficits on only one task: they had lower accuracy than normal in discriminating feature spacing in human faces. In contrast, they were normal in discriminating feature spacing in monkey faces and in houses. The results suggest that early visual experience is necessary to set up (or preserve) the neural architecture used for processing human faces, but not for processing objects in general. ! 2010 Wiley Periodicals, Inc. Dev Psychobiol 52: 775-781, 2010.

PURPOSE The investigation of patients treated for bilateral congenital cataracts allows to study the development of visual and multisensory functions after a period of visual deprivation in early infancy. In the present study, cataract patients were tested for their capability to recognize faces and to integrate auditory and visual speech information. METHODS In Experiment 1, 12 cataract patients were tested with the Benton Facial Recognition Test. In Experiment 2, a McGurk paradigm was used that investigated audio-visual interaction and lip-reading capabilities. Here, fifteen cataract patients participated and were compared to normally sighted controls and to visually impaired controls. RESULTS In the Benton Facial Recognition Test, cataract patients' performance was unimpaired when target and test face were identical. By contrast, they performed worse than a normally sighted control group when head orientation and/or lighting conditions of the test faces were changed. In the McGurk paradigm, cataract patients displayed impaired lip-reading abilities and a reduced audio-visual interaction compared to normally sighted controls. The latter deficit prevailed even in a sub-group matched for lip-reading capacities with a normally sighted control sub-group. CONCLUSION These results suggest that visual input in early infancy is a prerequisite for a normal development of visual and multisensory functions.

Higher visual functions were investigated in patients treated for bilateral congenital cataracts in two experiments. Participants were asked to detect either real or illusory contours (Kanizsa squares in Experiment 1 or one of four different Kanizsa contours in Experiment 2) among distractor items. Compared to normally sighted participants matched for age, gender and education, cataract patients treated after the age of 5–6 months took relatively longer to detect Kanizsa figures (Experiments 1 and 2) and they had higher miss rates (Experiment 2). The present results suggest that the ability of visual feature binding depends on early visual input and is permanently impaired if patterned vision is prevented in early infancy for 5 months or more.

We evaluated the importance of early visual input for the later development of expertise in face processing by studying 17 patients, aged 10 to 38 years, treated for bilateral congenital cataracts that deprived them of patterned visual input for the first 7 weeks or more after birth. We administered five computerized tasks that required matching faces on the basis of identity (with changed facial expression or head orientation), facial expression, gaze direction and lip reading. Compared to an age-matched control group, patients’ recognition of facial identity was impaired significantly when there was a change in head orientation (e.g. from frontal to tilted up), and tended to be impaired when there was a change in facial expression (e.g. from happy to surprised). Patients performed normally when matching facial expression and direction of gaze (e.g. looking left or right), and in reading lips (e.g. pronouncing ‘u’ or ‘a’). The results indicate that visual input during early infancy is necessary for the normal development of some aspects of face processing, and are consistent with theories postulating the importance of early visual experience (de Schonen & Mathivet, 1989; Johnson & Morton, 1991) and separate neural mediation of different components of face processing (Bruce & Young, 1986).

We report an unusual intraoperative finding in the case of a 4-month-old infant with bilateral congenital cataracts removed within a 1-week period. Surgery in the right eye was uneventful. During removal of the cataract in the left eye, signs of the intraoperative floppy-iris syndrome (IFIS) were observed; ie, iris floppiness, iris prolapse to the incisions, and progressive miosis. The surgical technique was identical in both eyes except that epinephrine was added to the irrigating solution in the right eye but inadvertently omitted in the left-eye surgery. Use of intracameral epinephrine has been documented to prevent IFIS in adult eyes at risk for developing the syndrome. Our case highlights the importance of epinephrine in the irrigating solution in pediatric cataract surgery.

Purpose: To present three cases having bilateral congenital posterior capsular defects accompanying bilateral congenital cataracts. Cases: Similar to the previous reports there were characteristic demarcation of thickened margins on the posterior capsule defects and white dots on the anterior vitreous face in all of our cases. In addition to previous reports, we detected a semi-transparent membrane at the location of the posterior capsule defect bilaterally in all of our cases. Observations: This membrane was loosely attached to the borders of the posterior capsular opening and we removed it with vitreus cutter in two cases and with forceps in the other. In two cases the membranes covered the entire posterior capsular defect area; but in one case the membrane covered only the half of the defect. The cases were managed by standard irrigation - aspiration and anterior vitrectomy. Conclusion: Ophthalmologists should be aware that in some congenital cataracts, they may notice congenital posterior capsular defects with accompanying membranes.

Congenital Cataracts with Facial Dysmorphisms and Neuropathy (CCFDN) is a complex autosomal recessive disorder characterized by bilateral congenital cataracts, developmental delay, peripheral; hypo-demyelinating neuropathy, mild facial dysmorphisms, and other rare signs. Cerebral and spinal cord atrophy is the main neuroimaging finding but other less common abnormalities have been previously described. We describe progressive focal lesions of supratentorial white matter in a 10-year-old boy affected by CCFDN. Other etiologies have been excluded and these lesions can be considered a new finding of the disease. We discuss a possible demyelinating mechanism affecting both peripheral and central myelin.

Purpose To determine optimal timing for operating on dense bilateral congenital cataracts and to define latent periods for binocular visual deprivation. Methods A retrospective review of the records of children that had undergone bilateral lensectomies at our center. Infants with bilateral, dense, visually significant cataracts who had undergone lensectomy within the first year of life from 1992 to 2000 were identified. Children with other ocular anomalies, neurological and systemic disorders, intraocular lenses, or with fewer than 5 years of follow-up were excluded. Results A total of 13 children were identified. The mean age at surgery was 8.7 weeks (range, 3–20; SD 5.3). The mean interval between surgeries of the 2 eyes was 3.8 days (range 0-7; SD 3.2). The median final visual acuity at 5 years of age was 6/18 (range, 6/5–6/36). There was a moderate correlation between (log) visual outcome and time to surgery ( r = −0.59, p = 0.002, r 2 = 0.35). Conclusions Visual acuity after surgery for bilateral congenital cataracts appears to decline exponentially with duration of visual deprivation.

To estimate the risk of aphakic glaucoma after lensectomy for congenital cataract and its association with surgery within the first month of life.A retrospective case notes review was conducted of all patients who had lensectomy for congenital cataract during their first year of life at Great Ormond Street Hospital between 1994 and 1997. Patients with pre-existing glaucoma, anterior segment dysgenesis, and Lowe syndrome were excluded. The risk of aphakic glaucoma after surgery was estimated using Kaplan-Meier survival analysis.80 patients, undergoing 128 lensectomies were eligible. Of these, six patients (nine eyes) were lost to follow up. Based on eye count, the risk of glaucoma by 5 years after lensectomy was 15.6% (95% CI 10.2 to 23.4). Based on patient count, the 5 year risk of glaucoma in at least one eye following bilateral surgery was 25.1% (95% CI 15.1 to 40.0). The incidence of glaucoma remained at a constant level for the first 5 years after surgery. After early bilateral lensectomy, within the first month of life, the 5 year risk of glaucoma in at least one eye was 50% (95% CI 27.8 to 77.1) compared to 14.9% (95% CI 6.5 to 32.1) with surgery performed later (log rank test, p = 0.012). There was no significant difference (Kolmogorov-Smirnov test: unilateral lensectomy p = 0.587, bilateral lensectomy p = 0.369) in 5 year visual outcomes between eyes operated before and after 1 month of age.Bilateral lensectomy during the first month of life is associated with a higher risk of subsequent glaucoma than with surgery performed later. The reason for this is unclear but it may be prudent, in bilateral cases, to consider delaying surgery until the infant is 4 weeks old. As the incidence of glaucoma is similar for each year after surgery, long term glaucoma surveillance is mandatory.

Subject Peter Doyle (PD) had congenital bilateral cataracts removed at the age of 43. Pre-operatively PD’s visual acuity was 20/ 80, with a resolution limit around 15 cpd, and he experienced monocular diplopia with high contrast stimuli. Post-operatively PD’s visual acuity improved to approximately 20/40, with a resolution limit around 25 cpd. Using a variety of pre- and post-operative tests we have documented a wide range of neural adaptations to his limited and distorted visual input, and have found a limited amount of post-operative adaptation to his newly improved visual input. These results show that the human visual system is capable of significant adaptation to the particular optical input that is experienced. 2002 Elsevier Science Ltd. All rights reserved.

Bilateral congenital cataracts are often characterized by morphology, etiology, and related conditions. We report a case of unique congenital cataracts with triangular morphology and associated prenatal methamphetamine exposure. Although this association is likely coincidental, the cataract's morphology in light of the specific timing of prenatal drug use deserves reporting.

Asteroid hyalosis, noted in 0.83% of routine eye examinations, 1 is uncommon in younger patients and is more frequently seen in patients more than 60 years old. It has been considered to be related to an aging process, and when it occurs in younger patients, ocular disease is typically associated. 1 We report a 4-week-old patient with Down syndrome and bilateral congenital cataracts who had unilateral asteroid hyalosis.

Two hundred thirty-eight consecutive patients with pediatric cataracts were fitted with a variety of aphakic contact lenses after cataract extraction. Thirty-nine patients did not return for follow up. Of the remaining 199 patients, 40 discontinued contact lens wear. None of the 78 patients with unilateral or bilateral acquired cataracts discontinued contact lens wear due to problems wearing their lenses, although nine discontinued contact lens wear due to poor vision or difficulty maintaining amblyopia treatment. Twenty-two of 84 patients with unilateral congenital cataracts discontinued lens wear, six directly due to difficulties wearing lenses. Sixteen had poor vision in their aphakic eye and an inability to maintain patching for amblyopia. Eight of 37 patients with bilateral congenital cataracts discontinued lens wear because of problems wearing their lenses, and one other discontinued lens wear because of poor vision. In summary, 26 of 40 patients (out of a total of 199) that discontinued aphakic contact lens wear did so because of poor vision, while only 14 did so because of difficulties wearing the contact lenses. Eleven of these 14 patients were able to wear aphakic spectacles in lieu of contact lenses. This study shows that most pediatric patients with cataracts fail at treatment because of problems related to treatment of amblyopia, and not problems related to the fitting and wearing of contact lenses.

Several studies have indicated that operation during the first months of life in children with dense congenital cataract improves the final visual acuity. In the current study seven otherwise healthy children operated on before the age of fifty-six days are compared with seven children operated on after the age of three months. The patients were followed by a team consisting of a paediatric ophthalmologist, a contact lens optician and an orthoptist. They were treated with contact lenses, spectacles with near addition and occlusion therapy when needed. Visual acuity was initially tested with preferential looking technique and later with Snellen optotypes. In the early treated group the visual development was almost normal with a final visual acuity of 20/20, while in the late treated group no patient obtained better visual acuity than 20/100. The findings indicate that dense congenital cataract should be treated before the age of three months.

A total of 25 patients with bilateral congenital cataracts, from three aetiological groups i.e. rubella (6 patients) hereditary (6 patients) and undetected aetiology (13 patients) were studied for sister chromatid exchanges (SCE). SCEs were markedly raised in the rubella groups as compared with age sex matched controls. The other two groups also showed raised SCE, more so in hereditary group.

In about one third of patients congenital cataracts are bilateral. If the cataracts are dense, and the patient an infant, bilateral simultaneous cataract removal reduces anesthetic risk, hospital stay, and cost and allows earlier optical correction. This study represents a retrospective survey of 32 eyes (16 cases) with simultaneous surgery for bilateral congenital cataracts. Bilateral simultaneous surgery can be performed to avoid a second general anesthesia in infants who have bilateral dense congenital cataracts.

Adult-like expertise in processing face information takes years to develop1 and is mediated in part by specialized cortical mechanisms2 sensitive to the spacing of facial features (configural processing)3. Here we show that deprivation of patterned visual input from birth until 2–6 months of age results in permanent deficits in configural face processing. Even after more than nine years' recovery, patients treated for bilateral congenital cataracts were severely impaired at differentiating faces that differed only in the spacing of their features, but were normal in distinguishing those varying only in the shape of individual features. These findings indicate that early visual input is necessary for normal development of the neural architecture that will later specialize for configural processing of faces.

Generally speaking, it can be said, when considering all operated bilateral congenital cataracts, that a visual acuity of over 20/100 is found in approximately 50% of patients. In this study, 230 eyes were followed; of these 100 (43%) had an acuity of 20/60 or better and 130 (57%) had 20/100 or less. In conclusion the functional prognosis is better for incomplete cataracts than for total congenital cataracts. The main reason for this is the fact that total cataracts are often associated with cerebroretinal lesions or anomalies. The patient's age at operation and the surgical technique chosen are secondary factors. When considered from the standpoint of visual acuity, the results of congenital cataract surgery are discouraging, at least in the eyes with complete cataract. The visual prognosis could be better for total congenital cataracts if we operate on them at birth or immediately after birth.

A case of polyostotic fibrous dysplasia in an African male is described. The case also had bilateral congenital cataracts and nerve deafness on the side affected by the dysplasia.

Using the method of limits, we measured spatial and temporal vision in 13 children who had been deprived of patterned visual input during infancy until they were treated for dense central cataracts in both eyes. Spatial vision was assessed with vertical sine-wave gratings, and temporal vision was assessed with an unpatterned luminance field sinusoidally modulated over time. Under these testing conditions, spatial contrast sensitivity at low and medium spatial frequencies (0.33‐2 c deg 1 ) was within normal limits, but sensitivity at higher spatial frequencies and grating acuity were reduced on average by 1.3 and 0.5 log units, respectively. Temporal vision was affected less severely, with losses in sensitivity only for low temporal frequencies (5 and 10 Hz), which averaged 0.4 log units. Thus, spatial and temporal vision are likely mediated by different neural mechanisms, that are differentially affected by deprivation. © 1999 Elsevier Science Ltd. All rights reserved.

Summary Coarctation of the aorta in two siblings is described. A third sib has bilateral congenital cataracts. The etiology of congenital heart disease with emphasis on its familial occurrence is briefly discussed.

Publisher Summary Even though the long term visual outcome of bilateral congenital cataracts has usually been more encouraging than those obtained in monocular congenital cataracts, a significant portion of the patients have persisted with major visual handicaps. Although immunization programmes for rubella have significantly reduced the incidence of the embryopathy and its associated ocular anomalies, binocular congenital cataracts remain a major cause of visually handicapped infants in developed countries. Neonatal and infantile cataracts are considered complete when no fundus details are ophthalmoscopically visible, even with full dilatation of the pupil. Effective visual stimulation in this setting is obviously impossible. There can be no question of the necessity for surgical removal of the cataracts if they are detected at such a time as to allow for visual rehabilitation. The more difficult and, unfortunately, more common clinical problem is the assessment of the infant with incomplete cataracts. No absolute rules or objective techniques are available for identifying the partial cataracts that will invariably produce severe visual deprivation and those that will spare the developing visual system.

In 24 infants who underwent surgery for bilateral congenital cataracts, 29 eyes (60%) achieved visual acuities of 6/18 (20/60) or better and 13 (27%) had visual acuities of 6/60 (20/200) or worse. The best results occurred in patients who underwent surgery before they were 8 weeks old. Only one patient of the seven operated on after the age of 8 weeks achieved a visual acuity of better than 6/60 (20/200). These favorable visual outcomes were the result of early surgery, short intervals between operations on fellow eyes (48 hours or less), total bilateral occlusion between operations, careful postoperative monitoring with retinoscopy and visual-evoked potentials, and early correction of aphakia.