Congenital cataract with multiple congenital anomalies in a sibship.
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Abnormalities, Multiple↗
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Management of congenital cataracts.
The management of congenital cataracts at the Wilmer Institute is presented, with emphasis on the need for careful preoperative assessment. The indications and techniques for surgery in uncomplicated cataracts are discussed, and the advantages of using vitrectomy instruments in complicated congenital cataracts, such as those associated with persistent hyperplastic primary vitreous, are described.
Cataract↗
Late results of congenital cataract surgery.
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.
Adult↗
Unilateral congenital cataracts and their management.
Eight cases of unilateral congenital cataract were operated in the first two years of life. They were treated with contact lenses fitted within the first three weeks postoperatively and with patching of the good eye. None of these eyes achieved better than 3/100 vision. This dense amblyopia would seem based on the marked refractive difference. In addition to the ocular aspects, the psychological aspects of this evaluation would indicate that such surgery and postoperative management is probably not indicated for unilateral congenital cataracts.
Cataract↗
[Congenital cataract in 1979 (author's transl)].
The authors try to point out the question dealing with the surgery of congenital cataract in 1979. They remember the visual prognosis of congenital cataract according to its aetiology, as well as the best time to operate the child. The basic principles of the aspiration of this particular cataract are documented. Advantages and draw backs of each surgical procedure are presented.
Adolescent↗
Visual functions following bilateral surgery of congenital cataracts in children.
The visual functions, before and after surgery, of 19 children with bilateral congenital cataract are evaluated. Some of the children, in spite of the poor vision before surgery and the late age at surgery, developed good vision with binocular functions. These children probably had an incomplete lens opacity in their early childhood which became completely opaque at an age at which irreversible stimulus deprivation amblyopia is unlikely to develop. Therefore, children over the age of 6 years with poor vision due to congenital cataract, on whom no information is available concerning the density of their lens opacities in early childhood, should not be regarded as having a poor visual prognosis.
Adolescent↗
[Intelligence findings in children and adolescents with congenital cataract].
In children and young people with congenital cataracts the verbal intelligence quotient gives higher values than the perceptive-practical IQ. Longitudinal studies show the IQv reduced, but not the IQH. Connections exist between visual acuity and the IQ value, and the causes can be assumed to be disturbances of verbal information processing.
Adolescent↗
[Congenital cataract in two families: Heredity and treatment (author's transl)].
A report is given on the inheritance of congenital cataract in two families. While in one family the males were mainly affected, in the second faimily the females were mainly affected with congenital cataract. In our experience an operation is only indicated if the visual acuity is no longer sufficient for day-to day living (less than 0.25).
Adolescent↗
Risk Factors and Predictive Model for Postoperative High Myopia in Children Undergoing Congenital Cataract Surgery With Intraocular Lens Implantation.
PURPOSE: To identify risk factors associated with the development of high myopia following congenital cataract surgery and to establish a robust predictive model. DESIGN: Retrospective clinical cohort study. SUBJECTS: This retrospective study included 106 pediatric patients who underwent congenital cataract surgery with primary IOL implantation (mean follow-up 8.19 years). The model was externally validated in an independent cohort of 72 patients with a mean follow-up of 7.83 years. METHODS: Preoperative and postoperative ocular biometric parameters were collected. Risk factors for postoperative high myopia were analyzed using Cox proportional hazards regression, which served as the basis for model construction. The predictive performance of the model was rigorously evaluated for discrimination and calibration. Discriminative ability was quantified using Harrell's C-index and the area under the receiver operating characteristic curve (AUC). Model calibration was assessed via calibration plots by comparing predicted probabilities with actual observed outcomes. Internal validation was performed using a bootstrapping method (500 iterations) to ensure model stability and adjust for potential overfitting. RESULTS: An initial postoperative refraction of <+0.75D, and a higher IOL Power to Axial length Ratio (IOL/AL ratio) were identified as significant risk factors for the development of postoperative high myopia. Shorter preoperative axial length was associated with a greater magnitude of postoperative myopic shift. The predictive model demonstrated robust performance, achieving a C-index of 0.711 (internal validation C-index: 0.713). The area under the receiver operating characteristic curve (AUC) values for predicting high myopia at 5 and 10 years were 0.858 and 0.745, respectively. Furthermore, calibration curves demonstrated excellent agreement between the predicted and observed outcomes throughout the follow-up period. In external validation, the model achieved a C-index of 0.825, 5-year AUC of 0.833, and 10-year AUC of 0.713. CONCLUSIONS: Our analysis established that initial postoperative refraction <+0.75D, and an elevated IOL/AL ratio are key determinants of high myopia risk following surgery. Shorter preoperative axial length was associated with a greater magnitude of postoperative myopic shift. This predictive framework provides clinicians with a practical tool to optimize preoperative IOL selection and identify high-risk infants who require vigilant myopia prevention and balanced amblyopia management.
Humans↗
Phenotype i associated with congenital cataract in Japanese.
A Japanese family with two siblings of phenotype i is presented. Both had a past history of surgical treatment for congenital cataract. In Japan, 18 individuals of phenotype i, including our case, have been found in ten unrelated families. Seventeen of them had congenital cataract. Cataract was not found in any of the 45 phenotype I members in these families. It is briefly discussed why these two linked and quite rare genes were found in combination only in Japanese persons.
Cataract↗
[Activity of enzymes of galactose metabolism in so-called congenital cataract (author's transl)].
Activity of galactokinase (69 subjects) and galactose-1-phosphate uridyl transferase (92 subjects) were measured in haemolysed blood from children (predominantly of school age) with congenital cataract. chi2 tests, gene-frequency determination and metabolic-kinetic studies indicated that the changes in the lens in congenital cataract are partly due to a manifest or latent disorder of galactose metabolism, in particular a glactose-1-phosphate uridyl transferase defect.
Austria↗
Rubella infection and other factors associated with congenital cataracts in Cape Town.
An analysis of 56 patients with congenital cataracts was made to determine which factors played a role in the causation of these lenticular defects in Cape Town. There is good evidence that 16% of the defects were caused by intra-uterine rubella virus infection and that a further 30% were hereditary in origin.
Adolescent↗
Hydrocephalus combined with congenital cataract and microphthalmia.
We describe a case of bilateral microphthalmia with bilateral congenital cataracts associated with hydrocephalus in a 9-month-old girl with consanguineous parents. The differential diagnosis included: (1) congenital rubella syndrome; (2) congenital toxoplasmosis; (3) chromosome alterations; and (4) metabolic disease. However, negative clinical, laboratory, and instrumental investigations excluded all of these hypotheses. We stress the usefulness of echography in establishing whether or not infection has occurred during intrauterine life.
Cataract↗
Nevus of Ota with ipsilateral congenital cataract.
A mongoloid woman had a nevus of Ota, an ipsilateral congenital cataract, and upper extremity hemiatrophy. To our knowledge, no similar patient has been previously reported. We review the literature concerning the nevus of Ota and cataracts in mongoloid indivuduals.
Adult↗
[Congenital cataract linked to the Y chromosome].
A possible Y linked form of congenital cataract is reported, but an autosomal dominant mode of inheritance cannot be ruled out.
Cataract↗
Isolation of rubella virus from lens material in cases of congenital cataracts.
Rubella virus cultures were carried on lens materials from seven cases of bilateral congenital cataracts and in one of them it was possible to isolate the virus. This case is one of those rare cases in which the virus was isolated.
Abnormalities, Multiple↗
Vitrophage in management of congenital cataracts.
We have used the vitrophage for combined lensectomy and vitrectomy in six eyes with congenital cataracts. No intraoperative complications were encountered. One week after operation four of the six eyes had clear corneas, and all eyes showed a bright red reflex. With follow-up ranging from 18 to 21 months all eyes had clear corneas and a view of the posterior pole consistent with 20/20 vision.
Adolescent↗
A family with X-chromosomal recessive congenital cataract, microphthalmia, a peculiar form of the ear and dental anomalies.
An investigation has been made in a family with X-chromosomal recessive congenital cataract, microphthalmia, a peculiar form of the ear, and dental anomalies. The carrier females show only slight symptoms. They all have lens opacities, and most of them show more symptoms. For genetic counseling it is of utmost importance to examine all family members and to re-examine the females from time to time for early lens opacificaiton, which is the most constant finding. In literature families have been described with similar clinical symptoms only a few times. We have been trying to demonstrate a linkage with the Xga locus, which might enable us to locate the mutant gene on the X-chromosome and to exclude with more certainty the carrier status. The results of this blood group specification were not informative. In future we hope to be able to demonstrate a linkage with other markers located on the X-chromosome.
Adolescent↗