Luxación bilateral de cristalino en octogenaria / Bilateral luxation of crystalline lens in an octogenarian

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Persistent fetal vasculature presenting with axial elongation and platyphakia.

Leukocoria in children should always raise the concern for retinoblastoma. However, a variety of non-neoplastic conditions can also present with leukocoria, including persistent fetal vasculature (PFV), a nonhereditary, congenital anomaly caused by a failure of the fetal intraocular vasculature to regress during development. Classically PFV presents with features that make it easily distinguishable from retinoblastoma, including microphthalmia, retrolental fibrovascular membrane, central dragging of ciliary processes, and cataract. We present an atypical case of PFV in a 9-month-old boy who presented with the unusual features of axial myopia and platyphakia.

Lack of FOXE3 coding mutation in a case of congenital aphakia.

PURPOSE: To report the findings in a patient with congenital primary aphakia, a rare disease known to be caused by mutations in the FOXE3 gene. METHODS: The clinical appearances and visual functions of the patient were determined from the medical records. Genetic analyses were performed to search for mutations in the FOXE3 gene by Sanger sequencing and whole exome sequencing. RESULTS: The 2-month-old male patient first presented with bilateral congenital aphakia associated with microphthalmia, corneal opacity, and dysplasia of the anterior segment. At the age of 2-years, his visual acuity in the left eye was 20/1000 at 30 cm, he was able to discriminate red, blue, and yellow light stimuli, and a b-wave was recorded by scotopic combined rod-cone electroretinograms. The right eye became blind during the follow-up period. No mutation in the FOXE3 gene was detected. CONCLUSION: Although congenital aphakia is known to be caused by mutations in the FOXE3 gene, the results of lack of coding mutation in this patient suggests a possible genetic heterogeneity of the disease.

Role of ultrasound biomicroscopy in the planning for secondary implantation of intra ocular lens in aphakia.

Purpose This is a prospective investigational study that was performed at Tanta University Eye Hospital, Tanta, Egypt to evaluate the role of ultrasound biomicroscopy (UBM) in planning secondary implantation of intra ocular lens (IOL) in aphakia. Methods Preoperative UBM was performed for the assessment of anterior segment of 30 aphakic eyes admitted for secondary IOL implantation with special attention to central corneal thickness (CCT), anterior chamber depth (ACD), ciliary sulcus (CS), anatomical changes, and posterior capsular (PC) integrity which had been assessed by measuring the remnants of PC with special attention to the 12, 3, 5, 6, 7, and 9 o'clock meridians. Results This study involved 30 eyes in 27 patients; 16 males and 11 females. The mean age was 8.39 ± 2.36 years. The causes of aphakia were: congenital cataract extraction in14 eyes (46.7 %); trauma in 14 eyes (46.7 %); and after extracapsular cataract extraction in 2 eyes (6.6 %). The mean CCT was 0.61 + 0.35 mm and the mean ACD was 3.03 + 0.41 mm. The ciliary sulcus was patent in 28 eyes (93.4 %). Posterior synechia was observed in 8 eyes (26.7 %), lens remnants in 11 eyes (36.7 %), corneal scars in 11 eyes (36.7 %), and vitreous in anterior chamber in 1 eye (3.3 %). The integrity of PC was illustrated with a diagram. Conclusions UBM is a useful device to evaluate aphakic eyes before secondary IOL implantation through good evaluation of the anterior segment with special attention to the posterior capsular integrity, ciliary sulcus, anterior chamber depth, corneal thickness, and detection of any structural changes in the anterior segment resulting from the remote cause of aphakia.

Patency of superior ciliary sulcus in aphakic silicone oil-filled eyes: an ultrasound biomicroscopy study.

OBJECTIVE: To assess the patency of the superior ciliary sulcus in aphakic silicone oil-filled eyes by ultrasound biomicroscopy (UBM). DESIGN: Prospective interventional series. PARTICIPANTS: Twelve eyes of 12 patients were included in this study. METHODS: Twelve aphakic silicone oil-filled eyes were evaluated using UBM. The UBM examination of the anterior segment was performed using the Humphrey Instruments UBM equipped with a high-frequency (50 MHz), high-resolution (50 microm) transducer. The patients were placed in a semisitting position in which their backs were inclined to a suitable angle (about 50 degrees ). They were also asked to look straight ahead, to bring the superior ciliary sulcus to the highest point of the globe. The probe approached the patients' eyes superiorly at a slight angle (about 10 degrees ). Images of the superior ciliary sulcus were captured and analyzed. RESULTS: The UBM images demonstrated an intact curved aqueous-silicone oil interface that appeared at some distance from and not filling the ciliary sulcus. A patent superior ciliary sulcus was thus found in all patients, even in those with a widely opened ciliary sulcus. CONCLUSIONS: The superior ciliary sulcus maintained its patency in aphakic silicone oil-filled eyes. These findings indicate that an iridectomy in a superior position may also be effective, although this has not been tested clinically in this report.

Central corneal thickness in children with congenital cataract and children with surgical aphakia: a case-control study.

AIM: To measure the central corneal thickness (CCT) of children with congenital cataract and surgical aphakia. METHODS: Children with congenital cataract or surgical aphakia were prospectively recruited and divided into four groups: unilateral cataract (group 1, n = 14), bilateral cataract (group 2, n = 17), unilateral aphakia (group 3, n = 32) and bilateral aphakia (group 4, n = 44). An age-, sex-, and race-matched control group of normal individuals was selected. Ultrasonic pachymetry was performed by the same observer. RESULTS: The mean CCT of the control group was not significantly different from the normal (p = 0.747) and cataractous eyes of group 1 (p = 0.252). The mean CCTs of both eyes of group 2 were significantly higher than the control group (p<0.01). The mean CCT of the aphakic eyes in group 3 was significantly higher than the contralateral healthy eyes and control eyes (p<0.001). The mean CCTs of both eyes of group 4 were significantly higher than the control group (p<0.001). The mean CCT was significantly higher in aphakic eyes of groups 3 and 4 than in cataractous eyes of groups 1 and 2 (p<0.001). CONCLUSIONS: Aphakic eyes due to congenital cataract show thicker corneas than normal phakic eyes. Aphakic eyes after congenital cataract extraction show thicker corneas than eyes with congenital cataracts, suggesting that the increase in CCT occurs postoperatively.

Retinoscopic (refractive) estimation of axial length in paediatric aphakia: a comparison with ultrasonic measurement.

AIM: To compare paediatric axial length values estimated from the aphakic refraction alone with axial length values measured by ultrasound. METHODS: Retrospective institutional medical record review of paediatric aphakic patients 12 years of age and younger with documented ultrasonic axial length and objective refraction (retinoscopy) within 3 months of each other. An estimate of axial length was made from the aphakic refraction alone (with an assumed average keratometry value of 44 dioptres) for all patients. RESULTS: 149 eyes of 102 paediatric aphakic patients were identified. The ultrasonic axial length values (mean 22.47 mm, SD 1.69, 95% confidence interval (CI) 0.27) and estimated axial length values (mean 22.41 mm, SD 1.53, 95% CI 0.25) had an average difference of 0.05 mm (SD 1.04, 95% CI 0.17) and were not significantly different (p = 0.56) by the two tailed paired t test. A histogram of the differences that did exist between the two values resembled a normal distribution. The nine eyes with the largest differences between the two values had either low hyperopic aphakic refractions or abnormal average keratometry values. CONCLUSIONS: There was no significant difference between the two groups of axial length values, and the distribution of differences that did exist seemed random. The greatest differences between the two values occurred in longer (less hyperopic) eyes and in eyes with abnormally steep or flat keratometry. Estimation of axial length from the aphakic refraction alone seems to be a useful technique in the average paediatric eye, especially if biometry is unavailable.

Congenital corneal staphyloma associated with aphakia.

A neonate had a globular mass bulging through the eyelids of the left eye. Computed tomography revealed a large ectatic corneal lesion and the absence of a lens. The enucleated globe revealed that the posterior surface of the ectatic cornea was lined by iris tissue, indicating corneal staphyloma.

Optical and ultrasound measurement of axial length and anterior chamber depth for intraocular lens power calculation.

PURPOSE: To evaluate the precision, reproducibility, and applicability of an optical method based on partial coherence interferometry for intraocular lens (IOL) power calculation. SETTING: Ultrasound laboratory of a university eye hospital. METHODS: A prospective comparison of measurements made by the IOLMaster optical instrument (Carl Zeiss) and Ultrascan Digital 2000 contact ultrasound A-scan (Alcon) for IOL calculations was performed. Examined were 255 eyes of 134 persons (204 phakic, 47 pseudophakic, and 4 aphakic). The mean age of the patients was 67.9 years (range 7 to 94 years). RESULTS: The IOLMaster measurements were successful in more than 80% of cases: in 82%, 99%, and 99% for axial length (AL), anterior chamber depth (ACD), and keratometry measurements, respectively. The reproducibility of the AL and ACD measurements was very high (coefficient of variation 0.13% and 2.20%, respectively). The AL and ACD values were significantly larger with the IOLMaster (P <.001) than with the Ultrascan Digital 2000. The correlation between ultrasound and optical AL measurements was high (r = 0.985; P <.001); however, there was no correlation between ACD measurements (r = 0.079; P =.397). The corneal refractive power measurements of a Javal-type keratometer and the IOLMaster were highly correlated (r = 0.955; P <.001), with a mean difference of 0.2 diopter. CONCLUSIONS: The results show that measurements for IOL calculation are easy and precise with the optical method. It is a noncontact method, so no anesthesia is needed and there is no risk of infection.