Children with weak or absent capsular support requiring lens removal and intraocular lens implantation present as one of the more challenging cases in pediatric ophthalmology practice. The authors present a case series with postoperative follow-up of at least five years after retropupillary fixation of iris-clipped lenses. All cases had improved visual acuity with only minor focal iris atrophy as complication in one case. Retropupillary fixation of iris-clipped IOL should be included in an ophthalmologist's armamentarium when operating on cases with weak or absent capsular support.
lens subluxation

OBJECTIVE: To evaluate the application of femtosecond laser technology in the management of subluxated lens. METHODS: We retrospectively analyzed the data of the patients with subluxated lens undergoing femtosecond laser- assisted surgery at the Cataract Center of Guangzhou Aier Eye Hospital between March, 2017 and May, 2019. The LenSx femtosecond laser-assisted cataract surgery system was used to perform capsulotomy and lens fragmentation. According to the patients' eye condition, anterior vitrectomy was performed and capsular retractors was used. After phacoemulsification, I/A and insertion of the tension rings, the intraocular lens (IOL) was implanted into the capsular bag. The perioperative data, complications, visual acuity and intraocular pressure after the operation were recorded, and the stability of the capsular bag and IOLs were assessed. RESULTS: We analyzed the data of 25 cases (29 eyes) of subluxated lens, including 16 (16 eyes; 55.17%) as the result of traumatic lens subluxation, 5 (9 eyes; 31.03%) of Mafan syndromes, 1 case (1 eye; 3.45%) of high myopia and 3 cases (3 eyes; 10.34%) of unknown causes. Thirteen 13 eyes (44.83%) showed mild subluxation, 7 (24.14%) had moderate subluxation, and 9 (31.03%) had severe subluxation. Femtosecond laser- assisted capsulorhexis, lens fragmentation and phacoemulsification were successfully completed for 29 eyes, of which 28 eyes (96.55%) retained the complete capsular bag and with successful implantation of the capsular tension devices and IOLs. Nine eyes (31.03%) were treated with anterior segment vitrectomy; iris hooks were used for 2 eyes (6.90%) and capsular bag hooks for 9 eyes (31.03%). The best corrected visual acuity was significantly improved in 29 eyes after operation ( < 0.05). At 1 month after the surgery, 26 eyes (89.66%) showed stably centered IOLs, 2 eyes (6.90%) showed slight tilt of the IOLs, and 3 eyes (10.34%) had anterior capsular contraction. The intraoperative complications included subconjunctival hemorrhage (75.87%), incomplete capsulotomy (17.24%) and contracted pupils (13.79%). CONCLUSIONS The application of femtosecond laser assisted technology enhances the surgical safety and effectiveness for subluxated lens, facilitates the choice of individualized surgical options, and promotes maximum recovery of the patients' visual function.
Humans ; Laser Therapy ; Lens Implantation, Intraocular ; Lens Subluxation ; surgery ; Lenses, Intraocular ; Phacoemulsification ; Retrospective Studies

PURPOSE: We evaluated the prognostic factors of open globe injuries in children and adolescents, and compared the ocular trauma score (OTS) and pediatric penetrating ocular trauma score (POTS). METHODS: We performed a retrospective review of 77 children under 18 years of age who visited our clinic with open globe injuries between May 1993 and April 2014. We investigated the factors that may affect final visual acuity. We also compared the OTS and POTS using receiver operating characteristic curves as a method to predict final visual acuity. RESULTS: By univariate analysis, an initial visual acuity less than 20/200, globe rupture, wound size greater than 7.0 mm, retinal detachment, lens dislocation, and total number of operations contributed to worse visual outcomes (<20/200). Conversely, central corneal involvement, traumatic cataract, wound size less than 7.0 mm, and initial visual acuity greater than 20/200 were better prognostic indicators (≥20/32). Both OTS and POTS had diagnostic value as a predictor of final visual acuity, although there were no statistically significant differences between the two scoring systems. CONCLUSIONS: Initial visual acuity and wound size are important prognostic factors for the final visual acuity in children and adolescent, following open globe injuries. Both OTS and POTS are reliable prognostic models for open globe injuries in children and adolescents.
Adolescent ; Cataract ; Child ; Humans ; Lens Subluxation ; Methods ; Retinal Detachment ; Retrospective Studies ; ROC Curve ; Rupture ; Visual Acuity ; Wounds and Injuries

PURPOSE: To report the results of a new technique for intraocular lens scleral fixation of temporary haptic externalization and four-point fixation for enhanced stability. METHODS: Two 10–0 polypropylene strands were fixed at two points 2 mm apart on each haptic of a conventional three-piece intraocular lens, using our previously reported method of temporary haptic externalization after injector implantation. Postoperative refractive outcome and stability were evaluated. RESULTS: Although the intraocular lens was fixed at a total of four points, no intraoperative difficulties were observed during the process. Patients showed successful fixation of the intraocular lens postoperatively. The fixed intraocular lens showed good centering and minimal tilting. When compared to the two-point fixation technique, postoperative astigmatism was significantly smaller in the four-point fixation group (1.80 ± 0.84 vs. 1.00 ± 0.0.50, p = 0.033). Lens-induced astigmatism calculated from subtraction of corneal astigmatism from total astigmatism was also significantly smaller in the four-point fixation group (2.23 ± 0.98 vs.1.17 ± 0.0.70, p = 0.043). No postoperative complications were identified during a mean follow-up period of 14.8 months (range, 10 to 19 months). CONCLUSIONS: By fixing the intraocular lens at two points on each side of the haptics, this method minimizes the tilting of the intraocular lens and thus decreases postoperative lens-induced astigmatism. Also, the possibility of intraocular lens dislocation in the long term might be decreased by this two-point fixation technique.
Aphakia ; Astigmatism ; Cataract ; Dislocations ; Follow-Up Studies ; Humans ; Lens Subluxation ; Lenses, Intraocular ; Methods ; Polypropylenes ; Postoperative Complications

Neonatal Marfan syndrome (nMFS) is considered to be on the most severe end of the spectrum of type I fibrillinopathies. The common features of nMFS include ascending aortic dilatation, severe mitral and/or tricuspid valve insufficiency, ectopia lentis, arachnodactyly, joint contractures, crumpled ear, loose skin, and pulmonary emphysema.We describe a newborn male diagnosed with nMFS. He presented several atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Molecular analysis revealed a missense mutation at nucleotide 3217 (c.3217G>A) in exon 26 of the fibrillin-1 (FBN1) gene, resulting in the substitution of a glutamate for a lysine at codon 1073 (E1073K) in the 12th calcium binding epidermal growth factor-like domain of the FBN1 protein. Here we report a rare case of Nmfs with several combined atypical features, such as diaphragmatic eventration, severe hydronephrosis with hydroureter, and dilated cisterna magna. Our report is the first atypical nMFS case with p.Glu1073Lys mutation of FBN1 in Korea and may help clinicians with the diagnosis and follow-up of atypical nMFS.
Arachnodactyly ; Calcium ; Cisterna Magna ; Codon ; Contracture ; Diagnosis ; Diaphragmatic Eventration ; Dilatation ; Ear ; Ectopia Lentis ; Exons ; Follow-Up Studies ; Glutamic Acid ; Humans ; Hydronephrosis ; Infant, Newborn ; Joints ; Korea* ; Lysine ; Male ; Marfan Syndrome* ; Mutation, Missense ; Skin ; Tricuspid Valve Insufficiency

PURPOSE: To report a case of bilateral simple ectopia lentis associated with FBN1 gene mutation. CASE SUMMARY: A 47-year-old women presented with a one-month history of ocular pain and decreased visual acuity of the right eye. She had a family history of crystalline lens dislocation but showed no systemic abnormality or trauma history. Intraocular pressure was 45 mmHg in the right eye, which showed a myopic shift (−6.5 D). The crystalline lens of the right eye was subluxated to the anterior chamber, and the angle was closed. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the right eye was performed. After that, best corrected visual acuity of the right eye was 1.0, and intraocular pressure was 15 mmHg. After 2 years, she presented with intermittent ocular pain and decreased visual acuity of the left eye. The crystalline lens of the left eye was subluxated to the anterior chamber. Phacoemulsification with scleral fixation of the posterior chamber intraocular lens of the left eye was performed. After that, intermittent ocular pain and visual acuity of the left eye were improved. Genetic testing confirmed an FBN1 gene mutation in the patient. CONCLUSIONS: A bilateral ectopia lentis patient without history of definite trauma should undergo complete systemic and ophthalmic examination to rule out accompanying disease, and a detailed family history should be collected. If hereditary ectopia lentis is suspected, genetic testing of probands and their family should be performed and will be helpful for genetic counseling and ophthalmic surveillance.
Anterior Chamber ; Dislocations ; Ectopia Lentis* ; Female ; Genetic Counseling ; Genetic Testing ; Humans ; Intraocular Pressure ; Lens, Crystalline ; Lenses, Intraocular ; Middle Aged ; Phacoemulsification ; Visual Acuity

PURPOSE: To determine the risk factors of intraoperative complications in femtosecond laser-assisted cataract surgery. METHODS: This study included 598 eyes of 337 patients who underwent femtosecond laser-assisted cataract surgery (FLACS) between July, 2012 and January, 2017. All eyes had corneal incisions, anterior capsulotomy, nuclear fragmentation, and limbal relaxing incisions (if required). Intraoperative complications were analyzed by watching videos, and the related factors of each complication were retrospectively reviewed alongside the medical records. RESULTS: The mean age of the patients was 62.1 ± 11.9 years. Among the study group, 18 eyes required manual creation of corneal incisions; because the corneal incisions could not be made due to corneal central opacity, corneal peripheral degeneration, ptreygium, conjunctival chalasis, or idiopathic. The anterior capsulotomy was incomplete in 43 cases and manual capsulorrhexis was required for completion. These cases were associated with various conditions, including hypermature cataract, anterior polar or subcapsular cataract, corneal central opacity, pupillary abnormality, lens subluxation, poor pupil dilation, and idiopathic. Overall, 22 eyes had difficulties with nuclear fragmentation, with either mature cataract, lens subluxation, corneal central opacity, anterior polar or subcapsular cataract, or pupillary abnormality. Using the Laser SoftFit™ patient interface decreased the incidences of incomplete corneal incision (from 3.56% to 2.24%, p = 0.367), anterior capsulotomy (from 9.31% to 4.03%, p < 0.05), and nuclear fragmentation (from 5.20% to 1.34%, p < 0.05). The incidences of complications in the experienced group was statistically lower compared with the novice group (p < 0.05 for all comparisons). CONCLUSIONS: The femtosecond laser platform was effective and safe for cataract surgery. However, in the presence of related factors, use of this platform might need to be re-assessed and should be considered for intraoperative complications. Additionally, with the Laser SoftFit™ patient interface and improved surgeon experience, better intraoperative results can be expected for FLACS surgery.
Capsulorhexis ; Cataract* ; Corneal Opacity ; Humans ; Incidence ; Intraoperative Complications ; Lens Subluxation ; Medical Records ; Pupil ; Retrospective Studies ; Risk Factors*

BACKGROUND AND OBJECTIVES: Marfan syndrome (MFS) is a connective tissue disorder with autosomal dominant inheritance and a highly variable clinical spectrum. However, there are limited data available on the clinical features of Korean patients with MFS. The aim of the present study was to describe the clinical characteristics and outcomes of Korean patients with MFS. SUBJECTS AND METHODS: We included all patients who were diagnosed with MFS between January 1995 and May 2015 at a single tertiary medical center. Patients with an MFS-related disorder including MASS phenotype (myopia, mitral valve prolapse, borderline and non-progressive aortic root dilatation, skeletal findings, and striae), mitral valve prolapse syndrome, and ectopia lentis syndrome were excluded. A total of 343 Korean patients aged ≥15 years who satisfied the revised Ghent nosology were included. RESULTS: The mean patient age at diagnosis was 35.9±12.6 years and 172 (50.1%) patients were male. Median follow-up duration was 52.8 months. A total of 303 patients (88.6%) had aortic root dilatation with Z score ≥2 or aortic root dissection. Ectopia lentis was relatively less common (163 patients, 55.1%) and systemic score ≥7 was found in 217 patients (73.8%). Among 219 probands, a family history of MFS was present in 97 patients (44.5%) and sporadic cases in 121 patients (55.5%). Among the 157 probands who underwent genetic analysis, 141 (89.8%) had an FBN1 mutation associated with aortic root aneurysm/dissection. Aortic dissection (AD) or intramural hematoma (IMH) was identified in 110 patients (32.1%). Among the 221 patients without AD or IMH, descending aortic aneurysms were identified in 19 patients (8.6%). Two hundred thirteen patients (62%) underwent cardiovascular surgery of any type. Eight patients died during follow-up. CONCLUSION: We described the clinical characteristics and outcomes of Korean MFS patients. Cardiovascular manifestations were commonly detected and FBN1 mutation was present in approximately 90% of patients. In contrast, ectopia lentis was identified in approximately half of patients. Our findings will be informative for the evaluation of patients with MFS.
Aortic Aneurysm ; Asian Continental Ancestry Group ; Connective Tissue ; Demography ; Diagnosis ; Dilatation ; Ectopia Lentis ; Follow-Up Studies ; Hematoma ; Humans ; Korea* ; Male ; Marfan Syndrome* ; Mitral Valve Prolapse ; Phenotype ; Wills

No abstract available.
Child, Preschool ; DNA Mutational Analysis ; Diseases in Twins/*genetics/metabolism ; Ectopia Lentis/*genetics/metabolism ; Extracellular Matrix Proteins ; Humans ; Male ; Microfilament Proteins/*genetics/metabolism ; *Mutation ; *Twins, Monozygotic

Humans ; Lens Subluxation ; Lenses, Intraocular ; Male ; Middle Aged ; Remission, Spontaneous