Pharmacologic pupillary constriction after dilated fundus examination for ectopia lentis to prevent further subluxation.
ABSTRACT: Purpose:To describe two cases of ectopia lentis with different preoperative management strategies following the initial diagnostic dilated exam. Observations:In both cases, the patients presented with bilateral subluxation of the crystalline lens. Neither patient had a known history of Marfan's disease, homocystinuria or other systemic disorders that affect the body's connective tissue possibly leading to lens subluxation. Patient 1 was sent home after dilated fundus examination with no special precautions. That same night, he developed severe right eye pain and further decreased vision. He was found to have complete dislocation of his right crystalline lens into the anterior chamber with corneal edema and an elevated pressure, requiring an urgent pars plana lensectomy. In our second case of ectopia lentis, patient 2 was examined prior to dilation and noted to have bilateral subluxation of the lens into the vitreous. Twenty minutes after dilation, the crystalline lens was noted to be in the anterior chamber in the right eye. The patient was laid supine for several minutes and once the lens was in the posterior cavity, she was given 1% pilocarpine in both eyes to constrict the pupil to prevent dislocation in the anterior chamber. Patient 2 had an uneventful perioperative period and did not suffer worsening subluxation after her initial visit. Conclusion and importance:Unlike patient 1, patient 2 did not suffer further subluxation after her initial dilated eye exam, therefore avoiding a more arduous clinical and surgical course. Patients presenting with completely dislocated lenses may benefit from the reversal of pupillary dilation by being placed in the supine position and given reversal drops such as 1% pilocarpine prior to leaving the office. This method may help prevent complications from anterior lens subluxation and pupillary block glaucoma until definitive surgical managment.
Project description:Megalocornea in isolation is a rare congenital enlargement of the cornea greater than 13 mm in diameter. Patients with megalocornea are prone to cataract formation, crystalline lens subluxation, zonular deficiencies and dislocation of the posterior chamber intraocular lens (PCIOL) within the capsular bag. A 55-year-old male with megalocornea in isolation developed subluxation of the capsular bag and PCIOL. The PCIOL and capsular bag were explanted, and the patient was subsequently implanted with an anterior chamber iris claw lens. An anterior chamber iris claw lens is an effective option for the correction of aphakia in patients with megalocornea.
Project description:Ectopia lentis is a genetically heterogeneous condition that is characterized by the subluxation of the lens resulting from the disruption of the zonular fibers. Patients with ectopia lentis commonly present with a marked loss in visual acuity in addition to a number of possibly accompanying ocular complications including cataract, myopia, and retinal detachment. We here describe an isolated form of ectopia lentis in a large inbred family that shows autosomal-recessive inheritance. We map the ectopia lentis locus in this family to the pericentromeric region on chromosome 1 (1p13.2-q21.1). The linkage region contains well more than 60 genes. Mutation screening of four candidate genes revealed a homozygous nonsense mutation in exon 11 of ADAMTSL4 (p.Y595X; c.1785T-->G) in all affected individuals that is absent in 380 control chromosomes. The mutation would result in a truncated protein of half the original length, if the mRNA escapes nonsense-mediated decay. We conclude that mutations in ADAMTSL4 are responsible for autosomal-recessive simple ectopia lentis and that ADAMTS-like4 plays a role in the development and/or integrity of the zonular fibers.
Project description:Purpose:This study evaluated bimanual intracapsular irrigation-aspiration for ectopia lentis with use of a small incision for 4-point scleral fixation of a foldable posterior-chamber intraocular lens (IOL) and anterior vitrectomy in patients with Marfan syndrome. Methods:We performed a retrospective study of 18 eyes from 10 patients with Marfan syndrome who underwent surgical intervention for ectopia lentis at our clinic between July 2012 and September 2018. In this study, intraoperative and postoperative complications, uncorrected visual acuity, best-corrected visual acuity, spherical equivalent, intraocular pressure, and endothelial cell density were evaluated. Results:No intraoperative complications were reported. In all cases, early postoperative evaluation revealed a clear cornea, round pupil, and well-centered IOL. Mean logMAR uncorrected visual acuity improved from 1.09 preoperatively to 0.56 postoperatively (P < 0.05). Mean logMAR best-corrected visual acuity improved from 0.45 preoperatively to 0.17 postoperatively (P < 0.05). Aside from transient ocular hypertension, no postoperative complications were reported. Conclusion:The combined surgical technique presented above yields excellent visual outcomes with an extremely low incidence of complications. This approach is simple, safe, and effective in the treatment of ectopia lentis in patients with Marfan syndrome.
Project description:Purpose:To compare ocular anatomy differences of lens subluxation between eyes with or without acute angle closure (AAC). Methods:This is a retrospective and case-control study. Sixty cases with mild lens subluxation were recruited. Among them, 30 eyes with acute angle closure were assigned to the AAC group and 30 eyes without AAC were assigned to the non-AAC group. The anterior segment was quantitatively evaluated by ultrasound biomicroscopy (UBM). The axial length (AL) was measured with IOL Master. All patients underwent lens extraction surgery and were followed up for six months. Results:The history of blunt trauma accounted for 22 (73.3%) cases in the AAC group and 21 (70%) cases in the non-AAC group. Fifteen (50%) patients in the AAC group had iridotomy history, and high intraocular pressure recurred. The UBM analysis showed that the average central chamber depth of the affected eyes in the AAC group was 1.82?mm, which was significantly shallower than that in the fellow eyes (2.58?mm, P < 0.05) or both eyes in the non-AAC group.Both eyes in the AAC group presented a shorter AL and shallower anterior chamber than the eyes in the non-AAC group. Conclusions:An asymmetrical anterior chamber between bilateral eyes is an important feature in lens subluxation-induced AAC. The crowded anterior chamber and shorter AL might be the anatomic basis for the eye with lens subluxation-induced AAC.
Project description:Purpose:To describe the occurrence of visual loss secondary to dispersion of cells deriving from an iris strand during pupil dilation in a 35-year-old woman. Observations:A 35-year-old woman presented complaining of blurring in the Left Eye 15-30 minutes following breastfeeding at night hours. On clinical examination, the uncorrected visual acuity was 20/20 in both eyes and slit-lamp examination demonstrated cells in the anterior chamber (26-50 cells in field/3+ SUN working group grading) whereas the examination of the right eye was unremarkable. Dilated examination revealed a thin pigmented strand of iris in the Left Eye spanning from one edge of the iris to the other (1st to 8th h). This strand was connected at a single point to the anterior capsule of the lens and from the point of connection red blood cells flowed in a linear configuration that ended at the lower part of the anterior chamber. Conclusions and importance:In this case report a patient with persistent pupillary membrane in the form of an individual iris strand connected to the anterior lens capsule presented with episodes of monocular vision loss. This occurred due to red blood cell dispersion from the iris strand during mesopic light conditions and pupil dilation while breastfeeding. To the best of our knowledge, this is the first report in the literature.
Project description:PURPOSE:To describe a five-grade classification of ectopia lentis in Marfan syndrome (MFS) and to evaluate the positive predictive value of the early grades of ectopia lentis. METHODS:We prospectively included MFS patients and their healthy relatives. The anterior segment examination was classified into grades 0 to 5, and we studied the sensitivity, specificity, and positive predictive value of ectopia lentis in this classification. RESULTS:Seventy-four MFS patients and thirty-six healthy controls were examined. In the MFS group, grades 1, 2, 3, and 4 were present in 15, 24, 17, and 7 patients, respectively, whereas 11 patients in this group did not present ectopia lentis. In the control group, grades 0 and 1 were observed in 30 and 6 individuals, respectively. Sensitivity to ectopia lentis of at least grade 2 was 64.9%, with 100% specificity, whereas sensitivity to ectopia lentis of at least grade 1 was 85.1%, with 83.3% specificity. The positive predictive value of ectopia lentis that was greater than or equal to grade 2 was 100%, whereas that of ectopia lentis greater than or equal to grade 1 was 91.3%. CONCLUSION:High positive predictive values s were found to be associated with grades 2 and higher of the five-grade classification of ectopia lentis. This classification should help to harmonize clinical practices for this major feature of MFS.
Project description:Femtosecond laser-assisted cataract surgery was performed in a case of posttraumatic cataract with six clock hours subluxation and vitreous in the anterior chamber (AC). Femtosecond laser pretreatment allowed a closed-chamber creation of corneal incisions, capsulotomy, and lens fragmentation with minimal sudden lens-diaphragm movements and zonular stress. Integrated imaging systems allowed customization of the size and position of capsulotomy and nuclear fragmentation, based on the extent and site of subluxation. Presence of vitreous in AC did not hamper femtosecond laser application. Triamcinolone-assisted vitrectomy was performed before phacoemulsification and after implanting the intraocular lens (IOL). Postoperative uncorrected visual acuity was 20/20 with a stable IOL.
Project description:PURPOSE:To investigate the effect of pupil dilation on ocular biometric parameters and intraocular lens (IOL) power calculation in schoolchildren using the Lenstar LS 900. METHODS:One hundred forty eyes of 140 healthy schoolchildren were included in the analysis. Axial length (AL), central corneal thickness (CCT), aqueous depth (AD), anterior chamber depth (ACD), lens thickness (LT), flat keratometry (K), steep K, astigmatism, white-to-white (WTW), and iris/pupil barycenter distance were measured, before and after pupil dilation. Anterior segment length (ASL) was defined as the sum of ACD and LT, and lens position (LP) was defined as ACD plus half of the LT. The relative lens position (RLP) was defined as LP divided by AL. IOL power was calculated using the eight formulas (Hill-RBF, Barrett, Haigis, Hoffer Q, Holladay, Olsen, SRK II, and SRK/T) integrated in the Lenstar LS 900. Parameters before and after pupil dilation were compared. RESULTS:AL, AD, ACD, LT, ASL, LP, RLP, flat K, iris barycenter distance, pupil barycenter distance, and PD differed significantly after pupil dilation (P < 0.001 in all cases), as compared to before dilation. The Olsen formula demonstrated significant differences in the magnitude of astigmatism (P = 0.010) and IOL power (P = 0.003) after pupil dilation. Using the different formulas, 23.6-40.7% of participants had IOL power changes of more than 0.50 diopters, while 0.7-1.4% had IOL changes of more than 1.0 diopter after pupil dilation. CONCLUSIONS:Dilated and undilated pupil size affected the Lenstar LS 900 measurement of some ocular biometric parameters, and pupil dilation led to IOL power changes exceeding 0.50 diopters with a high percentage (from 23.6% to 40.7%) in schoolchildren, which should be noticed in clinical practice.
Project description:We have previously described a syndrome characterized by facial dysmorphism, lens dislocation, anterior-segment abnormalities, and spontaneous filtering blebs (FDLAB, or Traboulsi syndrome). In view of the consanguineous nature of the affected families and the likely autosomal-recessive inheritance pattern of this syndrome, we undertook autozygosity mapping and whole-exome sequencing to identify ASPH as the disease locus, in which we identified two homozygous mutations. ASPH encodes aspartyl/asparaginyl ?-hydroxylase (ASPH), which has been found to hydroxylate aspartic acid and asparagine residues on epidermal growth factor (EGF)-domain-containing proteins. The truncating and missense mutations we identified are predicted to severely impair the enzymatic function of ASPH, which suggests a possible link to other forms of ectopia lentis given that many of the genes implicated in this phenotype encode proteins that harbor EGF domains. Developmental analysis of Asph revealed an expression pattern consistent with the proposed link to the human syndrome. Indeed, Asph-knockout mice had a foreshortened snout, which corresponds to the facial abnormalities in individuals with Traboulsi syndrome. These data support a genetic basis for a syndromic form of ectopia lentis and the role of aspartyl hydroxylation in human development.
Project description:PURPOSE:To report the long-term outcomes of transscleral fixation of capsular tension rings (CTR) with intraocular lens (IOL) placement in pediatric patients with ectopia lentis. SETTING:John A. Moran Eye Center, University of Utah, Salt Lake City, Utah, USA. DESIGN:Retrospective case series. METHODS:Pediatric patients requiring lens extraction with transscleral capsular bag fixation with a CTR and IOL for ectopia lentis between January 2006 and January 2016 were analyzed. RESULTS:Thirty-seven patients (67 eyes) who had transscleral fixation of the capsular bag using a CTR fixated with 9-0 or 10-0 polypropylene (Prolene), 8-0 polytetrafluoroethylene (Gore-Tex), or 9-0 nylon were identified. The mean age at time of surgery was 7.25 years (2 to 18 years) and the mean follow-up was 35.3 months (0.25 to 120 months). The proportion of eyes showing improvement in corrected distance visual acuity (CDVA) postoperatively was 78.5%, which demonstrated significance with a 95% confidence interval. In the immediate postoperative period, 1 eye developed a hyphema and 1 eye required IOL repositioning. Long-term complications included posterior capsule opacification in 35 eyes (52%) and uveitis-glaucoma-hyphema syndrome in 1 eye (1.5%). Three eyes (4.4 %) required IOL repositioning for spontaneous delayed IOL dislocation, 2 sutured with 8-0 polytetrafluoroethylene at postoperative month 8 and postoperative year 3 and 1 sutured with 9-0 polypropylene at postoperative year 7. CONCLUSION:Transscleral fixation of the capsular bag using a CTR improved CDVA and provided IOL stability in pediatric patients with ectopia lentis.