Birc7: A Late Fiber Gene of the Crystalline Lens

Purpose. To study the effectiveness of extrascleral filling (ESF) using endoillumination in the surgical treatment of patients with primary rhegmatogenous retinal detachment (RRD). Material and methods. The material for the study was the data of a comprehensive clinical examination and surgical treatment by ESF method using endoillumination in 17 patients (17 eyes) with RRD. In 7 cases (41%), the macular area was involved in the detachment process. In 5 cases (29.4%), local scleral filling was performed. In the remaining 12 cases (70.6%), the local ESF was supplemented with a circling silicone element. Surgical intervention was supplemented by transscleral drainage of subretinal fluid (SRF) in 10 cases (59%) and pneumatic retinopexy with SF6 gas 50% in 8 cases (48%). Results. In the general group of patients, best corrected visual acuity (BCVA) increased from 0.35 to 0.46. In the subgroup of patients with a detached macular area, the positive dynamics is more pronounced, BCVA increased from 0.1 to 0.28. The progression of proliferative vitreoretinopathy caused the recurrence of retinal detachment in two patients (11.8%). Recurrences were diagnosed after 3 and 5 months, respectively. In both cases, a vitrectomy with tamponade of the vitreous cavity with silicone oil 5000 Cst was performed. The percentage of successful anatomical outcome after the first operation in our study was 82%. The percentage of successful achievement of the final anatomical result was 94%. In two cases, additional injection of SF6 gas into the vitreous cavity was required. Conclusion. This type of surgical treatment is an effective method of surgical treatment of patients with RRD. In our study, the successful anatomical outcome after the first operation was recorded in 82% of patients, which correlates with the data of the authors who also used this method (83–92%). Surgeons who performed surgical treatment using this technique in our study note improved workplace ergonomics when visualizing the fundus using an operating microscope and endoillumination compared with indirect ophthalmoscopy. Other teams of authors came to this conclusion as well. In our study, there were no complications associated with the introduction of a light pipe into the vitreous cavity (iatrogenic crystalline lens injury, endophthalmitis), which indicates the safety of this type of surgical treatment.

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Outcomes of three surgical approaches for managing ectopia lentis in Marfan syndrome

European Journal of Ophthalmology ◽

10.1177/1120672121992950 ◽

2021 ◽

pp. 112067212199295

Author(s):

Gurkan Erdogan ◽

Nilay Kandemir Besek ◽

Betul Onal Gunay ◽

Alper Agca

Keyword(s):

Marfan Syndrome ◽

Crystalline Lens ◽

Surgical Approaches ◽

Posterior Chamber ◽

Ectopia Lentis ◽

Iol Implantation ◽

Capsular Tension Ring ◽

Group 3 ◽

Group 2 ◽

Group 1

Objective: To investigate the clinical outcomes of three surgical approaches for ectopia lentis in Marfan syndrome (MS) patients who had undergone crystalline lens removal with posterior chamber intraocular lens (IOL) implantation techniques comprising the intrascleral fixation of IOL, sutured scleral fixation of IOL, and IOL implantation with the use of a Cionni capsular tension ring (CTR). Methods: This is a retrospective comparative study, including 35 eyes of 21 patients who underwent the intrascleral fixation of IOL (group 1), scleral IOL fixation with the Z-suture (group 2), and IOL implantation with the use of a Cionni CTR (group 3) following crystalline lens removal. The surgical indications were as follows: no improvement in visual function after eyeglasses or contact lens application due to excessive irregular astigmatism and advanced crystalline lens decentration in which the edge of the crystalline lens came up to the optical axis, or dislocation of the crystalline lens resulting in aphakia and secondary glaucoma due to lens dislocation. The surgical outcomes and complications due to surgery were compared between the groups. Results: The mean age of the patients in the study was 12.3 ± 8.7 years (5–32 years). There were 10 eyes in group 1, 13 eyes in group 2, and 12 eyes in group 3. Visual acuity improved significantly in each group after surgery. Ocular residual astigmatism did not differ significantly between the groups ( p = 0.51). Conclusion: There were no significant differences between the three surgical approaches in the current study in terms of the postoperative results and complications.

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A 127 kb truncating deletion of PGRMC1 is a novel cause of X-linked isolated paediatric cataract

European Journal of Human Genetics ◽

10.1038/s41431-021-00889-8 ◽

2021 ◽

Author(s):

Johanna L. Jones ◽

Mark A. Corbett ◽

Elise Yeaman ◽

Duran Zhao ◽

Jozef Gecz ◽

...

Keyword(s):

Intellectual Disability ◽

Protein Interactions ◽

Cholesterol Biosynthesis ◽

Crystalline Lens ◽

Whole Genome Sequencing Data ◽

Mendelian Disease ◽

Cataract Formation ◽

Sequencing Data ◽

Zebrafish Model ◽

Paediatric Cataract

AbstractInherited paediatric cataract is a rare Mendelian disease that results in visual impairment or blindness due to a clouding of the eye’s crystalline lens. Here we report an Australian family with isolated paediatric cataract, which we had previously mapped to Xq24. Linkage at Xq24–25 (LOD = 2.53) was confirmed, and the region refined with a denser marker map. In addition, two autosomal regions with suggestive evidence of linkage were observed. A segregating 127 kb deletion (chrX:g.118373226_118500408del) in the Xq24–25 linkage region was identified from whole-genome sequencing data. This deletion completely removed a commonly deleted long non-coding RNA gene LOC101928336 and truncated the protein coding progesterone receptor membrane component 1 (PGRMC1) gene following exon 1. A literature search revealed a report of two unrelated males with non-syndromic intellectual disability, as well as congenital cataract, who had contiguous gene deletions that accounted for their intellectual disability but also disrupted the PGRMC1 gene. A morpholino-induced pgrmc1 knockdown in a zebrafish model produced significant cataract formation, supporting a role for PGRMC1 in lens development and cataract formation. We hypothesise that the loss of PGRMC1 causes cataract through disrupted PGRMC1-CYP51A1 protein–protein interactions and altered cholesterol biosynthesis. The cause of paediatric cataract in this family is the truncating deletion of PGRMC1, which we report as a novel cataract gene.

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Lovastatin has no effect on the crystalline lens

Inpharma Weekly ◽

10.2165/00128413-199107780-00051 ◽

1991 ◽

Vol &NA; (778) ◽

pp. 16

Author(s):

&NA;

Keyword(s):

Crystalline Lens

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ON THE CLEAVAGE PRODUCTS OF THE CRYSTALLINE LENS

Journal of Biological Chemistry ◽

10.1016/s0021-9258(18)85913-0 ◽

1922 ◽

Vol 51 (1) ◽

pp. 155-164

Author(s):

Yoshizumi Hijikata

Keyword(s):

Crystalline Lens

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Subtle changes of the crystalline lens after cycloplegia: a retrospective study

BMC Ophthalmology ◽

10.1186/s12886-021-01884-9 ◽

2021 ◽

Vol 21 (1) ◽

Author(s):

Cheng Dai ◽

Meng Liu ◽

Xiaodong Lv ◽

Binzhong Li

Keyword(s):

Retrospective Study ◽

Children And Adolescents ◽

Population Change ◽

Anterior Segment ◽

Anterior Chamber Depth ◽

Crystalline Lens ◽

Equivalent Diameter ◽

Crystalline Morphology ◽

Significant Difference ◽

Before And After

Abstract Background The purpose of this study was to evaluate the shape of the crystalline lens in terms of biometry and diopters before and after cycloplegia using the CASIA2 swept-source (SS) optical coherence tomography (OCT) system on the anterior segment. Methods This was a retrospective study. Children and adolescents (26 males and 29 females, aged 4–21 years) with simple ametropia were selected for optometry and CASIA2 imaging at 2 separate visits before and after cycloplegia. Diopter values were derived from the spherical power (S) obtained by optometry. Biometric parameters of the crystalline lens, including the anterior chamber depth (ACD), anterior and posterior curvature of the lens (ACL and PCL), lens thickness (LTH), lens decentration (LD), lens tilt (LT), and equivalent diameter of the lens (LED), were measured by the CASIA2 system. The differences in these parameters after compared with before cycloplegia were determined, and their relationships were analyzed. Results Fifty-five participants (106 eyes) were initially enrolled. There was a significant difference (P < 0.05) in the S (t=-7.026, P < 0.001), ACD (t=-8.796, P < 0.001), ACL (t=-13.263, P < 0.001) and LTH (t = 7.363, P < 0.001) after compared with before cycloplegia. The change in the PCL (t = 1.557, P = 0.122), LD (t = 0.876, P = 0.383), LT (t = 0.440, P = 0.661) and LED (t=-0.351, P = 0.726) was not statistically significant (P > 0.05). There was a significant (P < 0.05) correlation of the change in the S with that in the ACL (r = 0.466, P < 0.001), LTH (r=-0.592, P < 0.001), and LED (r = 0.223, P = 0.021) but not the PCL (r = 0.19, P = 0.051), LD (r=-0.048, P = 0.0628) or LT (r=-0.022, P = 0.822). Furthermore, the change in the ACD was closely related to the change in crystalline morphology. However, in children and adolescents, we found that the change in crystalline morphology was unrelated to age. Conclusions Changes in lens morphology after compared with before cycloplegia are mainly related to the ACL and LTH, but there is no difference in the PCL, LD, LT, or LED. In the adolescent population, change in the S is related to change in the ACL, LED and LTH. However, age is unrelated to the shape and tendency of the crystalline lens. Further research is required to determine whether the same conclusion applies to different age groups and different refractive states (myopia, hyperopia, emmetropia) .

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The simply modified intrascleral fixation using round flange (SMURF) technique for intrascleral intraocular lens fixation

Scientific Reports ◽

10.1038/s41598-021-81924-y ◽

2021 ◽

Vol 11 (1) ◽

Author(s):

Soa Kim ◽

Jee Taek Kim

Keyword(s):

Intraocular Lens ◽

Invasive Procedure ◽

Crystalline Lens ◽

Early Postoperative Period ◽

Cystoid Macular Oedema ◽

Modified Technique ◽

Intraocular Pressure Elevation ◽

Wound Leakage ◽

Iol Dislocation

AbstractWe describe a simply modified intrascleral fixation using round flange (SMURF) technique and report the clinical outcomes of the surgery. Forty-one eyes of 41 consecutive patients, with intraocular lens (IOL) dislocation, crystalline lens subluxation, and zonular weakness, who underwent surgery using the SMURF technique were included. The modified technique included the use of a conventional 27-gauge needle, a non-bent needle, oblique sclerotomy, direct threading of the leading haptic, and simple placement of the following haptic. IOLs were successfully placed and showed good centring. There were no cases of wound leakage or hypotony during the early postoperative period. Postoperative complications included vitreous haemorrhage in one eye (2.4%), intraocular pressure elevation in one eye (2.4%), and iris capture in six eyes (14.6%). There were no cases of postoperative retinal detachment, cystoid macular oedema, endophthalmitis, or IOL dislocation during the follow-up period. We proposed a few modifications in the intrascleral flanged technique for IOL fixation. The modified technique is a simple, easy, and minimally invasive procedure for successful IOL intrascleral fixation.

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Lens and Peripheral Retinal Relationships During Vitrectomy: Comparison of 23-, 25-, and 27-Gauge Vitrectomy and Curved Endolaser Probes

Journal of VitreoRetinal Diseases ◽

10.1177/2474126420971979 ◽

2020 ◽

pp. 247412642097197

Author(s):

Jacob S. Duker ◽

Michael J. Venincasa ◽

Pedro F. Monsalve ◽

Armando L. Garcia ◽

Sander R. Dubovy ◽

...

Keyword(s):

Insertion Site ◽

Crystalline Lens ◽

Peripheral Retina ◽

Small Gauge ◽

The Mean ◽

Deceased Donors ◽

23 Gauge ◽

Considerable Range ◽

Safe Access ◽

Phakic Eyes

Purpose: This work aims to compare spatial relationships between the crystalline lens and vitrectomy instruments of different gauges. Methods: Eight phakic eyes recovered from deceased donors were used after fixation. Valved trocars (27-gauge, 25-gauge, and 23-gauge) were sequentially placed in the superotemporal quadrant 4 mm posterior to the limbus in each eye. Intraocular relationships of vitrectomy and curved endolaser probes were measured for each gauge. Results: There were no significant differences in maneuverability between instruments of different gauges. The mean distance from instrument to lens at the geometric center of the globe was 5.5 mm. Vitrectomy probes of all gauges could access the peripheral retina on both sides of the sclerotomy in the 3 to 4 o’clock position adjacent to the sclerotomy without touching the lens. The instruments could be advanced without lens touch to contact the retina within at least 2 mm of the ora serrata 180° away from the insertion site. Conclusions: Vitrectomy and curved endolaser probes achieved similar maneuverability relative to the lens regardless of gauge. This study confirms that small-gauge vitrectomy instruments have a considerable range of safe access to the peripheral retina in phakic eyes from a single sclerotomy.

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