UNASSIGNED: To describe the use of FLACS (Femtosecond-Laser-Assisted Cataract Surgery) and pupiloplasty technique employed in a cataract surgery associated with iris-lens-zonule coloboma, as well as to report the advantages that FLACS can provide in this type of complicated surgery.
UNASSIGNED: During FLACS (Victus® - TECHNOLAS, Bausch and Lomb Incorporated, USA), after the docking procedure, the parameters of capsulotomy, fragmentation and incisions were manually adjusted. Iris retractors were anchored to the edge of the anterior lens capsule to provide stability to the bag during phacoemulsification maneuvers, and a capsular tension ring and intraocular lens (IOL) were implanted. Iris repair was approached using a modification of the slip-knot technique. For this purpose, a 10.0 Prolene on a straight needle was used and knotted extraocularly, over the conjunctiva. Finally, an anterior vitrectomy was performed as a precaution.
UNASSIGNED: In spite of the existing controversy regarding FLACS technology compared to conventional surgery, it seems to be beneficial in complicated cases such as cataracts associated with iris and lens coloboma, since it allows the modification of different parameters that facilitate and ensure surgery safety. On the other hand, the iris repair technique described facilitates manipulation of the iris sutures. In addition, complementary techniques such as the use of a capsular tension ring and pupiloplasty help to center and stabilize the IOL, and to reduce the symptoms derived from iris coloboma, obtaining a better visual quality.

Marfan\'s syndrome (MFS) is an autosomal dominant connective tissue disorder with defect in the fibrillin-1 gene. The most common ocular manifestation is subluxated lens in the superotemporal direction, accounting for 50%-85% of total cases. The association of lens coloboma with MFS has been described in literature, but the coexistence of lens coloboma with ectopia lentis is a rare feature. Here, we describe three cases of MFS including a case of bilateral lens coloboma with ectopia lentis: case 1 - a 39-year-old male with inferotemporal lens subluxation in the right eye and superotemporal lens subluxation in the left eye with open-angle glaucoma and high myopia, case 2 - a 15-year-old child with bilateral superonasal lens subluxation with lens coloboma, and case 3 - a 56-year-old female with bilateral lens coloboma. Case 1 and case 2 had clear lenses with good refractive correction; hence, they were optically rehabilitated with contact lenses, whereas case 3 was advised for cataract surgery. It is important to distinguish the lens coloboma from a more common entity, ectopia lentis as former usually remains stable while the latter might need a surgical intervention.

A ten-year-old male child was referred with complaints of blurring of vision and deviation of the eye. On examination, the right eye has an esodeviation squint with a best corrected visual acuity of 6/60 Snellen\'s acuity and 6/6 Snellen\'s acuity in the left eye. Slit-lamp biomicroscope of the right eye showed coloboma at the 9 o\'clock position with cataract. The rest of the anterior and posterior segments was normal in both eyes. Thus, a diagnosis of unilateral lens coloboma with amblyopia was made.

Congenital rubella syndrome (CRS) may affect all ocular structures in general, either in isolation or in combination. Typical ocular complications in CRS include cataracts, microcornea, microphthalmia, glaucoma, nystagmus, and retinopathy. We report a case of a four-year-old girl who presented with bilateral total cataracts with sensory nystagmus, a poorly dilating pupil, iris hypoplasia, high axial myopia in the right eye (RE), and a retinal detachment in the left eye (LE). The systemic evaluation revealed microcephaly with an associated patent ductus arteriosus (PDA) and mild pulmonary arterial hypertension (PAH). Based on these findings, the child was diagnosed with clinically confirmed CRS. The child was taken up for right-eye cataract surgery. Intra-operatively, a lens coloboma involving the temporal equator of the lens along with a highly tessellated fundus was noted. Two weeks post-cataract surgery, the child had a best corrected visual acuity (BCVA) of 6/24. However, four weeks after the surgery, the child developed total rhegmatogenous retinal detachment in the right eye, for which pars plana vitrectomy with endolaser and silicon oil tamponade was done. Four weeks later, the child\'s BCVA in the right eye was 6/36, which was maintained until the last follow-up of four months. Lens coloboma may be isolated or may occur in association with chorioretinal coloboma. Ours is the first case of unilateral atypical lens coloboma associated with high myopia and bilateral cataracts in a patient with congenital rubella syndrome. Lens colobomas with high myopia have not been reported previously as ocular associations of CRS. Our case highlights that in children with CRS presenting with unilateral or bilateral congenital cataracts, the possibility of lenticular coloboma as a coexistent association should be kept in mind while taking these cases for cataract surgery.

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Lens colobomas extending more than 4 clock hours and causing visual impairment require lens extraction along with capsular support devices with scleral fixation for adequate centration of the capsular bag and for prevention of capsular fornix aspiration with inadvertent extension of zonular dialysis intraoperatively. In this case series, we describe a technique for the management of isolated lens colobomas involving 4-5 clock hours by clear lens extraction and intraocular lens implantation using a combination of a capsular tension ring with a capsular tension segment (CTS) for the centration and stability of the capsular bag. Hoffman\'s corneoscleral pocket and half-bow sliding knot technique were used for scleral fixation of the CTS.

BACKGROUND: Marfan syndrome (MS), a connective tissue disorder that affects ocular, skeletal, and cardiovascular systems, is caused by heterozygous pathogenic variants in FBN1. To date, over 1800 different pathogenic variants have been reported.
METHODS: In the present study, FBN1 sequence analysis was performed in a family and two unrelated patients with MS.
RESULTS: Three novel pathogenic variants were detected. Two of these variants [c.6610T>C; p.(Cys2204Arg) and c.1956T>G; p.(Cys652Trp)], which affect a cysteine residue, were associated with MS with ectopia lentis, whereas the mutation causing a premature stop codon [c.2506delA; p.(Ser836ValfsX10)] leads to a classical MS of a milder phenotype.
CONCLUSIONS: We anticipate that the three novel pathogenic variants identified in this study will provide further support for the clinical relevance of variants in the large FBN1 gene.

A rare case of bilateral ectopia lentis with isolated lens coloboma in Marfan syndrome is reported. A 21-year-old female presented with decreased vision in both eyes. Her unaided visual acuity was 20/200 and 20/400 in the right and left eye, respectively, improving to 20/40 with -4.5 DS/-3.0 DC x 10° correction in the right eye and 20/80 with -10.0 DS/-6.5 Dc x10° correction in the left eye. On slit lamp examination under mydriasis, both eyes revealed ectopia lentis with lens coloboma and stretched zonules. Fundus examination revealed pigmentary changes at the fovea. On systemic evaluation, she was diagnosed with Marfan syndrome. She was prescribed a refractive correction in form of a contact lens and kept under observation.

OBJECTIVE: The aim of this study is to describe the management of cataract and refractory glaucoma in a case of congenital aniridia (AN)-1.
METHODS: In an 18-year-old female patient affected by congenital AN, bilateral coloboma of the zonula and lens, cataract and glaucoma, pars plana vitrectomy, cataract extraction and subsequent transcorneal ciliary body argon laser photocoagulation were performed.
RESULTS: Five years after laser treatment, the best-corrected visual acuity was 20/50 and the intraocular pressure was stable at 18 mm Hg with topical pharmacological management consisting of timolol 0.5% and latanoprost 0.005%.
CONCLUSIONS: From our experience, transcorneal ciliary body photocoagulation after cataract extraction and vitrectomy could be a useful technique to manage high ocular pressure in eyes affected by congenital AN.

A lens coloboma is not a true coloboma; it is just a zonular absence that causes a defect in the lens equator and hence a more spherical lens. It can be isolated or in association with iris, choroid or retinal colobomas. Typically the defect is present at the site of the embryonic fissure, but it can be present elsewhere. This congenital defect can cause amblyopia, especially when it is associated with cataract. We describe a case of a 39-year-old male with an atypical coloboma that was managed successfully with phacoemulsification, capsular tension ring and intraocular lens implantation. Scheimpflug image analysis and full ophthalmological assessment were performed. Scheimpflug images demonstrated a notching of the lens equator and an absence of zonulae. During the surgical procedure an ophthalmic viscosurgical device was used to tamponade the vitreous, and after continuous curvilinear capsulorhexis the capsular tension ring was inserted to allow the surgeon to perform a safe phacoemulsification.