Inside-out and outside-in: Tips and tricks in posterior lenticonus

Background: Posterior lenticonus is a congenital condition characterized by the thinning and bowing of posterior lenticular capsule. Cataract develops when normal intra?lenticular pressure bulges the posterior capsule at the circumscribed portion of inherent thin posterior lens capsule, leading to derangement of lamellar lens fibers. This condition poses a surgical challenge as the presentation varies from thin, bulging posterior capsule to large, pre?existing posterior capsular dehiscence. Purpose: This video highlights the tips for the surgical management of various scenarios of posterior lenticonus. Synopsis: In this video, tips for surgical management of various scenarios of posterior lenticonus are illustrated. Intraoperatively, ruptured posterior capsule is suspected by the presence of a fish?tail sign. The anterior capsulotomy is relatively easier in a flatter anterior lens capsule, owing to reduced intra?lenticular pressure. Hydro?dissection is avoided to prevent extension of pre?existing posterior capsular dehiscence or creation of rupture in a thinned?out capsule. The peripheral lens cortex aspiration is initiated first, followed by in each quadrant. In a presence of ruptured posterior capsule by vigilant inspection at this stage, the surgeon should change the direction of lens matter aspiration by aspirating the central lenticular matter first, followed by peripheral cortex like “inside?out”. In absence of posterior capsule dehiscence, peripheral cortex is aspirated first, followed by central lens matter aspiration like “outside?in”. Adequate anterior vitrectomy is performed until there are no vitreous tags. Highlights: In the presence of pre?existing posterior capsular defect, the lens matter is aspirated from the center (inside?out), whereas in the absence of capsular defect, the lens matter is aspirated from the periphery (outside?in).

Posterior optic capture of intraocular lens in difficult cases of pediatric cataract

We describe the technique of posterior optic capture without anterior vitrectomy in two difficult cases of pediatric cataract. We demonstrate how a three?piece foldable intraocular lens can be maneuvered behind the posterior capsule after an improvised posterior capsulotomy. This technique provided excellent intraocular lens (IOL) stability with the absence of lens epithelial cell proliferation in infants with altered posterior capsule morphology

Femtosecond laser-assisted cataract surgery in Alport's syndrome - A case report

Alport's syndrome with anterior lenticonus poses significant challenges during capsulorhexis with high chances of peripheral extension. We report a case of 23-year-old lady with Alport's syndrome with anterior lenticonus in the left eye that underwent successful femtosecond laser-assisted cataract surgery (FLACS). The anterior lenticonus was eccentric nasally; hence, the FLACS capsulotomy was positioned slightly nasally so that the capsulotomy margin was outside the cone base. The distance vision improved from 6/24 to 6/6 at one-month follow-up. In summary, FLACS improved the safety of cataract surgery in an eye with anterior lenticonus and yielded excellent visual outcomes.

Posterior lenticonus with persistent fetal vasculature

A 10 year old girl present with both eyes central cataract with posterior lenticonus. Intraoperative, she was noted to have both eyes persistent fetal vasculature (PFV). To the best of our knowledge, association of bilateral posterior lenticonus and PFV has not been reported before. This supports the hypothesis that PFV has a role in pathogenesis of posterior lenticonus.

Toric multifocal intraocular lens implantation in a case of bilateral anterior and posterior lenticonus in Alport syndrome.

We report the first case of toric multifocal intraocular lens (IOL) implantation in both the eyes of a young patient of Alport syndrome with anterior and posterior lenticonus with a successful outcome. An 18‑year‑old female patient presented with progressively blurred vision in both eyes since 4–5 years not improving with glasses. Refraction showed high myopia with astigmatism; however, the vision did not improve beyond 6/60 with glasses correction. Clinical examination on slit lamp showed anterior and posterior lenticonus bilaterally with a classical oil droplet appearance. We performed clear lens extraction by phacoemulsification with toric multifocal IOL implantation in both eyes. Postoperatively, the patient achieved an excellent refractive outcome with the unaided vision of 6/9, N6 in both eyes.

Multiple congenital ocular defects in a Bedlington terrier dog / 대한수의학회지

To report multiple congenital ocular defects in a Bedlington terrier dog aged 2.5 months with blindness. Routine ophthalmic examinations were performed for the clinical signs. Menace responses and cotton ball test were absent in both eyes (OU), but pupillary light reflexes were normal in OU. Slit lamp biomicroscopy reveled corneal dystrophy, posterior subcapsular cataract, microphthalmia in OU and lenticular coloboma in the right eye. In indirect ophthalmoscopy and ultrasonography, retinal detachment and posterior lenticonus were shown in OU. It is the first report of lenticular coloboma and posterior lenticonus in a Bedlington terrier dog.

Alport syndrome

Objective@#To present a case of Alport syndrome, its pathogenesis, etiology, clinical manifestation, diagnosis, and management.@*Methods@#This is a case report.@*Results@#A 22-year-old male presented with blurring of vision associated with bilateral anterior and posterior lenticonus. The patient had a history of blurring of vision on both eyes, occasional right lower-quadrant pain on urination, frothy urine, and bilateral hearing loss. Family medical history showed one brother who died at 15 years of heart disease, and another brother at 17 from chronic kidney disease. Slitlamp examination showed a conspicuous oil droplet reflex seen through retroillumination. Anterior and posterior bulging of the lens was noted, highly suggestive of anterior and posterior lenticonus. On indirect ophthalmoscopy, no perimacular dot-and-fleck retinopathy was seen. Further systemic workup revealed elevated serum levels of blood urea nitrogen (BUN) and creatinine, and marked proteinuria and hematuria. Ultrasound of the kidneys revealed bilateral renal parenchymal disease. Pure tone audiometry confirmed bilateral moderate sensorineural hearing loss. @*Conclusions@#There should be a high index of suspicion for Alport syndrome in any patient presenting with anterior and posterior lenticonus. A thorough history-taking and physical examination, including slitlamp examination through a dilated pupil, are necessary to fully support its diagnosis. There is no specifically defined treatment for Alport’s syndrome; management should be individualized and approached in a multidisciplinary fashion. Lenticonus can be treated by phacoemulsification with careful capsulorrhexis.

Ocular manifestations of Alport syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

·AIM: To analyze the clinical manifestation of Alport syndrome, especially the ocular features.·METHODS: The physical, ophthalmologic and audiologic examination results of thirty two patients with Alport syndrome were analyzed retrospectively.·RESULTS: Thirty (93.7%) patients had some family history. All patients had renal disease: eighteen(56.3%) patients with chronic renal failure, four(12.5%) patients with renal insufficiency, and the other ten(31.3%) patients with hematuria. Twenty (62.5%) patients had sensorineural deafness. Thirteen (40.6%) patients had ocular deformity, five(15.6%) patients had typical ocular changes: three patients with anterior lenticonus, and two patients with macular flecks.·CONCLUSION: Ocular anomalies are not requisite for the diagnosis of Alport syndrome. But its typical ocular features should be recognized by the ophthalmologists which supports the diagnosis.

The Surgical Management of Posterior Lenticonus Associated with Pediatric Cataract

PURPOSE: To evaluate clinical characteristics of posterior lenticonus associated with pediatric cataracts and to identify the factors associated with final visual acuities after cataract surgery. METHODS: Thirty-eight patients (46 eyes) who were diagnosed with posterior lenticonus preoperatively or during the operation were involved in this study. We investigated the age at diagnosis, the severity of lens opacity, the pre- and postoperative best corrected visual acuity, and the effectiveness of amblyopia treatment. RESULTS: The mean age at presentation was 39.0+/-37.4 months, and the mean follow-up period was 42.8+/-33.5 months postoperatively. Nine patients (23%) had bilateral posterior lenticonus, and the mean age of bilateral cases was lower (p<0.001) and the lens opacity more severe than those of unilateral cases (P=0.001). Fifteen eyes (32.6%) were surgically identified as having posterior lenticonus during lens aspiration, the mean age of surgically identified cases was lower (p<0.001) and the lens opacity more severe then those of preoperatively diagnosed cases (P=0.014). The age at initial examination was negatively correlated with final visual acuities in log MAR (P=0.04). CONCLUSIONS: Bilateral and surgically identified posterior lenticoni were associated with younger age and more severe lens opacity. The final visual acuities were better in patients with late onsets of lens opacities.

Visual outcome after phacoemulsification with intraocular lens implantation in patients with anterior lenticonus due to Alport syndrome / 国际眼科杂志(Guoji Yanke Zazhi)

· AIM: We describe visual improvement after phacoemulsification with intraocular lens (IOL) implantation in 7 eyes with anterior lenticonus secondary to Alport syndrome.· METHODS: 7 eyes from 4 patients with Alport syndrome underwent phacoemulsification with IOL implantation due to anterior lenticonus.· RESULTS: All of the patients resumed excellent visual acuity after surgery.· CONCLUSION: We recommend the phacoemulsification with IOL implantation as a safe procedure in anterior lenticonus due to Alport syndrome.

Anterior Lens Capsule Abnormalities in Alport Syndrome

Alport syndrome is a hereditary, progressive disease characterized by progressive nephritis, sensorineural deafness, and ocular abnormalities, including anterior lenticonus. The ultrastructure of the lens capsule abnormalities in Alport syndrome is reported. Four anterior lens capsules from 31-year-old patient and 26-year-old patient with lenticonus who were affected by the Alport syndrome were obtained at capsulectomy. And all four anterior lens capsules were examined by transmission electron microscopy. The histopathologic findings showed that the thickness of the anterior lens capsules was decreased (4~13 micrometer) and that there were many vascular dehiscences localized at the inner part of the lens capsule. There were large numbers of capsular dehiscences containing fibrillar materials and vacuoles. The anterior capsules were clearly fragile in this disease, forming the basis for the progressive lenticonus and anterior polar cataract.

A Case of Anterior Lenticonus in Alport's Syndrome

Alport's syndrome, first described by Alport in 1927, is a clinical entity which consists of lens abnormalities, hereditary hemorrhagic nephritis and nerve deafness. Anterior lenticonus is a rare structual anomaly of lens in which there is a conicalor spherical protrusion of the anterior lens surface into the anterior chamber as a characteristic finding of Alport's syndrome. The authers experienced a case of Alport's syndrome in a 22-year-old man associated with bilateral anterior and posterior lenticonus, hematuria, proteinuria, renal failur earld nerve deafness(complete form).