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Saudi Journal of Ophthalmology :... 2022To report the surgical outcome of early lens aspiration, posterior chamber intraocular lens (PC IOL), and capsular tension ring (CTR) in a case series of...
PURPOSE
To report the surgical outcome of early lens aspiration, posterior chamber intraocular lens (PC IOL), and capsular tension ring (CTR) in a case series of microspherophakia (MSP) and secondary glaucoma.
METHODS
Case series of 18 eyes of MSP cases presented with lenticular myopia and secondary glaucoma that underwent early lens aspiration, PC IOL and CTR by one ophthalmologist. Baseline, long-term postoperative outcomes and complications were documented.
RESULTS
All cases underwent successful surgery with lens aspiration PC IOL implantation and CTR insertion without intraoperative complications. One of the 18 cases was a delayed referral which had broad anterior synechiae and following lens aspiration developed corneal decompensation. In one eye, CTR implantation was not possible hence, lens aspiration with scleral fixation (SF) of 3 piece IOL was performed (excluded from the analysis). Overall there was an improvement in visual acuity (from 0.3 ± 0.1 to 0.2 ± 0.2 LogMar, = 0.006), intraocular pressure (IOP), and most notably, deepening of the anterior chamber. Some cases required subsequent glaucoma surgery to control IOP. After a long duration of follow-up, all cases had stable capsular lens complex and no capsular phimosis.
CONCLUSION
Early Lens aspiration with CTR and PCIOL alone in MSP with lens subluxation has a significant impact on the patient's quality of vision, deepening the anterior chamber and preventing complications or poor outcomes. In addition, good capsular-lens complex stability and absence of capsular phimosis or phacodonesis on long-term follow-up were obtained.
PubMed: 36211322
DOI: 10.4103/sjopt.sjopt_231_21 -
Frontiers in Medicine 2021To investigate the lens biometric parameters in congenital lens deformities, using a novel technique of swept-source anterior segment optical coherence tomography...
To investigate the lens biometric parameters in congenital lens deformities, using a novel technique of swept-source anterior segment optical coherence tomography (SS-ASOCT). This prospective study included patients with microspherophakia (MSP), coloboma lentis (CL), and posterior lenticonus (PL). For this cohort, 360-degree high-resolution lens images were obtained using the latest SS-ASOCT (CASIA2, Tomey Corp, Nagoya, Japan). The lens biometric parameters were calculated by the CASIA2 built-in software for anterior lens radius (ALR), posterior lens radius (PLR), anteroposterior distance (APD), anterior chamber depth (ACD), equatorial diameter (Eq Dia), rear projection length (RPL), and maximum diameter of the lesion (MDL). This study included two eyes each with MSP and CL and one eye with PL. The lens of MSP was spherical and posteriorly dislocated, with decreased ALR and PLR, Eq Dia, but increased APD. In patients with CL, the coloboma was isolated, bilateral, inferior, and located toward the maldeveloped ciliary body. High astigmatism was mainly lenticular, and this was calculated by the ALR and PLR. Regarding the site of coloboma, a significant decrease in ALR was observed, while the PLR and APD were not affected. The PL eyes had a cone-shaped protrusion of the posterior lens surface with a subtle cataractous region around the apex. An extremely high posterior surface curvature was observed with a mean PLR of 1.67 mm. The RPL and MDL were about 1.80 and 0.4 mm, respectively, which were homogenous at different sections. The CASIA2 is a valuable option for crystalline lens measurement for congenital lens deformities, enabling the accurate diagnosis and providing illuminating insights into the pathogenesis of MSP, CL, and PL.
PubMed: 34988093
DOI: 10.3389/fmed.2021.774640 -
Medicine Aug 2020Microspherophakia is characterized by a small, spherical crystalline lens with increased sagittal diameter. Because of the uncertainty about the outcome, as well as the...
RATIONALE
Microspherophakia is characterized by a small, spherical crystalline lens with increased sagittal diameter. Because of the uncertainty about the outcome, as well as the complexity of the operation and development of complications, the management and timing of surgical intervention for microspherophakia are still debated. Lens extraction is effective for avoiding the risk of pupillary blockage, but the outcome after operation is controversial. The iTrace (Tracey, USA) report shows the influence of low-order aberrations (LOA) and high-order aberrations (HOA), which may be valuable in predicting postoperative outcome. Our report concerns a child with microspherophakia who underwent lens extraction via the analysis of visual quality by iTrace.
PATIENT CONCERNS
Our report is on the case of a 7-year-old girl whose parents observed she had to bring her papers and books extremely close to her face to read. On examination, the girl was bilaterally diagnosed microspherophakia with a small tremble lens. The objective refraction was -15.0 diopter of spherical power (DS)/-1.00 diopter of cylindrical power (DC) × 180 right eye (OD) and -12.5 DS/-1.50 DC × 20 left eye (OS). The HOA of OD and OS were high up to 0.926 and 0.659, respectively by iTrace. The visual quality remained terrible after correcting LOA (high myopia and astigmatism). According to iTrace report, the patient would get a good visual quality by extracting the clear lens with HOA from cornea after correcting LOA. The girl's parent opted for surgery on the left eye.
DIAGNOSIS
Due to the patient's symptoms, examination results, she was diagnosed with microspherophakia.
INTERVENTIONS
The patient underwent clear lens extraction by ultrasonic phacoemulsification and intraocular lens implantation.
OUTCOMES
The first day after operation, total HOA was decreased to 0.077. Total LOA was 0.713. Corrected distance visual acuity (CDVA) is 20/20. One week after surgery, HOA was 0.110 and LOA was 0.328. CDVA was 20/25. CDVA was still 20/25 one month after surgery. The total HOA was 0.110 and the LOA was 0.334 by iTrace.
LESSONS
ITrace not only plays an important role in analyzing potential reasons of undesirable preoperative visual quality but also can predict postoperative outcomes. All these functions are helpful for determining surgical intervention of microspherophakia cases.
Topics: Aberrometry; Astigmatism; Child; Corneal Diseases; Corneal Topography; Ectopia Lentis; Female; Glaucoma; Humans; Iris; Lens Implantation, Intraocular; Myopia; Phacoemulsification; Treatment Outcome; Ultrasonics; Visual Acuity
PubMed: 32846863
DOI: 10.1097/MD.0000000000021937 -
Arquivos Brasileiros de Oftalmologia 2023To evaluate the stability and efficacy of the double-flanged 5-0 polypropylene suture to fixate subluxated cataracts at 18 months and the possible complications of this...
PURPOSE
To evaluate the stability and efficacy of the double-flanged 5-0 polypropylene suture to fixate subluxated cataracts at 18 months and the possible complications of this new technique.
METHODS
This technique uses a 5-0 polypropylene monofilament to create two flanges with a thermocautery, for fixation of a capsular tension segment to the sclera to fix the subluxated capsular bag. This technique was implemented in 17 eyes requiring intraocular lens implantation in a setting of zonular dialysis due to trauma, Marfan syndrome, microspherophakia, idiopathic disease, and post-phacoemulsification status.
RESULTS
Follow-up of the patients occurred at 18 months. Best-corrected visual acuity improved significantly from 0.85 to 0.39 (logMAR), whereas the spherical and cylindrical refractive errors and intraocular pressure remained stable from preoperation. No suture photodegradation or pseudophacodonesis were detected.
CONCLUSION
The double-flanged 5-0 polypropylene suture transscleral bag fixation technique has shown favorable long-term outcomes in terms of bag intraocular lens/complex fixation and stability. In eyes with zonular weakness or dialysis, this technique appears to be a safe and knotless option for cataract surgery.
Topics: Humans; Polypropylenes; Lens Capsule, Crystalline; Cataract Extraction; Cataract; Lenses, Intraocular; Sclera; Suture Techniques; Retrospective Studies
PubMed: 35417514
DOI: 10.5935/0004-2749.20230022 -
International Journal of Ophthalmology 2022To evaluate the safety and efficacy of scleral-fixated 3-looped haptics intraocular lens (IOL) implantation for surgical management of microspherophakia.
AIM
To evaluate the safety and efficacy of scleral-fixated 3-looped haptics intraocular lens (IOL) implantation for surgical management of microspherophakia.
METHODS
A retrospective case series include 10 microspherophakic patients (15 eyes) who underwent lens removal plus a modified surgical treatment of scleral-fixated 3-looped haptics IOL implantation. The primary outcomes involved visual acuity, intraocular pressure (IOP). Secondary outcomes were spherical equivalent (SE), anterior chamber depth (ACD), corneal endothelial cell density and postoperative complications.
RESULTS
After a postoperative follow-up of 17.60±15.44mo, improved visual outcomes can be observed. The uncorrected distance visual acuity (UCVA) logMAR improved from 1.54±0.59 preoperatively to 0.51±0.35 postoperatively (=0.001), and best corrected visual acuity (BCVA) logMAR improved from 0.97±0.91 preoperatively to 0.24±0.23 postoperatively (=0.003). Moreover, the SE decreased from -9.58±7.47D preoperatively to -0.65±2.21 D postoperatively (<0.001). In terms of safety profile, the average IOP decreased from 21.10±12.94 mm Hg preoperatively to 14.03±3.57 mm Hg postoperatively (=0.044), and the previously elevated IOP of three eyes decreased to the normal range. The ACD increased from 2.25±1.45 mm preoperatively to 3.35±0.39 mm postoperatively (=0.017). The density of corneal endothelial cells did not change significantly after surgery (=0.140). The posterior chamber IOLs were well centered and no severe complications were found.
CONCLUSION
Lens removal plus the modified surgical treatment of scleral-fixated 3-looped haptics IOL implantation can help in improvement of visual acuity, which can be regarded as a relative safe method for the surgical management of microspherophakia.
PubMed: 36536984
DOI: 10.18240/ijo.2022.12.08 -
The American Journal of Case Reports May 2021BACKGROUND Short stature is the second most common reason for referral to a pediatric endocrinology clinic. Numerous genetic causes have been identified.... (Review)
Review
BACKGROUND Short stature is the second most common reason for referral to a pediatric endocrinology clinic. Numerous genetic causes have been identified. Weill-Marchesani syndrome (WMS) is one of the rare genetic disorders that cause short stature. It is caused by homozygous mutations in the FBN1 gene, ADAMTS10 gene, ADAMTS17 gene, or LTBP2 gene. Despite genetic heterogeneity, WMS is clinically homogeneous. It is characterized by short stature, brachydactyly, joint stiffness, ocular abnormalities, mainly microspherophakia and glaucoma, and occasionally cardiac defects. CASE REPORT A 9-year-old boy had bilateral narrow-angle glaucoma with lens subluxation, elevated intraocular pressure, and severe myopia since early childhood. He had phenotypic dysmorphic features and radiological findings consistent with WMS. He underwent lensectomy and scleral-fixated intraocular lens implantation as well as drug treatment to control the intraocular pressure. He was a slow grower, and his growth parameters showed disproportionate short stature with brachydactyly and joint stiffness. Growth hormone provocation tests were subnormal with a peak value of 7.89 ng/mL. CONCLUSIONS The constellation of clinical presentation, radiological findings, and the molecular examination confirmed a homozygous familial variant of the ADAMTS10 gene identified by carrier gene testing. This known familial variant creates a premature termination codon classified as a likely pathogenic cause of WMS. In this syndrome, glaucoma treatment is considered the greatest challenge. The disease-causing mechanism in WMS is not known but thought to be due to abnormal actin distribution and organization in fibroblasts as a result of impaired connections between extracellular matrix components and the cytoskeleton.
Topics: ADAMTS Proteins; Child; Child, Preschool; Dwarfism; Ectopia Lentis; Glaucoma; Homozygote; Humans; Latent TGF-beta Binding Proteins; Male; Weill-Marchesani Syndrome
PubMed: 34057920
DOI: 10.12659/AJCR.930824 -
Eye (London, England) Sep 2019To assess the outcomes of intra-lenticular lens aspiration (ILLA) in paediatric cases with anterior dislocation of lens.
PURPOSE
To assess the outcomes of intra-lenticular lens aspiration (ILLA) in paediatric cases with anterior dislocation of lens.
METHODS
A retrospective review of medical records of cases with anterior dislocation of the lens in children (age < 16 years) that underwent ILLA between June 2017 and May 2018 was performed. Corrected distance visual acuity (CDVA), intraocular pressure (IOP), and anterior segment findings were noted at presentation and follow-up. Surgical notes were reviewed for all cases. Post-operative central corneal thickness (CCT) and central macular thickness (CMT) were recorded.
RESULTS
Eleven eyes of eight patients with a median age of ten years underwent ILLA. There were four males and four females. The median duration of symptoms was 2 months, CDVA was 1.77 logMAR, and IOP was 16 mm of Hg. Ten eyes had corneo-lenticular touch with corneal oedema, and two had raised IOP at presentation. Homocystinuria (n = 2/8), Microspherophakia (n = 2/8), Marfan syndrome (n = 1/8), Buphthalmos (n = 1/8) and Ectopia lentis et pupillae (n = 1/8) were the identifiable causes for anterior dislocation. There were no intra-operative complications in any case. Immediate post-operative corneal oedema and raised IOP was observed in nine and three cases respectively and was treated with medical therapy. The median post-operative CDVA and IOP at 6-months was 1 logMAR and 15 mm of Hg respectively. The median CCT and CMT were 516 and 248 μm respectively. Five eyes developed a central corneal descemet scar.
CONCLUSIONS
ILLA is a safe and effective technique for surgical removal of an anteriorly dislocated lens in paediatric cases.
Topics: Adolescent; Anterior Eye Segment; Capsulorhexis; Child; Child, Preschool; Chondroitin Sulfates; Drug Combinations; Eyeglasses; Female; Humans; Hyaluronic Acid; Infant; Intraocular Pressure; Lens Subluxation; Male; Retrospective Studies; Suction; Tonometry, Ocular; Visual Acuity
PubMed: 30944461
DOI: 10.1038/s41433-019-0426-y -
American Journal of Ophthalmology Case... Jun 2022The purpose of this case report is to describe the utility of portable femtosecond laser platform in a child with bilateral isolated microspherophakia and ectopia lentis...
PURPOSE
The purpose of this case report is to describe the utility of portable femtosecond laser platform in a child with bilateral isolated microspherophakia and ectopia lentis performed under general anesthesia.
OBSERVATION
An appropriately sized and well centered capsulotomy with well centered PCIOL could be achieved in both eyes of the patient.
CONCLUSION & IMPORTANCE
The advent of mobile femtosecond laser assisted cataract surgery might prove a useful and convenient platform for surgeries in pediatric patients with subluxated cataracts even under general anesthesia. It may circumvent the need for an additional laser suite and reduce surgical time by eliminating the need for patient movement.
PubMed: 35313468
DOI: 10.1016/j.ajoc.2022.101442 -
American Journal of Ophthalmology Case... Dec 2022The purpose of this report is to describe a case of a patient with microspherophakia (MSP) who had a scleral rupture during a retinal detachment (RD) repair with primary...
PURPOSE
The purpose of this report is to describe a case of a patient with microspherophakia (MSP) who had a scleral rupture during a retinal detachment (RD) repair with primary scleral buckle and cryoretinopexy.
OBSERVATIONS
A 48-year-old woman with MSP presented with six days of expanding loss of vision and photopsias. Examination revealed a superior retinal detachment involving the macula associated with two superior retinal tears. The patient underwent successful placement of a segmental buckle. During cryoretinopexy treatment of the tears, a 4 mm full-thickness scleral rupture occurred. The sclera was immediately closed with interrupted 8-0 nylon sutures and reinforced with a processed pericardium allograft. Subsequent combined phacoemulsification with capsulectomy, zonulectomy, and pars plana vitrectomy with retinal reattachment was performed nine days post buckle placement.
CONCLUSIONS AND IMPORTANCE
This case illustrates that a patient with MSP, even observed in the absence of a genetic syndrome or familial condition, may be at increased risk of scleral rupture during RD repair. Though future investigations are necessary to confirm this association, surgeons should take a conservative approach by having a high clinical suspicion for compromised scleral integrity in patients with MSP and proceeding with caution in procedures that may pose a risk of scleral rupture. A pericardium allograft can be an effective adjunct for scleral rupture repair.
PubMed: 36393910
DOI: 10.1016/j.ajoc.2022.101746 -
Eye (London, England) Mar 2020
Topics: Corneal Diseases; Ectopia Lentis; Glaucoma; Humans; Iris
PubMed: 31391543
DOI: 10.1038/s41433-019-0544-6