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Indian Journal of Ophthalmology Jul 2022Microspherophakia is a rare developmental abnormality of the crystalline lens with a myriad of ocular and systemic associations. Glaucoma is a serious complication... (Review)
Review
Microspherophakia is a rare developmental abnormality of the crystalline lens with a myriad of ocular and systemic associations. Glaucoma is a serious complication associated with this disorder. Early identification of the disease, timely visual rehabilitation, and appropriate management of the lens and glaucoma can help us prevent blindness from this condition. Multidisciplinary care with lifelong follow-up is recommended, as this typically affects the younger population. Current treatment protocols for this condition are mainly based on case reports and retrospective studies with shorter follow-up. Due to the rarity of this disease, designing a large randomized controlled trial to identify the merits and demerits of each management strategy is challenging. With cataract, glaucoma, and vitreoretinal specialists, each having their preferred way of managing microspherophakic lenses, we decided to do a comprehensive review of the existing literature to devise an integrated approach toward effective management of these patients. This review will collate all evidence and provide a very practical decision-making tree for its management.
Topics: Corneal Diseases; Ectopia Lentis; Glaucoma; Humans; Iris; Randomized Controlled Trials as Topic; Retrospective Studies; Visual Acuity
PubMed: 35791105
DOI: 10.4103/ijo.IJO_2888_21 -
Taiwan Journal of Ophthalmology 2023The crystalline lens is an important structure in the eye that starts to develop as early as the 22 day of gestation, with further differentiation that continues after... (Review)
Review
The crystalline lens is an important structure in the eye that starts to develop as early as the 22 day of gestation, with further differentiation that continues after the induction. Congenital anomalies of the lens may involve the size, shape, and position of the lens. They may sometimes be associated with anterior segment dysgenesis or persistence of the tunica vasculosa lentis and hyperplastic vitreous and hyaloid system. Manifestations of anomalies of the lens shape are usually seen in early or late childhood however may sometimes be delayed into adulthood based on the level of visual impairment or the presence or absence of any syndromic associations. While lens coloboma has more often been reported in isolation, the more commonly implicated genes include the PAX6 gene, lenticonus in particular anterior is often part of Alport syndrome with extra-ocular manifestations in the kidneys and hearing abnormalities due to mutations in the alpha 5 chain of the Type IV collagen gene. Recognition of these manifestations and obtaining a genetic diagnosis is an important step in the management. The level of visual impairment and amblyopia dictates the outcomes in patients managed either conservatively with optical correction as well as surgically where deemed necessary. This review discusses the various anomalies of the lens shape with its related genetics and the management involved in these conditions.
PubMed: 38249493
DOI: 10.4103/tjo.TJO-D-23-00076 -
American Journal of Ophthalmology Case... Dec 2020To report a case of bilateral spontaneous filtering bleb, scleral thinning, microspherophakia, and mild craniofacial dysmorphism.
PURPOSE
To report a case of bilateral spontaneous filtering bleb, scleral thinning, microspherophakia, and mild craniofacial dysmorphism.
OBSERVATIONS
An 18-year-old girl was referred to our clinic for evaluation of bilateral spontaneous filtering blebs. Her corrected distance visual acuity (CDVA) was 20/400 in the right eye (RE) and 20/100 in the left eye (LE). She had superior scleral thinning, multicystic filtering bleb, and microspherophakia bilaterally. The IOP was 9 mmHg in the RE and 8 mmHg in the LE. A mild craniofacial dysmorphism including downward slanting of palpebral fissures and malar hypoplasia was present. There was no sign of corneal ectasia in tomography. Anterior segment optical coherence tomography revealed the filtering blebs as subconjunctival low reflective fluid-filled spaces. Due to severe scleral thinning un the RE we performed a tectonic scleral patch graft. 6 months after surgery the depth of the anterior chamber increased and CDVA improved.
CONCLUSION AND IMPORTANCE
This case indicated that the formation of spontaneous filtering blebs may occur in a syndromic condition. In any case with this symptom, special attention should be paid to craniofacial features, sclera, crystalline lens, and IOP. Tectonic scleral patch graft could be a valuable option in selected patients.
PubMed: 33020746
DOI: 10.1016/j.ajoc.2020.100948 -
BMJ Open Ophthalmology Jan 2023To study the visual, refractive and surgical outcomes of intraocular lens (IOL) implantation versus aphakia in children with microspherophakia. (Clinical Trial)
Clinical Trial
OBJECTIVE
To study the visual, refractive and surgical outcomes of intraocular lens (IOL) implantation versus aphakia in children with microspherophakia.
DESIGN
Retrospective, comparative, non-randomised interventional study.
METHODS
All consecutive children with microspherophakia who satisfied the inclusion criteria were included. The eyes that underwent in-the-bag IOL implantation and those that were left aphakic were included in groups A and B, respectively. The postoperative visual outcomes, IOL stability and complications during the follow-up period were studied.
RESULTS
22 eyes (13 patients, male 76%), of which 12 eyes were in group A and 10 eyes in group B. The mean±SE of age at surgery was 9.4±1.4 and 7.3±0.9 years in group A and group B, respectively (p value 0.18). The mean follow-up of group A was 0.9±0.4 years (median 0.5 years; Q1 0.04, Q3 2.16) and group B was 1.3±0.9 years (median 0.147 years; Q1 0.08, Q3 0.39) (p value 0.76). All the baseline biometric variables including best-corrected visual acuity (BCVA) were comparable in each group. The final BCVA in logMAR adjusted for follow-up was comparable in both group A (0.29±0.06) and group B (0.52±0.09) (p value 0.06). Mean predictive error of IOL power in microspherophakia was 0.17±0.43.The most common complication in group A was visual axis opacification of two eyes (16.7%, 95% CI 2.9% to 49.1%), of which one eye (8.3%, 95% CI 0.4% to 40.2%) needed membranectomy. Vitreous in anterior chamber was the most common complication in group B, seen in two eyes (20%, 95% CI 3.5% to 55.8%), of which one eye (10%, 95% CI 0.5% to 45.9%) underwent YAG laser vitreolysis. The survival analysis (p value 0.18) was comparable in each group.
CONCLUSION
In-the-bag IOL is an option, which can be considered in selected cases of microspherophakia in developing nations where regular follow-up and economic constraints are a major concern.
Topics: Humans; Child; Male; Lens Implantation, Intraocular; Aphakia, Postcataract; Retrospective Studies; Visual Acuity; Follow-Up Studies; Postoperative Complications; Joint Dislocations; Anterior Chamber
PubMed: 37278431
DOI: 10.1136/bmjophth-2022-001049 -
Journal of Current Ophthalmology 2023To report a rare case of microspherophakia and lens subluxation in a young patient with Klinefelter syndrome who underwent cataract surgery, emphasizing the importance...
PURPOSE
To report a rare case of microspherophakia and lens subluxation in a young patient with Klinefelter syndrome who underwent cataract surgery, emphasizing the importance of a thorough general health assessment and anamnesis prior to cataract surgery.
METHODS
The case concerns a 52-year-old male referred for phacoemulsification under general anesthesia. A review of literature was performed.
RESULTS
Preoperative assessment revealed a corticonuclear cataract in both eyes, with dislocation of the crystalline lens in the left eye in an area of zonular dehiscence. Upon careful examination, both eyes showed a microspherophakic lens with an increased lens thickness and the lens equator being visible over 360° in the left eye. The patient denied any trauma or medical conditions. His medical health record revealed the coexistence of Klinefelter syndrome (47, XXY). The association between Klinefelter syndrome and microspherophakia has only once been reported in the literature. Intraoperatively, a more cautious approach was withheld and a capsular tension ring was used. Postoperative outcome was successful with good visual outcome and no interoperative complications.
CONCLUSIONS
This case report highlights the importance of proper preoperative assessment before cataract surgery, especially in unusual cases such as early-onset cataract and/or lens subluxation. In addition, it stresses the importance of a systemic and/or genetic evaluation in patients with microspherophakia and an ophthalmological examination in patients with Klinefelter syndrome.
PubMed: 38250495
DOI: 10.4103/joco.joco_291_22 -
Indian Journal of Ophthalmology Oct 2022We herein describe the kissing MVR technique in cases of microspherophakia for safe and effective endocapsular lens aspiration. Microspherophakia is associated with...
We herein describe the kissing MVR technique in cases of microspherophakia for safe and effective endocapsular lens aspiration. Microspherophakia is associated with abnormally lax and broken zonules, which pose a great challenge to the operating surgeon, especially while creating the openings in the capsular bag. In this novel technique, simultaneous use of two 23 G MVR blades reduces the above problem associated with the severely mobile lens. Here, one MVR blade stabilizes the capsular bag and, at the same time, provides counter-traction for the opposite MVR blade while puncturing the capsular bag. Furthermore, the creation of openings in the lens at the equator or just posterior to it is beneficial as the capsule is relatively thicker and stronger at this location. This also minimizes the risk of premature extension to the anterior or posterior capsule, thereby avoiding complications like dropped lens matter, vitreous prolapse, and vitreous traction.
Topics: Corneal Diseases; Ectopia Lentis; Glaucoma; Humans; Iris; Lens Capsule, Crystalline; Lenses, Intraocular
PubMed: 36190071
DOI: 10.4103/ijo.IJO_588_22 -
Indian Journal of Ophthalmology Nov 2023Microspherophakia is a rare developmental lens anomaly with increased anteroposterior and reduced equatorial diameter. It presents with refractive myopia, shallow...
BACKGROUND
Microspherophakia is a rare developmental lens anomaly with increased anteroposterior and reduced equatorial diameter. It presents with refractive myopia, shallow anterior chamber, and angle closure glaucoma. It is associated with subluxated or dislocated lens, progressive myopia, defective accommodation, and glaucoma. Glaucoma is the most common vision-threatening complication and mostly requires surgical management that includes trabeculectomy, lensectomy, and drainage implant. A staged or combined procedure can be performed. The purpose of this video is to highlight the advantages of combining parsplana vitrectomy (PPV) with parsplana lensectomy (PPL), scleral fixated intraocular lens (SFIOL), and Aurolab aqueous drainage implant (AADI) in a young patient with advanced glaucoma and gross subluxation. Drainage implants are preferred over filtering surgeries in eyes undergoing vitreoretinal procedures due to the risk of bleb fibrosis and hypotony seen in the latter. The combined procedures should be tailored according to the lens status and severity of glaucoma in each patient.
PURPOSE
The purpose of this video is to illustrate a combined quadruple procedure (PPL, PPV, SFIOL, and AADI) in microspherophakic patients with unstable glaucoma and video-based skill transfer to a novice surgeon.
SYNOPSIS
This video illustrates quadruple surgery in a microspherophakic patient with secondary angle closure glaucoma. The authors also emphasize the advantages of a combined quadruple procedure over staged procedure or combined PPL with filtering procedure.
HIGHLIGHTS
Quadruple procedure can be performed in young microspherophakic patients with advanced glaucoma or at risk of progression and losing central vision along with gross subluxation of lens. It eliminates the need for multiple procedures, the risk of hypotony, and bleb-related complications.
VIDEO LINK
https://youtu.be/KdFjb7acXCI.
Topics: Humans; Glaucoma, Angle-Closure; Intraocular Pressure; Visual Acuity; Glaucoma; Retrospective Studies
PubMed: 37870033
DOI: 10.4103/IJO.IJO_733_23 -
Journal of Family Medicine and Primary... Dec 2022Microspherophakia refers to a spherophakic lens with a decreased equatorial diameter. Microspherophakia can be found in systemic or ocular conditions, such as Marfan's...
Microspherophakia refers to a spherophakic lens with a decreased equatorial diameter. Microspherophakia can be found in systemic or ocular conditions, such as Marfan's syndrome, Weill-Marchesani syndrome, iridocorneal endothelial syndrome, and Axenfeld-Rieger syndrome. A 3-year-old girl was brought with complaints of eyes appearing larger, watering and inability to see bright light for 1 year. On examination, she had megalocornea; the cornea was clear with a shallow anterior chamber, and microspherophakic lens. Her intraocular pressure (IOP) was recorded as 43 and 32 mmHg in the right and left eyes, respectively. This article guides in classifying, categorizing, and managing a case with microspherophakia.
PubMed: 36994055
DOI: 10.4103/jfmpc.jfmpc_1212_22