[Transscleral fixation of intraocular lens in the treatment of lens subluxation in children]. / Transskleral'naya fiksatsiya intraokulyarnoi linzy v lechenii sublyuksatsii khrustalika u detei.

Congenital subluxation of the lens as a complication of Marfan syndrome, Weill-Marchesani syndrome, microspherophakia, etc. leads to the development of amblyopia and requires timely surgical treatment with removal of the subluxated lens and implantation of an artificial intraocular lens (IOL). IOL implantation in children with pathology of the ligamentous apparatus of the lens remains an urgent problem of ophthalmic surgery due to the lack of a consensus regarding the IOL fixation method among practitioners. PURPOSE: This study evaluated the effectiveness and safety of IOL implantation with transscleral fixation using the knotless Z-suture technique in pediatric patients with congenital lens subluxation. MATERIAL AND METHODS: The study included 24 children (36 eyes) with grade III congenital subluxation of the lens who underwent phacoaspiration of the subluxated lens with IOL implantation with transscleral fixation using the knotless Z-suture performed in the Kazakh Research Institute of Eye Diseases in Almaty in the period from 2017 to 2021. The average observation period was 31.7±11.3 months (2.0 to 4.5 years). The stability of the IOL position, the state of the intrascleral sutures, visual acuity after surgery, the presence and severity of complications in the long-term period were evaluated. RESULTS: All patients (100%) had a significant improvement in visual acuity after surgery. No intraoperative complications were registered in any of the cases. Postoperative complications were noted in 8.3% of cases (n=3). The final functional outcome of surgical treatment depended on the presence of concomitant pathology, the main cause of low vision was the development of refractive amblyopia due to refractive errors. CONCLUSIONS: The presented technique of transscleral fixation of IOL has proven to be reliable, which is especially important for pediatric patients considering their high physical activity and expected lifespan.

Sunrise in the eye: Bilateral superonasal lens subluxation in Marfan syndrome.

Aim: To describe the case of a patient with Marfan syndrome who had bilateral superonasal lens subluxation. Method: The case of a male patient, aged 18, who complained of having impaired vision in both eyes (BE) since he was a toddler, was presented. On examination of the patient, features suggestive of Marfan syndrome were revealed, as well as bilateral intraocular lens subluxation. Results: The patient was refracted and glasses were recommended, which improved his vision. The patient was referred to the cardiology, orthopedic, and dental departments for a multidisciplinary approach to prevent complications and further management. Discussion: Lens subluxation is frequently presented as a primary clinical manifestation of Marfan syndrome. It can vary from asymptomatic, which is seen only after pupillary dilation, to significant subluxation, in which the equator of the lens in the pupillary axis causes diplopia or decreased vision. Conclusion: This case underscored the importance of considering the rare feature of Marfan syndrome.

Zonular fibre Insertion-to-Limbus Distance (ZLD): normative data to assess lens position and diagnose ectopia lentis.

PURPOSE: Subluxation of the crystalline lens (Ectopia Lentis, EL) can lead to significant visual impairment and serves as a diagnostic criterion for genetic disorders such as the Marfan syndrome. There is no established criterion to diagnose and quantify EL. We prospectively investigated the distance between the zonular fibre insertion and the limbus (ZLD) in healthy subjects as a parameter to assess the position of the lens, quantify EL and provide normative data. METHODS: This prospective, observational, cross-sectional study includes one-hundred-fifty eyes of 150 healthy participants (mean age 28 years, range 4-68). Pupils were dilated with tropicamide 0.5% and phenylephrine 2.5% eyedrops. ZLD was measured in mydriasis at the slit lamp as the distance between the most central visible insertions of the zonular fibres on the lens surface and the corneoscleral limbus. Vertical pupil diameter (PD) and refractive error were recorded. If zonular fibre insertions were not visible, the distance between limbus and the pupillary margin was recorded as ZLD. RESULTS: 145 right and 5 left eyes were examined. 93% of study subjects were Caucasian, 7% were Asian. In eyes with visible zonular fibre insertions (n = 76 eyes), ZLD was 1.30 ± 0.28 mm (mean ± SD, range 0.7-2.1) and PD was 8.79 ± 0.57 mm (7.5-9.8). In the remaining 74 eyes, ZLD was 1.38 ± 0.28 mm (0.7-2.1), and PD was 8.13 ± 0.58 mm (6.7-9.4). For all eyes, ZLD was 1.34 ± 0.29 mm (0.7-2.1), and PD was 8.47 ± 0.66 mm (6.7-9.8). Refractive error and sex did not significantly affect ZLD. Smaller PD and older age were associated with larger ZLD (P < 0.001 and P = 0.036, respectively). CONCLUSION: Average ZLD was 1.34 mm in eyes of healthy subjects. Older age correlated with larger ZLD. These normative data will aid in diagnosing and quantifying EL.

Lens subluxation combined with parry-romberg syndrome: case report.

BACKGROUND: Parry-Romberg syndrome (PRS) is a rare progressive degenerative disorder of unknown etiology. Here we report a rare case of PRS combined with lens subluxation in Eye and ENT hospital of Fudan University, Shanghai. To our knowledge, it is the first reported case of PRS combined with lens subluxation that has been managed surgically with phacoemulsification and CTR placement and IOL implantation in Shanghai. CASE PRESENTATION: A 60-year-old woman was referred for "right visual blur for 2 years" and had persistent right facial paralysis of unknown etiology since the age 12. She had right facial muscle atrophy and paralysis. Eye examination also showed the right eyelid pseudoptosis, enophthalmos, age-related cataract combined with lens subluxation existed in the right eye. The patient was diagnosed as age-related cataract and lens subluxation in the right eye and progressive hemifacial atrophy (Parry-Romberg syndrome). We conducted a combined phacoemulsification, IOL and CTR implantation and pupilloplasty surgery for the patient under general anesthesia and the postoperative UCVA was 20/30 and remained for 1 year's follow up. CONCLUSIONS: Here we reported a rare case of PHA combined with lens subluxation in China. After appropriate eye surgery, the patient achieved satisfying vision result in the right eye.

Sutured scleral fixation of existing subluxated/dislocated acrylic one-piece intraocular lenses.

AIM: To evaluate the visual and refractive outcomes in cases after sutured scleral fixation of existing subluxated or dislocated acrylic one-piece intraocular lenses (IOLs). METHODS: This study retrospectively enrolled a consecutive series of patients who underwent a surgery of sutured existing subluxated or dislocated IOLs from October 2018 to June 2020. All patients underwent comprehensive preoperative and postoperative ophthalmologic examination, and data were collected including age, sex, surgical indications, best-corrected visual acuity, refractive error, intraocular pressure. Presence of intraoperative and postoperative surgical complications was documented. RESULTS: A total of 20 consecutive cases were enrolled for analysis with mean final follow-up period 9.8±5.3mo. Visual acuity improved from a mean of 0.35 (0.46±0.32 logMAR) preoperatively to 0.61 (0.21±0.18 logMAR) at the 3-month follow-up (P=0.002). The mean amount of preoperative keratometric astigmatism and total postoperative refractive astigmatism was -1.24±0.80 diopters (D) and -1.42±0.97 D, respectively. There was no statistically significant difference between preoperative and postoperative astigmatism (P=0.156). The mean IOL-induced astigmatism was -0.23±0.53 D. The mean spherical equivalent at the 3-month follow-up was -0.1±0.94 D. No major complications were noted during the follow-up period. CONCLUSION: Surgical techniques using sutured scleral fixation of existing subluxated or dislocated acrylic one-piece IOLs result in favorable visual and refractive outcomes without major complications.

Intrascleral intraocular lens fixation with ab interno trabeculotomy in patients with exfoliation glaucoma with lens subluxation.

PURPOSE: To evaluate the surgical outcomes of intrascleral intraocular lens (IOL) fixation using ab interno trabeculotomy (LOT) in patients with exfoliation glaucoma with lens subluxation. STUDY DESIGN: Retrospective case series. METHODS: Twenty eyes of 18 patients with exfoliation glaucoma and lens or IOL subluxations were included. Three success criteria were based on postoperative intraocular pressure (IOP) (A, ≤15 mmHg; B, ≤18 mmHg; C, ≤21 mmHg). The IOP, number of glaucoma medications, and visual acuity were compared before and after surgery. Success probability was analyzed using Kaplan-Meier survival curves. Cox proportional hazards' regression models were used to examine prognostic factors for surgical failure. RESULTS: The mean follow-up period was 23.4 ± 7.8 months. The mean IOP significantly decreased from 23.2 ± 6.8 mmHg preoperatively to 14.0 ± 4.4 mmHg at 1 year postoperative (P<0.001). Postoperative hyphema and vitreous hemorrhage were observed in seven and 15 eyes, respectively, and washout in the anterior chamber or vitreous cavity was performed in four eyes. Postoperative IOP spikes and hypotony were observed in four eyes each. Glaucoma reoperation was performed in two eyes. The success rates at 12 months were 65%, 85%, and 90% using criteria A, B, and C, respectively. The IOP at 1 month after surgery was a significant prognostic factor for surgical failure according to criterion A (hazard ratio: 1.08; P=0.034). CONCLUSION: Intrascleral IOL fixation combined with microhook LOT is a promising option in cases of exfoliation glaucoma with subluxated lens/IOL; however, the high rate of postoperative hyphema and vitreous hemorrhage should be noted.

A refined total capsular bag suspension technique for lens subluxation from cystathionine beta-synthase deficiency: A case report and literature review.

Purpose: To report the application of a refined total capsular bag suspension technique for lens subluxation from Cystathionine beta-synthase (CBS) deficiency. Observations: A 15-year-old CBS deficiency male patient with a history of intracranial venous thrombosis presented to our clinic due to bilateral vision loss. The patient was treated with lens aspiration, intraocular lens (IOL) implantation, and total capsular bag suspension in both eyes respectively. During the six months postoperative follow-up, the patient exhibited improved visual acuity and minor refractive error. Conclusions and importance: The refined total capsular bag suspension technique is recommended for CBS deficiency patients with lens subluxation as a safe and effective surgical approach.

Novel diagnostic indicators for acute angle closure secondary to lens subluxation based on anterior segment and lens parameters.

Purpose: To explore stable and sensitive indicators for clinical diagnosis of acute angle closure (AAC) secondary to lens subluxation (LS) through quantitative analysis of CASIA 2 imaging.Design: A prospective cross-sectional study. Methods: Setting: Clinical practice.Participants: 23 patients with unilateral acute angle closure secondary to lens subluxation and 23 cataract patients without lens subluxation were recruited. Lens subluxation was confirmed by ultrasound biomicroscope diagnosis. The contralateral eyes without LS served as fellow control group. The cataract eyes without LS were enrolled in blank control group.Intervention: Participants underwent ophthalmologic examinations including slit-lamp biomicroscope, best corrected visual acuity, intraocular pressure, central corneal thickness measurement, axial length, gonioscopy, ultrasound biomicroscope and 360-degree anterior chamber and crystalline lens scan protocols of CASIA 2 system.Main outcome measures: Automated circumferential anterior segment and lens morphological parameters under anterior segment optical coherence tomography were analyzed via three-dimensional analysis. Results: Significant differences were found in the front and back radius of the lens, the front and back radius of steep curvature of the lens, lens thickness, lens decentration, lens diameter, iris-trabecular contact (ITC) index, ITC area, anterior chamber depth (ACD), lens vault (LV), and iris volume between LS and controls. Among these parameters, LV, the anterior radius of steep curvature of the lens and ACD demonstrated the highest prediction power (AUC = 0.87, 0.89, and 0.86, respectively). The prediction power of tilt/axis was much higher in the Gaussian Naive Bayes model (AUCs = 0.90) than in the logistic model (AUCs = 0.74). Combination of LV_mean, LV_std, tilt and tilt axis in Gaussian Naive Bayes model presented as most stable and excellent diagnostic markers for AAC secondary to LS (AUCs = 0.98). Conclusions: The combination of markers including lens tilt and lens vault in the mathematic model facilitate clinical work as it not only provides novel diagnostic indications and possible prompt treatment for AAC secondary to lens subluxations, but also enhances our understanding of the pathogenic role of zonulopathy in angle closure glaucoma.

Causes of intraocular pseudophakic lens explantation in Spain.

PURPOSE: To report the causes of pseudophakic intraocular lens (IOL) explantation in Spain over a 20 year period. METHODS: Multicenter observational prospective study of a consecutive series of cases corresponding to pseudophakic intraocular lenses explantations performed in 22 Spanish clinical centres from 2002 to 2021, within a national clinical research network. The clinical data of all IOL explantation patients was evaluated, and the different causes for IOL explantation were systematically analyzed. RESULTS: From a total of 1013 pseudophakic explantations, 919 cases were included in this investigation. The mean age of the patients at the time of explantation was 66.3 years, and 142 (15.45%) were women. Over the 20 years of the study, the five main reasons for explanting lenses in the last 20 years in Spain according to the Iberia RETICS database were: dislocation / decentration / misalignment (43.08%), bullous keratopathy (16.79%), neuroadaptation failure (13.85%), IOL opacification (9.71%), refractive surprise (6.87%). Other causes, less frequent, were uveitis / endophthalmitis / infection with 39 cases (4.25%); IOL substitution for reasons other than multifocal IOL neuroadaptation failure 3 cases (0.33%); and other causes, 47 cases (5.13%). Hydrophobic lenses were the most frequently explanted with a ratio of 47.1%. CONCLUSIONS: Over the 20 years of the study, the five main reasons for explanting lenses in the last 20 years in Spain according to the Iberia RETICS database were: dislocation / decentration / misalignment, bullous keratopathy, neuroadaptation failure, IOL opacification, and refractive surprise.

Ocular Involvement and Treatment Pattern in Korean Patients with Marfan Syndrome: A Population-Based Study.

PURPOSE: This study aimed to investigate the incidence and prevalence of, and treatment patterns for ocular complications in Korean patients with Marfan syndrome. METHODS: Incidence and prevalence of Marfan syndrome was calculated from 2010 to 2018, based on data from the Korean National Health Insurance Service (KNHIS). Diagnosis codes (for cataract, ectopia lentis, retinal detachment, etc.) and surgery reimbursement codes (lensectomy, phacoemulsification, buckling, vitrectomy, etc.) in the patients with Marfan syndrome were retrieved by complete enumeration of the data. RESULTS: The annual prevalence of Marfan syndrome adjusted by age and sex was gradually increased from 2.44 per 100,000 in 2010 to 4.36 per 100,000 in 2018. The age group of 10-19 years showed the highest prevalence. The prevalence of ectopia lentis was 21.7%, of whom 43.0% underwent surgeries. Surgery for RD was performed in 253 (14.1%) of 2044 patients during the study period. CONCLUSION: Although the most prevalent ophthalmologic manifestation was ectopia lentis, total prevalence rate of RD was more than 10% in the study period; thus, regular fundus examination is recommended for the patients with Marfan syndrome.

A new approach for single-haptic retropupillary iris-claw lens subluxation - The "fencing" technique.

INTRODUCTION: Single-haptic iris-claw intraocular lens (IOL) dislocation is not an uncommon complication. A few different surgical techniques are available for its refixation but usually involve a more invasive approach. We aim to demonstrate an original and simple approach for refixation of single haptic retropupillary iris-claw IOL subluxations. METHODS: We present a case of an 80-year-old male with a single haptic retropupillary iris-claw IOL subluxation in a previously vitrectomized eye. We used a new surgical approach to refix this type of subluxation. RESULTS: This new technique uses only two 30-gauge needles (one of them connected to a viscosurgical device) to re-enclavate the subluxated haptic of the retropupillary iris-claw IOL. By minimising surgical manipulation, the patient's postoperative period was uneventful. CONCLUSIONS: We highlight a new, quick, safe, and unusual surgical approach to single-haptic retropupillary iris-claw IOL dislocation in vitrectomized eyes. Because of its characteristics, we named it the "fencing" IOL repositioning technique.

Traction suture fixation technique using a capsular tension ring fragment for severe lens displacement during cataract surgery in dogs.

OBJECTIVES: To describe the traction suture fixation technique for severely displaced lenses (≥180°) using a capsular tension ring (CTR) fragment during cataract surgery in dogs, and to retrospectively investigate its clinical outcomes. ANIMALS STUDIED: Eight dogs (nine eyes). PROCEDURES: The medical records of dogs with severe lens displacement (≥180°) due to cataracts that underwent traction suture fixation during cataract surgery using a CTR fragment at the Grand Animal Hospital (Hyogo, Japan) between November 2019 and September 2022 were retrospectively reviewed. The retrieved data included the signalment, type of CTR, and postoperative outcomes. RESULTS: A single CTR fragment was used to fix the capsular bag to the sclera in six eyes, and two CTR fragments were used at two sites in three eyes. The lengths of the CTR fragments used were 1 one-fifth fragment, 3 one-fourth fragments, and 8 one-third fragments. Postoperative complications included uveitis (nine eyes), glaucoma (one eye), posterior iris adhesion (one eye), corneal ulcer (one eye), and intracorneal stromal hemorrhage (one eye). Vision was maintained in all dogs during the follow-up period (2 months to 2 years and 8 months) without recurrent capsular bag displacement. CONCLUSIONS: This technique can correct and stabilize capsular bag displacement relatively easily through a small incision during cataract surgery in dogs with severe lens displacement. Intraocular lens implantation was also possible.

Modified intrascleral haptic fixation of the light adjustable lens in a case of spontaneous adult-onset bilateral lens subluxation.

Purpose: To describe the application of the light adjustable lens (LAL) using an intrascleral haptic fixation (ISHF) technique for the correction of aphakia and post-operative refractive error. Observation: The LAL was placed using a modified trocar-based ISHF technique for visual rehabilitation following removal of bilateral cataracts in a patient with ectopia lentis. She ultimately obtained an excellent refractive outcome after adjustment with micro-monovision. Conclusions and Importance: Secondary intraocular lens placement has a much higher risk of residual ametropia than traditional in-the-bag lens placement. The ISHF technique with the LAL presents a solution for eliminating postoperative refractive error in patients requiring scleral-fixated lenses.

[Phaco surgery in disorders of the capsular-zonular apparatus of the lens]. / Fakokhirurgiya pri narusheniyakh svyazochno-kapsulyarnogo apparata khrustalika.

The article presents authors' original methods of phaco surgery that can be used in disorders of the capsular-zonular apparatus of the lens. The cataract surgery techniques for lens subluxation developed and introduced into clinical practice allow using the most physiologically correct intracapsular intraocular lens (IOL) fixation in the vast majority of cases. The introduction of femtosecond laser at key stages of phacoemulsification in complicated clinical situations reduces the dependence of the results on the human factor and allows performing removal of complicated cataracts at a qualitatively new level.

Pars plana lensectomy and iris-claw Artisan intraocular lens implantation in patients with Marfan syndrome.

RATIONAL: The rationale of this study was to evaluate the visual and anatomical outcomes of pars plana lensectomy and iris-claw Artisan intraocular lens (IOL) implantation in patients with subluxated crystalline lenses secondary to Marfan syndrome. MATERIALS AND METHODS: In this retrospective case series, we evaluate the records of 21 eyes of 15 patients with Marfan syndrome and moderate-to-severe crystalline lens subluxation who underwent pars plana lensectomy/anterior vitrectomy and implantation of iris-claw Artisan IOL at referral hospital from September 2015 to October 2019. RESULTS: Twenty-one eyes of 15 patients (10 males and five females) with a mean age of 24.47 ± 19.14 years were included. Mean best-corrected visual acuity was improved from 1.17 ± 0.55 logMAR to 0.64 ± 0.71 logMAR at the final follow-up visit (P < 0.001). The mean intraocular pressure did not change significantly (P = 0.971). The final refraction showed a mean sphere of 0.54 ± 2.46 D and a mean cylinder of 0.81 ± 1.03 at the mean axis of 57.92 ± 58.33 degrees. One eye developed rhegmatogenous retinal detachment 2 months after surgery. CONCLUSIONS: Pars plana lensectomy and iris-claw Artisan IOL implantation seem to be a useful, impressive, and safe procedure with a low rate of complications in Marfan patients with moderate-to-severe crystalline lens subluxation. Visual acuity was significantly improved with acceptable anatomical and refractive outcomes.

Knobloch Syndrome in Siblings with Posterior Fossa Malformations Along with Cerebellar Midline Cleft Abnormality Caused by Biallelic COL18A1 Mutation: Case-Based Review.

Knobloch syndrome (KS) is an autosomal recessive disorder caused by biallelic pathogenic variants in COL18A1 . KS clinically manifests with the typical eye findings (high myopia, vitreoretinal degeneration, retinal detachment, and lens subluxation), variable neurological findings (occipital encephalocele, polymicrogyria, cerebellar malformations, epilepsy, and intellectual disability), and the other uncommon clinical manifestations. Literature review of all KS patients (source PubMed) was done with special reference to cerebellar abnormalities. Here, we report two siblings with typical KS with posterior fossa malformations and novel cerebellar midline cleft abnormality analyzed by whole exome sequencing. Known pathogenic homozygous variant c.2908C > T; (p.Arg970Ter) in exon 26 of COL18A1 was found as a cause for KS. These two siblings presented with early-onset severe ocular manifestations, facial dysmorphism, and variable central nervous system manifestations along with novel cerebellar midline cleft abnormality. The presence or absence of structural brain malformations and genotypes does not absolutely predict cognitive functions in KS patients. However, the presence of posterior fossa abnormality may be predictive for the development of ataxia in later life and needs further studies.

Supracapsular scleral sutured intraocular lens implantation in anterior segment dysgenesis patients with ectopia lentis.

OBJECTIVE: To describe a new strategy to manage ectopia lentis in ASD patients assessing the visual outcomes and safety of supracapsular scleral sutured intraocular lens implantation and analyzing the accuracy of different intraocular lens (IOL) power calculation formulae. METHODS: Eight patients with ASD (13 eyes) were underwent supracapsular scleral suture fixation of posterior chamber (PC) IOL without capsular extirpation. The preoperative and postoperative clinical features were compared. The prediction error values from four formulae (SRK/T, Holladay 1, Hoffer Q, Haigis), with or without Wang-Koch (WK) adjustment, were calculated for the cases. RESULTS: Zonulodialysis and premature cataracts could be the main reason for the decreased vision in patients with ASD. There was a significant improvement in best corrected visual acuity on 3-month follow-up after applying supracapsular scleral suture fixation of PC IOL. The prediction errors of the different formulae showed a slight tendency towards postoperative myopia. The Haigis formula with WK adjustment showed the best performance. CONCLUSIONS: Supracapsular scleral suture fixation of IOLs for retaining the capsule-zonule barrier is a good option for ASD patients. The Haigis formula is recommended for ASD patients treated with supracapsular scleral suture fixation of IOLs. The predicted IOL power should be reduced based on the effect of the new anatomic position of the IOL to achieve a satisfactory visual outcome.

Utilizing perioperative patient positioning to correct intraocular lens implant subluxation.

Purpose: to describe how preoperative and intraoperative positioning techniques can be used to manage subluxed intraocular lenses (IOL) whilst saving patients from pars plana vitrectomy. Observations: An 88-year-old man with a complex past medical history including mild cognitive decline from early Alzheimer's dementia and pertinent ocular history of pseudoexfoliation syndrome and previous cataract surgery with IOL presented with decreased vision secondary to an inferiorly subluxed IOL/bag complex. The IOL was not visible in the operating room when he was supine but was visible in clinic the next day after he had slept in the prone position the night before. The patient was returned to the operating room the next day and a fixation suture was used to capture the IOL while he was upright. The IOL was then fixated to the sclera in standard position. Conclusions and Importance: Both pre and intraoperative positioning techniques can help anterior segment surgeons fixate subluxed IOLs that otherwise seem inaccessible from an anterior approach and thus avoiding the inherent risks associated with vitrectomy.

Evaluation of Multimodal Biometric Parameters for Diagnosing Acute Angle Closure Secondary to Lens Subluxation.

INTRODUCTION: To evaluate the clinical characteristics and multimodal biometric parameters from ultrasound biomicroscopy (UBM) and IOL Master biometry of patients with acute secondary angle-closure due to lens subluxation (ASAC-LS), acute primary angle-closure (APAC), and cataract. METHODS: This retrospective study included 22 eyes with ASAC-LS, 27 eyes with APAC, and 39 eyes with cataract. Gender, age, affected eye, best corrected visual acuity, axial length, central corneal thickness, and anterior chamber depth (ACD) assessed by UBM and IOL Master were measured and compared between the three groups. In addition, we compared the ratio of ACD (ACD ratio) and the difference of ACD (ACD difference) measured by the two instruments. Logistic regression analysis was conducted to evaluate the predictive factors for lens subluxation. Receiver operating characteristic (ROC) curves were plotted to obtain a suitable cutoff value of biometric parameters to separate ASAC-LS cases from APAC and cataract cases. RESULTS: In the ASAC-LS group, the median (interquartile range [IQR]) ACD measured by IOL Master was 2.47 (IQR 1.85-2.92) mm while the median ACD measured by UBM was 3.11 (IQR 2.60-3.76) mm. The difference of ACD measured by the two instruments was statistically significant in the ASAC-LS group (P < 0.001) whereas the differences were not statistically significant in the APAC group (P = 0.521) and cataract group (P = 0.204). Subsequent pairwise comparison revealed that only the ACD difference (0.40 [IQR 0.22-1.08] mm) and ACD ratio (1.18 [IQR 1.07-1.40]) in the ASAC-LS group were significantly different from those in the APAC group (ACD difference 0.02 [IQR 0.01-0.07] mm; ACD ratio 1.01 [IQR 1.00-1.04]) and cataract group (ACD difference 0.09 [IQR 0.01-0.14] mm; ACD ratio 1.03 [IQR 1.00-1.04]) (all P < 0.001). The ACD difference and ACD ratio were significantly associated with lens subluxation in the multivariate logistic regression analysis (P < 0.001 and P = 0.001, respectively). Additionally, the ROC curve analysis showed that the ACD difference at 0.235 mm and the ACD ratio at 1.080 were the respective cut-off values for lens subluxation, with a sensitivity of 77.3% and specificity of 100.0%. CONCLUSION: Our findings provide a new option for identifying lens subluxation. Specifically, combining the ACD from UBM and IOL Master may be helpful for differential diagnosis of ASAC-LS.

Bilateral Subluxation of Microspherophakic Lens in a Child with Cohen Syndrome.

Cohen Syndrome is a rare autosomal recessive condition characterized by facial abnormalities with or without microcephaly, non-progressive intellectual delay, hypotonia, ophthalmic abnormalities, and neutropenia. Due to its low incidence and variable presentation, much about the disorder, including ophthalmic manifestations, is not fully understood. Here, we present the first documented case of a 5-year-old Amish child with Cohen Syndrome who presented with bilateral subluxation of microspherophakic lenses - rare findings themselves, let alone coexisting in a patient with a rare genetic syndrome. The child underwent bilateral lensectomy and is being managed with aphakic spectacles.