Risk Factors for Retinal Detachment in Marfan Syndrome After Pediatric Lens Removal.

PURPOSE: To determine retinal detachment (RD) risk factors after lens removal surgery in children with Marfan syndrome (MS). DESIGN: Retrospective, case control study. METHODS: This was an institutional case series including children (age <18 years) with MS who underwent lens removal surgery. Clinical and surgical characteristics were extracted from the children's electronic files: age, axial length (AL), gender, number of surgeries received, intraocular lens (IOL) implantation at the first surgery, complete removal of the capsular bag, and final best-corrected visual acuity. Risk factors associated with RD occurrence were identified. RESULTS: Among 158 eyes included (85 children), 35 eyes (22.2%) developed RD during follow-up. Bilateral detachment occurred in 11 patients (45.8%). Age at the time of the lens removal surgery was not different between groups. Children in the RD group had a higher AL (P < .001), longer follow-up, IOL implantation, and capsular residue. Multivariate analysis identified capsular residue (odds ratio, 16.8; 95% CI, 1.9-148.8; P = .01) and AL (odds ratio, 1.3; 95% CI, 1.01-1.7; P = .03) as predictors for RD. CONCLUSIONS: Children with MS and increased AL were more likely to develop an RD after lens surgery. When considering lens removal surgery in a pediatric population presenting with MS, a complete capsular removal seemed to be the safer option regarding RD risk.

Incidence and Outcomes of Eye Trauma Associated With Recreative Use of Nonpowder Toy Guns: A 12-Year Retrospective Study.

PURPOSE: Nonpowder toy guns (NPTGs) are responsible for many ocular traumas. This study aims to detail the outcomes of these injuries depending on the causative NPTG. DESIGN: Retrospective, observational case series. METHODS: Cases of NPTG-associated ocular trauma managed in a Parisian eye emergency department between August 1, 2010, and January 1, 2023, were reviewed. The date of trauma, causative NPTG, patient demographics, initial and follow-up eye examinations, any surgical procedure, and visual outcomes for each ocular trauma were analyzed. RESULTS: Over 12 years, NPTGs were responsible for 324 eye injuries and 980 visits. Patients were mostly male (77.5%), and mean age at trauma was 16.2 years. Foam bullets or foam dart blasters accounted for 54.9% of traumas and were mainly responsible for corneal injuries and hyphema (30.9% and 27%, respectively). BB guns and airsoft guns were frequently responsible for anterior segment lesions, as well as intravitreal hemorrhages (14.7%) and commotio retinae (21.1%). Paintball guns accounted for the largest proportion of posterior segment lesions (eg, intraretinal or subretinal hemorrhages leading to macular atrophy/contusion maculopathy), and one-third of casualties had undergone ocular surgery. Among all traumas, final visual acuity was lower than 20/200 in 6.5% of cases. Phthisis occurred in 8 cases: Two were related to foam bullets or foam dart blaster injuries (1 contusion and 1 rupture), 2 other cases followed a rupture due to BB guns/airsoft guns, 1 case occurred after a rupture related to a paintball gunshot, and 3 others were due to other types of compressed air guns (1 rupture, 1 intraocular foreign body, and 1 total retinal detachment). CONCLUSIONS: NPTG-related ocular trauma outcomes differ according to the causative toy. Paintball guns and BB guns/airsoft gun-related traumas were more likely to be associated with severe lesions, but an increasing number of ocular injuries related to the use of foam bullets or foam dart blasters are reported in younger and younger children. Public health policies should promote the use of protective eyewear.

Outcomes of Modified Limbal Lensectomy-Vitrectomy in Stages 4B and 5 Retinopathy of Prematurity with Extended Retrolental Fibroplasia.

PURPOSE: To report on the anatomical and functional outcomes of a modified limbal lensectomy-vitrectomy (LV) approach for stages 4B and 5 retinopathy of prematurity (ROP) as defined in the International Classification of Retinopathy of Prematurity, 3rd Edition (ICROP 3). DESIGN: Retrospective, monocentric, consecutive case series. PATIENTS: Infants with ROP that underwent limbal LV for diffuse retrolental fibroplasia. METHODS: Clinical charts and Retcam photographs were reviewed. Surgical approach consisted of a limbal LV through peripheral iridectomies with centripetal dissection of the preretinal fibrosis. MAIN OUTCOME MEASURES: Anatomical success and visual function at last follow-up were evaluated. Multivariate logistic regression was used to explore potential prognostic factors affecting the anatomical outcome. RESULTS: A total of 128 eyes of 81 patients with a mean gestational age of 28.7 ± 3.0 weeks and a mean birthweight of 1244 ± 429 g were included. Eighteen eyes (14.1%) had a stage 4B, 24 (18.8%) a stage 5B, and 86 a stage 5C (67.2%) ROP. Mean age at surgery was 57.4 ± 36.3 weeks and mean postoperative follow-up was 22.7 ± 20.4 months. Only 5 eyes (3.9%) had prior peripheral retinal ablation. Macular reattachment was achieved in 74 eyes (57.8%). Controlling for other baseline factors, a stage 5C (versus stage 4B, odds ratio [OR] = 6.9 [1.5-32.1], P = 0.01 and versus stage 5B, OR = 7.4 [1.5-37.1], P = 0.02), the presence of vascular activity (OR = 6.4 [2.3-18.1], P < 0.001), and the presence of Schlieren sign (OR = 13.0 [2.1-82.2], P = 0.006) were associated with a failure of macular reattachment. Visual acuity was assessed in 92 eyes (71.9%), among which 59 eyes (64.1%) had light perception or better. CONCLUSIONS: Modified limbal LV resulted in macular reattachment in more than half of eyes with ROP-related retinal detachment and diffuse retrolental fibrosis. A stage 5C based on ICROP 3, the presence of vascular activity, and a Schlieren sign were significantly associated with a failure of macular reattachment. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Management and Outcomes of Posterior Persistent Fetal Vasculature.

PURPOSE: To describe clinical features, management, and outcomes of posterior persistent fetal vasculature (PFV) and suggest a management algorithm. DESIGN: Retrospective, consecutive case series. PARTICIPANTS: All children diagnosed with posterior PFV and treated or followed at the Rothschild Foundation Hospital in France between June 2011 and September 2021. METHODS: Retrospective analysis of the clinical characteristics of posterior PFV. We reported age, gender, presenting symptoms, intraocular pressure, and visual acuity (VA) at diagnosis. Patients were divided into 4 groups depending on the severity and involvement or not of anterior segment. We reported the vitreoretinal surgical techniques used. MAIN OUTCOME MEASURES: Anatomic results, ocular hypertension, best-corrected distance visual acuity (BCDVA), presence of postoperative adverse events, and additional surgical interventions were recorded at each follow-up visit. RESULTS: Ninety-six patients were included. The median age at diagnosis was 8 months (IQR = 12), mean 18.9 ± 30.9 months) with a mean follow-up of 27 ± 31.2 months. Although PFV is often an isolated disease, it was associated with a systemic disease in 8% of cases. There was anterior involvement in 62 (64%) of eyes. Forty-one eyes (42.7%) were microphthalmic and more frequently associated with severe PFV (53% vs. 25%; P = 0.01). Surgery was performed in 85 patients (89%). Of them, 69 (81%) had a total success, 5 (6%) had a partial success due to persistent limited peripheral retinal detachment (RD), and 11 (13%) had a failure due to persistent total RD after surgery. Postoperative adverse events occured in 38 eyes including ocular hypertension requiring eye drop medication (7.1%), secondary cell proliferation around the intraocular lens (8.2%), intravitreal hemorrhages (7.1%), and persistent tractional RD (10.6%). Second surgery was performed in 18 patients (21%). At last follow-up, VA could be measured in logarithm of the minimum angle of resolution in 43 children (45%), light perception in 21 eyes (22%), and no light perception or impossible to assess in 32 eyes (33%). CONCLUSIONS: In our case series, most patients presenting with posterior PFV received complex vitreoretinal surgery. Goals of the surgery vary and include retinal flattening, reduction of vitreoretinal traction, freeing of visual axis, and aesthetic concerns. We propose a surgical and medical management algorithm for PFV. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found after the references.

Surgical removal of submacular choroidal neovessels in children: Outcome and literature review.

PURPOSE: To report on the outcome of surgical submacular choroidal neovascular membrane (CNV) removal in children and to perform a comprehensive review of literature concerning this intervention in children. METHODS: In this retrospective, noncomparative, interventional case series, we included 8 eyes of 7 consecutive children with subfoveal choroidal neovascularization treated by pars plana vitrectomy (PPV) and CNV removal. Main outcome measures were visual acuity and complications. RESULTS: Mean age at surgery was 8.6 ± 5.2 years (range: 2-16). Two out of 8 eyes were idiopathic. Corrected-distance visual acuity (CDVA) improved from 1.01 ± 0.45logMAR (range:0.3-1.5) at presentation to 0.60 ± 0.37 (range:0-1) at last follow-up (p = 0.03). Mean follow-up was 3.9 ± 3.9 years. Six eyes received at least one intravitreal injection of bevacizumab prior to surgery. Recurrence occurred in one eye with Best's disease.Literature review revealed a total of 42 cases with the most frequent etiologies being Presumed ocular histoplasmosis syndrome (POHS) and idiopathic CNV. Considering all cases together, mean CDVA improved from 1.00 ± 0.37logMAR to 0.52 ± 0.42 (p < 0.01). CNV recurrence occurred in 11 eyes (22.0%), 7 of which had an inflammatory etiology. Other complications included pigment epithelium tear, atrophy and retinal tear. CONCLUSION: Surgical removal of CNV is a viable, effective and safe option in children with persistent submacular neovascular membranes.

Prospective cholestanol screening of cerebrotendinous xanthomatosis among patients with juvenile-onset unexplained bilateral cataracts.

BACKGROUND: Cerebrotendinous xanthomatosis (CTX) is a rare genetic disorder related to CYP27A1 biallelic mutations, leading to decreased synthesis of bile acids and increased cholestanol. Juvenile bilateral cataracts are one of the most common findings in the disease, frequently occurring before the onset of neurological manifestations. While early treatment with chenodeoxycholic acid can prevent the onset of neurological impairment, poor awareness of CTX accounts for a markedly delayed diagnosis. The objective of this study was to evaluate the utility of plasma cholestanol analysis at the moment of cataract diagnosis and before the onset of neurological impairment in CTX. METHODS: Multicenter prospective cohort study of patients with juvenile-onset unexplained bilateral cataracts recruited from seven French ophthalmology departments. Plasma cholestanol analysis was performed at diagnosis from January 2018 to January 2020. CYP27A1 genetic testing was performed at the ophthalmologist's discretion. Cholestanol levels were compared with those of a similar population of patients without cataracts (control cohort). RESULTS: 30 patients were finally recruited, with a mean age at cataract diagnosis of 7.1 years (± 4.8 SD, range 1-19 years). One patient had a very high cholestanol level (68 µmol/L, reference < 10) and carried two pathogenic heterozygous mutations in CYP27A1 confirming CTX. This patient was a 19-year-old female, reporting chronic diarrhea only in childhood, and diagnosed with bilateral posterior cataracts with cortical fleck-like opacities. Therefore, the incidence of CTX in our cohort of patients was 3.3%. Five further patients (5/29; 17.2%) had moderate elevations of cholestanol level (between 10.3 and 16.5 µmol/L), compared to 12/286 (4.2%) in the control cohort (p = 0.014) after adjustment for age. CONCLUSION: Our study argue for the relevance of plasma cholestanol CTX screening in all patients with juvenile-onset unexplained cataracts, even without other CTX identified manifestations. Whether moderate elevations of plasma cholestanol unrelated to CTX may be a risk factor for bilateral cataracts occurrence needs further examination.

Congenital cataract surgery: long-term refractive outcomes of a new intraocular lens power correction formula.

PURPOSE: The final refraction after intraocular lens (IOL) implantation remains a challenge in the management of paediatric cataracts. No consensual guidelines exist for the choice of IOL power. The aim of this study was to validate a method of IOL power calculation by evaluating the final refractive error in all patients with IOL implantation operated at our institution. METHODS: We retrospectively studied all children under 7 years of age who underwent cataract surgery with IOL implantation at our institution between 2010 and 2015. Intraocular lens (IOL) power was calculated as follows: After B-scan determination of the emmetropic IOL power, a reduction of 40%, 35%, 30%, 25%, 20%, 15%, 10% and 5% was applied to children 0-3, 3-6, 6-12, 12-18, 18-24, 24-30, 30-36, 36-48 months, respectively. The following data were collected: follow-up, age at surgery, uni- or bilaterality, implanted IOL power and final refraction. RESULTS: During this period, 81 children (125 eyes) met the inclusion criteria with a median follow-up of 60 months (36-97). The median age at surgery was 6.61 months (0.76-48). We included 52 children with bilateral cataract (96 eyes) and 29 children with unilateral cataract (29 eyes). The mean implanted IOL power was 23.3 ± 4.6 diopters (D). The mean spherical equivalent at last follow-up was 0.07 ± 3.5 D. CONCLUSION: Our undercorrection formula for IOL implantation after congenital cataract surgery leads to long-term refractive results globally close to emmetropia.

Immunomodulatory treatment and surgical management of idiopathic uveitis and juvenile idiopathic arthritis-associated uveitis in children: a French survey practice.

BACKGROUND: Surgeries for idiopathic uveitis and juvenile idiopathic arthritis-associated uveitis in children are complex because of the high risk of inflammatory postoperative complications. There is no consensus about treatment adaptation during the perioperative period. The objectives of this study are to report the therapeutic changes made in France and to determine whether maintaining or stopping immunosuppressive therapies is associated with an increased risk of surgical site infection or an increased risk of uveitis or arthritis flare-up. METHODS: We conducted a retrospective cohort study between January 1, 2006 and December 31, 2018 in six large University Hospitals in France. Inclusion criteria were chronic idiopathic uveitis or chronic uveitis associated with juvenile idiopathic arthritis under immunosuppressive therapies at the time of the surgical procedure, operated before the age of 16. Data on perioperative treatments, inflammatory relapses and post-operative infections were collected. RESULTS: A total of 76 surgeries (42% cataract surgeries, 30% glaucoma surgeries and 16% posterior capsule opacification surgeries) were performed on 37 children. Adaptation protocols were different in the six hospitals. Immunosuppressive therapies were discontinued in five cases (7%) before surgery. All the children in the discontinuation group had an inflammatory relapse within 3 months after surgery compared to only 25% in the other group. There were no postoperative infections. CONCLUSIONS: The results of this study show varying practices between centres. The benefit-risk balance seems to favour maintaining immunosuppressive therapies during surgery. Further studies are needed to determine the optimal perioperative treatments required to limit post-operative inflammatory relapses.

Incidence and Risk Factors of Glaucoma Following Pediatric Cataract Surgery With Primary Implantation.

PURPOSE: To determine the incidence and risk factors for glaucoma after pediatric cataract surgery with intraocular lens (IOL) implantation. DESIGN: Retrospective, consecutive case series. METHODS: In this single-center study, we reviewed 136 children (199 eyes) who underwent pediatric cataract surgery before 1 year of age with a minimum of 1 year of follow-up. The intervention used was pediatric cataract surgery with IOL implantation, and the primary outcome measure was the presence or absence of secondary glaucoma. RESULTS: The mean age at surgery was 148 ± 93 days (range 30-359 days) with a mean follow-up of 6.3 ± 3.6 years (range 1.1-12.8 years). Glaucoma developed in 31 eyes (16%) with 5- and 10-year incidence rates of 12% and 28%, respectively. The incidence of glaucoma seemed to be bimodal, with a first peak occurring after a mean delay of 2.5 months (range 1.6-4.1 months) and a second peak occurring after a mean delay of 5.7 years (range 2.6-11.7 years). Younger age at surgery, shorter axial length, longer follow-up, use of trypan blue, reintervention, and bilateral surgery were associatied with a higher incidence of glaucoma. Multivariate analysis including the aforementioned variables indentified longer follow-up (odds ratio [OR] = 1.3 [95% confidence interval {CI} 1.1-1.6], P = .001), reintervention (OR = 4.1 [95% CI 1.2-13.4], P = 0.02), and the use of trypan blue (OR = 4.1 [95% CI 1.3-13.1], P = .02) as predictors for the development of glaucoma. CONCLUSION: Glaucoma is a common complication after pediatric cataract surgery. It seemed to have a bimodal incidence. Risk factors for glaucoma development were reintervention, the use of trypan blue, and a long follow-up.

Outcome and risk factors of vitreoretinal surgery in pediatric patients with familial exudative vitreoretinopathy.

PURPOSE: To evaluate the outcome for vitreoretinal surgery in children with familial exudative vitreoretinopathy (FEVR) and to evaluate the risk factors associated with failure. METHODS: This is a retrospective interventional case series of 43 consecutive eyes (34 patients) with vitreoretinal surgery for FEVR. Ocular status prior to intervention and at last follow-up and all surgical steps were recorded. Follow-up time was at least 6 months. Main outcome measure was surgical failure (defined as one of the following: (1) deterioration of visual acuity and stage, (2) persistence or development of total retinal detachment, (3) phthisis). RESULTS: After a mean follow-up of 3.3 ± 3.4 years (median 2.3; 0.5-15.7 years), surgery was successful in 30 eyes (70%) and failed in 13 eyes (30%). Twenty-one eyes (49%) improved, 13 (30%) remained stable, and 9 (21%) deteriorated. Postoperatively, stages and VA improved significantly (p = 0.001; p = 0.04, respectively). Surgical failure was only observed on patients with stages 4 and 5. Mean macular thickness decreased significantly in eyes (stages 2 and 3) with tractional epiretinal membrane. CONCLUSION: Eyes with tractional epiretinal membrane in stages 2 and 3 seem to benefit from vitrectomy and membrane peeling with a positive risk-benefit profile. Advanced stages have a low success rate and limited functional improvement, but in selected cases, surgery seems beneficial.

Ocular Spiroplasma ixodetis in Newborns, France.

Cataract and uveitis are rare in newborns but potentially blinding. Three newborns with cataract and severe anterior uveitis underwent cataract surgery. Spiroplasma ixodetis was detected in lens aspirates using bacterial 16S-rRNA PCR and transmission electron microscopy. These findings, which suggest maternal-fetal infection, are consistent with previous experimental Spiroplasma-induced cataract and uveitis.

Cataract Surgery with Primary Lens Implantation in Children with Chronic Uveitis.

PURPOSE: To evaluate the evolution of chronic uveitis in children undergoing cataract surgery with primary intraocular lens (IOL) implantation. METHODS: Twelve children with chronic uveitis underwent cataract surgery with primary posterior chamber intraocular lens (IOL) implantation. RESULTS: Fourteen eyes were implanted with a foldable hydrophobic acrylic IOL. The mean follow-up was 35.39 months (8.72-69.57). The mean BCDVA before surgery and at the end of follow-up was 1.11 (0.40-2.30; SD: 0.57) and 0.48 (0-3; SD: 0.77; p=0.007) respectively. The mean oral corticosteroids dosage after surgery and at the end of follow-up was 0.80 mg/kg/day (SD: 0.37) and 0.17 mg/kg/day (SD: 0.24; p=0.001) respectively. All patients except one were treated with methotrexate. Four patients (5 eyes) were additionally treated with anti-tumor necrosis factor agent. CONCLUSIONS: Cataract surgery with primary posterior chamber hydrophobic IOL implantation is possible and leads to a good visual recovery in cases of pediatric chronic uveitis. This surgery requires aggressive anti-inflammatory management with immunosuppressive drugs to control inflammation and reduce the corticosteroids dosage.

Pseudo-accommodation in non-amblyopic children after bilateral cataract surgery and implantation with a monofocal intraocular lens: prevalence and possible mechanisms.

BACKGROUND: Some pseudophakic patients implanted with a monofocal intraocular lens (IOL) have good near visual acuity (VA) with their distance correction. The objective was to evaluate the prevalence of pseudo-accommodation in children after bilateral cataract surgery, without amblyopia, and to define its mechanisms. METHODS: Observational study that took place in a pediatric ophthalmology department, Paris, France. A total of 68 eyes were included, 40 from 23 children and 28 from 14 adults, with a corrected distance VA above 20/25 and a normal near VA (20/25) with +3 addition. Pseudo-accommodation was defined as a near VA better than 20/50 with the distance correction and without addition. Prevalence of pseudo-accommodation was calculated in each group. In order to determine the possible mechanisms of pseudo-accommodation in children, we compared children with pseudo-accommodation and adults without pseudo-accommodation regarding several parameters: refraction, axial length, corneal topography, aberrometry, pupillary diameter and IOL shift after cyclopentolate instillation. RESULTS: Among the children group, 36 (90 %) had pseudo-accommodation versus 2 (7 %) in the adult group. We found that spherical equivalent, implant power, corneal multifocality and corneal higher-order aberrations (mainly coma and trefoil) were significantly higher in the pseudo-accommodation group, while pupil diameter and implant shift were not significantly different. CONCLUSIONS: Pseudo-accommodation has a high prevalence among non-amblyopic pseudophakic children. Several possible mechanisms have been found to explain pseudo-accommodation in children: a high power of the IOL and a small axial length, maximizing the effect of the IOL shift, corneal multifocality and corneal higher-order aberrations.

LONG-TERM OUTCOME OF EPIRETINAL MEMBRANE SURGERY IN YOUNG CHILDREN.

PURPOSE: To describe the clinical characteristics of epiretinal membranes (ERMs) secondary to nonprogressive diseases in very young children and to assess their surgical outcome. METHODS: Retrospective interventional case series study of 13 pediatric patients (aged less than 12 years) operated on for ERM more than a 6-year period. Visual acuity measurement, ophthalmic examination, and optical coherence tomography imaging were assessed preoperatively and postoperatively. RESULTS: Mean age was 6.5 years (3-12 years). Mean follow-up duration was of 28.5 months (12-69 months). Among the 13 patients, there were 7 cases of idiopathic ERM, 4 cases of combined hamartoma of the retina and retinal pigment epithelium, and 2 cases of posttraumatic ERM. The diagnosis of ERM was fortuitous in 10 cases (77%). Twelve children (92%) experienced a functional improvement. All patients received adjuvant treatment of amblyopia. Best-corrected visual acuity improved significantly after surgery from 20/160 to 20/40 (P = 0.001). CONCLUSION: Even in very young children, surgery resulted in a significant long-term improvement. Children screening was essential for diagnosis and treatment in most cases.

Should unilateral congenital corneal opacities in Peters' anomaly be grafted?

PURPOSE: To evaluate the surgical outcomes and visual results after penetrating keratoplasty for congenital unilateral corneal opacities in Peters anomaly. METHODS: This was a retrospective study of 14 children who underwent keratoplasty for a unilateral Peters anomaly between 1999 and 2009. All patients received a topical treatment of cyclosporine and corticosteroids. Suture ablation was performed between the first and second postoperative month and all patients had spectacle correction and amblyopia treatment. Age at time of surgery, posterior segment status evaluated by ultrasonography, axial length, preoperative intraocular pressure, fellow eye status, mean follow-up time, early and late complications, final graft outcome, refraction, and visual acuity upon the last visit were noted. RESULTS: Mean age at the time of surgery was 9 months. Posterior segment was normal in all cases. Mean axial length was 18 mm and mean intraocular pressure was 11 mmHg. Mean follow-up was 30 months. Four children had graft rejection episodes, 2 of which responded to medication. Four underwent cataract surgery, one developed pupil deformation requiring a pupilloplasty, and 2 cases were complicated by surgical glaucoma. A total of 11 eyes (78.6%) had clear grafts at the end of the follow-up. Mean spherical equivalent was -1.5 D. Visual acuity was measurable in 3 cases and was 20/50 in one case, 20/63 in the second, and 20/2000 in the third. Concerning preverbal children, central, steady, and maintained fixation was found in 8 cases. CONCLUSIONS: Operating on a unilateral congenital corneal opacity is a challenging decision and should be taken after explaining the need for long-term follow-up to the parents. Useful vision can be achieved and maintained after strict amblyopia therapy. A multidisciplinary ophthalmologic follow-up is necessary.

Vitreoretinal dysplasia masquerading as Peters' anomaly.

PURPOSE: Vitreoretinal dysplasia is characterized by a congenital bilateral nonattachment of the retina. In some cases, anteroposition of the iris and lens can lead to corneolenticular contact and corneal opacity, masquerading as Peters' anomaly. We report 3 cases of vitreoretinal dysplasia initially diagnosed as Peters' anomaly. METHODS: Case report. RESULTS: Case 1: In a 3-year-old boy with bilateral corneal opacities, high frequency ultrasound showed iridolenticular adherences as in Peters' anomaly, but color Doppler imaging of the posterior pole revealed a bilateral total retinal detachment. Case 2: In an 18-month-old boy with bilateral corneal opacity and dense cataract, ultrasound revealed a shallow anterior chamber and bilateral vitreous hemorrhage with retinal detachment. Case 3: In a 6-month-old girl with bilateral central corneal opacity, shallow anterior chamber, iridocorneal synechiae, and a cataract, ultrasound revealed a total retinal detachment in the right eye, and was uncertain in the left eye. Corneal transplantation was attempted in the left eye, but a retinal detachment was discovered peroperatively. There were associated central nervous system anomalies. CONCLUSIONS: Vitreoretinal dysplasia is a potentially evolving disease and can lead to anterior chamber thinning, corneal opacity, and secondary glaucoma. Anterior and posterior segment ultrasound is essential to fix the correct diagnosis and prognosis.

Management of retinal detachment in Coats disease. Study of 15 cases.

PURPOSE: To correlate the final outcome with the initial presentation and treatment in Coats disease retinal detachment. METHODS: The records of 15 patients with retinal detachment were evaluated retrospectively regarding the age at the time of diagnosis, initial presentation, methods of treatment, visual and anatomic results, and complications. Changes in vision and retinal status were noted and correlated with the different methods of treatment to propose a therapeutic strategy. RESULTS: In 15 patients (15 eyes), with a mean follow-up of 28 months (range, 6 months to 7 years), Coats disease was diagnosed at a mean age of 3.4 years (range 3 months to 15 years). Primary management was laser photocoagulation in seven patients, cryotherapy in two, and vitreoretinal surgery in six. Additional treatment was necessary in nine patients of whom six had laser photocoagulation, one had cryotherapy, and two had vitreoretinal surgery. Visual stability was achieved in 12 cases. Anatomic improvement was achieved in 12 eyes (3 cases of phthisis bulbi). No enucleation was ultimately necessary. CONCLUSIONS: Carefully selected treatment can improve almost each eye with Coats disease complicated by retinal detachment. Although visual outcome is poor, anatomic improvement or stability is the main goal of the management.

Pathophysiology of zonular diseases.

PURPOSE OF REVIEW: The mechanisms implicated in the clinical manifestations of zonular diseases, especially ectopia lentis, are reviewed. RECENT FINDINGS: The molecular mechanisms involve fibrillin in a large spectrum of heritable diseases characterized by zonular stretching. The usual complications are refractive errors, especially myopia, glaucoma (either primary open angle, secondary angle closure and pupil block by anterior displacement of the lens) and retinal detachment. SUMMARY: The genetics and molecular understanding provide information for genetic counseling. Treatment of myopia and glaucoma depend on the underlying mechanism, and lens surgery techniques are continuously improved.

Non-penetrating deep sclerectomy for glaucoma associated with Sturge-Weber syndrome.

PURPOSE: To report the results of non-penetrating deep sclerectomy (NPDS) in the treatment of glaucoma associated with Sturge-Weber syndrome (SWS). METHODS: We carried out a retrospective case series analysis of patients who underwent NPDS for glaucoma associated with SWS between 1998 and 2003. The control of glaucoma after NPDS, the results of surgery on intraocular pressure, the need for additional medical treatment and surgical complications were studied. RESULTS: Twelve eyes of nine patients, aged 11 days to 24 years, underwent filtering surgery: nine NPDS procedures were performed and three surgical procedures had to be converted to trabeculectomy because NPDS was not technically achievable. The mean follow-up after surgery was 26.3 months (range 6-48 months). Two trabeculectomies were complicated by choroidal effusion, which resolved in both cases. Good control of glaucoma was obtained during follow-up. CONCLUSIONS: Non-penetrating deep sclerectomy is transiently efficient in the treatment of SWS-associated glaucoma. Further studies of NPDS for the treatment of glaucoma associated with SWS are warranted.

Iris fixation of foldable intraocular lenses for ectopia lentis in children.

PURPOSE: To describe a technique for iris fixation of acrylic intraocular lenses (IOLs) in ectopia lentis and evaluate the medium-term anatomical and functional results in children. SETTING: Pediatric Ophthalmology Department, Fondation Rothschild, Paris, France. METHODS: This retrospective study included 17 eyes of 9 children. The technique consisted of lens ablation, leaving in place the capsule in the zone of nonruptured zonule, and an IOL placement in the remaining sulcus with 2 iris sutures. The age of patients, postoperative complications, follow-up, anatomical results, final refraction, and visual acuity were noted. RESULTS: Median age was 4.8 years. One case of postoperative hyphema and 1 case of aseptic endophthalmitis occurred and resolved after treatment. The anatomical outcome was favorable in all cases, with centered IOLs and round pupils. Mean follow-up was 16.3 months. Mean final refraction was +0.18 diopter, and mean final best corrected visual acuity was 20/32 (range 20/50 to 20/20). CONCLUSIONS: The results suggest that iris fixation of foldable IOLs for ectopia lentis in children can lead to good anatomical and functional results. The use of the remaining part of the sulcus is helpful in reaching correct positioning of the IOL. The complication rate is similar to that with other techniques described in the literature, whereas the small incision and the absence of transscleral sutures could simplify the short-term and long-term evolution.