Long-term anatomical and functional outcomes of surgical treatment of retinal complications in children and adolescents with Stickler syndrome between 2004 and 2021.

BACKGROUND: Stickler syndrome (STL) is an inherited progressive connective tissue collagen disorder. STL is the most common hereditary cause of retinal complications, retinal tears, and the development of retinal detachment (RD) in childhood. The aim of the study was to evaluate the long-term anatomical and functional results of surgical treatment of retinal complications in children and adolescents affected by STL. METHODS: A retrospective, single-center study was performed a cohort of children with STL who underwent retinal surgery between 2004 and 2021. RESULTS: The study group consisted of nine children; the mean age at the time of the retinal tear with/without retinal detachment was 7.2 (2-10) years, and the mean follow-up period was 9.6 (5-16) years. Pathogenic variants COL2A1 (5 children) and COL11A1 (3 children) were confirmed in our cohort. In total, we operated on 13 eyes, 11 eyes with complicated RD and two eyes with multiple retinal defects, but without RD. At the end of the follow-up period, an attached retina was achieved 77% (10 eyes) with or without silicone oil tamponade: cryopexy alone was successful in one eye (10%), scleral buckling (EB) in five eyes (50%), and vitrectomy with silicone oil tamponade combined with EB in four eyes (40%). The mean number of surgeries was 2.3 per eye. The resulting best corrected visual acuity ranged from 0.03 to 0.1 in one eye, from 0.16 to 0.4 in two eyes, and from 0.5 to 1.0 in 7 eyes. CONCLUSION: Repair of retinal tears with/without retinal detachment in patients with Stickler syndrome often requires multiple surgeries with combinations of cryopexy, scleral buckling, and/or vitrectomy with silicone oil tamponade. Treatment of the ocular complications arising from STL requires long-term comprehensive care.

Long-term outcomes of "open iridectomy" for secondary anterior chamber epithelial iris cysts.

Epithelial cysts run a high risk of recurrence and conversion to sheet-like ingrowth after surgical intervention. In this retrospective study, we introduced a modified iridectomy for treatment of secondary epithelial iris cysts (EICs) in the anterior chamber. Twenty-nine patients (29 eyes) aged 2-61 years received "open iridectomy" for EICs between April 1995 and July 2019. After viscodissection, most of the cyst wall was cut using a 20-gauge aspiration cutter via a 2.5-mm clear corneal incision. The residue closely adhering to the iris stroma was remained to avoid photophobia and diplopia. At 3 months, best corrected visual acuity was ≥ 20/100 in 55.5% (15/27, except two pediatric patients with poor cooperation) of patients. Among the eight patients suffering partial corneal edema preoperatively, six patients received surgery treatment at 3-6.5 months, and the cornea in the other two patients became transparent after medication. In a mean follow-up of 47.4 months, recurrence occurred in 3 patients at 7, 37, and 118 months, respectively. The percentage of treatment success was 96%, 87%, and 65% at 1, 5, and 10 years, respectively. "Open iridectomy" was effective for EICs, with a minimal invasion, less damage to the corneal endothelium, and a low recurrence rate.

Role of ICAM-1 in impaired retinal circulation in rhegmatogenous retinal detachment.

Many studies have demonstrated that rhegmatogenous retinal detachment (RRD) leads to impaired retinal circulation. However, the involvement of inflammation in the RRD-induced worsening of retinal circulation was obscure. This retrospective observational study included 150 patients with primary RRD (macula-on, n = 63; macula-off, n = 87) who underwent 25-gauge microincision vitrectomy surgery (25G MIVS). Total retinal blood flow was represented by the mean blur rate (MBR) of the optic nerve head vessel, measured by laser speckle flowgraphy preoperatively and until 6 months postoperatively. Aqueous humor samples were obtained during surgery to determine cytokine concentrations by enzyme-linked immunosorbent assay. At 3 and 6 months postoperatively, there were no significant differences between eyes with macula-on RRD and fellow eyes. However, in macula-off RRD, MBR remained significantly lower in RRD eyes 6 months postoperatively (P < 0.05). Log-transformed levels of soluble intercellular adhesion molecule-1 (sICAM-1) were negatively correlated with relative MBR (r-MBR, RRD eye/fellow eye) before surgery (r = - 0.47, P = 0.01) in macula-on, but not macula-off, RRD. Six months postoperatively, r-MBR correlated significantly with sICAM-1 levels (r = - 0.36, P = 0.02) in macula-off RRD. ICAM-1 may play a role in RRD-induced deterioration of retinal circulation.

Management of a case of Enhanced S-cone syndrome with massive foveoschisis treated with pars plana vitrectomy with silicone oil tamponade.

Introduction: Goldmann Favre Syndrome (GFS) is a vitreoretinal degenerative disease with macular retinoschisis. The current treatment of foveoschisis is topical and oral carbonic anhydrase inhibitors.Case: A 22-year-old male diagnosed with GFS presented a progressive decrease in vision of the right eye. The optical coherence tomography showed a significant macular schisis. A medical treatment with topical and oral carbonic anhydrase inhibitors was ineffective. We performed a pars plana vitrectomy and silicone oil placement which led to an improvement of the visual acuity and a reduction of the foveoschisis.Conclusion: We describe here the first case of surgical treatment for macular schisis in a patient with GFS.

Glaucoma-Related Adverse Events at 10 Years in the Infant Aphakia Treatment Study: A Secondary Analysis of a Randomized Clinical Trial.

Importance: Glaucoma-related adverse events constitute serious complications of cataract removal in infancy, yet long-term data on incidence and visual outcome remain lacking. Objective: To identify and characterize incident cases of glaucoma and glaucoma-related adverse events (glaucoma + glaucoma suspect) among children in the Infant Aphakia Treatment Study (IATS) by the age of 10.5 years and to determine whether these diagnoses are associated with optic nerve head (ONH) and peripapillary retinal nerve fiber layer (RNFL) assessment. Design, Setting, and Participants: Analysis of a multicenter randomized clinical trial of 114 infants with unilateral congenital cataract who were aged 1 to 6 months at surgery. Data on long-term glaucoma-related status and outcomes were collected when children were 10.5 years old (July 14, 2015, to July 12, 2019) and analyzed from March 30, 2019, to August 6, 2019. Interventions: Participants were randomized at cataract surgery to either primary intraocular lens (IOL), or aphakia (contact lens [CL]). Standardized definitions of glaucoma and glaucoma suspect were created for IATS and applied for surveillance and diagnosis. Main Outcomes and Measures: Development of glaucoma and glaucoma + glaucoma suspect in operated-on eyes up to age 10.5 years, plus intraocular pressure, axial length, RNFL (by optical coherence tomography), and ONH photographs. Results: In Kaplan-Meier analysis, for all study eyes combined (n = 114), risk of glaucoma after cataract removal rose from 9% (95% CI, 5%-16%) at 1 year, to 17% (95% CI, 11%-25%) at 5 years, to 22% (95% CI, 16%-31%) at 10 years. The risk of glaucoma plus glaucoma suspect diagnosis after cataract removal rose from 12% (95% CI, 7%-20%) at 1 year, to 31% (95% CI, 24%-41%) at 5 years, to 40% (95% CI, 32%-50%) at 10 years. Risk of glaucoma and glaucoma plus glaucoma suspect diagnosis at 10 years was not significantly different between treatment groups. Eyes with glaucoma (compared with eyes with glaucoma suspect or neither) had longer axial length but relatively preserved RNFL and similar ONH appearance and visual acuity at age 10 years. Conclusions and Relevance: Risk of glaucoma-related adverse events continues to increase with longer follow-up of children following unilateral cataract removal in infancy and is not associated with primary IOL implantation. Development of glaucoma (or glaucoma suspect) after removal of unilateral congenital cataract was not associated with worse visual acuity outcomes at 10 years. Trial Registration: ClinicalTrials.gov Identifier: NCT00212134.

Outcomes of primary rhegmatogenous retinal detachment repair with extensive scleral-depressed vitreous removal and dynamic examination.

There are multiple surgical approaches to the repair of rhegmatogenous retinal detachment (RRD). Here, we evaluated the outcomes of small-gauge pars plana vitrectomy (PPV), alone or in combination with scleral buckle (SB-PPV), for RRD repair using a standardized technique by 3 vitreoretinal surgeons: "extensive" removal of the vitreous with scleral depression and dynamic examination of the peripheral retina. One hundred eighty seven eyes of 180 consecutive patients treated for primary RRD by three vitreoretinal surgeons at a tertiary academic medical center from September 2015 to March 2018 were analyzed. Most RRDs occurred in males (134 [71.3%] eyes), affected the left eye (102 [54.3%]), and were phakic (119 [63.3%]). PPV alone was performed in 159 eyes (84.6%), with a combined SB-PPV used in the remaining 29 eyes (15.4%); focal endolaser was used in all (100%) cases. The single surgery anatomic success rate was 186 eyes (99.5%) at 3 months, and 187 (100%) at last follow up. Overall best-corrected visual acuity (BCVA) had significantly improved at 3 months ([Snellen 20/47] P<0.00005) and last follow up ([Snellen 20/31] P<0.00005), as compared to day of presentation ([Snellen 20/234]). Our findings suggest that "extensive" removal of the vitreous and dynamic peripheral examination with scleral depression may lead to high single surgery success in primary uncomplicated RRD repair.

Update in Pediatric Pseudotumor Cerebri Syndrome.

Pseudotumor cerebri syndrome (PTCS) is a rare condition in children presenting with headache and papilledema from increased intracranial pressure that can cause significant morbidity. This can be idiopathic, also known as idiopathic intracranial hypertension or primary intracranial hypertension, or can be secondary to medications and associated medical conditions. Given the threat to vision, early detection and treatment is needed in all age groups. However, identifying papilledema or pseudopapilledema in children presents unique challenges sometimes as a result of differences between prepubertal and postpubertal children, further elucidating the complex pathophysiology. Management requires brain imaging, lumbar puncture, and frequent eye exams with medical and rarely surgical treatment. Visual outcomes in children are favorable if caught early and management can be prolonged over years. Pediatric PTCS is different from adult PTCS in many ways, and this review will focus on the most updated definitions of the disease, theories of pathophysiology, management, and treatment in the pediatric population.

Human allograft refractive lenticular implantation for high hyperopiccorrection.

Hyperopia is a common form of refractive error in the United States. Many refractive errors can be treated with refractive surgery methods such as laser in-situ keratomileusis and photorefractive keratectomy; however, in patients with large degrees of hyperopia (≥+5.0 diopters [D]), these surgical methods are limited because of higher rates of refractive regression. Lenticule Intrastromal Keratoplasty (LIKE) is a surgical procedure that can be used to correct refractive errors in patients with high hyperopia. The authors describe the first intrastromal implantation of an allograft lenticule performed for the primary correction of hyperopia in the United States, and demonstrate that LIKE is potentially an effective procedure for the correction of high hyperopia. Mechanisms for achieving the intended refractive correction and the complications our patient experienced, including epithelial ingrowth and flap necrosis, are discussed.

Outcome of vitrectomy for advanced proliferative vitreoretinopathy complicating primary rhegmatogenous retinal detachment among Nigerians.

AIM: To present the anatomical and visual outcome and compare different techniques in the surgical treatment of proliferative vitreoretinopathy (PVR) in Nigerians. METHOD: Comparative retrospective review of PVR grade C and D eyes that had vitreoretinal surgery with silicone oil between April 2005 and December 2012. Data was extracted from consecutive case notes after exclusion of eyes with PVR associated with proliferative diabetic retinopathy (PDR), proliferative sickle cell retinopathy (PSCR) and eyes with nonuse of silicone oil. A comparison of the outcome of vitrectomy alone (Vit.), versus combined with a scleral buckle (Vit.+SB), versus with retinectomy (Vit.+RT), versus with all three procedures (Vit.+SB+RT) was done. Statistical analysis was done using the Statistical Package for Social Sciences version 16 software. Pearson Chi-square test and Fisher's exact T-test were used to determine the effect of relationships. RESULTS: 138 eyes of 138 patients had grades C (100 eyes) and grade D (38 eyes) PVR. Surgery involved vitrectomy and membrane peel in 53% of eyes, additional scleral buckle in 22%, and retinectomy was performed in 17%. Retinal reattachment rate was 86% for PVR C eyes and 87% in PVR D eyes. There was no statistically significant difference in anatomical outcome between vitrectomy alone and the combination surgeries. In the vitrectomy only category, the postoperative vision was noted to improve (> preoperative), in 48% of PVR C and in 31% of PVR D. 33% of PVR C and 44% of PVR D eyes had a worse vision (< preoperative). Visual outcome was similarly poor in the combination surgeries with improved vision noted in 12%, 44%, and 33% of the Vit.+SB, Vit. +RT, and Vit.+SB+RT PVR C eyes, respectively. In PVR D eyes, improved vision was seen in 57% and 12% of Vit.+SB and Vit.+RT eyes, respectively. CONCLUSION: Surgery results in anatomical reattachment and there is nonsuperiority of any technique. Visual outcome is poor as previously reported. Recent trials of pharmacological adjuncts may show promise for improved visual outcomes.

New application of membrane blue-dual dye for retinal or iatrogenic break staining in retinal detachment surgery.

INTRODUCTION: The principles of surgery for managing primary rhegmatogenous retinal detachment (RRD) are to precisely identify and correctly treat all causative retinal breaks. Estimates regarding unidentified breaks complicating RRDs vary from 2.2%-22.5%. PURPOSE: To evaluate the efficacy of membrane blue-dual heavy dye solution for staining of undetected preoperatively retinal and iatrogenic breaks. SUBJECTS, MATERIAL AND METHODS: 1. This is a prospective interventional study. 23 and 27-gauge vitrectomy surgeries were evaluated for primary repair of 5 patients with rhegmatogenous retinal detachment (RRD). No breaks where identified prior to surgery despite meticulous pre-operative examination using binocular indirect ophthalmoscopy with indentation. 0.1ml of MembraneBlue-Dual™ was applied onto the vitreous cavity, while it was completely filled with fluid, and all excess dye was immediately aspirated with a blunt backflush instrument. In all eyes with RRD, the surgery was completed by gas tamponade (C3F8 or SF6). Follow up was 6 months. RESULTS: We compared the number of breaks identified when examined intraoperative with internal peripheral indentation before and after injection of the dye and found that in all cases (100%) at least one more break was found after injection of dye which was subsequently treated with cryotherapy or endolaser. At last follow up six months after surgery the success rate was 100% and none of our cases re-detached. CONCLUSIONS: The greatest advantage of use of this "heavy" dye solution membrane blue-dual is improved intraoperative identification of ILM at the edges of retinal breaks and the discrimination of them from surrounding intraocular structures. Due to its increased molecular weight and viscosity properties it eliminates the need for fluid-air exchange, injection of PFCL or subretinal injection.

Phakic intraocular lens implantation for the correction of hyperopia.

The prevalence of myopia is much higher than hyperopia. Hence, there are relatively few studies investigating phakic intraocular lens (pIOL) implantation for the correction of hyperopia. This review aimed to summarize the available relevant literature on the efficacy and safety of pIOL implantation for the correction of hyperopia and hyperopic astigmatism. At present, two types of pIOLs are used to correct hyperopia and hyperopic astigmatism: anterior chamber iris-fixated pIOLs and posterior chamber implantable collamer lenses. Both have been found to be safe and effective. No serious events (eg, retinal or choroidal detachment, endophthalmitis) were reported in the reviewed articles. Implantation of pIOLs might be the optimal refractive surgery for the correction of high hyperopia.

Rieger Syndrome: Rehabilitation With Dental Implants.

INTRODUCTION: Axenfeld-Rieger syndrome (ARS), also known as Rieger syndrome, is a rare autosomal dominant condition defined by craniofacial, ocular, dental, periumbilical, and systemic anomalies. CASE PRESENTATION: This case report describes in detail a multidisciplinary approach to successfully restore the oral function and esthetics of a 22-year-old patient diagnosed with ARS. The patient's clinical evaluation revealed that the area corresponding with teeth #13, #12, #11, #21, #22, and #23 was occupied by four malformed and/or deciduous teeth. The four anterior teeth were extracted, and socket preservation was performed using bovine-derived porous bone mineral. Six months after extractions, two implants were placed in the location of the lateral incisors and additional bone graft was performed. Two months after the initial healing, a temporary fixed partial was delivered and 9 months after implant placement the implants were restored with a porcelain-fused-to-metal fixed partial denture. CONCLUSIONS: The use of implant-supported fixed partial dentures to restore missing teeth in patients with ARS provides biological and mechanical advantages over conventional, fixed, or removable prosthodontics. Further evaluation is needed to determine the longevity and long-term prognosis of dental implants in patients with ARS.

Historical, Current and Future Approaches to Surgery for Rhegmatogenous Retinal Detachment.

Rhegmatogenous retinal detachment (RRD) is a serious condition that can cause blindness without surgical treatment. RRD occurs when a retinal tear or hole allows fluid to accumulate below the retinal surface, causing the retina to separate from the underlying layers. RRD is difficult to treat because each case is unique, varying with the location, size, and duration of the detachment, as well as patient age. The first successful methods to reattach the retina in RRD used thermocautery to repair the detachment. Many renowned ophthalmologists continued to study RRD and developed many new surgical approaches, notably: scleral buckling (SB), in which a silicone band is placed around the eye to reduce traction on the retina caused by the vitreous humor that fills the eye; pars plana vitrectomy (PPV), which eliminates traction on the retina by removing the vitreous; and pneumatic retinopexy (PR), in which the retina is reattached by pushing it back into place with an expanding gas bubble injected into the eye. However, no consensus has been reached on which approach is ideal. Furthermore, recent surgical and non-surgical breakthroughs, such as artificial vitreous substitutes and neuroprotective drugs, must also be considered. Thus, this review provides a guide for ocular specialists and non-specialists on the historical background of RRD, summarizes the three current main techniques (SB, PR and PPV) compares these three techniques, and provides an overview of new technologies that promise to greatly improve outcomes after RRD surgery.

Congenital fibrosis of the extraocular muscles: review of recent literature.

PURPOSE OF REVIEW: Congenital fibrosis of the extraocular muscles (CFEOM) is caused by abnormal development of the innervation of extraocular muscles. We update the recent literature regarding the clinical, anatomic, genetic, and molecular characteristics of CFEOM. Surgical considerations are addressed. RECENT FINDINGS: CFEOM is broken down into three main subtypes, CFEOM1, CFEOM2, and CFEOM3. Several recent reports of individuals, as well as family pedigrees, highlight the phenotypic heterogeneity of CFEOM. Intracranial and intraorbital radiologic findings have enhanced our understanding of the disease pathophysiology. Molecular genetics research has increased our understanding of the development of extraocular muscles and their innervation as well as pathophysiology of CFEOM. SUMMARY: Our understanding of the pathophysiology of CFEOM has increased with the recent contributions from neuroimaging, molecular genetics, and pedigree analysis. Surgical management of patients with CFEOM continues to be challenging.

Urrets-Zavalia Syndrome following cataract surgery in a case of anterior megalophthalmos / Síndrome de Urrets-Zavalia após cirurgia de catarata em um caso de megaloftalmo anterior

ABSTRACT Intraoperative and postoperative cataract surgery in eyes with anterior megalophthalmos are challenging procedures. Herein we describe the case of a 53-year-old male with anterior megalophthalmos who developed unilateral Urrets-Zavalia Syndrome following cataract surgery.

RESUMO O intraoperatório e o pós-operatório de cirurgia de catarata em olhos com megaloftalmo anterior é desafiador. Descrevemos o caso de um homem de 53 anos com megaloftalmo anterior que desenvolveu a Síndrome de Urrets-Zavalia unilateral após cirurgia de catarata.

Six-Month Outcomes After High Hyperopia Correction Using Laser-Assisted In Situ Keratomileusis With a Large Ablation Zone.

PURPOSE: To evaluate refractive and visual outcomes of laser-assisted in situ keratomileusis (LASIK) to treat high hyperopia using an aberration-neutral profile and large ablation zone. METHODS: This was a retrospective, consecutive observational case series at Helios Ophtalmologie, St. Jean-de-Luz, France. One hundred forty-six consecutive eyes of 77 patients who underwent LASIK with mechanical microkeratome to correct hyperopia with correction in the maximum hyperopic meridian strictly higher than +5 D (mean + 6.6 ± 1.0 D) were included. Procedures were performed with an Amaris 750S excimer laser (Schwind eye-tech-solutions GmbH, Kleinostheim, Germany) using an aberration-neutral profile, a 6.7 ± 0.1 mm optical zone, and a 9.2 ± 0.1 mm total ablation zone. Refractive results, predictability, safety, and efficacy were evaluated at 6 months postoperatively. RESULTS: At 6 months postsurgery, the mean manifest refraction spherical equivalent was -0.06 ± 0.83 D and the mean cylinder was 0.42 ± 0.35 D. Sixty-six percent of eyes were within ±0.50 D of the attempted spherical equivalent correction. Six months postoperatively, 60% of eyes achieved an uncorrected distance visual acuity of 20/20 or better. Ten percent of eyes lost 1 line of corrected distance visual acuity and 4% gained a line. No eyes lost more than 2 Snellen lines of corrected distance visual acuity at any follow-up. CONCLUSIONS: High hyperopia correction with LASIK using an aberration-neutral profile and large ablation zone provides good efficacy, safety, predictability, and visual outcomes.

Diagnosis of complicated FEVR preoperatively and intra-/post-operatively: characteristics and risk factors for diagnostic timing.

BACKGROUND: To delineate the characteristics of complicated familial exudative vitreoretinopathy (FEVR) patients diagnosed before surgery or intra-/post-operatively and to analyze the risk factors for the diagnostic timing. METHODS: Forty-eight patients who underwent surgery and were diagnosed as FEVR in our department were retrospectively reviewed. Data were collected including the demographic and clinical characteristics of these patients. FEVR patients were divided into 2 groups according to the diagnostic timing: FEVR diagnosed pre-operatively (23 patients), FEVR diagnosed intra-/post-operatively (25 patients). Multivariable analysis was applied for analyzing the risk factors for diagnostic timing. RESULTS: The clinical characteristics of the FEVR patients were of great variability, including retinal detachment (RD), disappear of anterior chamber, retrolental membrane, epiretinal membrane (ERM), vitreous hemorrhage (VH), myopic foveoschisis (MF), lamellar macular hole (LMH), high myopia (HM). And the referral diagnosis or pre-operative diagnosis were always non-specific. The majority of the referral or preoperative diagnosis were unilateral RD (52.1%), bilateral RD (8.3%), unilateral persistent fetal vasculature (PFV) (8.3%), bilateral PFV (4.2%). There are two risk factors for the complicated FEVR cases diagnosed as FEVR preoperatively: pre-operative ocular manifestations with RD only (OR, 0.104; p-value, 0.022), positive parent's fluorescein angiography (FA) (OR, 0.105; p-value, 0.035). CONCLUSIONS: The phenotypes of FEVR were greatly variable, they can mimic many non-specific vitreoretinal disorders. The most non-specific referral diagnosis/pre-operative diagnosis was unilateral RD, bilateral RD, unilateral PFV, bilateral PFV. A positive family history or a simple ocular presentation with RD only could contribute to diagnose FEVR preoperatively.

Spontaneous suprachoroidal hemorrhage in a high myopia patient with rhegmatogenous retinal detachment: a case report and literature review.

Purpose: To report a rare case of spontaneous suprachoroidal hemorrhage (SSCH) in a high myopia patient with rhegmatogenous retinal detachment (RRD) and successful treatment.Methods: We present a case of SSCH that occurred in a 73 woman with high myopia with RRD and discuss the results of a systemic review of the literature published from 1999 to 2017.Results: Phacoemulsification without intraocular lens implantation and vitrectomy combined with silicone oil injection was performed and retinal detachment and choroidal detachment were reattached after oil removed. In the literature review, we found that among a total of 36 patients (37 eyes), acute secondary glaucoma was a complication in 70.3% (26 eyes) of the cases, and over half of the cases (24 eyes, 64.9%) were treated with surgery. Eighteen cases (50%) were characterized by systemic hypertension and 21 cases (58.3%) had abnormal hemostasis. Age-related macular degeneration (ARMD) was the most common (12 eyes, 32.4%) ocular disease and was followed by glaucoma (7 cases, 18.9%). Visual acuity was classified as hand motion (HM) or worse in 25 eyes (out of 34 eyes, 73.5%) at initial presentation and in 25 eyes (out of 36 eyes, 69.4%) upon final examination. Nine cases experienced significant visual improvement, including six that underwent vitrectomy.Conclusion: Advanced age, systemic anticoagulation, and hypertension are strong risk factors. RRD associated with massive SSCH is an extremely rare event. Vitrectomy and choroidal blood drainage can effectively remove suprachoroidal hemorrhage (SCH) and promote retinal reattachment in these eyes. However, the final visual prognosis usually remains poor.

Progressive High Hypermetropic Shift as a Refractive Surprise Following Glaucoma Filtration Surgery in a Phakic Child With Early-Onset Childhood Glaucoma Associated With Axenfeld-Rieger Anomaly.

PURPOSE: We report a case of progressive high hypermetropia following glaucoma filtration surgery in a child with Axenfeld-Rieger syndrome and congenital glaucoma. METHODS/RESULTS: We report a case of a 4-day-old female child presented as a case of Axenfeld-Rieger anomaly with secondary glaucoma in both eyes (OU), who underwent combined trabeculotomy with trabeculectomy in OU at the age of 3 weeks. On postoperative third month, cornea cleared and posterior embryotoxon was noted in OU with Habb's striae in OS. Intraocular pressure (IOP) was controlled and fundus was normal in OU. Refraction was +2.00 D sph. in OD and 3.00D sph. in OS and was observed. On postoperative eighth month, IOP was controlled in OU, whereas retinoscopy showed refraction of +4.00D sph./-2.00D cyl at 30 degrees in OD and +10.00 D sph/-3.00 cyl at 120 degrees in OS, glasses were prescribed and was asked to review. After 4 years, the patient presented with blurring of vision, the best-corrected visual acuity noted was 20/100 in OD and 20/320 in OS. IOP was 28 and 14 mm Hg in OD and OS, respectively, but with healthy optic disc in OU. Refractive error had increased and was +5.50D sph in OD and +14.00D sph/-5.00 D cyl at 90 degrees in OS. Corneal topography showed cornea plana in OU, which was more in OS (K1: 32.6D and K2: 38.9D) compared with OD (K1: 38.7D and K2: 40.1D). The patient was started on glaucoma medications in OD. Four months later, IOP was controlled and refraction was stable in OU. CONCLUSIONS: Present case is the first to describe the unusual presentation of progressive high hypermetropia in a child with Axenfeld- Rieger anomaly with congenital glaucoma after surgical intervention for glaucoma. Childhood glaucoma is classically associated with myopic shift in refraction and refraction is one of the most important clinical parameter measured at every follow-up visit. Although progressive hypermetropic shift is a rare occurrence, clinicians should be aware of this possibility. Keratomerty should be performed when such refractive surprises arise, which may help detect the clinical condition and the etiology for such presentation.