[Painful anisocoria in a five-year-old child: A rare diagnosis which must not be missed]. / Anisocorie douloureuse chez un enfant de cinq ans : un diagnostic rare à ne pas méconnaître.

Congenital ectropion uveae (CEU) is a rare anomaly of the embryonic development of the anterior segment of the eye. We report the case of a 5-year-old child with an undiagnosed CEU who was treated urgently for an acute angle closure attack. CASE DESCRIPTION: A 5-year-old child was referred urgently for evaluation of anisocoria with mydriasis of the right eye and severe headache. Brain imaging with contrast injection was initially performed in the pediatric emergency department and ruled out central nervous system pathology. The initial examination of the right eye revealed an intraocular pressure (IOP) of 37mmHg, corneal edema, congenital ectropion uveae, mydriasis with pupillary block, a closed angle on gonioscopy, and a clear lens. The examination of the left eye was unremarkable, with no visible CEU. The initial management consisted of medical treatment with topical glaucoma drops and miotics and acetazolamide at 10mg/kg/d. Re-evaluation under general anesthesia showed persistent mydriasis and no resolution of the pupillary block. Filtering surgery was performed in the absence of a complete response to medical treatment, allowing control of IOP without drops and complete regression of the corneal edema. DISCUSSION: CEU is a rare malformation, and pressure complications represent an insignificant proportion of pediatric glaucoma cases. The acute presentation of acute angle closure in this potentially blinding short-term setting, however, makes detection and management difficult in very young children in a great deal of pain. Only one similar case has been reported in the pediatric literature. CONCLUSION: Acute angle closure complicating CEU is exceptional and difficult to diagnose in a pediatric context. Parents of children with this predisposing condition should be informed of the need to consult urgently when clinical signs of elevated intraocular pressure appear.

Small Incision Lenticule Extraction in Myopic Eyes With Corectopia After Intraocular Lens Implantation.

ABSTRACT: We present a case of ametropia corrected by small incision lenticule extraction (SMILE) in a corectopia eye after cataract surgery. The patient's optical zone, nomogram, and centration were carefully considered. The intended corrections were -6.60 - 1.25 × 175 OD and -6.85 - 0.50 × 10 OS. M-sized and S-sized cones were recorded in the right and left eye, respectively. The diameter of the right eye optical zone was 7.00 mm, whereas that of the left eye was 6.50 mm. The corneal vertex was determined using the film mark method as the scanning center. Three months after surgery, the uncorrected distance visual acuities were 20/20 OD and 20/13 OS. The effective optical zone was 6.5 and 5.5 mm in the right and left eyes, respectively. It is feasible to correct ametropia with SMILE in patients with corectopia after intraocular lens implantation. The large optical zone will compensate for the visual disturbance.

Development of neovascular glaucoma after intraocular surgery in Pierson syndrome.

Purpose: To report a patient with Pierson syndrome who presented with neovascular glaucoma (NVG) after cataract surgery.Methods: Retrospective case report.Results: A 17-year old monocular female presented with sudden onset of pain and decreased vision in the right eye. On examination, she had intraocular pressure (IOP) of 50 mmHg, aggressive iris neovascularization (NVI) and 3-piece IOL. Fundus examination revealed pale disc with tessellated fundus and parapapillary atrophy. Vascular arcades were vertically stretched with avascular ischemic retina starting from the near periphery. Macula appeared thin and atrophic. An intravitreal injection of 0.05 mg/0.1 ml bevacizumab was given to the right eye followed by Ahmed glaucoma valve (AGV) implantation. Assessment of her brother revealed similar posterior segment changes. A subsequent urine analysis showed proteinuria and high albumin to creatinine ratio. Next-generation sequencing for LAMB2 gene revealed a homozygous c.4573 + 1 G > A variant confirming the diagnosis of Pierson syndrome.Conclusion: This case expands our knowledge on retinal ischemia in the setting of Pierson syndrome. Close monitoring after intraocular surgery is recommended to look for the development of NVG.

Phacoemulsification and 1% atropine eye drops for treatment of antimetropic congenital microcoria associated with cataracts / Facoemulsificação e colírio de atropina a 1% para tratamento de microcoria congênita antimetrópica associada a catarata

ABSTRACT A rare case of bilateral congenital microcoria associated with antimetropia in a 47-year-old man is here described. The patient presented with a chief complaint of progressive vision loss in his right eye over the past five years. A slit-lamp examination and ultrasound biomicroscopy confirmed congenital microcoria and cataracts. Phacoemulsification was performed using an iris expansion device and the anterior capsule was stained using the "trypan down under" technique. Preoperative considerations, the surgical approach, and postoperative management are discussed.

RESUMO Um caso raro de microcoria congênita bilateral associada à antimetropia em um homem de 47 anos de idade é descrito aqui. O paciente queixava-se de perda visual progressiva em seu olho direito nos últimos 5 anos. Um exame com lâmpada de fenda e biomicroscopia ultrassônica confirmaram microcoria congênita e catarata. A facoemulsificação foi realizada usando dispositivo de expansão iriana, e a cápsula anterior foi corada através da técnica "trypan down under". Considerações pré-operatórias, abordagem cirúrgica e manejo pós-operatório são discutidos.

The Aravind Pseudoexfoliation Study: Surgical and First-Year Postoperative Results in Eyes without Phacodonesis and Nonmiotic Pupils.

PURPOSE: To compare intraoperative complication rates, 1-year visual outcomes, and postoperative complication rates over the first postoperative year in eyes with and without pseudoexfoliation undergoing cataract surgery. DESIGN: Prospective, comparative, interventional study. PARTICIPANTS: Nine hundred thirty eyes with cataract and uncomplicated pseudoexfoliation (without phacodonesis, clinically shallow anterior chambers, or pupil size <4 mm) and 476 controls with cataract but without pseudoexfoliation recruited from 4 centers of the Aravind Eye Care System in Southern India. The 2 groups were randomized separately to receive either a single-piece acrylic intraocular lens (IOL; SA60AT; Alcon Laboratories, Fort Worth, TX) or a 3-piece acrylic IOL (MA60AS; Alcon Laboratories). The pseudoexfoliation group also was randomized to receive or not receive a capsular tension ring. METHODS: All eyes underwent phacoemulsification with IOL implantation and were followed up at 1 day, 1 month, 3 months, and 1 year after surgery. MAIN OUTCOME MEASURES: Association of pseudoexfoliation status with intraoperative complication rates, 1-year best-corrected visual acuity, and any other complications. RESULTS: Mean ages were 63.0±6.9 years and 57.9±7.3 years in the pseudoexfoliation and control groups, respectively (P < 0.001). Pseudoexfoliation patients were more likely to be men (P = 0.014), to have a nuclear opalescence grade of more than 4 (P = 0.001), and to have a pupil size of less than 6 mm (P < 0.001) when compared with controls. Intraoperative complication rates were 2.9% and 1.9% in the pseudoexfoliation and control groups, respectively (P = 0.29). One-year postoperative best-corrected visual acuity was comparable (P = 0.09). Complication rates at 1 year were 2.7% and 2.5% in the pseudoexfoliation and control groups, respectively (P = 0.82). Average endothelial cell loss was 14.7% in the pseudoexfoliation group and 12.7% in the control group at 1 year (P = 0.066) when adjusting for age and nuclear opacity. CONCLUSIONS: Pseudoexfoliation eyes without shallow anterior chamber, small pupils, or apparent zonulopathy may represent eyes with lower risks of complications. Despite smaller pupils and denser cataracts, pseudoexfoliation eyes without clinically apparent preoperative zonulopathy were not at a higher risk of intraoperative or postoperative complications or worse visual outcomes after cataract surgery.

Neuro-Ophthalmic and Clinical Characteristics of Brain Tumours in a Tertiary Hospital in Ghana.

BACKGROUND: Anecdotally, increasing number of patients are seen at Korle Bu Teaching Hospital (KBTH) with brain tumour. Neuro-ophthalmic symptoms and signs may help in timely diagnosis and intervention. OBJECTIVE: To evaluate the neuro-ophthalmic and clinical characteristics of brain tumour in patients presenting at a tertiary hospital in Ghana. STUDY DESIGN: A prospective case series involving 36 consecutive patients newly diagnosed with brain tumour from November 2010 to October 2011, at the Ophthalmology, Neurosurgery and Endocrine units of KBTH, Ghana. All patients had clinical diagnosis of brain tumour with confirmation by computerized tomography (CT) or magnetic resonance imaging (MRI). Thirteen patients had histological confirmation of diagnosis. OUTCOME MEASURES: Presenting Visual acuity, Colour vision, Visual fields and Cranial nerve deficits. RESULTS: Data of 36 patients were analyzed. Ages ranged from 3 to 69 years, mean (SD) 42.56 (±16.6 years). Twenty-six (72%) were females. Tumours included pituitary adenoma (20, 55.5%), meningioma(10, 27.8%), choroid plexus tumour(1, 2.8%), medulloblastoma(1, 2.8%), craniopharyngioma(1, 2.8%), haemangioblastoma(1, 2.8%), thalamic tumour(1, 2.8%) and haemangioma(1, 2.8%). Histologically confirmed tumours included pituitary adenoma (9, 69.2%), meningioma (3, 23.1%), craniopharyngioma (1, 7.7%). One patient had both a pituitary adenoma and meningioma. Blurred vision (30, 83.3%), headache (28, 77.8%) and photophobia (13, 36.1%) were predominant symptoms. Commonest neuro-ophthalmic signs were impaired colour vision (62 eyes, 88.6%), optic atrophy (26, 74.3%), unilateral or bitemporal hemianopia (15, 41.5%) and relative afferent pupillary defect (12, 34.3%). Seven (19.4%) patients were visually impaired and nine (25%) blind. Thirty-three of 72 (45.8%) eyes had monocular blindness. CONCLUSIONS: Common neuro-ophthalmic characteristics were blurred vision, headache, impaired colour vision, optic atrophy, and relative afferent pupillary defect (RAPD). Significant numbers of patients were blind or visually impaired at presentation.

Mechanism and management of angle closure in uveitis.

PURPOSE OF REVIEW: To summarize the multiple mechanisms responsible for angle closure in uveitis and to outline the management principles and treatment modalities. RECENT FINDINGS: Angle closure in uveitis is a heterogeneous disease with multiple mechanisms. Recent advances in anterior segment imaging have provided insights into the mechanisms of angle closure in uveitis. Uveitic eyes with angle closure from pupil block require surgical iridectomy with mobilization of the peripheral iris and viscogoniosynechiolysis of both posterior synechiae and peripheral anterior synechiae. Systemic conditions associated with uveitis can result in anterior displacement of the iris-lens diaphragm, and present as acute angle closure. Pupil block is not the predominant mechanism in these eyes, and management is primarily medical. Data are limited on the optimal treatment of angle closure in uveitis, and the role of glaucoma filtration surgery, cataract extraction, minimally invasive glaucoma surgery and newer modalities of cycloablation require evaluation. SUMMARY: The management of angle closure in uveitis should adhere to the principles of managing both uveitic glaucoma and angle closure. Identification of the mechanism of angle closure in uveitic eyes may enable treatment to be targeted at the responsible mechanism.

PERSISTENT PUPILLARY MEMBRANE OR ACCESSORY IRIS MEMBRANE?.

Frequently, in literature and curent practice, accessory iris membrane (AIM) and persistant pupillary membrane (PPM) are confused. Both AIM and PPM are congenital iris anomalies in which fine or thick iris strands arrise form the collarette and obscure the pupil. AIM, which is also called iris duplication, closely resembles the normal iris tissue in color and thickness and presents a virtual second pseudopupil aperture in the centre while PPM even in its extreme forms presents as a translucent or opaque membranous structure that extends across the pupil and has no pseudopupil. Mydriatiscs, laser treatment or surgery is used to clear the visual axis and optimize visual development. Surgical intervention is reserved for large, dense AIMs and PPMs. Our patient, a 29 year old male, has come with bilateral dense AIM, bilateral compound hyperopic astigmatism, BCVA OD = 0.6, BCVA OS = 0.4, IOP OU = 17 mmHg. To improve the visual acuity of the patient we decided to do a bilateral membranectomy, restoring in this way transparency of the visual axis. After surgery, the visual acuity improved to BCVA OD= 0.8, BCVA OS=0.8.

Acute angle-closure glaucoma in a patient with miller fisher syndrome without pupillary dysfunction.

PURPOSE: To report a case of an angle-closure glaucoma in a patient with Miller Fisher syndrome (MFS) without pupillary dysfunction. METHODS: We present a case report of a 75-year-old male presenting with total ophthalmoplegia, complete bilateral ptosis, and gait disturbance. He was diagnosed with MFS without pupillary dysfunction, which precipitated unilateral acute angle-closure glaucoma (AACG) due to complete lid ptosis. RESULTS: The initial ocular examination revealed hand motion in the right eye. Intraocular pressure, as assessed by Goldmann applanation tonometry, was 50 mm Hg, and gonioscopic findings revealed a closed angle on the right eye. After maximal tolerated medical therapy, laser peripheral iridotomy was performed. The unilateral AACG with MFS resolved without further incident. CONCLUSIONS: This is the first reported case of a patient with MFS without autonomic dysfunction and AACG. We believe that pupillary dysfunction or lid ptosis due to neurological disorders may increase the possibility of AACG.

Laser-assisted cataract surgery following insertion of a pupil expander for management of complex cataract and small irregular pupil.

We report a case in which laser-assisted cataract surgery was performed safely and successfully following manual division of posterior synechiae and mechanical dilation of the pupil with a Malyugin ring in a patient with an irregular, small stuck-down pupil.

Femtosecond laser-assisted cataract surgery in eyes with a small pupil.

PURPOSE: To evaluate the efficacy of different treatment modalities in eyes with small pupils before femtosecond laser-assisted cataract surgery. SETTING: Ruhr University Eye Clinic, Bochum, Germany. DESIGN: Prospective clinical trial. METHODS: Eyes with an intraoperative pupil size smaller than 5.5 mm received sequential treatments to achieve a pupil larger than 5.5 mm in 3 steps: (1) intracameral administration of epinephrine solution, (2) additional viscomydriasis, and (3) implantation of a Malyugin ring pupil expander. When a step enlarged the pupil to at least 5.5 mm, femtosecond laser-assisted cataract surgery with an anterior capsulotomy diameter of at least 4.5 mm and 350 µm nuclear fragmentation grids, ultrasound phacoemulsification, and intraocular lens implantation were performed. Main outcome measures were achieved preoperative pupil size in each sequential treatment group and analysis of intraoperative complications. RESULTS: Of 850 eyes scheduled for cataract surgery, 40 received sequential treatments. To achieve a pupil larger than 5.5 mm, epinephrine was sufficient in 7% of the eyes; additional viscomydriasis was necessary in 25%, and the pupil expander was implanted in 68%. The most frequent comorbidities were pseudoexfoliation of the lens capsule (30.0%) and intraoperative floppy-iris syndrome (12.5%). Tongue-like lesions of the capsulotomies were detected in 5 eyes. CONCLUSIONS: The 3-step treatment allowed the surgeon to increase the efficiency and safety of femtosecond-assisted cataract surgery in eyes with a small preoperative pupil, providing good safety margins at the pupil boundary for the capsulotomy and the softened nuclear volume.

Primary orbital teratoma with tooth in an adult: a rare association with cataract and corectopia.

INTRODUCTION: Orbital teratoma is a known cause of proptosis in neonates but it is rare in adults. CASE REPORT: An adult female presented with primary orbital teratoma with a well formed tooth inside the multicystic mass. Anterior orbitotomy with cyst excision was done with retrieval of a canine tooth. COMMENT: This report documents a unique case of orbital teratoma presenting in an adult with well formed canine tooth inside. Microcornea, corectopia and cataract were other distinctive coexisting features of our case.

Results of phacoemulsification with Malyugin Ring in comparison with manual iris stretching with hooks in eyes with narrow pupil.

PURPOSE: To evaluate the results of phacoemulsification in eyes with a narrow pupil dilated with Malyugin Ring, in comparison with manual pupillary stretching with hooks. SETTING: Department of Ophthalmology, Medical University of Lodz, Poland. MATERIALS AND METHODS: The examined group (group 1) consisted of 23 eyes of 23 patients whose pupil was dilated using Malyugin Ring. The reference group (group 2) consisted of 17 eyes of 17 patients whose pupil was dilated manually by stretching with hooks. All patients underwent uneventful standard phacoemulsification. Patients were examined preoperatively, 1 day, and 1 month after the surgery. Best-corrected visual acuity (BCVA), intraocular pressure, anterior and posterior segment of the eye, and corneal endothelial cell density were evaluated. Statistical analysis was done using nonparametric tests. RESULTS: Mean preoperative BCVA in group 1 was 0.31±0.27 and in group 2 it was 0.26±0.26 (p>0.05). In both groups there was a significant improvement in BCVA after the surgery (p<0.05). Mean postoperative BCVA in group 1 was 0.75±0.30 and in group 2 it was 0.56±0.56 (p<0.05). Mean corneal endothelial cell loss measured 30 days postoperatively amounted to 9.35±11% in group 1 and 13.77±8.0% in group 2 (p<0.05). No serious complications were found. CONCLUSIONS: In eyes with narrow pupil, the use of Malyugin Ring makes the surgery easier and allows for better functional results and smaller corneal endothelial cell loss in comparison with manual pupillary stretching with 2 hooks.

Fixed, dilated pupils following traumatic brain injury: historical perspectives, causes and ophthalmological sequelae.

Pupillary abnormalities are commonly seen in patients presenting with severe traumatic brain injury (TBI). The objectives of this study were to determine the underlying condition responsible, the natural history of recovery of third nerve palsy and the ultimate clinical outcome in 60 patients admitted to a regional neurosurgical centre with a diagnosis of TBI and unilateral or bilateral fixed, dilated pupils (FDP). In approximately three-quarters of cases, some form of road traffic incident was the mechanism of injury. In patients presenting with a unilateral FDP, the CT-defined condition was most commonly diffuse brain injury (49%) with no obvious lateralising condition. In 34% of cases CT demonstrated a lateralising condition ipsilateral to the side of the FDP and in 9% cases the FDP was contralateral to the side of the CT abnormality. Of those patients who survived an FDP, 72% were left with some form of ophthalmological deficit. Most patients with bilateral FDP did not survive (88%); however, of those who did survive, none was left in a persistent vegetative state or with any ophthalmological sequelae. A FDP is a grave prognostic sign following TBI commonly resulting in long term ophthalmological sequelae; however, a favourable outcome is still attainable.

Keratopigmentation (corneal tattooing) for the management of visual disabilities of the eye related to iris defects.

AIM: To investigate the functional and cosmetic outcomes of keratopigmentation (KTP) in cases of moderate to severe visual dysfunctions owing to different iris disorders. METHODS: 11 eyes with moderate to severe visual disabilities related to iris defects underwent KTP for functional and cosmetic restoration using micronised mineral pigments and assisted with modern technologies such as femtosecond laser and new automated keratopigmentation instruments for the intrastromal and superficial application of the pigments. RESULTS: Following the KTP surgery, the visual-function-related symptoms improved in all cases, from significant improvement to total elimination. Eight patients were asymptomatic after the surgery. In two patients, minimal non-disabling symptoms remained after surgery. One patient with traumatic aniridia complained of significant residual glare at the 3-month postoperative visit and was reoperated to reduce the simulated pupil to 4 mm. The cosmetic outcomes were analysed and classified as excellent in eight patients and good in three. CONCLUSION: KTP using new micronised mineral pigments and new surgical protocols has proven in this series to be an effective surgical technique for the management of moderate to severe visual dysfunctions related to iris defects.