UVEAL EFFUSION ASSOCIATED WITH LOCALIZED SCLERODERMA BECAUSE OF SCLERAL FIBROSIS.

BACKGROUND/PURPOSE: To report a case of uveal effusion associated with localized scleroderma because of scleral collagen fibrosis. Partial-thickness sclerectomy treatment was successful in acquiring the resolution of the uveal effusion. METHODS: Case report. RESULTS: A 44-year-old Chinese woman with known localized scleroderma visited the retinal clinic complaining of insidious onset blurring of vision in both eyes for 8 months. The best-corrected visual acuity was 20/200. Ophthalmoscopy revealed apparent inferior bullous serous retinal detachments in the right eye. Optical coherence tomography showed subretinal fluid and folds of the retinal pigment epithelium layer in both eyes. B-scan ultrasonographic image of the right eye confirmed a 360-degree serous retinal detachment in the right eye accompanied with increased thickness of the ocular wall. Ultrasound biomicroscopy of the anterior segment detected a shallow ciliary body detachment in the right eye. Fluorescein angiography and indocyanine green angiography demonstrated the leopard-spot pattern in all phases. Partial-thickness sclerectomy treatment was successful in acquiring the resolution of the uveal effusion. Histopathologic examinations of the sclera flaps revealed scleral collagen fibrosis. CONCLUSION: This clinicopathologic report first describes a patient with localized scleroderma and scleral collagen fibrosis, resulting in uveal effusion that responded to partial-thickness sclerectomy.

IDIOPATHIC PENETRATION OF CILIA INTO THE POSTERIOR SEGMENT PRESENTING AS SECTORAL SCLERITIS WITH PROGRESSIVE INTRAOCULAR INFLAMMATION.

PURPOSE: To report two cases of idiopathic intraocular cilia presenting as sectoral scleritis with progressive intraocular inflammation. METHODS: Both patients were treated with intravitreal antibiotics and underwent pars plana vitrectomy where the cilia were removed and identified on histopathology. RESULTS: One patient developed a retinal detachment while being treated for presumed endophthalmitis. The intraocular cilium was discovered during pars plana vitrectomy. In the second case, the cilium was detected on dilated fundus exam and was believed to be the cause of the patient's scleritis and vitritis. Therapeutic vitrectomy was performed. In both cases, the cilia were positively identified on histopathology. CONCLUSION: Idiopathic intraocular penetration of cilia should be considered in the differential diagnosis of sectoral scleritis with progressive intraocular inflammation.

Iris and ciliary body cysts and phakic intraocular lenses.

Phakic intraocular lenses are used to correct refractive errors. The procedure is predictable and potentially reversible. The procedure is not free of complications though. The occurrence of iris cyst after implantation of a phakic intraocular lens was not described previously in the literature. We describe two cases of iris cysts in the presence of a phakic intraocular lens; the first case describes a cyst which was not present prior to the anterior chamber phakic intraocular lens implantation. The second case describes a hidden iris cyst that affected the posterior chamber phakic intraocular lens position and lead to glaucoma.

Persistent Fetal Vasculature With Elongated Ciliary Processes in Children.

PURPOSE: Outcomes and reoperation rates in infants with unilateral persistent fetal vasculature (PFV) with elongated or stretched ciliary processes have not been extensively studied and were excluded from the Infant Aphakia Treatment Study (IATS). The purpose of this study is to analyze the preoperative measurements, reoperation rates, and complications after surgery in unilateral anterior PFV with stretched processes. DESIGN: Retrospective case series. METHODS: Inclusion criteria consisted of unilateral cataract surgery prior to 7 months of age and anterior PFV with elongated ciliary processes. Eyes with posterior retinal involvement and less than 6 months of follow-up were excluded. All patients underwent lensectomy, posterior capsulectomy, and vitrectomy. RESULTS: Eleven eyes of 11 patients were included. Patients had a mean age at surgery of 2.4 ± 1.4 months. Average follow-up was 4.5 ± 3.7years. Globe axial lengths were 18.6 ± 1.9 mm. Ten patients (91%) were initially left aphakic. Three patients (27%) later received a secondary intraocular lens (IOL), and 1 patient underwent an IOL exchange. Six out of 10 (60%) aphakic patients developed visual axis opacification. One aphakic patient required topical therapy for glaucoma. One additional patient developed neovascular glaucoma and retinal detachment. The eye was subsequently enucleated. Three patients underwent strabismus surgery. Two patients underwent pupilloplasty. Two patients (18.2%) had a final visual acuity better than 20/200. CONCLUSION: Eyes operated for PFV with elongated ciliary processes are unlikely to have a final visual acuity greater than 20/200 and many will need additional surgery. Postoperative visual axis opacification occurred in 60% and glaucoma developed in 18%.

Implantable collamer lens surgery in patients with primary iris and/or ciliary body cysts.

BACKGROUND: The prevalence of primary iris and/or ciliary body cysts is common in myopia, though asymptomatic in nearly all cases. It's a very valuable thing to study the clinical safety and reliability of implantable collamer lens (ICL) surgery in patients with primary iris and/or ciliary body cysts. METHODS: A total of 108 patients (201 eyes) were included in this retrospective study. All eyes had been implanted with V4c implantable collamer lens (ICLV4c). According to the eyes with or without primary iris and/or ciliary body cysts, all eyes were divided into two groups. We observed preoperative and postoperative uncorrected distance visual acuity (UDVA), corrected distance visual acuity)(CDVA), intra-ocular pressure(IOP), anterior chamber volume(ACV), anterior chamber depth(ACD), trabecular-iris angle (TIA), angle opening distance at 500 µm (AOD500),vertical central distance between the corneal endothelium and the front surface of ICL(CE-ICL), and the central vault. The follow-up periods covered 12 months. RESULTS: Among all the 201 eyes, primary iris and/or ciliary body cysts were detected in 54 eyes (26.87%),but the prevalence was account to 36.11%(18males,21females).There were 30 eyes (55.56%) with unilateral single cyst, 12 eyes (22.22%) with unilateral double cysts, 12 eyes (22.22%) eyes with unilateral multiple and/or multi-quadrants cysts, the mean size of cysts was (0.714 ± 0.149)mm(range from 0.510 to 1.075 mm).30.4% of the cysts were located at iridociliary sulcus, 65.5% in pars plicata, and 4.1% in midzonal iris, which showed a characteristic distribution pattern, with cysts found predominantly in the inferior and temporal quadrants.The postoperative size and the number of cysts showed nearly no changes. The postoperative ACV, AOD500 and TIA showed a statistical reduction in both two groups (P < 0.05), but with no statistical significant between the two groups (P > 0.05), the parameters of postoperative IOP,CE-ICL and central vault also showed the same results as which. We did not observe serious complication and IOP elevating in the whole follow-up periods. CONCLUSION: Primary iris and/or ciliary body cysts are not absolutely contraindication for ICL surgery. For some single cyst smaller than 1.075 mm or single quadrant cysts located at ciliary body are rare to lead some serious complications. But, for some multiple cysts, especially multi-quadrants cysts located at iridociliary sulcus, we still should remain cautions.

USE OF FIBEROPTIC-GUIDED CO2 LASER IN THE TREATMENT OF UVEAL EFFUSION.

BACKGROUND/PURPOSE: To report a new technique for treating patients with uveal effusion syndrome by the fiberoptic-guided CO2 laser. METHODS: Interventional case report. A 74-year-old man presented with exudative detachment of the choroid secondary to uveal effusion syndrome. Partial-thickness sclerotomy and full-thickness sclerotomy were performed to treat the disease using a fiberoptic-guided CO2 laser. RESULTS: After the surgery, the patient's visual acuity improved and choroidal folds disappeared. CONCLUSION: This technique allows concomitant coagulation and cutting, thereby reducing the risk of bleeding and providing better depth control.

Recurrent lacrimal gland choristoma of the ciliary body in an infant mimicking a medulloepithelioma.

PURPOSE: Choristoma is a congenital tumor made up of ectopic normal tissue. Different histopathologic subtypes have been described. Among them, lacrimal gland choristoma is found mainly in infants and can affect the iris, the ciliary body, or the choroid and epibulbar region. Our aims were to report a case of lacrimal gland choristoma, review the published cases, and present the main differential diagnoses. METHODS: A local resection of a limited mass of the ciliary body was performed on a 12-month-old girl who had a 6-month history of visual loss, leukocoria, and pupillary deformation. RESULTS: Histopathologically, we observed a well-demarcated lesion involved under the epithelium of the ciliary body. It was composed of acini delineated by a well-differentiated epithelium without atypia and mitotic figures. Immunohistochemical analyses confirmed the lacrimal nature with the expression of epithelial markers (cytokeratin 7 positive and cytokeratin 20 negative) and neuron-specific enolase without immunoreactivity for other neuronal markers. Two years later, a local recurrence appeared and was resected. It showed nearly the same histopathologic features. CONCLUSIONS: Lacrimal gland choristoma is a very rare lesion of the infant. Diagnosis is based on a histopathologic analysis with immunohistochemical studies to exclude other differential diagnoses such as a more common malignant tumor in childhood, medulloepithelioma. This observation shows an atypical clinical presentation of this benign lesion characterized by local recurrences.

Traumatic open globe injury in young pediatric patients: characterization of a novel prognostic score.

BACKGROUND: Open globe injury is a common cause of monocular blindness in children. Current formulas to predict outcomes of open globe injury often rely heavily on visual acuity and presence of an afferent pupillary defect, examination elements that are difficult to assess in young children. We aimed to analyze the features of open globe injuries in children aged 0-6 years to facilitate development of a novel algorithm for predicting visual outcomes in this age group. METHODS: The medical records of patients 0-6 years of age presenting at a single institution with open globe injury from 2000 to 2013 were retrospectively reviewed. Epidemiology, physical examination, and intervention data were used to develop a prognostic algorithm. RESULTS: A total of 28 patients were included. Mean age at presentation was 4.2 years (range, 1.9-6.7). Glass was the most common mechanism of injury. Associated findings included uveal prolapse (93%), choroidal detachment (39%), hyphema (32%), and retinal detachment (11%). In addition to primary repair, 43% patients required a lensectomy, and 7% underwent surgery to repair retinal detachment. Complicating cataract (P < 0.005) and a wound >6 mm (P < 0.05) were associated with a final visual acuity worse than 20/40. A novel algorithm for predicting visual outcome was devised with a sensitivity of 81% and a specificity of 92%. CONCLUSIONS: Patients 0-6 years of age with open globe injuries present unique risk factors for poor outcome. The trauma score generated by our algorithm is not reliant on presenting visual acuity and may be useful in predicting prognosis in very young children.

Case report: imaging and treatment of ophthalmic manifestations in oculodentodigital dysplasia.

BACKGROUND: Diagnostic and surgical management of severe chronic angle- closure glaucoma secondary to ciliary body cysts can be difficult to manage in a patient with oculodentodigital dysplasia. CASE PRESENTATION: A 6-year old girl with oculodentodigital dysplasia, with progressive chronic angle- closure glaucoma secondary to ciliary body cysts presented to our clinic. The initial examination revealed counting fingers vision in the left eye. Intraocular pressure (IOP), as assessed by tonopen, was 31 mm Hg. Ultrasound biomicroscopy revealed ciliary body cysts in the left eye, and gonioscopy confirmed chronic angle closure. A tube shunt was placed to control the elevated IOP. A year after her tube shunt placement in the left eye, ultrasound biomiscropy was performed on her right eye and showed no ciliary body cysts. Gonioscopy in the right eye revealed an open angle to the ciliary body band. Subsequent serial gonioscopy every 3 months showed gradual narrowing of the right eye angle and finally three-and-a-half years after tube placement of the left eye, her right eye IOP became uncontrolled with medications alone and a tube shunt was similarly placed in the right eye. Intraoperative ultrasound biomicroscopy performed at the time of the right eye tube shunt revealed extensive ciliary body cysts in the right eye. Her IOP in both eyes have been well controlled since the placement of tube shunts. CONCLUSIONS: This is one of the first reported cases of severe chronic angle- closure glaucoma secondary to ciliary body cysts in a patient with oculodentodigital dysplasia. We believe that early screening for ciliary body cysts is important in patients with oculodentodigital dysplasia.

Uveal effusion syndrome mimicking severe chronic posterior uveitis: a case series of seven eyes of four patients.

PURPOSE: To describe the clinical findings and management of eyes affected by uveal effusion syndrome (UES) presenting with clinical features mimicking inflammatory ocular diseases, treated using individualized surgical approaches. METHODS: We report a consecutive interventional case series of seven eyes of four patients affected by UES. On presentation in our clinic, all patients showed signs of steroid effects as a consequence of a presumptive diagnosis; one eye had undergone vitrectomy for retinal detachment (RD), without benefit. Diagnosis of UES was based on ophthalmic examination, ultrasonography, fluorescein angiography, biometry and magnetic resonance imaging. Five eyes with active disease were treated using scleral thinning surgical procedures based on the extent and characteristics of the disease: sclerectomy sites were ultrasound-guided to the area of maximal choroidal swelling, associated with evacuative puncture in the case of bilateral funnel-shaped RD. RESULTS: One patient was diagnosed with type 1 UES, two with type 2, and one with type 3. Mean postoperative follow-up was 26 months. In all eyes, surgery resolved the ciliochoroidal and retinal detachment and improved visual acuity. In two eyes, visual restoration was limited by a prolonged disease course. CONCLUSION: UES may be mistaken for other sources of ciliochoroidal effusion. Early diagnosis and treatment is critical to avoid unnecessary procedures and to prevent severe visual loss as a result of neuroretinal damage. Surgical treatment based on the extent and characteristics of the disease may be effective for the resolution of ciliochoroidal effusion, even in type 3 UES, where conventional surgery has proved unsuccessful.

The best of the best: a review of select glaucoma case reports published in 2015.

This review article summarizes four key case reports published in the field of glaucoma in the year 2015. The first article describes a novel technique for draining choroidal fluid in patients with uveal effusion syndrome. The second article describes 2 cases of recurrent vitreous block despite adequate surgical intervention. The third article describes 2 cases of endogenous steroid response glaucoma. The last article describes the treatment of visual phenomena following iridectomy using femtosecond laser assisted keratopigmentation.

Inadvertent cyclodialysis cleft and annular ciliochoroidal detachment after hyperopic phakic intraocular lens implantation and prophylactic surgical iridectomy.

UNLABELLED: We present the case of a 26-year-old man with severe early hypotony after implantation of the Implantable Collamer Lens phakic intraocular lens (pIOL) in the left eye for hyperopia. To our knowledge, this is the first documented case of cyclodialysis cleft and secondary annular ciliochoroidal detachment after implantation of a pIOL, presumably provoked by a straightforward prophylactic surgical iridectomy. The initial diagnosis was determined using swept-source Fourier-domain anterior segment optical coherence tomography as gonioscopy and ultrasound biomicroscopy did not reveal the cleft. Conservative treatment was not effective. Argon laser photocoagulation resolved the problem. FINANCIAL DISCLOSURE: No author has a financial or proprietary interest in any material or method mentioned.

Full-thickness sclerotomy for uveal effusion syndrome.

To report the surgical outcome of full-thickness sclerotomy in five cases of uveal effusion syndrome (UES). Full-thickness sclerotomy without sclerectomy was performed on five eyes of four patients with UES with or without nanophthalmos. In four of the eyes, exudative retinal detachment associated with UES resolved after the sclerotomy. The subretinal fluid in one eye, which had a normal axial length, was relieved after undergoing three sclerotomy procedures. Full-thickness sclerotomy without vortex vein decompression or sclerectomy is an effective surgical option for the management of significant UES.

Long-term outcomes following the surgical repair of traumatic cyclodialysis clefts.

PURPOSE: To evaluate the long-term visual prognosis and intraocular pressure (IOP) control following direct and indirect cycloplexy for the surgical treatment of traumatic cyclodialysis clefts. METHODS: Retrospective consecutive case series of 17 eyes of 17 patients. All eyes showing signs of ocular hypotony were treated with either cleft cyclocryotherapy and/or direct surgical cycloplexy. Cycloplexy was performed by directly suturing the ciliary body to the scleral spur under a double-lamellar limbal-based scleral flap. The main outcome measures were IOP, best-corrected visual acuity (BCVA), and the occurrence of postoperative complications. RESULTS: The cyclodialysis clefts were post-traumatic in all the 17 eyes and extended for 2.1 ± 1.6 clock-hours (range, 0.5-6 clock-hours). The mean follow-up time was 43.7 ± 24.6 months (range, 12-110 months). Preoperatively, the mean IOP was 6.9 ± 4.0 mm Hg (range, 2-14 mm Hg). Postoperatively, painful reversible IOP spikes of up to 70 mm Hg developed in 13 eyes. The final mean postoperative IOP was 12.2 ± 4.1 mm Hg with no cases of secondary glaucoma. Preoperatively, BCVA was 6/12 or better in 4 eyes (24%), which rose to 12 eyes (71%) at final follow-up. Of the 12 patients who underwent direct cycloplexy, 75% achieved a final BCVA of 6/12 or better. There were no serious complications related to direct cycloplexy, including suprachoroidal haemorrhage or endophthalmitis. CONCLUSIONS: Successful cyclodialysis cleft repair can lead to a good long-term visual prognosis and stable IOP control, even in cases with a protracted history of ocular hypotony.