Outcomes of Anti-vascular Endothelial Growth Factor Treatment for Foveal Serous Retinal Detachment Associated with Inferior Staphyloma.

PURPOSE: To evaluate the efficacy of anti-vascular endothelial growth factor (VEGF) treatment of eyes with foveal serous retinal detachment (SRD) associated with inferior staphyloma and to investigate choroidal thickness changes following anti-VEGF therapy. METHODS: In this observational case series, eyes with inferior staphyloma accompanied by foveal SRD were treated with a single intravitreal anti-VEGF injection, followed by further injections as needed. Changes in height and width of subretinal fluid (SRF) and visual acuity after treatment were assessed. Choroidal thickness was measured at the subfovea, 1.5 mm superior and inferior to the fovea using enhanced depth imaging optical coherence tomography at baseline and 1 month after initial anti-VEGF therapy. RESULTS: Six eyes from six patients were included. One month after the initial injection, the mean SRF height and width had decreased significantly from 112.5 ± 40.1 to 44.5 ± 48.7 µm (p = 0.046) and from 1,401.8 ± 627.3 to 690.7 ± 634.7 µm (p = 0.028), respectively. Mean choroidal thickness at the superior point decreased from 218.7 ± 59.3 to 200.5 ± 61.0 µm (p = 0.046). SRF resolved completely in three of the six eyes (50%) with a mean of 6.8 ± 5.9 injections (range, 1 to 15). All eyes experienced at least one recurrence of exudation, at a mean interval of 4.8 months. Mean visual acuity improvement was 0.17 logarithm of the minimum angle of resolution units at a mean of 28.7 months follow-up. CONCLUSIONS: Anti-VEGF therapy resulted in an SRF decrease and modest visual improvement in eyes with foveal SRD associated with inferior staphyloma. Reduction in superior choroidal thickness appeared to contribute to the clinical improvements that were observed.

Presence of Posterior Staphyloma in Congenital Cataract Children.

Purpose: To investigate the prevalence of posterior staphyloma (PS) in congenital cataract children and its role in predicting postoperative axial elongation.Materials and Methods: Preoperative prevalence of PS in 520 congenital cataract patients was reviewed and compared with that of the healthy eyes of 300 unilateral traumatic cataract children after 1:1 propensity score matching. Then, 32 pseudophakic children with preoperative PS and 48 age-matched pseudophakic controls without preoperative PS were followed up after the surgery, to compare their axial growth rates and refractive changes.Results: Congenital cataract was significantly associated with the presence of PS (OR: 14.88, P = .009) after propensity score matching. Even in congenital cataract eyes with axial length <26 mm, 5% were identified with PS on B-scan: ≤22 mm: 3%, 22-24 mm: 5% and 24-26 mm: 13%. Eyes with preoperative PS exhibited faster postoperative axial growth than those without, especially in bilateral cases or in children undergoing surgery before 8 years old (≤4 years: 0.53 ± 0.33 vs 0.30 ± 0.21 mm/y P = .028; 4-8 years: 0.37 ± 0.26 vs 0.23 ± 0.15 mm/y P = .044). Myopic shift after surgery was also more significant in children with preoperative PS than in those without (-1.10 ± 0.50 vs -0.60 ± 0.47D/y, P < .001).Conclusions: Congenital cataract is a risk factor for PS. Preoperative PS in pediatric cataract eyes may be an indicator of excessive postoperative axial elongation, especially in bilateral cases or in cases undergoing cataract surgery at a younger age. Our findings may also promote better clinical decision-making in intraocular lens power selection for pediatric population.

Progressive Intrascleral Epithelial Cyst With Intracorneal Extension.

The authors report a case of primary sclerolimbal cyst with corneal extension in a 3-year-old boy. The cyst enlarged and dissected into the cornea over time. Surgical excision with tectonic allograft was performed. Preoperative, intraoperative, and postoperative anterior segment findings were shown, and results from histologic and cytologic assays were presented. [J Pediatr Ophthalmol Strabismus. 2019;56:e20-e23.].

Radius-Maumenee syndrome: a rare cause of glaucoma.

Case presentation of a 41-year-old woman with long-standing bilateral eye injection whose clinical findings included bilateral episcleral vessel engorgement and tortuosity, raised intraocular pressure with open iridocorneal angles and left optic disc cupping. All remaining objective examination was normal. She underwent cranial and orbits CT and CT-angiography scans which were unremarkable. Optical coherence tomography of the peripapillary nerve fibre layer and automated perimetry were compatible with advanced glaucomatous damage on the left eye. The patient was diagnosed with idiopathic elevated episcleral venous pressure or Radius-Maumenee syndrome. If medical therapy is unable to prevent disease progression, left eye filtering surgery will be proposed.

[Diagnostic imaging for sclerochoroidal calcifica-tion]. / Vizualiziruiushchie metody issledovaniia v diagnostike sklerokhorioidal'noi kal'tsifikatsii.

The article presents two clinical cases of sclerochoroidal calcification. This is a rare benign condition that usually does not display itself and is only occasionally diagnosed. It can also be accompanied by systemic disorder of mineral metabolism. The data obtained through medical imaging indicate partial destruction of the choroid at the site of the damage that causes secondary changes in the overlying retina.

Pericardial patch graft repair of severe localized scleral thinning encountered during strabismus surgery.

This article presents a surgical technique using a pericardial patch for the permanent repair of severe scleral thinning encountered during strabismus surgery. In the present case scleral thinning resulted from buckle removal. Familiarity with this technique may prove important for the strabismus surgeon treating patients with a history of surface ocular hardware or disease-induced scleral thinning. This video article may be viewed atjaapos.org.

Bilateral Blebs Secondary to Spontaneous Scleral Perforations.

PURPOSE: To report an unusual case of bilateral spontaneous scleral perforations. METHODS: A 34-year-old woman presented with bilateral conjunctival blebs and decreased vision in both eyes. There was no history of ocular trauma or surgery and no previous ophthalmic or systemic conditions that may have contributed to the scleral perforations. RESULTS: Best-corrected visual acuity was 20/40 in both eyes and intraocular pressures were 6 mm Hg in the right and 5 mm Hg in the left. There was associated bilateral hypotonous maculopathy confirmed with optical coherence tomography. Slit-lamp biomicroscopy revealed bilateral inferonasal conjunctival blebs in otherwise normal anterior segments. Ultrasound biomicroscopy suggested an opening between the anterior chambers and subconjunctival spaces of both eyes. The patient underwent sequential repair of the perforations with sclerocorneal patch grafts. Intraoperatively crescentic perforating scleral clefts were noted in both eyes, with abnormal and friable surrounding tissue. Histopathology revealed degeneration of the sclera with absence of normal lamellar collagenous architecture and structure. At 9 months postoperative for each eye, the best-corrected visual acuity had improved to 20/20 on both eyes and intraocular pressures were 20 mm Hg on the right and 10 mm Hg on the left. Macular anatomy and thickness were restored in both eyes. CONCLUSIONS: Spontaneous scleral perforations without prior history of trauma or surgery is extremely rare. The underlying cause in our patient is hypothesized to be a coloboma with spontaneous perforation given its location and histopathology. If symptomatic it can successfully be repaired with a patch graft.

Hypotonic maculopathy secondary to scleral defect in atypical retinochoroidal coloboma.

PURPOSE: To describe a patient with unilateral hypotonic maculopathy and optic disc edema after spontaneous bulbar perforation of a full-thickness scleral defect. METHODS: An 11-year-old girl underwent scleral buckling surgery. Preoperative and postoperative evaluation included optical coherence tomography and high-resolution 3T magnetic resonance imaging. RESULTS: The scleral defect was covered with Tenon capsule sheaths and a meridional silicone buckle. One year postoperatively, visual acuity and macular and optic disc morphology were completely restored. CONCLUSIONS: Ocular hypotony related to a defect of the sclera forming the roof of a retinochoroidal coloboma is a rare event. Hypotony was a consequence of a defect at the level of an atypical retinochoroidal coloboma where the scleral wall was found to be absent.

[Diffuse brown discoloration of skin, mucosa and urine]. / Diffuse Braunfärbung von Haut, Schleimhaut und Urin.

A 45-year-old woman presented with diffuse melanosis, icteric sclera and melanuria. Physical examination revealed a massive nodular melanoma with ulceration and satellite metastases on the back. Further investigation showed distant cutaneous and visceral metastasis. After palliative debulking along with postoperative multidrug chemotherapy, the patient has shown objective disease regression for more than 11 months. However, it remains to be seen if disease regression will translate into increased survival.

[Sclerochoroidal calcifications with vision-threatening choroidal neovascularisation]. / Sklerochoroidale Kalzifikationen mit visusbedrohender choroidaler Neovaskularisation.

BACKGROUND: Sclerochoroidal calcification is an uncommon ocular condition in elderly patients. The lesions are frequently bilateral and located at the superotemporal quadrant. The diagnosis is made by fundoscopic appearance, angiography and echography. HISTORY AND SIGNS: A 75-year-old man was referred with bilateral choroidal lesions and visual deterioration in the right eye. Examination revealed apart from old signs of a multifocal choroiditis also bilateral, elevated, yellow lesions located at the upper temporal arcades. In the right eye one lesion was accompanied by hemorrhages, edema and lipid exudates. A subsequent fluorescein angiography disclosed choroidal neovascularisation. Ultrasonography showed the characteristic findings of highly reflective lesions with acoustic shadowing. Serum calcium and phosphate levels were normal. THERAPY AND OUTCOME: Because of rapid visual deterioration on the right eye Ranibizumab (Lucentis) was injected intravitreal. CONCLUSIONS: Despite their good prognosis, sclerochoroidal calcifications associated with neovascular membranes can become a vision-threatening disorder.

Ciliary body melanoma with limited nodular extrascleral extension and diffuse iris-angle infiltration treated by whole anterior segment plaque radiotherapy.

BACKGROUND: Primary uveal malignant melanoma of the ciliary body associated with nodular extrascleral extension, diffuse iris-angle infiltration, and secondary glaucoma is usually treated by prompt enucleation. We report a patient with ciliary body melanoma associated with nodular extrascleral extension and diffuse infiltration of the iris and angle treated conservatively because the fellow eye was blind. METHODS: The clinical features and surgical management of a melanoma of the ciliary body with extrascleral extension and diffuse infiltration of the iris and angle are presented. The tumor was treated with focal I-125 plaque radiotherapy followed by supplemental whole anterior segment I-125 plaque radiotherapy. RESULTS: The patient has been followed for over 2.5 years since the initial plaque radiotherapy and over 1.5 years since the supplemental whole anterior segment radiotherapy. His visual acuity is correctable to 20/40 OD and there is no evidence of metastatic disease. His glaucoma is well controlled following trabeculectomy and tube shunt procedure. CONCLUSION: Whole anterior segment plaque radiotherapy for ciliary body melanoma with diffuse iris-angle infiltration provided palliative local tumor control without significant local complications through available follow-up.

Polypoidal choroidal vasculopathy in tilted disk syndrome and high myopia with staphyloma.

PURPOSE: To describe polypoidal choroidal vasculopathy as a complication of tilted disk syndrome and high myopia with staphyloma. DESIGN: Retrospective interventional case series. METHODS: This report was a multicenter evaluation of six patients (eight eyes) with tilted disk syndrome or high myopia that was complicated by posterior staphyloma. Complete ophthalmic examination that included fluorescein angiography, optical coherence tomography (OCT), and indocyanine green angiography (ICG-A) was performed in all patients. RESULTS: All patients had macular abnormalities and visual loss. Fundus examination and fluorescein angiography showed typical features of tilted disk syndrome (five patients; six eyes) or high myopia (one patient; two eyes) with staphyloma that was associated with polypoidal choroidal vasculopathy. OCT and ICG-A confirmed the presence of polypoidal dilations in the choroid. Seven eyes were treated with laser photocoagulation or verteporfin-photodynamic therapy (V-PDT), although one eye did not require treatment. Visual acuity at the final visit had improved in three eyes, deteriorated in three eyes, and remained unchanged in two eyes. CONCLUSION: Polypoidal choroidal vasculopathy is a potential cause of visual loss in tilted disk syndrome and high myopia. We postulate that choroidal abnormalities at the border of staphylomas induce blood-flow disturbances that are similar to those disturbances that are observed in chronic central serous chorioretinopathy, which is another condition that occasionally is associated with polypoidal choroidal vasculopathy. The pathogenesis remains unclear, and further study is required to better understand the formation of choroidal polypoidal dilations in these conditions.

Scleral ossification in phthisical eyes.

Heterotopic secondary ossification of the eye usually affects intraocular tissues. Although calcium deposition in the scleral lamellae is not uncommonly observed, bone formation is only rarely associated with chromosomal abnormalities or colobomatous eyes. Herein two cases of both scleral and intraocular ossification in patients with long-standing ocular phthisis are reported. The cases of idiopathic scleral ossification, albeit exceedingly rare, suggest that osteogenic precursor cells may reside in the sclera, as well and though very rarely, these cells can also be stimulated to form heterotopic bone by a traumatic or inflammatory local event.