Management of bilateral idiopathic healed sclerokeratouveitis with ciliary and intercalary staphyloma with complicated cataract and secondary glaucoma

Secondary choroidal neovascularization in multifocal choroiditis and panuveitis (MCP) is readily visible as a hyper-autofluorescencent area originating from a hypoautofluorescent spot (scar). With increasing follow-up time, the hyper-autofluorescence associated with CNV decreases. AF imaging provides a method to image the appearance of new or enlarging spots that appear, which is more sensitive than using ophthalmoscopically visible signs.

Inflammatory CNVM, which is usually of the classic type (type 2), is seen on AF photography as a precise hyper-autofluorescent area due to the hyperplastic retinal pigment epithelium (RPE). Following treatment, CNVM may contract and leave a zone of absent RPE causing a hypoautofluorescence.
Secondary choroidal neovascularization in multifocal choroiditis and panuveitis (MCP) is readily visible as a hyper-autofluorescencent area originating from a hypoautofluorescent spot (scar). With increasing follow-up time, the hyper-autofluorescence associated with CNV decreases. AF imaging provides a method to image the appearance of new or enlarging spots that appear, which is more sensitive than using ophthalmoscopically visible signs.
More studies are required to understand the role of AF in inflammatory diseases. AF imaging could be an important noninvasive tool to reduce the need for angiography and to help in early diagnosis, as also in the follow-up of inflammatory CNVM patients.

Use of Anti VEGF in Inflammatory CNVM
There are potential disadvantages of treating inflammatory CNV with photodynamic therapy (PDT). PDT can cause localized inflammation and increase VEGF production. Local release of VEGF, after PDT, may be associated with a higher incidence of recurrent choroidal neovascularization (CNV). [2] Tran et al studied 10 patients with CNV, who were refractory to previous immunosuppression and PDT or intravitreal triamcinolone (IVTA). They reported that intravitreal bevacizumab improved the best corrected visual acuity (BCVA) and reduced the central macular thickness in the eyes with 2.5 injections (mean), and this was seen at 7.5 months of follow up. In this study they included a patient who recurred three months after pegaptanib injection and responded well. [3] Fine et al reported successful treatment of inflammatory CNVM with ranibizumab in a patient with multifocal choroiditis and pan uveitis (MCP), who did not responded to bevacizumab or the PDT and IVTA therapy. [4] The recent and largest case series by Monsour et al reported significant improvement of 2.2 lines in BCVA at 24 months, with a significant decrease in foveal thickness (265 microns), with only 1.3 injections (mean). This study confers the long-term benefits of intravitreal bevacizumab. [5] Serious side effects and chances of recurrence make PDT an obsolete treatment option. Recent promising results favor intravitreal bevacizumab as a primary / mono therapy in inflammatory CNVM.

Management of bilateral idiopathic healed sclerokeratouveitis with ciliary and intercalary staphyloma with complicated cataract and secondary glaucoma
Dear Editor, We read the article by Parihar et al. [1] and agree with their conclusions and would like to share the course of a uveitic glaucoma with intercalary staphyloma which was managed well with phacoaspiration, posterior chamber intraocular lens (PCIOL), and Ahmed glaucoma valve (AGV) implantation. Uveitic glaucoma is challenging due to the early onset and the numerous mechanisms of pathogenesis including steroidinduced intraocular pressure (IOP) elevation. [2] Medical and surgical interventions though initially successful eventually fail due to fibrosis. [3,4] A 32-year-old lady with recurrent episodes of pain and redness in both eyes (BE) for 3 years was referred to our center with uncontrolled high IOP in left eye. She was on topical timolol maleate 0.5%, brimonidine tartrate 0.15%, and dorzolamide 2% twice daily in BE since 6 months and complained of progressive diminution of vision, dark coloration of the sclera, and white opacities on the cornea for 1 year. There was not any history of ocular injury, fever, joint pain, skin lesions, or any other systemic illness.
On examination, vision in the right and left eye was 1/60 and 20/200, respectively. Both eyes had vascularized maculoleucomatous corneal opacities, normal anterior chamber depth, annular posterior synechiae, patent peripheral iridectomies, and cataract. She also had bilateral ciliary and intercalary staphyloma more in the right than the left eye. Tonopen reading in the right and left eye was 20 mmHg and 40 mmHg, respectively. Fundus and gonioscopy examination was not possible due to media opacities [ Fig. 1].
We diagnosed this patient as bilateral idiopathic healed sclerokeratouveitis with ciliary and intercalary staphyloma with complicated cataract and secondary glaucoma. We planned phacoaspiration with PCIOL and AGV implantation as a single-stage procedure in the left eye under cover of oral prednisolone (1 mg/kg) tapered in 4 weeks. The plate of AGV was placed in the superotemporal quadrant and the tube was covered with the cadaveric scleral graft. Fixing the AGV tube and plate was an extremely uphill task due to thinness of sclera. Using iris retraction hooks, temporal phacoaspiration was performed and a multipiece acrylic foldable PCIOL was implanted in the bag. On the first postoperative day, left eye IOP was 17 mmHg and vision was 20/200. However, on the second day, there were a severe anterior chamber reaction, an exudative membrane over the IOL and iridectomy with an IOP of 22 mmHg and vision of finger counting at 1 m. We performed YAG membranectomy and pigment sweeping of the IOL on the fifth postoperative day. By the seventh day the IOP was 14 mmHg and vision improved to 20/63 and has remained in the lower teens in 2-year follow-up [Figs. 2 and 3].