Dome-shaped maculopathy | IMCRJ
Dome-shaped maculopathy (DSM) was first described in very myopic patients by Gaucher et al in 2008.1 It is associated with thickening of the sclera, which leads to a convex protrusion of the macula creating a dome.2 A common complication of DSM is serous retinal detachment (SRD) that develops on the macula,3.4 although its exact mechanism remains a mystery. It has been postulated that DSM may be an adaptive or compensatory response to great axial length in high myopic people.5.6 and serous detachment may be the result of retinal dysfunction and fluid obstruction caused by scleral thickening.5 Attempts to treat this with various interventions, including lasers, diuretics, anti-inflammatory drops, and even surgery, have met with varying success.7–13 Recently, we came across a case of dome-shaped maculopathy associated with the formation of macular holes. A standard surgical approach including vitrectomy, internal limiting membrane (ILM) peel with triamcinolone acetate visualization and gas tamponade has been successful.
A 62-year-old myopic woman with keratoconus complained of changes in visual acuity in her right eye for 5 months with distortion and difficulty reading the fine print. She had a history of Intacs placed in the right eye 10 years previously, and had Intacs placed in the left with corneal reticulation in both eyes when she noticed a change in vision in the right eye. His best corrected visual acuity (BCVA) was 20 / 200-2 OD and 20/50 OS. His intraocular pressures (IOP) were 15 mmHg and 14 mmHg, respectively. The axial length measured 26.85 mm OD and 26.89 mm OS, and there were more than 2 sclerotic nuclear cataracts (NSC) in both eyes. The initial optical coherence tomography (OCT) showed a macular bulge indicating DSM in both eyes with a full-thickness macular hole in the right eye and subretinal fluid in the left eye (Figure 1). The central macular thickness (CMT) was 215 OD and 318 OS. The patient started taking spironolactone 25 mg BID PO and suffered pars plana in his right eye 2 weeks later. The operation was performed on September 10, 2018 at the California Hospital in Los Angeles, California.
Figure 1 (From left to right) Horizontal OCT sections (with maps of difference between the images) preoperative, postoperative at 2 months and s / p vitrectomy at 1 year. The CMT was 215, 317, 203 OD and 318, 361, 335 OS respectively.
At the time of surgery, the posterior hyaloid membrane had been elevated and the Weiss ring removed. Attention turned to the macula where triamcinolone acetate was injected into the macular area and the epiretinal membrane and underlying internal limiting membrane were removed with ILM forceps. An air-fluid exchange with C3F8 gas was carried out. On postoperative day 1, the patient’s IOP was 30 OD and 500 mg acetazolamide QD PO was added for 2 months to treat both the pressure in the right eye and the subretinal fluid that was present. in the left eye. The patient was compliant with 2 weeks of strict face-down positioning.
At 2 months postoperatively, the BCVA was 20 / 60-1 OD and 20/40 + 1 OS. There were 3+ NSC, as well as 1+ cortical cataract and 1+ posterior subcapsular OD cataract, and 2+ OS NSC. The CMT was 317 OD and 361 OS. The patient had persistent subretinal fluid in both eyes, and then started taking one drop of NSAIDs twice daily in both eyes. The patient underwent cataract surgery on his right eye 9 months post-op. At 1 year of s / p vitrectomy, CMT was 203 OD and 335 OS. At the most recent examination, 32 months after vitrectomy and 23 months after cataract surgery, the BCVA was 20/20 OD and 20/30 OS.
We report a successful case of macular hole repair associated with DSM. A standard approach to repairing macular holes has been used with success. DSM subretinal fluid persisted after surgery but responded to a combination of oral spironolactone and a drop of topical NSAIDs. The mechanism of DSM remains a mystery, but effective treatment options may involve an altered fluid transport mechanism through the RPE14 probably creates the buildup of fluid in the subretinal space associated with DSM. NSAID drops can speed up the absorption of fluids through the RPE,15 as are diuretics, including spironolactone and acetazolamide.16-19 Our patient is nearsighted which may make macular hole surgery less effective because a staphyloma can further stretch the retina and impair the closure.20 The dome-shaped scleral support in the DSM can actually facilitate closing the hole in the same way as a macular loop.21 The subretinal fluid, however, would interfere with closure and the use of adjunct therapies in this case may have contributed to our success.
The patient recovered from BCVA at 20/20 OD and 20/30 OS. The patient has a closed macular hole with resolved subretinal fluid in the right eye. The left eye has persistent subretinal fluid, although less nasally from its peak at 2 months postoperatively (Figure 1). The decrease in visual acuity in the left eye may be due in part to a combination of factors: persistent FRS, her cataract, and her history of keratoconus. The presence of persistent subretinal fluid in the left eye may be secondary to the slightly longer axial length of 0.04 mm or vitreomacular adhesion, which may cause traction on the macula and prevent further absorption of fluid from the left eye. through the EPR. It is difficult to determine to what extent each of these factors play a role in final visual acuity, but the fact that his vision is improved from his initial presentation with a decrease in subretinal fluid suggests that fluid may play. a bigger role in the end result.
We confirm that any aspect of the work covered in this manuscript involving human patients has been conducted with the ethical approval of all relevant organizations. Written informed consent was obtained from all patients for case details and all accompanying images to be released.
There is no recognition that we would like to do. No institutional approval was required for the publication of this case report.
All authors have made significant contributions to the reported work, whether in study design, design, execution, data acquisition, analysis and interpretation, or in all of these areas; participated in the writing, editing or critical review of the article; gave final approval to the version to be published; agree on the journal to which the article was submitted; and accepting to be responsible for all aspects of the job.
No grant or funding has been awarded.
The following authors have no financial or other disclosure: SL, RPG.
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