Bilateral Exudative Retinal Detachments
A suspicion of VKH in a patient with a stem cell disorder.
Daniel A. Chruscicki, MD • Stephanie Sutton, MS • Elizabeth Sutton, MS
A 57-year-old Caucasian woman was referred for “sudden decrease of vision” in the left eye that occurred one week earlier. She reported no associated pain, flashing lights, or floaters. Her medical history was significant for primary myelofibrosis, first diagnosed in 2006.
At the time of the exam, the patient was not receiving any treatments, and her only medications were occasional acetaminophen and ibuprofen. Typically, her only manifestation of myelofibrosis was mild thrombocytosis.
The external exam revealed no signs of alopecia, poliosis, or vitiligo. Her visual acuity with correction was 20/40 OD and CF at 6 feet OS. IOPs by applanation were 14 mm Hg OD and 12 mm Hg OS. Slit-lamp biomicroscopy was only significant for trace nuclear sclerotic changes in the lens. No anterior-chamber cells appeared in either eye.
A dilated fundus exam of the right eye was significant for rare vitreous floaters and a healthy-appearing optic nerve with a 0.2 cup-to-disc ratio. The macular exam revealed two small exudative retinal detachments (Figure 1). One detachment was adjacent to the optic nerve head, and the second detachment was located in the inferior periphery. No vitreous cells, snowballs, or snow banking were evident. There was no evidence of subretinal or choroidal masses.
Figure 1. Inferior exudative retinal detachment in the right eye.
The dilated fundus exam of the left eye was significant for rare vitreous floaters and a healthy-appearing optic nerve with a 0.2 cup-to-disc ratio. The macular exam revealed a large exudative retinal detachment involving the optic nerve and the fovea (Figure 2). There was a second exudative retinal detachment identified in the inferior retinal periphery.
Figure 2. Exudative retinal detachment involving the macula.
A systemic workup was initiated. The patient tested negative for tuberculosis, syphilis, toxoplasmosis, and Lyme disease. The patient's tests revealed normal ESR, CRP, ANA, RF, C-ANCA, P-ANCA, serum protein electrophoresis, and serum viscosity. A complete blood count indicated mild anemia, slightly elevated platelets, and slightly decreased lymphocytes. A comprehensive metabolic panel was significant for only a mild elevation in ALK phosphatase.
After a discussion with the patient's hematologist and primary care physician, the patient was started on prednisone 80 mg P.O. After three weeks of treatment, her visual acuity had markedly improved to 20/30 OD and 20/40 OS. The exudative detachments exhibited significant improvement. Only a small amount of subretinal fluid remained inferiorly in the left eye.
At this point, the patient was offered a spinal tap to look for pleocytosis, in addition to HLA testing, to attempt to confirm a diagnosis of Vogt-Koyanagi-Harada (VKH) syndrome. However, she declined any further testing.
The patient was continued on the 80 mg of steroids for a second period three weeks. Follow-up vision at this point was 20/25 OD and 20/50 OS. There was further reduction of the subretinal fluid in the left eye. A prednisone taper was instituted. Four weeks later, the patient returned with worsening vision. Visual acuity at that time was 20/70 OD and 20/200 OS.
A fundus exam was performed, along with fluorescein angiography, which showed complete resolution of the exudative retinal detachments (Figures 3 and 4), in addition to a normal foveal depression in both eyes (Figures 5 and 6). She was sent to her primary care physician, who diagnosed her with high blood sugar, and she was subsequently hospitalized for blood glucose control.
Figure 3. Retinal pigment changes the right eye showed improvement of exudative retinal detachment.
Figure 4. Retinal pigment changes in the left eye also showed improvement of exudative retinal detachment.
Figure 5. Improvement in retinal thickness after steroids in the right eye.
Figure 6. Improvement in retinal thickness in the left eye.
Two weeks after her hospitalization, her vision improved to 20/30 OD and 20/40 OS. The exudative retinal detachments were still resolved, and she was finishing her steroid taper.
Chronic idiopathic myelofibrosis (CIMF) is a clonal hematopoietic stem cell disorder characterized by increased bone marrow collagen fibrosis, leukoerythro-blastic anemia with teardrop-shaped red cells, splenomegaly, and extramedullary hematopoiesis.1 Retinal hemorrhages can result from inefficient blood cell production, and blood cell irregularities have been reported as part of CIMF.2
There is one case report that was found in which bilateral retinal neovascularization was associated with chronic idiopathic myelofibrosis.3 To our knowledge, this is the first case involving bilateral exudative retinal detachments in the setting of primary myelofibrosis.
Vogt-Koyanagi-Harada syndrome, also called Harada's disease, is an acute inflammatory, immune-mediated disorder that typically affects both eyes of middle-aged adults and involves melanocyte-containing organs, such as the skin, ears, meninges, and eyes. Acute exudative panuveitis occurs in both eyes and causes ocular irritation and visual loss. It is a disorder that typically affects Latinos, Japanese, and pigmented individuals.
Vogt-Koyanagi-Harada syndrome can be subdivided into Vogt-Koyanagi disease, characterized mainly by skin changes and anterior uveitis, and Harada disease, in which neurological features and exudative retinal detachments predominate. Although HLA testing and a spinal tap were not performed, a Harada-type picture is a consideration in the differential diagnosis. RP
1. Tefferi A. Myelofibrosis with myeloid metaplasia. N Engl J Med. 2000;342:1255-1265.
2. Haskes C, Gagnon K. Retinal manifestations of idiopathic myelofibrosis, a hematologic disorder. J Am Optom Assoc. 1998;319-328.
3. Kim MJ, Yu HG. Case report of bilateral retinal neovascularization associated with chronic idiopathic myelofibrosis. Korean J Ophthalmol. 2010;24:131-133
|Daniel A. Chruscicki, MD, practices with Eye Surgical Associates in Lincoln, NE. Stephanie Sutton, MS, and Elizabeth Sutton, MS, are third- and first-year medical students, respectively, at the University of Nebraska Medical Center. None of the authors reports any financial interest in any products mentioned in this article. Dr. Chruscicki can be reached via e-mail at firstname.lastname@example.org.|