Dmitry Pyatetsky

Epidemiology and Course of Disease in Childhood Uveitis

PURPOSE To describe the disease characteristics and visual outcome of pediatric uveitis. DESIGN Retrospective, longitudinal observation. PARTICIPANTS Five hundred twenty-seven pediatric uveitis patients from the National Eye Institute, University of Illinois, Chicago, and Oregon Health Sciences University. METHODS Retrospective chart review. MAIN OUTCOME MEASURES Demographics, uveitis disease characteristics, complications, treatments, and visual outcomes were determined at baseline and at 1-, 3-, 5-, and 10-year time points. RESULTS The patient population was 54% female; 62.4% white, 12.5% black, 2.7% Asian, 2.1% multiracial, and 14.61% Hispanic. Median age at diagnosis was 9.4 years. The leading diagnoses were idiopathic uveitis (28.8%), juvenile idiopathic arthritis-associated uveitis (20.9%), and pars planitis (17.1%). Insidious onset (58%) and persistent duration (75.3%) were most common. Anterior uveitis was predominant (44.6%). Complications were frequent, and cystoid macular edema (odds ratio [OR] 2.94; P = 0.006) and hypotony (OR, 4.54; P = 0.026) had the most significant visual impact. Ocular surgery was performed in 18.9% of patients. The prevalence of legal blindness was 9.23% at baseline, 6.52% at 1 year, 3.17% at 3 years, 15.15% at 5 years, and 7.69% at 10 years. Posterior uveitis and panuveitis had more severe vision loss. Hispanic ethnicity was associated with a higher prevalence of infectious uveitis and vision loss at baseline. CONCLUSIONS The rate and spectrum of vision threatening complications of pediatric uveitis are significant. Prospective studies using standard outcome measures and including diverse populations are needed to identify children most at risk.

Acute retinal necrosis secondary to varicella zoster virus in an immunosuppressed post-kidney transplant patient.

A woman, aged 42 years, presented to the ophthalmologist complaining of right eye redness, tearing, photosensitivity, and blurred vision of a 1-month duration. Her past medical history was significant for a kidney transplant for which she was immunosuppressed with mycophenolate mofetil 750 mg twice a day and prednisone 10 mg daily. Ophthalmologic examination of the right eye revealed visual acuity of 20/60, intraocular pressure of 16 mmHg, scleral injection, and a significant inflammatory reaction in the anterior chamber. Funduscopy revealed vitreous haze, optic nerve edema, arteriolar sheathing and attenuation, and peripheral 360° of retinal whitening – a clinical picture consistent with retinitis (figure 1). The left eye examination was entirely normal. Figure 1 (A) Examination of the fundus on day of presentation shows peripheral whitening and vasculitis as evidenced by optic nerve edema, vitreous haze, and retinal arteriolar narrowing. The decrease in clarity of the photograph is secondary to the vitreous haze, ... The differential diagnosis for retinitis in an immunocompromised host includes acute retinal necrosis (ARN), progressive outer retinal necrosis, cytomegalovirus (CMV) retinitis, and toxoplasmosis. Less common causes of acute retinitis include syphilis,1 Bechets disease, and lymphoma. Aqueous fluid obtained from the anterior chamber was sent for polymerase chain reaction (PCR) viral DNA analysis, and the patient was treated with 2 mg of ganciclovir injected intravitreally on the day of presentation. A 2-week course of renal-dose adjusted intravenous (IV) acyclovir was started with subsequent regression of the retinal whitening (figure 2). Varicella zoster virus (VZV) infection with a viral load of 6.5 × 107 IU was later confirmed with PCR of the aqueous fluid. The vision initially improved to 20/40 in the right eye. She was transitioned from IV acyclovir to oral valacyclovir. Figure 2 (A) After treatment with anti-viral medication, there is regression of retinal whitening and atrophic retinal tissue. (B) Areas of regression are indicated by white asterisks. The arterioles that previously were narrowed, indicated by the white arrows, ... Seven weeks later, the patient developed a rhegmatogenous retinal detachment that was diagnosed after she complained of an abrupt decrease in vision. Despite successful surgical reattachment of the retina with pars plana vitrectomy, scleral buckle, and silicon oil, the patients vision remained at light perception level only. Necrotizing herpetic retinopathies are caused by VZV, herpes simplex virus 1 and 2, CMV, and rarely, Epstein Barr virus. A spectrum of clinical presentations may occur depending on the virus and the hosts immunity.2 Immunosuppressed patients are at risk for development of necrotizing retinopathies typically associated with immunocompromised states, such as CMV retinitis and progressive outer retinal necrosis. They may also develop syndromes that are typically seen in immunocompetent hosts, such as ARN. Acute unilateral decrease in vision, photophobia, and eye pain are the usual presentations of ARN.3 The key feature seen on examination is white-yellow, multifocal, peripheral patches that later coalesce into diffuse areas of full-thickness, peripheral retinal necrosis.4 Other signs of ocular inflammation including vitritis, vasculitis, optic disc edema, keratic precipitates, and posterior synechiae may also be seen. Untreated ARN results in blindness from retinal scarring, retinal detachment, or optic atrophy; therefore, ARN is an ophthalmic emergency. One-third of patients develop the disease in the fellow eye within one month of presentation, if left untreated.5 The diagnosis of ARN is made on the basis of clinical appearance, and antiviral treatment should be initiated promptly. Timely referral to a uveitis or retina specialist experienced in the care of such patients and collaboration with an infectious disease specialist are essential.

Poststreptococcal syndrome presenting as posterior scleritis in a child

Posterior scleritis in children is very rare. In contrast to the adult form, pediatric posterior scleritis has not previously been associated with any systemic disorder. We describe a case of an 11-year-old girl who presented with left eye pain and redness and was found to have posterior scleritis on ultrasonography. Her laboratory work-up revealed a highly elevated antistreptolysin O titer; the rest of her serologic and radiologic evaluation was unremarkable. She was diagnosed with presumed poststreptococcal posterior scleritis and improved with a slow taper of oral steroids.

Unilateral Nodular Scleritis Secondary to Latent Syphilis

Acaucasian man, aged 47 years, was referred to an ophthalmologist for evaluation of a painful lesion on his left eye. On examination, there was a solid, injected, non-mobile 5 mm × 7 mm nodule located on the inferotemporal sclera of the left eye (figure 1). The best corrected visual acuity was 20/30, and intraocular pressure was normal. The anterior chamber was deep and quiet, and fundoscopic examination was unremarkable. Extraocular motility was full. The right eye examination was normal. Figure 1 A large elevated erythematous sclera nodule at the inferotemporal limbus with a nearby feeder vessel. His medical history was significant for a penile lesion in the distant past, as well as a full-body rash sometime thereafter. He denied being diagnosed with a sexually transmitted disease. A review of systems at presentation was negative for fever, night sweats, rash, genital lesion, or weight loss. The differential diagnosis for nodular scleritis includes infectious etiologies such as tuberculosis and syphilis, autoimmune diseases such as rheumatoid arthritis, sarcoidosis, systemic lupus erythematosus, granulomatosis with polyangiitis, and polyarteritis nodosa, as well as masquerades such as lymphoma. Laboratory tests performed included complete blood count, metabolic panel, quantiFERON, Treponema pallidum antibodies, rheumatoid factor, angiotensin converting enzyme, antinuclear antibodies, and anti-neutrophil cytoplasmic antibodies. A chest radiograph was also obtained. Initial treatment with 60 mg oral prednisone provided no clinical improvement in the lesion, and one week later his testing revealed a positive Treponemal pallidum antibody. He was treated for latent syphilis with three doses of intramuscular Penicillin G. Additional recommendations were made for HIV and hepatitis testing, which the patient refused. Rapid and marked improvement in the lesion followed the first penicillin injection (figure 2). Following three weekly injections of penicillin, the nodule had completely resolved (figure 3), and his best corrected visual acuity returned to 20/20. The rapid and complete clinical response to antibiotic therapy in the setting of his past medical history and laboratory testing strongly supports the likelihood that his condition was a manifestation of latent syphilitic infection. Figure 2 Following one dose of intramuscular penicillin. There is flattening of the nodule and marked improvement in the degree of erythema. Figure 3 Resolution of the scleral nodule following three doses of intramuscular penicillin. The sclera is flat with minimal residual erythema. Nearly one-half of all patients diagnosed with scleritis are found to have an associated illness or underlying condition.1 Scleritis is a rare finding in ocular syphilis, accounting for only 3% of cases2 and presents most commonly in an anterior nodular form.1,3 Diagnosis is often difficult, because there is no clear history of extraocular symptoms. Of patients with ocular syphilis, 25% to 50% have no other clinical signs.4 Ocular lesions were the initial manifestations of syphilis in 87% of the confirmed cases in one published review.5 Scleritis secondary to latent syphilis responds only minimally to steroids but demonstrates a dramatic response to penicillin therapy. Previously documented cases report full and rapid resolution of the nodules 1 to 2 weeks after the first dose of antibiotic.1,3 Emergency room physicians, ophthalmologists, internists, and infectious disease specialists should keep a high index of suspicion for syphilis in all patients with scleritis. A thorough history eliciting exposure risks is essential given the close association to HIV and for rapid diagnosis and treatment.

Asymmetric Anterior Uveitis as a Delayed Complication of Treatment with Systemic High-dose Cytosine–Arabinoside: A Case Report and Literature Review

A 36-year-old male was admitted with left flank pain and during his workup was diagnosed with acute myeloid leukemia (AML) with granulocytic maturation and translocations between chromosomes 8 and 21 (M2 t(8; 21)). Evaluation of cerebrospinal fluid via lumbar puncture demonstrated no evidence of leukemic involvement. The patient underwent standard induction chemotherapy with idarubicin (12 mg/m daily for 3 days) and cytarabine (100 mg/m continuous infusion for 7 days), which resulted in complete remission as demonstrated by bone marrow biopsy. Since the presence of 8;21 translocation, in the absence of c-kit mutation, confers favorable prognosis and exquisite sensitivity to cytarabine in patients with AML, the decision was made to proceed with 4 cycles of consolidation chemotherapy consisting of high doses of cytarabine (HIDAC). The first cycle of HIDAC chemotherapy (3 g/m, q12 h, days 1, 3, 5) administered with prednisolone acetate 1% eyedrops (starting 24 h prior to initiation of chemotherapy, given every 6 h throughout chemotherapy until 48 h after completion of chemotherapy) was uneventful. Four weeks after completion of the second cycle of HIDAC with the same prophylaxis regimen, he was admitted to an outside hospital with gastroenteritis and complained of redness and pain in the right eye. The workup for the gastroenteritis included stool studies for occult blood, WBC count, and microscopy for protozoa and stool cultures, which were all negative, and he was thought to have viral gastroenteritis. He was afebrile during his hospitalization. His ocular complaints were attributed to bacterial conjunctivitis and he was started on tobramycin drops without improvement. Several days later he presented to the ophthalmology clinic and was found to have severe anterior uveitis in his right eye (Figure 1A). His best-corrected visual acuity (BCVA) was 20/30 in the right eye and he had diffuse keratic precipitates with 4þ cell (according to the standardization of uveitis nomenclature criteria), and fibrin in the anterior chamber but no hypopyon, 360 degrees of posterior synechiae, and no view to the fundus. A B-scan was performed, which was unremarkable. He also had mild anterior uveitis in his left eye with 0.5þ cell and trace flare (BCVA 20/20). A detailed review of systems was negative. A uveitis workup was performed consisting of serology for Cytomegalovirus, Epstein– Barr virus, and human immunodeficiency virus, QuantiFERON-TB Gold In-Tube, fluorescent treponemal antibody-absorption (FTA-ABS) test, human leukocyte antigen B-27 evaluation, and a chest x-ray. All the blood tests were negative and the x-ray was normal. With infectious and autoimmune etiology ruled out by history, examination, and workup, the prime suspect for the etiology became cytarabine toxicity. He was started on topical prednisolone acetate 1% every 2 h and cyclopentolate 1% 2 times a day in his right eye and prednisolone acetate 1% 4 times a day in his left eye with significant improvement at 1 week and his BCVA improved to 20/20 in the right eye (Figure 1B).

Posterior uveitis secondary to syphilis.

A 24-year-old man presented to the ophthalmologist complaining of decreased vision and intermittent dull pain in the left eye of 5-months duration. Ophthalmologic examination revealed visual acuity of 20/70, mild inflammatory reaction in the anterior chamber, vitreous haze (blur in figure 1 ▶), optic nerve edema, and retinal arteriolar narrowing and sheathing (figure 1 ▶). Additional history was significant for recurrent facial zoster, anal condyloma acuminatum, and a recent episode of pneumonia. General examination revealed multiple umbilicated lesions on facial skin consistent with a diagnosis of molluscum contagiosum. There was a fading macular rash on the palms of his hands and the soles of his feet. The differential diagnosis for this combination of long-standing vitritis and retinal arteritis in the setting of multiple comorbidities suggestive of human immunodeficiency virus (HIV) is highly suspicious for syphilis, although it also includes necrotizing herpetic retinopathies, toxoplasmosis retinochoroiditis and non-infectious etiologies such as sarcoidosis.1,2 Serological testing confirmed the clinical diagnosis of tertiary syphilis and HIV. The eye pain and vitritis responded to intravenous penicillin G, however the vision remained unchanged. Figure 1. Examination of the fundus shows evidence of long-standing vitritis and retinal arteritis as evidenced by the presence of (a) mild inflammation, optic nerve edema (arrow) and (b) vitreous haziness and presence of retinal arteriolar narrowing and sheathing ...