Spontaneous bilateral peripapillary, subhyaloid and vitreous hemorrhage with severe anemia secondary to idiopathic thrombocytopenic purpura

A 42-year-old female presented to us with a complaint of sudden painless loss of vision in both eyes of three days duration. Visual acuity was 20/100 for distance in both eyes. Fundus examination showed bilateral peripapillary hemorrhages, with subhyaloid and vitreous hemorrhage in both eyes. Hematological investigations revealed hemoglobin (HB 7 gm %) and severe thrombocytopenia (12,000/ ul). She was referred to a hematologist where a diagnosis of idiopathic thrombocytopenic purpura (ITP) was made. She was treated for systemic condition with regular ophthalmic follow-up. Over the next nine months, retinal hemorrhages completely resolved and the patient regained her visual acuity. The purpose of this case report is to highlight the clinical presentation of severe anemia, which is different from previous reports and the role of an ophthalmologist in first detecting the Idiopathic thrombocytopenic purpura (ITP), which led to successful recovery. We report the case of a three-year-old male child who presented with a single painless subcutaneously located upper lid mass of 1.2 × 1.5 cm with a central depression. The mass could be easily separated from overlying skin on complete excision biopsy and showed a never described before whitish brain like appearance consisting of multiple lobes and gyri, which histopathologically proved to be molluscum contagiosum (MC). Tests to investigate underlying immunodeficiency did not show any evidence of immunocompromised state.

have been caused by the effect of TA as a corticosteroid because corticosteroids are known to alter the function of RPE cells. [6] A third possible reason might be, because the patient was young, the vitrectomy was performed under general anesthesia and therefore the patient was not moved to a facedown position for several hours, and the TA crystals within the vitreous cavity may have settled in the MH while the subject was in the faceup position immediately after surgery. However, conflicting outcomes exist in literature. The deposition of residual TA crystal on fovea after MHS has not been associated with poor visual outcome or interference with the closure of hole. [7,8] Mahmoud and associates reported that foveolar lucencies observed by OCT were a common finding after macular hole surgery and that the findings gradually decreased and eventually resolved over time without additional surgical intervention and with further improvement of visual acuity. [9] In our case report, a young 12-year-old boy who sustained a traumatic stage 4 macular hole of size of 700μ closed despite poor compliance with postoperative positioning. We did not attempt to remove the residual TA in the macular hole during vitreous surgery. Although the TA was aspirated with a 23G soft silicon tipped cannula over the macular hole, we did not try to insert the vitrector or a silicon tipped cannula needle into the macular hole to remove the steroid crystals, as the procedure carries the risk of damaging the RPE on the floor of the macular hole, which may prevent the visual acuity from improving after closure of the macular hole. OCT image of the eyes with residual TA, observed in the fovea on the seventh day after surgery clinically, showed a hyper reflective mass corresponding to the TA on OCT. However, a repeat OCT carried out four weeks after surgery showed recovery of the foveal morphologic features to an almost normal depression, with closure of the hole.
Although the number of reported cases is too small to conclude that TA is non toxic to photoreceptor cells or the RPE, residual TA in the macular hole post vitreous surgery may not interfere with ultimate macular hole closure or visual improvement.

Ajit Babu Majji, Kapil Bhatia, Annie Mathai
A 42-year-old female presented to us with a complaint of sudden painless loss of vision in both eyes of three days duration. Visual acuity was 20/100 for distance in both eyes. Fundus examination showed bilateral peripapillary hemorrhages, with subhyaloid and vitreous hemorrhage in both eyes. Hematological investigations revealed hemoglobin (HB 7 gm %) and severe thrombocytopenia (12,000/ ul). She was referred to a hematologist where a diagnosis of idiopathic thrombocytopenic purpura (ITP) was made. She was treated for systemic condition with regular ophthalmic follow-up. Over the next nine months, retinal hemorrhages completely resolved and the patient regained her visual acuity. The purpose of this case report is to highlight the clinical presentation of severe anemia, which is different from previous reports and the role of an ophthalmologist in first detecting the Idiopathic thrombocytopenic purpura (ITP), which led to successful recovery. is an autoimmune disease in which antibodies directed against one's own platelets cause their peripheral destruction, resulting in a low platelet count and, occasionally, bleeding complications. Such patients can develop severe grades of anemia, with resultant ocular manifestations. Ophthalmic involvement is exceptionally rare. [2] Severe anemia, when associated with thrombocytopenia can frequently result in ocular manifestations. We present a patient of ITP with severe thrombocytopenia and anemia presenting with sudden painless loss of vision due to bilateral peripapillary, subhyaloid and vitreous hemorrhage.

Case Report
A 42-year-old lady presented to us on 11.09.2008 with complaint of sudden painless loss of vision in both eyes. She had a history of severe menorrhagia for the past two months. No history of head or ocular trauma was present. She was not a hypertensive or diabetic. Her best corrected visual acuity was 20/100 in both eyes. Anterior segment examination was normal. On fundus examination, both eyes showed peripapillary retinal hemorrhages, subhyaloid and vitreous hemorrhage [ Fig. 1 a, b]. An initial impression of anemic retinopathy was made. She was advised hematological evaluation, which revealed hemoglobin 7.2 gm %, RBC count was 2.3 lakh/ cu mm, platelets 12,000 / ul, rest of blood profile was normal (bleeding time, clotting time, prothrombin time and partial thromboplastin time). She was referred to a hematologist, where peripheral smear showed megalokaryocytes and lymphocytes. A bone marrow biopsy confirmed the diagnosis of ITP. Antinuclear antibodies and anti cardiolipin antibodies were negative. She was infused with single donor platelets and IV immunoglobulins till her platelets level returned to normal. She was started on systemic steroids 1 mg/kg/day and hematinics. With this treatment her hemoglobin and platelets

Discussion
Ocular manifestations of severe anemia have been increasingly recognized and anemia of varied reasons can result in different ocular manifestations, flame-shaped hemorrhages being the commonest type of hemorrhage followed by sub-hyaloid hemorrhage. [1] Severity of retinal manifestations in anemia depends upon severity of anemia. Our case had severe anemia but associated venous tortuosity and cotton-wool spots were absent. This led to suspicion of associated problems. A systemic work up, revealed diagnosis of ITP, an autoimmune disease, in which antibodies directed against one's own platelets cause their peripheral destruction, resulting in a low platelet count and, occasionally, bleeding complications. Such patients can develop severe grades of anemia, with resultant ocular manifestations and decrease in vision, which can often be bilateral. However ophthalmic involvement is exceptionally rare. [2] Hemorrhagic ophthalmic manifestations associated with ITP include vitreous hemorrhage associated with intracranial bleeding in a Terson type phenomenon, [3] hemorrhage within the optic tract, [4] nonarteritic anterior ischemic optic neuropathy [5] and subconjunctival hemorrhage. [6] Thrombocytopenia alone, even severe (a platelet count <50 000), is rarely sufficient to cause significant retinal hemorrhage. However, thrombocytopenia combined with anemia is a known risk factor, and retinal hemorrhages in association with ITP have only been reported to occur with concurrent severe anemia. [7] There are a few isolated case reports in literature describing similar retinal hemorrhages associated with ITP. Meyer et al. [8] described similar retinal findings associated with ITP, but the patient had co-existent diabetic retinopathy. Okuda et al. [9] and Paulin et al. [10] also described similar findings. In all three reports, patients had to undergo splenectomy along with medical management. In addition, vitrectomy was done for vitreous hemorrhage So, as clearly demonstrated in our patient, ophthalmic manifestations do not need any specific treatment other than controlling ITP and associated anemia. Good comprehensive examination of the patient with a high index of suspicion can clinch a the systemic diagnosis, and control of systemic parameters will help improve retinopathy associated with ITP and severe anemia.