sir,

Aplastic anaemia1 has many ophthalmic manifestations,2 but scleral necrosis has not been previously reported. We report a patient with aplastic anaemia who developed severe, progressive, right eye scleral necrosis.

Case report

A 25-year-old male presented with progressively decreased vision in the right eye, of 10 days duration. He had developed sudden onset idiopathic aplastic anaemia 4 weeks ago, and was on irradiated packed cell transplants and oral cyclophosphamide.

On examination, there was no perception of light in the right eye. There was proptosis of 4 mm, on Hertel's exophthalmometry. Slit-lamp examination of the right eye showed extensive areas of scleral necrosis nasally (Figure 1). There was hyphema filling the entire anterior chamber, rendering fundus examination impossible. Left eye examination was unremarkable except for the presence of severe conjunctival blanching, with segmentation and severely reduced conjunctival vessel calibre.

Figure 1
figure 1

External photograph showing proptosis of the right eye with extensive areas of scleral necrosis nasally, with uveal tissue exposure.

Full size image

Computed tomography scan showed a choroidal and an orbital haemorrhage. Blood investigations revealed haemoglobin of 6.70 g%, haematocrit of 10.10%, total leucocyte count of 1400, and very low platelet count. There was no evidence of blasts, on peripheral smear or on bone marrow biopsy, which was dry. Rheumatological disease was ruled out by rheumatological consultation and investigations.

The scleral necrosis progressed rapidly, with auto-evisceration of all ocular contents, within 24 h. Histopathological examination showed disorganized ocular contents, with dark choroidal blood.

The patient's systemic condition improved post bone marrow transplantation. The left eye conjunctival blanching had completely reversed on final visit, 2 months post transplantation.

Comment

To the best of our knowledge, there are no published reports of occurrence of scleral necrosis in aplastic anaemia. Perforating scleromalacia as a complication in a patient with refractory anaemia with excess blasts3 and scleral melt in a patient with carotid artery obstruction4 were found to be the only related Medline reports.

We report a case of a 25-year-old male patient with aplastic anaemia who developed progressive right eye scleral necrosis, followed by auto-evisceration. Auto-evisceration was thought to be secondary to ischaemic necrosis and loss of integrity of the scleral coats, along with increased intraocular and intraorbital pressure due to choroidal and orbital haemorrhage. Severe blanching of the other eye possibly heralded the onset of a similar process, but significantly improved post marrow transplantation. We present a hitherto unreported case of devastating ocular complication of aplastic anaemia. Patients with aplastic anaemia should be kept under closed observation and the systemic condition controlled, as soon as possible, to prevent disastrous consequences.