Aplastic anemia induced disc edema and visual loss in pregnancy: a case report
© Gupta et al; licensee BioMed Central Ltd. 2008
Received: 24 September 2008
Accepted: 18 November 2008
Published: 18 November 2008
A case of aplastic anemia diagnosed during pregnancy, which developed bilateral disc edema and acute pre-retinal hemorrhage leading to vision loss.
A 20 year old primagravid female developed acute vision loss in her right eye, during hospitalization for treatment of aplastic anemia diagnosed during her pregnancy. Her best-corrected visual acuity (BCVA) was hand motions and fundus evaluation revealed a large pre-macular hemorrhage in the right eye (OD) and bilateral disc edema. Neuro-imaging studies did not reveal any signs of intracranial mass lesion or edema.
There was resolution of the disc edema with improvement in the pre-macular hemorrhage resulting in 20/50 vision in the right eye, following supportive transfusions. Ophthalmic manifestations developing in a pregnant patient with aplastic anemia can be successfully managed with supportive care including red blood cell and platelet transfusions.
Aplastic anemia, a serious hematological disorder characterized by pancytopenia and hypoplastic bone marrow is often exacerbated during pregnancy [1–3]. Hormonal imbalance between hematopoietic placental lactogen and erythropoietin and marrow suppressive estrogen result in this association . We report an unusual case of bilateral disc edema and visual loss due to pre-retinal hemorrhage in a patient with aplastic anemia, diagnosed during pregnancy.
A 20 year-old primagravid Caucasian female at 20 weeks of gestation presented to the emergency department with complaints of weakness, dizziness, headaches and palpitations. Complete blood count (CBC) analysis revealed white blood cell count (WBC) of 1.9 × 109 cells/litre, hemoglobin of 4.9 gm/dl, and platelet count of 5 × 109 cells/litre. Further investigation showed normal liver function tests and prothrombin time and an INR of 13.3s and 0.9 respectively. Bone marrow biopsy confirmed the etiology of the pancytopenia as aplastic anemia and subsequent red blood cell and platelet transfusions stabilized the patient's hematologic status.
The first reported case of aplastic anemia was in a pregnant individual in 1888 . Other conditions associated with aplastic anemia include idiosyncratic drug reactions (chloramphenicol), chemical exposure, eosinophilic fasciitis, and seronegative hepatitis . 78% of cases of aplastic anemia exhibit ophthalmic manifestations. Typical ophthalmic manifestations include eyelid hematoma, subconjunctival hemorrhage, cotton wool spots, retinal nerve fiber layer hemorrhage, Roth's spots, pre-retinal hemorrhage, vitreous hemorrhage, and disc edema. Of these, retinal hemorrhage and cotton wool spots are the most common, 67% and 38% respectively .
Vision loss in our case is secondary to pre-retinal hemorrhage overlying the fovea in the right eye. Visual impairment secondary to pre-retinal hemorrhages can be a presenting symptom in previously undiagnosed cases of aplastic anemia . The presence of hemorrhages in the setting of anemia and thrombocytopenia has been described as part of the constellation of retinal findings in patients with anemic-thrombocytopenic retinopathy. Carraro et al reported an increased prevalence of retinopathy in patients with hemoglobin levels <8 mg/dL and platelet counts <50 × 109/L, which is the case for many of the patients affected by aplastic anemia. 2% of patients with retinal hemorrhages exhibited pre-retinal lesions and 1 of 65 patients with ocular findings exhibited bilateral disc edema .
Optic disc edema has been reported to occur in 6% of cases of aplastic anemia, with the etiology most likely being related to elevated intracranial pressure . Two separate case reports describe the occurrence of idiopathic intracranial hypertension in 3 adolescent patients with aplastic anemia, which responded to management with acetazolamide and correction of the anemia [8, 9]. A recent retrospective case series further highlighted this relationship by describing 7 cases of improvement of papilledema associated with anemia by correction of anemia alone. In another case from the same series, the patient's papilledema only responded to the correction of the anemia despite prior interventions to lower the intracranial pressure . In our patient, we could not establish raised intracranial pressure as the cause for optic disc edema as the lumbar puncture resulted in a dry tap. None the less, our patient's disc edema and enlarged blind spot on HVF testing gradually improved as the hemoglobin levels stabilized.
In summary, this report illustrates a case of visual loss associated with pregnancy related aplastic anemia which was successfully managed with supportive care including red blood cell and platelet transfusions.
Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.
Complete blood count
Best-corrected visual acuity
White blood cell count
Magnetic Resonance Imaging.
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