Aplastic Anemia Presenting as Spontaneous Orbital Hemorrhage

Abstract

We report a case of a 51-year-old female presenting with a three-day history of a painful swollen right eye associated with loss of vision. She presented with no light perception in the affected eye, periocular ecchymosis, hemorrhagic chemosis, and an axial proptosis. CT scan revealed a unilateral proptosis with tension on the optic nerve and accompanying diffuse retrobulbar hemorrhage. Bone marrow studies confirmed aplastic anemia. A review of the English literature failed to reveal previous cases of aplastic anemia presenting with spontaneous orbital hemorrhage. We suggest that aplastic anemia be included in the differential diagnosis of a patient presenting with painful spontaneous orbital hemorrhage.

Efficacy of Transcutaneous Transseptal Orbital Decompression in Treating Acute Retrobulbar Hemorrhage and a Literature Review

“…30 In some case reports, lateral canthotomy with inferior cantholysis failed to sufficiently decompress the orbit because of severe tension of the globe and periorbital swelling; thus, visual acuity could not be maintained or reconstituted in these cases. 20 Taken together, these findings indicate that transcutaneous transseptal orbital decompression provides a rapid intervention which safely releases intraorbital pressure and efficiently drains hemorrhage from the orbital compartment, particularly, in extensive orbital hemorrhage, periorbital swelling or when imaging (CT or magnetic resonance imaging) is not available. Pulsating exophthalmos, which may be a sign of carotid-cavernous sinus fistula, is the only contraindication for surgical treatment.…”

“…In most published cases, conservative treatment was considered an adjuvant option. In addition to steroids, the use of mannitol and acetazolamide is included in almost all published protocols, 20,35,52 although the administration protocols vary. In contrast, our conservative therapy of choice for the treatment of acute RBH or other sudden expanding retrobulbar lesions or traumatic optic nerve lesions is the methylprednisolone megadose regimen (see Methods).…”

“…13,14 In nonpenetrating orbital trauma, fractures of the orbit following blunt facial trauma may be associated with RBH, and loss of vision occurs in 0.3% of these patients. 15,16 However, drug therapy 17 or hemorrhage due to congenital or systemic conditions [18][19][20] may lead to nontraumatic RBH. Drug-related nontraumatic RBH occurs in hemorrhagic diatheses due to circulating anticoagulation medication, 21,22 whereas vessel malformations are congenital causes of RBH.…”

Diagnosis and Treatment of Optic Nerve Trauma

“…22,23 However, drug therapy or hemorrhage due to congenital or systemic conditions may lead to nontraumatic RBH. [24][25][26][27] Drug-related nontraumatic RBH occurs in hemorrhagic diatheses due to circulating anticoagulation medication, whereas vessel malformations are congenital causes of RBH. [28][29][30] RBH can occur spontaneously in drug-related pharmacological or congenital conditions.…”

Spontaneous Periocular Ecchymosis in Children: Differential Diagnosis and Current Trends in Evaluation and Management

“…However, the spontaneous appearance of periocular ecchymosis in a child, without an attributable traumatic or surgical etiology, should alert the pediatrician and ophthalmologist to a differential diagnosis that includes both life-threatening and vision-threatening conditions (Table 1). Life-threatening conditions include pediatric malignancies such as neuroblastoma, rhabdomyosarcoma, and leukemia; as well as hematologic disorders such as aplastic anemia and thrombocytopenia [4][5][6][7][8]. Neuroblastoma and rhabdomyosarcoma are uncommon, with incidences ranging from 4 to 9 cases per million [9,10], but they represent 3% to 7.5% of malignancies in children, and are fatal if undiagnosed [11].…”