Purpose:
To report a case of progressive, submacular hemorrhage due to a rare hereditary prothrombin deficiency that was spontaneous and isolated.
Methods:
Retrospective case report including ultra-wide-angle fundus imaging (Optos, Marlborough, MA), clinical course, and pathology results.
Results:
A 55-year-old woman with a past medical history of hereditary prothrombin deficiency presented with sudden onset of photopsias in her left eye. Examination revealed a small submacular hemorrhage. Fluorescein angiogram showed no detectable underlying vascular abnormality in either eye. Coagulation panel was within normal limits. Despite receiving 14 units of fresh frozen plasma the hemorrhage progressed to involve the suprachoroidal space. Intraocular surgical drainage with gas tamponade was attempted without success. Two weeks later, an evisceration of the left eye was performed due to pain from intractable intraocular pressure elevation in the blind eye. Pathologic examination of the eye to detect any predisposing lesion was limited and revealed corneal blood staining only. Six months later, the patient was doing well with an ocular prosthesis and no systemic complications.
Conclusions:
To our knowledge, this is the first reported case of a spontaneous and progressive submacular hemorrhage in a hemophiliac that was not associated with systemic bleeding. Ultra-wide-field fluorescein angiography was useful to confirm the absence of underlying retina vascular abnormalities.
Keywords: macula/fovea • imaging methods (CT, FA, ICG, MRI, OCT, RTA, SLO, ultrasound)