Suprachoroidal Hemorrhage with Delayed Rebleeding as the First Presentation of Bullous Pemphigoid Associated Acquired Hemophilia

Abstract

We report a case of phacoemulsification conversion complicated by suprachoroidal haemorrhage (SCH) and delayed rebleeding at day 5 postoperation, as the first presentation of bullous pemphigoid associated acquired hemophilia. This association has been reported sparingly in the past, and almost exclusively only in hematology literature. To the best of our knowledge, this is the first case of delayed SCH rebleed presenting in a patient with previously undiagnosed Acquired Hemophilia reported. Although exceedingly rare, acquired hemophilia leads to drastic complications and has a high mortality rate. Advice to allow early recognition and prevention of complications is summarized.

Ocular bleeding in an undiagnosed hemophiliac neonate causing irreversible loss of vision: A case report with review of the literature

“…Due to this uncommon event, further blood testing was carried out and factor VIII inhibitors were discovered, which confirmed the diagnosis of acquired hemophilia; yet, the visual prognosis was poor. 4 Another case report by Hon et al narrated a similar story in which an 80-year-old patient presented with a sudden spontaneous macular hemorrhage followed by SCH and intracranial bleeding resulting in death shortly afterwards. 5 It is noteworthy to mention that hemorrhages involving the posterior eye segment showed poor visual outcome in the majority of the reported cases, hence, it is a major challenge for ophthalmologists to prevent ocular bleeding episodes by proper assessment and hematological workup in suspicious patients.…”

“…To the best of our knowledge, the literature in English has a few published cases of undiagnosed hemophilia patients presenting initially with postoperative SCH. 2–5 This current report presents the first case of delayed SCH occurring post-PKP in a neonate with undiagnosed hemophilia A.…”

“…Expectedly, many hemophilia patients usually give a positive history of prolonged bleeding after minor procedures such as tooth extraction or phlebotomies when asked retrospectively. 3 , 4 , 6 Second, neonates usually exhibit physiologic immaturity of their hemostatic system (prolonged PTT); subsequently, the reference ranges used to interpret their coagulation test results should take into consideration the gestational and postnatal age. 13 Any detected prolongation of the PTT thereafter demands measuring factor VIII level to confirm the suspected diagnosis of hemophilia A.…”