Post-Partum Hemorrhage (PPH) as Implicated by Osler-Weber-Rendu Syndrome

Abstract

Obstetrical management of patients with genetic disorders bears its own challenges in the overall care and outcomes of the patient. Hereditary hemorrhagic telangiectasia (HHT), or Osler-Weber-Rendu Syndrome, is an inherited genetic disorder that, due to the nature of the pathophysiology of arteriovenous malformation and dysplasia, has been highly associated with an increased tendency of bleeding. This case presents a patient diagnosed with HHT prior to pregnancy who developed severe postpartum hemorrhage (PPH) after cesarean section delivery. Clinical considerations were made proactively with the knowledge and understanding of the genetic disorder, but due to unforeseen changes in the manifestation of the PPH, clinical decisions were dynamically modified in order to save the patient from exsanguination, ultimately resulting in an emergency hysterectomy and a total of 15 units of transfused packed red blood cells (pRBC). As a result, this case report serves as a preliminary recount for future research and clinical management of similar cases.