Powers Patrick, Combs Shanna
Medical Education, Burnett School of Medicine at Texas Christian University, Fort Worth, USA.
Obstetrics and Gynecology, Burnett School of Medicine at Texas Christian University, Fort Worth, USA.
Cureus. 2023 Aug 12;15(8):e43388. doi: 10.7759/cureus.43388. eCollection 2023 Aug.
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.
患有遗传性疾病患者的产科管理在患者的整体护理和预后方面有其自身的挑战。遗传性出血性毛细血管扩张症(HHT),即奥斯勒-韦伯-伦杜综合征,是一种遗传性疾病,由于动静脉畸形和发育异常的病理生理性质,与出血倾向增加高度相关。本病例介绍了一名在怀孕前被诊断为HHT的患者,剖宫产术后发生了严重的产后出血(PPH)。在了解和认识该遗传性疾病的情况下进行了积极的临床考虑,但由于PPH表现出现意外变化,为了使患者避免失血过多而对临床决策进行了动态调整,最终导致急诊子宫切除术,共输注了15单位的浓缩红细胞(pRBC)。因此,本病例报告为未来类似病例的研究和临床管理提供了初步叙述。