Department of Surgery, University of Medicine and Dentistry of New Jersey- Robert Wood Johnson Medical School, One Robert Wood Johnson Way, New Brunswick, NJ 08903, USA.
J Pediatr Surg. 2013 Aug;48(8):e9-e12. doi: 10.1016/j.jpedsurg.2013.06.014.
Peliosis hepatis (PH) is a rare condition characterized by multiple blood-filled spaces within the hepatic parenchyma that can lead to fatal hemorrhage. There is no consensus on the best treatment algorithm for PH, and therapy is directed at removing the potential causative agent with operative intervention when necessary. Here we present the first known case of PH in a child with myotubular myopathy who was successfully treated with angiography and hepatic artery embolization as a first line therapy, without the need for operative intervention. Awareness of this condition and the available treatment modalities may lead to favorable outcomes in future cases.
肝血窦扩张症（PH）是一种罕见的疾病，其特征是肝脏实质内有多个充满血液的腔隙，可导致致命性出血。目前对于 PH 的最佳治疗方案尚无共识，治疗方法是在必要时通过手术干预去除潜在的病因。在这里，我们报告首例肌小管肌病患儿 PH 病例，该患儿经血管造影和肝动脉栓塞治疗作为一线治疗成功，无需手术干预。对这种疾病的认识和现有的治疗方法可能会导致未来病例的良好结果。
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