Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Ave, Cincinnati, OH 45229, United States.
Division of Pediatric General and Thoracic Surgery, Cincinnati Children's Hospital Medical Center, University of Cincinnati, 3333 Burnet Ave, Cincinnati, OH 45229, United States.
Semin Pediatr Surg. 2020 Oct;29(5):150977. doi: 10.1016/j.sempedsurg.2020.150977. Epub 2020 Sep 16.
Visceral vascular anomalies are common in patients with vascular malformations in other parts of the body and can include lymphatic, venous, and arteriovenous malformations. Depending on the organ or organs involved they may present differently and pose different treatment challenges. Defining the malformation and understanding its extent is paramount in devising management regimens. Medical, interventional, and surgical therapies are often required in combination to treat these complex lesions. There are new and promising advances in the development of therapeutic agents targeting the PI3K/AKT/mTOR pathway. Due to the complex nature of these lesions a coordinated, multi-disciplinary approach is necessary to manage and mitigate symptoms and complications of this diverse group of vascular malformations.
内脏血管异常在身体其他部位存在血管畸形的患者中很常见,包括淋巴管畸形、静脉畸形和动静脉畸形。根据受累的器官或器官的不同,它们可能表现不同,并带来不同的治疗挑战。明确畸形并了解其范围对于制定治疗方案至关重要。通常需要联合使用药物、介入和手术治疗来治疗这些复杂的病变。针对 PI3K/AKT/mTOR 通路的治疗药物有新的、有前途的进展。由于这些病变的复杂性,需要采取协调的多学科方法来管理和减轻这群多样化的血管畸形的症状和并发症。