Lange Undine G, Bucher Julian N, Schoenberg Markus B, Benzing Christian, Schmelzle Moritz, Gradistanac Tanja, Strocka Steffen, Hau Hans-Michael, Bartels Michael
Undine G Lange, Christian Benzing, Hans-Michael Hau, Michael Bartels, Department of Visceral, Transplant, Thoracic and Vascular Surgery, Leipzig University Hospital, 04103 Leipzig, Germany.
World J Transplant. 2015 Dec 24;5(4):354-9. doi: 10.5500/wjt.v5.i4.354.
In liver haemangiomas, the risk of complication rises with increasing size, and treatment can be obligatory. Here we present a case of a 46-year-old female who suffered from a giant haemangioma causing severe portal hypertension and vena cava compression, leading to therapy refractory ascites, hyponatremia and venostasis-associated thrombosis with pulmonary embolism. The patients did not experience tumour rupture or consumptive coagulopathy. Surgical resection was impossible because of steatosis of the non-affected liver. Orthotopic liver transplantation was identified as the only treatment option. The patient's renal function remained stable even though progressive morbidity and organ allocation were improbable according to the patient's lab model for end-stage liver disease (labMELD) score. Therefore, non-standard exception status was approved by the European organ allocation network "Eurotransplant". The patient underwent successful orthotopic liver transplantation 16 mo after admission to our centre. Our case report indicates the underrepresentation of morbidity associated with refractory ascites in the labMELD-based transplant allocation system, and it indicates the necessity of promptly applying for non-standard exception status to enable transplantation in patients with a severe clinical condition but low labMELD score. Our case highlights the fact that liver transplantation should be considered early in patients with non-resectable, symptomatic benign liver tumours.
在肝脏血管瘤中,并发症的风险随肿瘤大小的增加而上升,因此可能需要进行治疗。在此,我们报告一例46岁女性患者,她患有巨大血管瘤,导致严重的门静脉高压和腔静脉受压,进而引发治疗难治性腹水、低钠血症以及与静脉淤滞相关的血栓形成并伴有肺栓塞。患者未出现肿瘤破裂或消耗性凝血病。由于未受影响的肝脏存在脂肪变性,无法进行手术切除。原位肝移植被确定为唯一的治疗选择。尽管根据患者的终末期肝病实验室模型(labMELD)评分,患者的病情可能会逐渐恶化且不太可能获得器官分配,但患者的肾功能仍保持稳定。因此,欧洲器官分配网络“Eurotransplant”批准了非标准例外状态。患者在入住我们中心16个月后成功接受了原位肝移植。我们的病例报告表明,在基于labMELD的移植分配系统中,与难治性腹水相关的发病率未得到充分体现,这表明对于临床状况严重但labMELD评分较低的患者,有必要及时申请非标准例外状态以实现移植。我们的病例突出了一个事实,即对于不可切除的、有症状的良性肝脏肿瘤患者,应尽早考虑肝移植。