Tsue C, Sakamoto Y, Nakajima Y, Kishi K
Keio University School of Medicine, Japan.
J Surg Case Rep. 2012 Oct 1;2012(10):13. doi: 10.1093/jscr/2012.10.13.
Proteus syndrome is a rare congenital hamartomatous condition that is characterised by a wide range of malformations. Skin and skeletal developmental malformations are common and may manifest as significant physical anomalies. However, few reports have discussed surgical treatment. A 37-year-old woman presented with disproportionate enlargement of the right buttock, which required volume reduction. The overgrown tissue was combined with vascular malformations, which in our patient were extremely thick. The patient experienced massive blood loss during the procedure, due to which a blood transfusion was performed. Pathological findings revealed partial lipohyperplasia with venous malformations. Thus, during operations including incomplete excision, massive bleeding is always a possibility, but is not commonly described in these instances.
变形综合征是一种罕见的先天性错构瘤病症，其特征为存在多种畸形。皮肤和骨骼发育畸形很常见，可能表现为显著的身体异常。然而，很少有报告讨论其外科治疗。一名37岁女性因右臀部不成比例地增大前来就诊，需要进行减容手术。过度生长的组织合并有血管畸形，在我们的患者中这些血管畸形非常粗大。患者在手术过程中出现大量失血，因此进行了输血。病理检查结果显示为部分脂肪组织增生伴静脉畸形。因此，在包括不完全切除的手术过程中，总是有可能发生大量出血，但在这些情况下这种情况并不常见。
