Department of Pediatrics, Texas Children's Hospital, Houston, Texas, USA.
Division of Neonatology, Department of Pediatrics, University of Texas Medical Branch at Galveston, Galveston, Texas, USA.
BMC Pediatr. 2019 Apr 6;19(1):95. doi: 10.1186/s12887-019-1480-0.
Klippel-Trénaunay syndrome (KTS) is a rare congenital condition characterized by the clinical triad of capillary malformations (port wine stains), varicose veins with or without venous malformations, and bony and/or soft tissue hypertrophy.
Here we report the first case of a one-day-old male with KTS presenting with crossed-bilateral limb hypertrophy and post-axial polydactyly.
This case serves to highlight the variable presentation and multiple problems faced by patients with KTS and why multidisciplinary management is mandatory.
Klippel-Trénaunay 综合征(KTS)是一种罕见的先天性疾病,其特征为毛细血管畸形(葡萄酒色斑)、伴有或不伴有静脉畸形的静脉曲张以及骨和/或软组织肥大的三联征。
本文报告了首例 1 天男婴 KTS 病例,表现为双侧肢体交叉性肥大和轴后多指。
该病例强调了 KTS 患者临床表现的多变性和所面临的多种问题,也说明了多学科管理的必要性。