PDF(Pubmed)
Abstract：
BACKGROUND: Klippel-Trenaunay syndrome (KTS) is a congenital vascular malformation with a complicated etiology. It is sporadic and clinically rare in occurrence. The typical characteristics are capillary malformation (also known as port-wine stain), varicose veins and malformations, and bony and/or soft tissue hypertrophy with or without lymphatic malformation, which are known as the \"classic clinical triad\". Herein, a rare case of KTS characterized by crossed-bilateral limb hypertrophy accompanied by intermittent hematochezia and hematuria is reported.
METHODS: We described a 37-year-old female with KTS. She was admitted to our hospital owing to the gradual enlargement of the left lower extremity along with intermittent hematochezia and hematuria. The patient was diagnosed to have hemorrhoid bleeding by other hospitals and treated with conventional hemostatic drugs, but continued to have intermittent gastrointestinal bleeding and hematuria. Therefore, she visited our hospital to seek further treatment. During hospitalization, relevant imaging and laboratory examinations and colonoscopy were performed. In combination with the patient\'s history and relevant examinations, we considered that the patient had a complex form of KTS. We recommended a combined diagnosis and treatment from the vascular, interventional, anorectal, and other departments, although she declined any further treatment for financial reasons.
CONCLUSIONS: The clinical manifestations of KTS are extensive and diverse and chiefly include the typical triad. However, Vascular malformations of KTS can also involve several parts and systems such as digestive and urogenital systems. Therefore, the atypical manifestations and rare complications necessitate the clinician\'s attention and are not to be ignored.
METHODS: We described a 37-year-old female with KTS. She was admitted to our hospital owing to the gradual enlargement of the left lower extremity along with intermittent hematochezia and hematuria. The patient was diagnosed to have hemorrhoid bleeding by other hospitals and treated with conventional hemostatic drugs, but continued to have intermittent gastrointestinal bleeding and hematuria. Therefore, she visited our hospital to seek further treatment. During hospitalization, relevant imaging and laboratory examinations and colonoscopy were performed. In combination with the patient\'s history and relevant examinations, we considered that the patient had a complex form of KTS. We recommended a combined diagnosis and treatment from the vascular, interventional, anorectal, and other departments, although she declined any further treatment for financial reasons.
CONCLUSIONS: The clinical manifestations of KTS are extensive and diverse and chiefly include the typical triad. However, Vascular malformations of KTS can also involve several parts and systems such as digestive and urogenital systems. Therefore, the atypical manifestations and rare complications necessitate the clinician\'s attention and are not to be ignored.
摘要：
背景：Klippel-Trenaunay综合征（KTS）是一种病因复杂的先天性血管畸形。它是偶发性的，临床上很少见。典型特征是毛细管畸形（也称为葡萄酒色斑），静脉曲张和畸形，骨和/或软组织肥大伴或不伴淋巴畸形，被称为“经典临床三合会”。在这里，据报道，一例罕见的KTS病例，其特征是交叉双侧肢体肥大，伴有间歇性便血和血尿。
方法：我们描述了一名37岁女性患有KTS。她因左下肢逐渐扩大并伴有间歇性便血和血尿而入院。患者被其他医院诊断为痔疮出血，并接受常规止血药物治疗，但仍有间歇性消化道出血和血尿。因此,她到我们医院寻求进一步治疗。住院期间,进行了相关的影像学和实验室检查和结肠镜检查.结合患者病史和相关检查,我们认为患者患有复杂形式的KTS。我们建议从血管进行综合诊断和治疗，介入,肛门直肠,和其他部门，尽管出于经济原因，她拒绝了任何进一步的治疗。
结论：KTS的临床表现广泛多样，主要包括典型的三联征。然而,KTS的血管畸形还可以涉及多个部分和系统，例如消化和泌尿生殖系统。因此,不典型的表现和罕见的并发症需要临床医生的注意，不容忽视。
方法：我们描述了一名37岁女性患有KTS。
结论：KTS的临床表现广泛多样，主要包括典型的三联征。
