Analysis of Clinical Characteristics of 17 Cases of Chinese Patients With Klippel-Trenaunay Syndrome Involving Digestive Tract
10.3969/j.issn.1008-7125.2024.11.008
- VernacularTitle:我国17例消化道受累的Klippel-Trenaunay综合征患者临床特点分析
- Author:
Zongxiang CHEN
1
;
Anlong YUAN
Author Information
1. 武汉科技大学附属孝感医院消化内科(432000);武汉科技大学医学部医学院
- Publication Type:Journal Article
- Keywords:
Klippel-Trenaunay Syndrome;
Gastrointestinal Tract;
Clinical Characteristics;
Diagnosis;
Therapy
- From:
Chinese Journal of Gastroenterology
2024;29(11):685-689
- CountryChina
- Language:Chinese
-
Abstract:
Background:Klippel-Trenaunay syndrome(KTS)is a rare vascular malformation syndrome that can involve the gastrointestinal tract,and the characteristics of the disease are still not fully understood,and there is no clinical research data about the disease in the gastrointestinal tract in China.Aims:To summarize and analyze the clinical characteristics of Chinese patients with KTS involving the digestive tract.Methods:Relevant literature was searched using English keywords such as"Klippel-Trenaunay syndrome"and"K-T syndrome",and Chinese subject terms including"Klippel-Trenaunay syndrome"and"KTS".Each study reporting cases of KTS involving the digestive tract in China was reviewed,and clinical data of patients were collected and summarized for analysis.Results:A total of 17 Chinese patients with KTS involving the digestive tract were identified through the search.After data compilation and analysis,it was found that the majority of patients were females,with the age group of 20-29 being the most affected.60.0％of the patients presented with the typical triad of symptoms,usually affecting the right lower limb unilaterally,with cutaneous capillary malformations and limb hypertrophy as the primary clinical signs.The clinical manifestations of gastrointestinal involvement in KTS patients mainly include symptoms such as rectal bleeding,abdominal pain,and melena,with 58.9％of patients experiencing rectal bleeding within 10 years after birth,and some patients being initially misdiagnosed as having hemorrhoidal bleeding.The most commonly affected sites were the rectum and sigmoid colon,with rare cases involving the entire gastrointestinal tract.Most patients also presented with vascular abnormalities in other abdominal organs,leading to complications such as hematemesis and liver failure.Abdominal CT findings showed thickening of the affected intestinal wall and the presence of venous stones or vascular tumor lesions.Endoscopic examination revealed submucosal bluish or purplish vascular lesions and dilated vascular structures,along with disrupted mucosal vascular patterns.The majority of patients had their condition managed through conservative treatment,while severe bleeding or extensive lesions required surgery.Sclerotherapy,embolization,and other treatments have also been proven effective.Conclusions:KTS involving the digestive tract is a rare disease,commonly characterized by abdominal pain and gastrointestinal bleeding,which can be misdiagnosed as hemorrhoids.Abdominal CT or endoscopy may present specific features.Treatment mainly focuses on symptomatic management,with a subset of patients requiring surgical or interventional procedures.
