Multidisciplinary approach to Klippel-Trenaunay syndrome: a case report

Klippel-Trenaunay综合征的多学科诊疗:病例报告

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Abstract

Klippel-Trenaunay syndrome (KTS) is a rare inherited disorder presenting as a triad of capillary malformations (cutaneous hemangiomas), soft tissue hypertrophy, and varicosities. Two out of three signs are enough to make a diagnosis. It is associated with gastrointestinal, hematological, neuro-ophthalmic, dermatological, pulmonary, oro-dental, renal cardiac, and vascular complications. There is no curative treatment for the disease. Complications are currently managed symptomatically and thorough documentation of all signs and symptoms is essential for timely identification and management of potential complications, thereby improving patient outcomes. KTS was diagnosed in a 16-year-old female, with dyspnea and fever associated with hemoptysis and abdominal pain. On physical examination, cutaneous findings and signs of respiratory compromise as well as evidence of vascular disease were observed. After appropriate medical care, the patient was soon discharged from the hospital. Following initial treatment, the patient's symptoms arbitrarily recurred requiring continued medical management and blood product administration to manage complications resulting from KTS. KTS is a chronic disorder with no cure. Patients with KTS are managed symptomatically and require frequent hospitalizations for the same.

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