International journal of surgery case reports · 2026
A 20-year follow-up of a patient with Klippel-Trenaunay syndrome: case report.
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Dombrovskyi Dmytro B, Ivanchuk Maria A
Аннотация
INTRODUCTION: Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation characterized by a clinical triad: capillary malformations, varicose veins, and hypertrophy of soft tissues or bones in one or more limbs. Differential diagnosis from Parkes Weber syndrome is crucial; the primary distinguishing feature is the presence of arteriovenous fistulas. KTS is progressive. Management of KTS is predominantly conservative, involving lifelong elastic compression therapy and regular courses of venotonics. This case report presents the clinical follow-up of a patient with KTS over a period of more than 20 years.
CASE PRESENTATION: A female patient with KTS presented with a congenital venous malformation of the right lower extremity, characterized by predominant deep vein hypoplasia and superficial venous varicosities, with grade III chronic venous insufficiency on the right side. She also had pelvic varicosities and superficial venous varicosities of the left lower extremity. During the reported period, the patient underwent two surgical interventions for the condition and had three deliveries.
DISCUSSION: KTS follows a progressive course characterized by unilateral varicose veins, persistent embryonic veins, anomalies of the superficial and deep venous systems, low-flow venolymphatic malformations, and hypertrophy of bone and soft tissue. Pregnancy in women with KTS is considered a high-risk condition. Management of KTS is predominantly conservative, supplemented by minimally invasive procedures if necessary.
CONCLUSION: The study supports the conclusion that open surgery for KTS can lead to recurrences. Long-term use of elastic compression therapy, combined with consistent daily walking and regular courses of venotonics, may slow disease progression.
CASE PRESENTATION: A female patient with KTS presented with a congenital venous malformation of the right lower extremity, characterized by predominant deep vein hypoplasia and superficial venous varicosities, with grade III chronic venous insufficiency on the right side. She also had pelvic varicosities and superficial venous varicosities of the left lower extremity. During the reported period, the patient underwent two surgical interventions for the condition and had three deliveries.
DISCUSSION: KTS follows a progressive course characterized by unilateral varicose veins, persistent embryonic veins, anomalies of the superficial and deep venous systems, low-flow venolymphatic malformations, and hypertrophy of bone and soft tissue. Pregnancy in women with KTS is considered a high-risk condition. Management of KTS is predominantly conservative, supplemented by minimally invasive procedures if necessary.
CONCLUSION: The study supports the conclusion that open surgery for KTS can lead to recurrences. Long-term use of elastic compression therapy, combined with consistent daily walking and regular courses of venotonics, may slow disease progression.