Ref Number = PITIKA-ASPR0002
Elisa Joana Ramos Lim, M.D.
Objective: Klippel-Trenaunay Syndrome (KTS) is a rare and sporadic congenital condition defined as the triad of vascular malformation, in most cases presents with port wine stain, venous varicosity and limb overgrowth, with or without lymphatic malformation. The infrequency with which it is encountered makes KTS difficult to recognize. This paper aims to increase awareness about the disease, how it can put one’s health and future in “triple jeopardy”, and the need for a multidisciplinary approach in addressing these anticipated problems.
Case: born full term to non-consanguineous apparently healthy parents with an uneventful maternal and birth history, was noted to have a right lower limb that was larger than the left. This was often misdiagnosed as hemangioma during routine health check-ups with no specific management advised until she presented at a tertiary pediatric government hospital with a chronic wound leading to osteomyelitis on the affected limb at the age of four years old. Diagnosis was confirmed through x-ray, ultrasound with doppler studies and MRI with angiogram. Treated conservatively with antibiotics, and physiotherapy by means of compression stockings and orthopedic modalities, the patient was also referred to vascular and orthopedic surgery, rehabilitation medicine, and dermatology service for ample care and further monitoring until the child reaches adulthood.
Conclusion: this case illustrates the features of KTS and its predisposition to mild and severe complications such as infection, bleeding, limb length discrepancy, venous insufficiency, pain, depression and thromboembolism. KTS is a lifelong and potentially debilitating condition, requiring further monitoring and medical, surgical and psychologic care with a multidisciplinary team.
Keywords: Klippel-Trenaunay syndrome, features, predisposition, complications, monitoring
Disclaimer: The Views and opinions expressed in the articles are of the authors and not of the journal.
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