Adult Hip Manifestations in Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is rare and sporadic disorder. Clinical manifestation of KTS is heterogenous. KTS patients often suffer degenerative joint disease at an early age. Arthroplasty performed in extremities with KTS is challenging due to vascular malformations and increased risk of intraoperative and post-operative complications.

We report two patients of KTS with varied musculoskeletal manifestations with one patient treated operatively and one patient managed conservatively, both predominantly affecting the hip joint. We present this report due to rarity of presentation and to discuss varied articular manifestations in two patients with KTS. Both patients fulfilled the criteria and were diagnosed to have KTS. The first patient was a 36 year-old woman who presented with left hip pain since 3 years which was progressively worsening. As this patient did not have any vascular malformations around the hip, a left total hip arthroplasty was planned and successfully executed. The second patient was a 42-year-old gentleman who had polyarticular involvement, mainly affecting the hip and knee. Due to the severe presentation of a subluxated hip, vascular malformations, gross knee laxity, and the patient's occupational demands; it was decided to opt for conservative management in this patient.

KTS often presents a challenging scenario due to the rarity of the condition, multiple systems involved and varied manifestations from patient to patient which makes it essential to individualize treatment goals based on the severity, functional demands, and associated complications.