Klippel-Trenaunay Syndrome Complicated by Osteomyelitis – A Report of Two Cases

Klippel-Trenaunay syndrome (KTS) is a rare syndrome, consisting of a triad of malformations involving the capillary, venous, and lymphatic vessels. The anomaly is present at birth and usually involves a lower limb but may involve more than one limb, as well as portions of the trunk or face. The vascular lesion most often is a capillary malformation, generally localized to the hypertrophied area. KTS has been associated with various complications including hemorrhage, thrombosis and seizures. The association with osteomyelitis has been rarely reported. Here, we describe two cases of patients with KTS who presented following osteomyelitis.

Patient I, an 11 years-old girl, was admitted due to left lower limb pain and walking difficulty. On examination, diffuse port-wine lesions and left leg hemi-hypertrophy were noted and led to the clinical diagnosis of KTS. Subsequent bone scan demonstrated an active infectious process in the distal femur and a MRI study showed findings consistent with osteomyelitis and Brodie abscess. The patient required long term IV antibiotic as well as orthopedic surgery.

Patient II is a four years-old boy with recent diagnosis of KTS involving his left lower limb. He was admitted with fever, pain and swelling of his left leg. Blood culture was positive for Staphylococcus Aureus infection and subsequent bone scan showed findings of osteomyelitis involving the distal tibia. The patient required long term IV antibiotics.

In summary, these cases suggest an increased risk of osteomyelitis among patients with KTS and highlight this possible underappreciated association.