Klippel-Trenaunay-Weber syndrome (KTWS) is a rare condition characterized by capillary malformations, port-wine staining, bone hypertrophy, or, occasionally, hypotrophy. Typically, lesions occur in the lower extremities in 95% of cases, with only 5% occurring in the upper extremities [1]. Several case reports have described the treatment of fractures in the lower extremities with KTWS. However, the risk of massive bleeding, bone deformity, and poor bone quality can lead to suboptimal surgical outcomes [2-5].

To date, there have been no reports in the English literature regarding the treatment of forearm shaft fractures in patients with KTWS. Conventional methods such as open reduction and internal fixation (ORIF) may be hesitant in patients with KTWS due to the risk of intraoperative bleeding and wound problems. Intramedullary nailing is also challenging because of bone deformities and the risk of nonunion.

In this report, we present a case of a 60-year-old patient with KTWS who sustained a fracture of both forearm bones. Satisfactory results were achieved with ORIF following preoperative vascular embolization.

This study design was approved by the Institutional Review Board of Wonkwang University Hospital (WKUH 2023-04-014) and adhered to the ethical principles of the Declaration of Helsinki (2013 amendment). All participants provided written informed consent for publication, including clinical images.

A 61-year-old male patient presented with a both-bone fracture of the left forearm caused by being struck by a pipe while working. Physical examination revealed port-wine stains and edema on the left upper extremity and chest, as well as along with vein hypertrophy and capillary malformations (Fig. 1). The patient had never been diagnosed or treated for these lesions before this presentation.

Preoperative radiography showed a forearm both-bone fracture with bone deformity, classified as AO type 22-B3 (Fig. 2). Additionally, multiple calcifications were observed in the soft tissue on the volar side of the left forearm. Magnetic resonance angiography and computed tomographic angiography revealed angiomatosis and several venous malformations affecting the forearm, wrist, hand muscles, and subcutaneous fat tissues (Fig. 3). Also, bowing of shaft and deformation of the medullary canal were observed.

Based on the clinical presentation and imaging findings, the patient was diagnosed with KTWS. Given the inadequacy of conservative treatment for forearm both-bone fracture, surgical treatment was planned to proceed. Several surgical options were considered, intramedullary (IM) nailing was deemed infeasible due to the bowing and bony destruction of the radius. ORIF was considered but posed a significant risk of carrying massive bleeding. To mitigate this risk, therefore, preoperative vascular embolization was performed before surgery.

General anesthesia was used because a regional block could not be performed due to the presence of skin and vascular lesions. To further reduce the risk of intraoperative bleeding, a tourniquet was applied. The radius fracture was accessed using a volar Henry approach, while a subcutaneous approach with a separate incision was used for the ulna. Care was taken to avoid vascular injury by careful dissection through the intermuscular plane. Additionally, we identified a bleeding focus and electro-cauterized it. We used an anatomic locking plate that was pre-bent to accommodate bone deformity and bowing was used for fixation. After securing the plate fixation, the tourniquet was briefly released to identify and cauterize bleeding points, which were then ligated. Preoperative hemoglobin level was 13.7 g/dL and postoperative one day hemoglobin was 12.1 g/dL.

Compression stockings were applied to the upper extremity to prevent complications such as deep vein thrombosis, the patient wore compression stockings on the upper extremity from the time of admission. A long arm splint was applied for 2 weeks to minimize wound problems, followed by initiation of elbow and wrist range of motion with a removable brace. There were no postoperative complications such as wound problem, bleeding, or infection. In the 4 months after the surgery, bone union was progressing well and one year after surgery, the patient demonstrated signs of bone union (Fig. 4).

This report details the successful management of a forearm both-bone fracture in a 61-year-old KTWS patient using ORIF after preoperative vascular embolization.

There are several case reports have documented the treatment of fractures in the lower extremities in patients with KTWS (Table 1) [2-8]. In most cases of lower extremity fractures of patients with KTWS, no definitive treatment guidelines are established. Vascular malformations and bone destruction present significant challenges in the treatment of fractures. Mahjoub et al. [6] reported pain relief and ambulation can be facilitated through en-bloc resection and reconstruction for nonunion of the femoral shaft. Gupta et al. [2] reported a case in which a 12-year-old KTWS patient with a femoral shaft fracture experienced a 5 cm long lower limb discrepancy despite achieving bone union through skeletal traction with external fixation. Tsaridis et al. [7] reported that IM nailing was performed on a femoral shaft fracture, but massive transfusion and intensive care unit care were required due to massive bleeding. Nahas et al. [3] performed ORIF for femur shaft fracture, but reported delayed union, and reported that bone union was obtained through low-intensity pulsed ultrasound.

In this case, unlike the previous reports focusing on the lower extremity, fractures occurred in the upper extremity, not the lower extremity.

Multiple options were considered, including IM nail, when deciding the optimal fracture fixation technique for this patient. Patel et al. [8] reported that femoral shaft fracture in a patient with KTWS managed by flexible IM nails. However, since the forearm is generally considered as an independent joint and is a non-weight-bearing portion, anatomical reduction throughout ORIF is a more popular treatment than IM nailing for forearm both-bone fracture [9] Additionally, IM nailing is difficult to perform in this patient due to bone deformity, and complications such as nonunion or limitation of motion may occur.

Preoperative vascular embolization has generally been considered a safe and effective means of reducing intraoperative blood loss with recent studies and advances in technique reported. Choi et al. [5] reported satisfactory results using preoperative and postoperative angioembolization and IM fixation for a femoral shaft fracture in a 42-year-old patient with KTWS. Deshpande et al. [4] reported good clinical results using preoperative sclerotherapy and the minimally invasive osteosynthesis technique for femoral shaft fractures in patients with KTWS. In this case, a single-stage operation was performed after embolization. If the risk is to be reduced more, a sequential two-stage operation can be considered as an option.

Careful preoperative planning, meticulous surgical technique and postoperative management resulted in favorable outcomes without complications. In KTWS patients, particularly when vascular malformations and bone deformities are present, a precise individualized treatment strategy is thought to be important.