Management
In this patient, symptomatic LIC was initially treated with aspirin. Aspirin may be helpful in selected patients, although no prospective studies have analyzed safety and efficacy. A retrospective survey of patients with VMs on aspirin reported 45% of participants obtaining a positive response, manifested as either less aching pain, less shooting pain, decreased fullness/swelling, and shrinkage of VM. Bleeding and bruising were reported complications (51). If aspirin does not improve symptoms, anti-coagulation can be considered. A thorough personal and family history of bleeding should be completed prior to initiation of anti-coagulation. Prior studies have documented a lack of response to vitamin K antagonists (46), and generally low molecular weight heparin (LMWH) is the agent of first choice. Patients are typically started on prophylactic dosing (0.5 mg/kg twice a day) of LMWH, with appropriate monitoring of complete blood count, renal function, and LMWH levels. Treatment dosing of LMWH (1.0 mg/kg twice a day) may be needed depending on patient response. Duration of treatment is variable, with patients requiring either episodic or continuous dosing (52). As the direct oral anti-coagulants become approved for use in children, these agents may play a more significant role in anti-coagulation for VM associated LIC. Case reports of their use in adult patients with VMs have shown improvement in LIC symptoms (53,54).
Given the location on the hand, medical management was chosen. Surgical resection would be difficult and could lead to nerve or muscle damage, and similar concerns were present in regards to the potential complications of sclerotherapy. This patient was started on prophylactic dosing of lovenox with improvement in pain. If this patient had undergone sclerotherapy or surgical resection, discussion regarding the peri-procedural management of her LIC would be warranted. Unfortunately, there is a lack of evidence-based data to guide approach. The d-dimer threshold which places patients at risk for procedural complications, including both bleeding and thrombosis, is not known. Prior published guidelines from the Vascular Anomalies Special Interest Group of the American Society of Pediatric Hematology and Oncology recommended that patients with d-dimer greater than five times the upper limit of normal be administered LMWH for two weeks pre- and post-procedure (55).