Case of adolescent with Paget-Schroetter syndrome and underlying thrombophilia due to an elevated lipoprotein (A).

Upper extremity deep vein thrombosis (UEDVT) is a rare disorder in children and is most often associated with a central venous catheter (CVC), cancer, or an underlying thrombophilia. In adults, repetitive or strenuous upper extremity activity has been linked to mechanical compression of the thoracic outlet resulting in subclavian vein thrombosis, also known as Paget-Schroetter Syndrome (PSS). We present the case of a 14 year old female who was found to have an UEDVT consistent with PSS. On subsequent thrombophila screening, she was noted to have a significantly elevated lipoprotein (a) level of 83 (normal: <30 mg/dL). She underwent 3 months of anticoagulation therapy prior to her first rib resection and in follow-up was noted to have complete patency of her subclavian vein. This case illustrates the necessity for thrombophilia screening in the pediatric patient with UEDVT despite evidence of anatomic abnormality or mechanical venous compression.