GP55 Going out on a (clotted) limb – an interesting case of paget-schroetter syndrome in a young adolescent

Introduction A 13-year-old female, presented to the Paediatric Emergency Department (PED), with a 4 week history of bruising to her right upper limb. There was no concurrent history of trauma to the limb. Bruising was progressive, and the arm had become increasingly painful in the three days prior to presentation. On the night prior to presentation, swelling of the arm was noted with associated pain, which was worse on movement. Paraesthesia of the fingers of the affected limb was also noted. Past history Her past history was notable for asthma (mild, no previous admissions) and hypermobility syndrome. Medications There was no known drug allergies. She was not on any regular medications, including any form of contraceptive. Vaccinations were up to date. Family history was notable for Raynaud’s disease. There was no history of venous thromboembolism or any coagulation disorders. Examination On examination, a visibly swollen right upper limb was noted, with a 3 cm difference in circumference and bruising as described. Dilated tortuous veins were visible. The patient was tender to palpation over the biceps muscle and site of tendon insertion. Full power and range of motion was noted at both shoulder and elbow joints. Radial pulses were present and equal. The remaining systemic examination was unremarkable. Investigations Bloods normal, including coagulation US Doppler upper limb no obvious DVT. However, dilatation of basilic vein proximally, with a possible varicosity was noted MRA right upper limb large thrombus of right subclavian vein, protruding into right brachiocephalic vein and axillary vein CT pulmonary angiogram: significant reduction in costoclavicular distance (right>left), suggestive of a thoracic outlet obstruction Treatment Therapeutic subcutaneous low molecular weight heparin was commenced. The patient was admitted for further management, which included thrombectomy and heparin infusion. Discussion Paget-Schroetter syndrome, or primary ‘spontaneous’ DVT of the upper limb, is a condition which typically affects otherwise healthy individuals. It occurs as a result of anatomical abnormalities of the thoracic outlet, which results in compression of the axillosubclavian venous system and subsequent thrombosis. It is a rare condition that is, however, important to recognize promptly as early detection and treatment have been found to reduce long-term sequelae. This case, while rare, highlights the importance to us, as paediatric physicians, to consider in differential diagnosess, disorders and problems that would typically be more attributable to adult populations.