PAGET-SCHROETTER SYNDROME

INTRODUCTION

HISTORIC IMPORTANCE:- The earliest description of spontaneous

ASVT was by Cruveilhier in 1816, and the first elaborate account was provided by James Paget in 1875.In 1894, von Schroetter was the first to identify vascular trauma from muscle strain as a potential etiologic factor. In 1948, Hughes coined the term Paget-Schroetter Syndrome (PSS)

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In order of incidence, neurogenic (NTOS), venous (VTOS), and arterial (ATOS) thoracic outlet syndromes are three distinct entities and should be conceptualized, discussed, analyzed, and treated as such. VTOS is further divided into three different categories: intermittent/positional venous obstruction, secondary subclavian vein thrombosis (in the setting of catheters or pacemaker leads), and primary “effort thrombosis” The last entity, primary effort thrombosis, is the topic of the current review.

PATHOPHYSIOLOGY

Normal anatomy of the thoracic outlet.

Abnormal lateral insertion of the costoclavicular ligament in Paget-Schroetter syndrome

DIAGNOSIS:- Compression ultrasonography

with color Doppler Contrast venography Radionuclide, magnetic

resonance and computed tomographic venography

MANAGEMENT:-

CONSERVATIVE:- Anticoagulation Systemic fibrinolysis Catheterdirected thrombolysis Angioplasty Stenting

SURGICAL:- Surgical thrombectomy Trans-axillary TOD Clavicular(supra, para, infra) Sternal disarticulation with first

rib resection Medial claviculectomy Vascular reconstructive surgery

CASE SUMMARY: 35 year male, an agricultural labourer presented with the history of swelling and visible dilated veins over right arm and shoulder for last 6 days. he was having pain in the right armand gave a history of working as labourer on manual forage chopper machine for 7-8 hrs a day from last 9-10 days. The patient denied any trauma or past injuries to his upper extremities or trunk. He denied chest pain, shortness of breath, fever, easy bruising, bleeding, palpitation, bone pain, lymph node or joint swelling. The patient was not on any medications. The patient’s family history was negative. The patient smoked half a pack of cigarettes per day and drank alcohol socially.

On physical examination, the patient had prominent superficial veins visible over the right subclavian area and a palpable cord over the basilic and axillary veins. The right upper extremity had nonpitting edema with homogeneous erythema that blanched to palpation. His radial and brachial pulses were 2 at rest. He had a positive Wright test (attenuation of brachial/radial pulses with hyperabduction of the arm)

a negative Adson test and a negative Halsted test. The extremities were compared, and there was no directly observed or relative atrophy of any group of muscles. Tinel and Phalen signs were absent, and no tenderness was noted over the supraclavicular fossa. The left upper extremity showed no abnormalities

. On haematological examination he was Hbsag positive. Colour doppler examination revealed right axillary and subclavian vein thrombosis with multiple collaterals around the shoulder. There was no evidence of pulmonary embolism clinically and on CT chest.

The patient was treated with injection heparin for seven days followed by oral anticoagulation therapy and advise of avoiding sternous activity. The edema and collateral veins disappeared after one week of therapy with heparin. Patient was followed on 1 month and 3 month interval with no evidence of recurrence, after which unfortunately patient lost to follow-up.

SUMMARY

● Definitive outcomes with and without thoracic outlet decompression after thrombolysis by means of a true prospective randomized trial.

● Definitive answer to the timing of decompression – acute or delayed?

● The role of angioplasty vs observation for residual defects after decompression.

● The role of stents in the decompressed thoracic outlet.

● Duration of postoperative anticoagulation.

● Results after claviculectomy: functional and cosmetic perception and reality.

● Natural history of the contralateral side. ● Long-term functional outcome –

occupational and recreational. ● Best treatment of the vein that cannot be

opened with thrombolysis.

● When venous reconstruction is needed, and the best method thereof.

● Cellular and molecular events at the diseased costoclavicular junction.

● More information on the role of hypercoagulable states in effort thrombosis.

REFFREVCES:- Roche-Nagle G, Ryan R, Barry M, et al; Effort thrombosis of the

upper extremity in a young sportsman: Paget-Schroetter syndrome. Br J Sports Med. 2007 Aug;41(8):540-1; discussion 541. Epub 2007 Feb 8. [abstract]

Spencer FA, Emery C, Lessard D, et al; Upper extremity deep vein thrombosis: a community-based perspective. Am J Med. 2007 Aug;120(8):678-84. [abstract]

Amir-Us-Saqlain H, Javaid A, Hashmi I, et al; Upper extremity deep vein thrombosis. J Coll Physicians Surg Pak. 2005 May;15(5):309-10. [abstract]

Spiezia L, Simioni P; Upper extremity deep vein thrombosis. Intern Emerg Med. 2009 Sep 26. [abstract]

Kovacs MJ, Kahn SR, Rodger M, et al; A pilot study of central venous catheter survival in cancer patients using low-molecular-weight heparin (dalteparin) and warfarin without catheter removal for the treatment of upper extremity deep vein thrombosis (The Catheter Study). J Thromb Haemost. 2007 Aug;5(8):1650-3. Epub 2007 May 7. [abstract]

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