rare case presentation

1. PAGET-SCHROETTER
SYNDROME

2. 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)

3. CLASSSIFICATION:-
In order of
incidence, neurogeni
c (NTOS), venous
(VTOS), and arterial
(ATOS) thoracic
outlet syndromes are
three distinct entities
and should be
conceptualized, discu
ssed, analyzed, and
treated as such.
VTOS is further
divided into three
different categories:
intermittent/positional
venous
obstruction, secondar
y 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.

4. PATHOPHYSIOLOGY
Normal anatomy of the
thoracic outlet.
Abnormal lateral insertion of the
costoclavicular ligament in PagetSchroetter syndrome

5. DIAGNOSIS: Compression ultrasonography with
color Doppler
 Contrast venography
 Radionuclide, magnetic resonance
and computed tomographic
venography


6.  MANAGEMENT: CONSERVATIVE:-
Anticoagulation
 Systemic fibrinolysis
 Catheterdirected thrombolysis
 Angioplasty
 Stenting


7.  SURGICAL:-
Surgical thrombectomy
 Trans-axillary TOD
 Clavicular(supra, para, infra)
 Sternal disarticulation with first rib
resection
 Medial claviculectomy
 Vascular reconstructive surgery


8. 
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.

9. 
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)

10. 
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

12. 
. 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.

13. 
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.

14. 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.


15. ● 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.


16. ● 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.


18. 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-molecularweight 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]

19. 
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