Via Research Recognition Day Program VCOM-Carolinas 2025

Case Reports

CASE REPORT: PAGET SCHROETTER SYNDROME IN TWO BIOLOGICAL TEENAGE BROTHERS LEADING TO IPSILATERAL FIRST RIB RESECTION

Samson Mumber OMS II, John Mumber OMS II, Dylan Ledbetter OMS II, Dr. Daniel Cawley DC PhD VCOM-Auburn, Dr. Trent Prault MD Floyd Atrium

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BACKGROUND Paget-Schroetter Syndrome (PSS) is the venous type of thoracic outlet syndrome (TOS), a group of disorders caused by neurovascular dysfunction in the thoracic inlet of the upper body. 1 PSS, also known as primary effort thrombosis, involves thrombosis of the deep veins of the upper extremities due to compression of the subclavian vein by surrounding anatomical structures, such as the first rib and anterior scalene muscles. 1 This syndrome is rare, occurring in 1-4% of all venous thrombosis cases and with an incidence of 1 per 100,000 people . 1 The etiology of PSS is typically considered to be sporadic and the likelihood of two PSS cases occurring in biological siblings is incredibly rare. EPIDEMIOLOGY ● Cases of PSS are more commonly seen in males, with an incidence of 2:1 compared to females. 1 ● PSS is most commonly seen in the age range of 20 years to 30 years old. 2 ● Cases are most commonly documented in the right arm. 2 ● Most commonly those afflicted with PSS are athletes or undergo consistent active exercise with overhead motion leading to subclavian compression. 3 ● The adjacent anatomic structures of the thoracic outlet are the clavicle, the first rib, subclavius muscle, and anterior scalenes . Any of these anatomical structures may lead to compression of the subclavian vein with consistent overhead strain and repetitive motion. 3 ● Typically patients present with swelling and pain in their affected arm, the arm may be cyanotic and the fingers may have transient numbness and tingling. In some cases there may be venous collaterals identified grossly on the arm which is referred to as Urschel’s sign . 4 ● Complications include long term venous insufficiency, recurrent thrombosis if there is no surgical intervention, pulmonary embolism, and chronic pain from post thrombotic syndrome. 5

CASE 1: The first case involves a 17-year-old male basketball player who presented with persistent swelling in his right arm that initially worsened with exercise but later developed into a persistent issue. A venous ultrasound confirmed a deep vein thrombosis (DVT) in the patient’s axillary vein. A venogram revealed further extension of the thrombus into the basilic vein, with compression of the subclavian vein between the first rib and clavicle. The patient was then admitted to the hospital for thrombolysis with tissue plasminogen activator (tPA), which was followed by a first rib resection three days later. This course of treatment led to the prompt resolution of his symptoms. There were no genetic components nor obvious anatomical deformities noted that led to these cases. CASE 2: Four years later, his 18-year old younger brother developed a similar presentation that had a high suspicion of PSS. He was an active swimmer who noticed left arm swelling, anterior shoulder pain, and a positive Adson’s test. Given his family history, a venous duplex ultrasound was ordered which demonstrated an acute thrombus involving the subclavian, axillary, and brachial veins. The patient underwent a left upper extremity venogram with catheter-directed thrombolysis using tPA and heparin. Thrombolysis therapy was discontinued after only 8 hours due to a significant fibrinogen drop below 100 mg/dL. Two days later, the patient underwent a first rib resection alongside an anterior scalenectomy and venous thrombectomy. These procedures lead to the resolution of his symptoms. Testing was performed for common clotting disorders in both cases which all returned negative. CASE PRESENTATIONS

CONCLUSIONS

1. Golan, E., Rayan, N., & Bogoch, E. (2017). Paget-Schroetter Syndrome: A Comprehensive Review of Diagnosis and Management. Journal of Vascular Surgery , 66(2), 591-601. 2. Jones, N. R., Fisher, C. R., & Lewis, J. (2019). Paget-Schroetter Syndrome: Current Perspectives and Management Strategies. Vascular Medicine , 24(6), 558-564. 3. Rooke, T. W., Majo, J. R., & Aird, W. C. (2018). Primary Effort Thrombosis (Paget Schroetter Syndrome): A Review of Current Management Guidelines. Clinical and Applied Thrombosis/Hemostasis , 24(5), 738-744. 4. Vijayan, V., Pappas, P., & Hirsch, A. T. (2020). Upper Extremity Deep Vein Thrombosis: A Review of Paget-Schroetter Syndrome and Related Disorders. Seminars in Thrombosis and Hemostasis , 46(7), 747-754. 5. Alder, A. C., et al. (2017). "Long-term outcomes after surgical treatment of Paget Schrotter syndrome." Journal of Vascular Surgery , 65(4), 1026-1032. 6. Kahn, S. R., et al. (2009). "Quality of life in patients with post-thrombotic syndrome: a review." Thrombosis and Haemostasis , 101(2), 372-378. 7. Torbicki, A., et al. (2014). "Guidelines on the diagnosis and management of acute pulmonary embolism." European Heart Journal , 35(45), 3155-3173. 8. Illig KA, Doyle AJ. A comprehensive review of Paget-Schroetter syndrome. J Vasc Surg. 2010 Jun;51(6):1538-47. doi: 10.1016/j.jvs.2009.12.022. Epub 2010 Mar 20. PMID: 20304578. ACKNOWLEDGEMENTS We would like to thank Floyd Atrium and Dr. Trent Prault MD for allowing us to write this report. We would also like to thank VCOM and Dr. Daniel Cawley DC for guidance. ● The primary risk factor for PSS is mechanical compression. 2 As is common in PSS, both brothers were highly active and participated in athletics that required repetitive overhead motions, basketball and swimming.. ● Rare features for PSS in these cases included the younger brother having a left arm incidence while being right hand dominant, and both brothers being younger than 20 years old. ● To our knowledge, these are the first cases of PSS in biological brothers not related to a genetic or coagulopathy disorder. ● These case report raises questions on the direct etiologies of PSS in these patients outside of a history of activity. Should family history be pertinent in cases of PSS as well as whether similar build and body composition could predispose to the effort thrombosis? ● This report could prove beneficial by encouraging physicians to maintain a high degree of suspicion for PSS in patients with a strong family history and strenuous activity. REFERENCES

Fig. 1 Basic anatomy of the thoracic outlet. (m. = muscle and v. = vein) Illig, Karl A., and Adam J. Doyle. “A comprehensive review of paget-schroetter syndrome.” Journal of Vascular Surgery , vol. 51, no. 6, June 2010, pp. 1538 – 1547, https://doi.org/10.1016/j.jvs.2009.12.022.

Fig. 2 X-ray anterior-posterior view of the chest in the younger sibling (Case 2) one day post-left first rib resection demonstrating removal of the left first rib and mild post surgical atelectasis in the left lung base.

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