Paget-Schroetter Syndrome in a Young Female

Paget-Schroetter syndrome or effort thrombosis is a relatively rare primary spontaneous thrombosis of upper extremity deep veins secondary to entrapment of axillary subclavian veins from an abnormality of the thoracic outlet. It is commonly seen in young adults who lift heavy weights or strenuous use of the upper extremities during athletic activities. Repetitive microtrauma to the subclavian vein secondary to narrow costoclavicular space and strenuous activities leads to intimal layer inflammation, hypertrophy, fibrosis, and coagulation cascade activation. Management of Paget-Schroetter syndrome differs from the venous thrombosis of the lower extremity as treatment includes anticoagulation, thrombolysis, and surgical decompression. Early recognition and timely management are required to prevent significant disability from post-thrombotic syndrome and long-term morbidity from recurrent thromboembolism and pulmonary embolism. Internists and emergency physicians should be aware of the disease’s presentation, treatment options, and early referral to vascular surgeons since prompt initiation of appropriate treatment will have better outcomes than delayed treatment. We discussed a case of a 31-year-old female who lifts heavyweight at work, presented with right arm swelling and pain for 2 weeks, and diagnosed with axillary subclavian vein thrombosis secondary to thoracic outlet obstruction. She received a high-dose heparin drip followed by catheter-directed thrombolysis and underwent surgical decompression of axillary subclavian vein via resection of the first rib, subclavius muscle resection, partial anterior scalenectomy, and venolysis. In our review of the literature, randomized controlled studies lack the efficacy and safety of surgical decompression. However, the results are promising based on accumulated experience from vascular surgery experts and small case series. Extensive studies are needed further to delineate the protocol for the management of Paget-Schroetter syndrome.

Download Full-text

The infraclavicular approach for Paget–Schroetter syndrome

Annals of The Royal College of Surgeons of England ◽

10.1308/rcsann.2017.0154 ◽

2018 ◽

Vol 100 (2) ◽

pp. 83-91 ◽

Cited By ~ 4

Author(s):

G Samoila ◽

CP Twine ◽

IM Williams

Keyword(s):

Subclavian Vein ◽

Thrombotic Event ◽

Young Male ◽

Symptom Duration ◽

Secondary Patency ◽

Supraclavicular Approach ◽

First Rib ◽

Extrinsic Compression ◽

Catheter Directed Thrombolysis ◽

Effort Thrombosis

Introduction Paget–Schroetter syndrome is a rare effort thrombosis of the axillary-subclavian vein, mainly occurring in young male patients. Current management involves immediate catheter directed thrombolysis, followed by surgical decompression of the subclavian vein. This has been invariably performed using a transaxillary or supraclavicular approach. However, the subclavian vein crosses the first rib anteriorly just behind the manubrium and can also be accessed via an infraclavicular incision. Methods MEDLINE® and Embase™ were searched for all studies on outcomes in patients undergoing infraclavicular first rib resection for treatment of Paget–Schroetter syndrome. Measured outcomes included freedom from reintervention, secondary patency and symptom resolution. Studies on neurogenic, arterial and iatrogenic venous thoracic outlet syndrome were not included. Findings Six studies (involving 268 patients) were eligible. The overall secondary venous patency rate was 98.5%. There was freedom from reintervention in 89.9% of cases and among those patients with reocclusion, 84.0% had chronic thrombosis (symptom duration >14 days), with 76.2% having a venous segment stenosis of >2cm. Only 3 of the 27 patients remained occluded despite reintervention. The infraclavicular approach provides excellent exposure to the subclavian vein and allows reconstruction when required. Moreover, this approach enables complete resection of the extrinsic compression that precipitated the initial thrombotic event, with excellent long-term patency rates. In conclusion, the infraclavicular route may have significant advantages compared with the transaxillary or supraclavicular approaches for successful and durable treatment of Paget–Schroetter syndrome.

Download Full-text

Bilateral Upper Extremity DVT in a 43-Year-Old Man: Is It Thoracic Outlet Syndrome?!

Case Reports in Medicine ◽

10.1155/2014/758010 ◽

2014 ◽

Vol 2014 ◽

pp. 1-3 ◽

Cited By ~ 1

Author(s):

Hadoun Jabri ◽

Sarbajit Mukherjee ◽

Devang Sanghavi ◽

Shyam Chalise

Keyword(s):

Upper Extremity ◽

Subclavian Vein ◽

Thoracic Outlet Syndrome ◽

Rare Condition ◽

Surgical Decompression ◽

Upper Extremities ◽

Vein Thrombosis ◽

First Case ◽

Arm Swelling ◽

Deep Vein

Recurrent deep venous thrombosis, involving bilateral upper extremities, is an extremely rare phenomenon. We are presenting a 43-year-old man who was diagnosed with left upper extremity deep vein thrombosis (UEDVT) and was treated with anticoagulation and surgical decompression in 2004. 9 years later, he presented with right arm swelling and was diagnosed with right UEDVT using US venous Doppler. Venogram showed compression of the subclavian vein by the first rib, diagnosing thoracic outlet syndrome (TOS). He was treated with anticoagulation and local venolysis and later by surgical decompression of the subclavian vein. Bilateral UEDVT, as mentioned above, is an extremely rare condition that is uncommonly caused by TOS. To our knowledge, we are reporting the first case of bilateral UEDVT due to TOS. Diagnosis usually starts with US venous Doppler to detect the thrombosis, followed by the gold standard venogram to locate the area of obstruction and lyse the thrombus if needed. The ultimate treatment for TOS remains surgical decompression of the vascular bundle at the thoracic outlet.

Download Full-text

Recurrent Upper Extremity Thrombosis Associated with Overactivity: A Case of Delayed Diagnosis of Paget-Schroetter Syndrome

Case Reports in Vascular Medicine ◽

10.1155/2017/8764903 ◽

2017 ◽

Vol 2017 ◽

pp. 1-4

Author(s):

Himani Sharma ◽

Abhinav Tiwari

Keyword(s):

Upper Extremity ◽

Subclavian Vein ◽

Delayed Diagnosis ◽

Surgical Decompression ◽

Upper Extremities ◽

Coagulation Cascade ◽

First Episode ◽

Occupational History ◽

External Compression

Paget-Schroetter syndrome is thrombosis of the axillary-subclavian vein that is associated with strenuous and repetitive activity of the upper extremities. Overuse of the arm coupled with external compression results in microtrauma in the intima of the subclavian vein, resulting in the activation of the coagulation cascade. Diagnosis is usually made by Doppler ultrasound and the treatment involves thrombolysis, while routine surgical decompression of the thoracic outlet is controversial. In this report, we present a case of a patient who presented with a second episode of spontaneous right upper extremity deep venous thrombosis. The first episode was inadequately treated with oral anticoagulation alone. During the second episode, Paget-Schroetter syndrome was diagnosed, after careful review of his occupational history. He subsequently underwent angioplasty and decompression of thoracic outlet with no recurrence of thrombosis in a 12-month follow-up period.

Download Full-text

Endovascular Management of Paget-Schroetter Syndrome

Vascular ◽

10.2310/6670.2007.00064 ◽

2007 ◽

Vol 15 (5) ◽

pp. 290-296 ◽

Cited By ~ 17

Author(s):

Gregory J. Landry ◽

Timothy K. Liem

Keyword(s):

Cross Sectional ◽

Common Component ◽

Rib Resection ◽

First Rib ◽

Catheter Directed Thrombolysis ◽

Effort Thrombosis ◽

Arm Swelling ◽

Arm Motion ◽

Minimal Treatment ◽

First Rib Resection

Spontaneous thrombosis of the axillary and subclavian venous segments in young, healthy adults (effort thrombosis or Paget-Schroetter syndrome) is a rare but potentially disabling affliction. The diagnosis should be suspected in any young patient presenting with unilateral arm swelling. Typically, the dominant arm is affected, and frequent, repetitive arm use is a common component of the patients' history. Although the diagnosis can often be confirmed with a venous duplex evaluation, the central location of the venous abnormality occasionally mandates cross-sectional imaging or contrast venography to confirm the diagnosis. The underlying pathophysiology of this disorder is felt to be repetitive venous trauma owing to arm motion in the narrow anatomic space between the clavicle and first rib. The treatment of Paget-Schroetter syndrome is controversial and varies according to individual, institutional, and regional preferences. In general, the trend is toward more aggressive endovascular treatment. Prompt anticoagulation is generally accepted as the minimal treatment offered. Catheter-directed thrombolysis has also acquired a prominent role in reestablishing venous patency. The importance of relieving the anatomic compression of the subclavian vein by first rib resection remains controversial, with some experts advocating surgical intervention in all affected patients, whereas others perform this procedure selectively in cases of persistent venous stenosis or ongoing symptoms. Angioplasty with or without stenting is generally discouraged in the absence of anatomic decompression but may play an adjunctive role in patients undergoing first rib resection.

Download Full-text

Melatonin as adjuvant treatment for coronavirus disease 2019 pneumonia patients requiring hospitalization (MAC-19 PRO): a case series

Melatonin Research ◽

10.32794/mr11250063 ◽

2020 ◽

Vol 3 (3) ◽

pp. 297-310 ◽

Cited By ~ 8

Author(s):

Rafael Ricafranca Castillo ◽

Gino Rei A. Quizon ◽

Mario Joselito M. Juco ◽

Arthur Dessi E. Roman ◽

Donnah G De Leon ◽

...

Keyword(s):

Adjuvant Therapy ◽

Hospital Stay ◽

Clinical Improvement ◽

Case Series ◽

High Dose ◽

Chest Imaging ◽

Imaging Findings ◽

Rt Pcr ◽

Average Hospital ◽

Sleeping Problems

Treatment for coronavirus disease 2019 (COVID19) pneumonia remains empirical and the search for therapies that can improve outcomes continues. Melatonin has been shown to have anti-inflammatory, antioxidant, and immune-modulating effects that may address key pathophysiologic mechanisms in the development and progression of acute respiratory distress syndrome (ARDS), which has been implicated as the likely cause of death in COVID19. We aimed to describe the observable clinical outcomes and tolerability of high-dose melatonin (hdM) given as adjuvant therapy in patients admitted with COVID19 pneumonia. We conducted a retrospective descriptive case series of patients who: 1) were admitted to the Manila Doctors Hospital in Manila, Philippines, between March 5, 2020 and April 4, 2020; 2) presented with history of typical symptoms (fever, cough, sore throat, loss of smell and/or taste, myalgia, fatigue); 3) had admitting impression of atypical pneumonia; 4) had history and chest imaging findings highly suggestive of COVID19 pneumonia, and, 5) were given hdM as adjuvant therapy, in addition to standard and/or empirical therapy. One patient admitted to another hospital, who one of the authors helped co-manage, was included. He was the lone patient given hdM in that hospital during the treatment period. Main outcomes described were: time to clinical improvement, duration of hospital stay from hdM initiation, need for mechanical ventilation (MV) prior to cardiopulmonary resuscitation, and final outcome (death or recovery/discharge). Of 10 patients given hdM at doses of 36-72mg/day per os (p.o.) in 4 divided doses as adjuvant therapy, 7 were confirmed COVID19 positive (+) by reverse transcription polymerase chain reaction (RT-PCR) and 3 tested negative (-), which was deemed to be false (-) considering the patients’ typical history, symptomatology, chest imaging findings and elevated bio-inflammatory parameters. In all 10 patients given hdM, clinical stabilization and/or improvement was noted within 4-5 days after initiation of hdM. All hdM patients, including 3 with moderately severe ARDS and 1 with mild ARDS, survived; none required MV. The 7 COVID19(+) patients were discharged at an average of 8.6 days after initiation of hdM. The 3 highly probable COVID19 patients on hdM were discharged at an average of 7.3 days after hdM initiation. Average hospital stay of those not given hdM (non-hdM) COVID19(+) patients who were admitted during the same period and recovered was 13 days. To provide perspective, although the groups are not comparable, 12 of the 34 (35.3%) COVID19(+) non-hdM patients admitted during the same period died, 7/34 (20.6%) required MV; while 6 of 15 (40%) non-hdM (-) by RT-PCR but highly probable COVID19 pneumonia patients also died, 4/15 (26.7%) required MV. No significant side-effects were noted with hdM except for sleepiness, which was deemed favorable by all patients, most of whom had anxiety- and symptom-related sleeping problems previously. HdM may have a beneficial role in patients treated for COVID19 pneumonia, in terms of shorter time to clinical improvement, less need for MV, shorter hospital stay, and possibly lower mortality. HdM was well tolerated. This is the first report describing the benefits of hdM in patients being treated for COVID19 pneumonia. Being a commonly available and inexpensive sleep-aid supplement worldwide, melatonin may play a role as adjuvant therapy in the global war against COVID19.

Download Full-text

Potential Benefits From Heating the High-Dose rtPA Boluses Used in Catheter-Directed Thrombolysis for Acute/Subacute Lower Limb Ischemia

Journal of Endovascular Therapy ◽

10.1583/1545-1550(2003)010<0739:pbfhth>2.0.co;2 ◽

2003 ◽

Vol 10 (4) ◽

pp. 739-744 ◽

Cited By ~ 1

Author(s):

Dimitrios K. Tsetis ◽

Asterios N. Katsamouris ◽

Athanasios D. Giannoukas ◽

Adam A. Hatzidakis ◽

Theodoros Kostas ◽

...

Keyword(s):

Lower Limb ◽

Limb Ischemia ◽

High Dose ◽

Lower Limb Ischemia ◽

Catheter Directed Thrombolysis ◽

Potential Benefits

Download Full-text

Bilateral dysthyroid compressive optic neuropathy responsive to teprotumumab

European Journal of Ophthalmology ◽

10.1177/1120672121991042 ◽

2021 ◽

pp. 112067212199104

Author(s):

Catherine J Hwang ◽

Erin E Nichols ◽

Brian H Chon ◽

Julian D Perry

Keyword(s):

Side Effects ◽

Optic Neuropathy ◽

Surgical Decompression ◽

Thyroid Eye Disease ◽

Eye Disease ◽

High Dose ◽

Compressive Optic Neuropathy ◽

Traditional Management ◽

Immune Mediated ◽

Orbital Radiation

Thyroid eye disease is an auto-immune mediated orbitopathy which can cause dysthyroid compressive optic neuropathy. Traditional management of active thyroid eye disease includes temporizing high-dose steroids, orbital radiation and surgical decompression, which each possess significant limitations and/or side effects. Teprotumumab is an IGF-IR inhibitor recently FDA-approved for active thyroid eye disease. The authors report reversal of bilateral dysthyroid compressive optic neuropathy managed medically utilizing teprotumumab.

Download Full-text

ETMR-08. INTERNATIONAL CONSENSUS PROTOCOL FOR EMBRYONAL TUMOR WITH MULTILAYER ROSETTES

Neuro-Oncology ◽

10.1093/neuonc/noaa222.212 ◽

2020 ◽

Vol 22 (Supplement_3) ◽

pp. iii324-iii324

Author(s):

Derek Hanson ◽

Nicolas Andre ◽

Susan Chi ◽

Mariella Filbin ◽

Michael Fisher ◽

...

Keyword(s):

Induction Chemotherapy ◽

Clinical Investigation ◽

Case Series ◽

High Dose ◽

Consensus Protocol ◽

Retrospective Case ◽

International Consensus ◽

Small Series ◽

Dismal Prognosis ◽

Biological Studies

Abstract Embryonal tumors with multilayer rosettes (ETMR) are rare and highly-aggressive central nervous system (CNS) neoplasms which occur primarily in young children and carry a dismal prognosis. To date, no large clinical investigations have been conducted to determine the optimal therapy for ETMR. Data from retrospective case series suggest that our most aggressive standard therapies are not sufficient for cure in the majority of cases. New treatment approaches incorporating pre-clinical data and the known biology of ETMR are therefore urgently needed. A German drug screen using the patient-derived ETMR BT183 cell line and its xenograft revealed anti-tumor activity of topotecan, doxorubicin, and actinomycin D; three agents used infrequently for treating infant CNS tumors. Additional results from a small series of ETMR patients suggest that optimization of induction chemotherapy using these active agents may improve response and survival outcomes. In 2019, an international panel of pediatric neuro-oncology experts convened to advance therapy for ETMR. A consensus protocol was developed incorporating maximal safe surgical resection, induction chemotherapy with active pre-clinical agents, intrathecal chemotherapy, radiotherapy, and high-dose chemotherapy. This international consensus protocol represents the first prospective clinical investigation specific to ETMR and will be available through a treatment registry globally and as a clinical trial at select centers. The study aims to improve survival by providing aggressive, optimized therapy for ETMR and will serve as a platform to explore new biologically-promising agents. The investigation will also provide valuable prospective outcome data and correlative biological studies to serve as baseline comparators for future clinical trials.

Download Full-text