Endovascular Treatment of Paget-Schroetter Disease

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The paper describes a case of endovascular treatment of a patient with Paget Schroetter syndrome (PSS), who had verified thrombosis of the brachial, axillary and subclavian veins. The main etiological factor of venous thrombosis was hyperabduction syndrome – compression of the subclavian vein by the pectoralis minor muscle during arm movement. The indication for endovascular treatment was acute venous insufficiency of the upper limb with the developing threat of phlegmasia cerulea dolens. Regional catheter thrombolysis was performed using alteplase. There was a lysis of thrombotic masses with beneficial long-term clinical outcomes of the patient's treatment.

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THE CASE OF ENDOVASCULAR TREATMENT OF PAGET-SCHRETTER DISEASE (CLINICAL OBSERVATION) B.S. Sukovaty1, N.V. Bolomatov2, M.Y. Gordov2, I.V. Larina2. 1FGBOU HPE "Kursk State Medical University" K. Marx str., 3 Kursk, 305041, Russian Federation 2OBUZ "Kursk City Clinical Hospital of Emergency Medical Care", ul. Pirogova, 14, Kursk, 305035, Russian Federation ENDOVASCULAR TREATMENT OF PAGET-SCHRETTER DISEASE (CLINICAL OBSERVATION)B.S. Sukovatykh1 , N.V. Bolomatov2, M.Y. Gordov2, I.V. Larina2. 1 Kursk State Medical University, K. Marx str., 3 Kursk, 305041, Russian Federation1Kursk City Clinical Hospital of Emergency Medical Care, Pirogova str., 14, Kursk, 305035, Russian Federation Currently, in the context of a pandemic coronavirus infection, there has been a sharp increase in venous thromboembolic complications worldwide. In Russia, the number of patients among the elderly and senile with venous thrombosis has reached 300 per 100,000 thousand of the population. In the vast majority of cases (92-94%), thrombosis develops in the inferior vena cava system due to its large extent and high hydrostatic and hydrodynamic pressure in it. Thrombosis of the veins of the superior vena cava basin occurs only in 5-10% of patients. They are mainly of a secondary nature and are caused by conducting electrodes through the superior vena cava system into the heart cavity, catheters for infusion therapy through the subclavian vein. The superior vena cava may be compressed by a tumor or lymph nodes. Usually, in these cases, acute venous insufficiency develops to a mild or moderate degree, which requires anticoagulant therapy [1].Primary thrombosis of the subclavian vein (Paget –Schretter's disease) can occur due to injury of the vein during physical exertion with the stair muscles (scalenus syndrome), between the first rib or clavicle (rib–clavicle syndrome), the pectoralis minor muscle with a sharp withdrawal of the arm (hyperabduction syndrome). Trauma of the subclavian vein wall leads to the formation of an occlusive thrombus, followed by the continuation of the thrombotic process on the axillary and brachial veins with the development of severe acute venous insufficiency [ 2].The frequency of primary subclavian vein thrombosis varies from 2 to 5 people per 100,000 thousand of the population per year, which indicates a rare pathology. However, this disease in the absolute majority of cases occurs in people of working age, mainly in men, which determines its social nature.Primary thrombosis occurs when the muscles of the shoulder girdle and neck are overstressed during intense physical exertion. More often it develops in athletes or in people engaged in heavy physical labor. A certain role is played by innate anatomical features of a person that limit the mobility of the subclavian vein: additional cervical ribs, a high location of the first rib with a narrow space between the clavicle and the first rib, hypertrophy of the tendons of the stair muscles.In the presence of anatomical features during physical exertion, chronic traumatization of the vein wall occurs with the development of aseptic inflammation. The vein becomes inactive due to the development of scar connective tissue in the paravasal fiber. At the moment of excessive physical exertion, the endothelium of the inner lining of the subclavian vein is damaged and the process of thrombosis is triggered [ 3].For the syndrome Paget -Schretter is characterized by rapid development of the disease. After physical exertion, usually in the right, dominant hand a person has a feeling of heaviness in the limb. Pain and edematous syndromes develop within the next few hours. If during the first day the pains are localized in the subclavian region and have a bursting character, then later they spread to the entire limb and become discontinuous. Active and passive movements in the limb increase the pain syndrome.Edema of the limb in the first hours of the disease is moderate, but with a pronounced tendency to progression. Within 24-48 hours, the circumference of the affected limb on the shoulder and forearm increases sharply and exceeds the contralateral limb by several centimeters. Dense edema is characteristic, indicating the accumulation of fluid in the soft tissues of the affected limb. The color of the skin becomes cyanotic [ 4].In the absence of adequate conservative treatment, the thrombotic process can spread to the microcirculatory bed of the affected limb. Massive impregnation of the liquid part of the blood of soft tissues causes compression of arterioles and small peripheral arteries with the development of secondary arterial ischemia. The amplitude of pulsation of peripheral arteries decreases, bubbles filled with serous hemorrhagic fluid appear, and then areas of soft tissue necrosis on the cyst and the lower third of the forearm. Venous gangrene develops [5].The diagnostic program of the disease consists of laboratory, ultrasound, and radiological methods. When examining the coagulogram, hypercoagulation is detected: a decrease in APTT, INR, an increase in the prothrombed index, thrombosed time, fibrinogen. Ultrasound duplex scanning of subclavian, axillary and brachial veins occupies a leading place in the non-invasive diagnosis of Paget-Schretter syndrome. Scanning allows you to determine the location of thrombotic masses, places of narrowing of veins, blood outflow routes, flotation of the tip of the thrombus. Ultrasound imaging can be difficult when the clavicle overlaps the ultrasound window and the vein is inaccessible for examination. In these cases, they resort to computed tomography with contrast of the superior vena cava system. In cases of pronounced venous insufficiency with the transition of the process to the microcirculatory bed and flotation of the tip of the thrombus with the threat of pulmonary embolism, traditional upper limb phlebography is resorted to. Usually, they begin with distal ascending phlebography by injecting a contrast agent into the subcutaneous veins of the forearm with a previously proximally applied venous tourniquet. This study provides accurate information about the localization of deep vein thrombosis. When flotation of the top of the thrombus for its detailed characterization, retrograde phlebography is resorted to through venous femoral access [ 6].The subject of discussion is the choice of the method of treatment of the syndrome Paget-Schretter. In Russia, in most cases, traditional conservative treatment is carried out according to Russian clinical guidelines for the diagnosis, treatment and prevention of venous thromboembolic complications [7]. Unfortunately, the effectiveness of conservative therapy leaves much to be desired. In most patients, either occlusion of a thrombosed vein occurs, or its partial recanalization with the development of chronic venous insufficiency of the affected limb. Considering that the majority of patients, persons of physical labor, often there is a need to change working conditions or transition patients to disability [ 8]. At the same time, back in 2012, the American Society of Vascular Surgeons recommended regional catheter thrombolysis as the main method of treatment of deep vein thrombosis, both in the basin of the lower and in the basin of the upper vena cava. [9]. The European Society adheres to the same principles of treatment in its clinical recommendations [10]. The essence of the method consists in the introduction of a fibrinolytic drug through a catheter into the thickness of a venous thrombus. Regional catheter thrombolysis has found wide application abroad, and there are isolated reports of its use in Russia [11]. In the department of X-ray surgical methods of diagnosis and treatment, endovascular treatment of a patient with the syndrome was performed Paget-Schretter. Description of the syndrome treatment case Paget-Schretter.Patient K. 46 years old, medical history No. 2982 was admitted to the department of vascular surgery of the OBUZ KGKB SMP. Kursk 25.06.2022 with complaints of pain and swelling of the right upper limb. A day ago, after physical exertion (I was weeding the garden with a hoe for several hours), I began to notice a pulling pain in my right arm. By the evening of the same day, moderate swelling of the right hand and forearm appeared. The next day, the pain syndrome intensified, the swelling spread to the armpit. During the day, the patient was self-medicating: she took painkillers, rubbed her hand with troxevazine ointment. The treatment did not bring relief, the intensity of pain and edematous syndromes increased, the skin acquired a cyanotic color. The patient independently applied to the emergency department of the KGKB SMP in Kursk.Upon admission, the patient's condition is closer to satisfactory, the body temperature is normal. Pulse 72ud./ min., blood pressure 125/80 mm Hg. From the cardiovascular, respiratory, digestive, urinary and nervous systems without pathological disorders. The right upper limb is enlarged in volume due to edema, the circumference of the right forearm is 1.5 cm, and the shoulder is 3 cm larger than the left. The subcutaneous venous pattern is enhanced, the skin of the right hand is cyanotic. Pulsation on the arteries of the right hand is distinct at all levels. There is no pathology in clinical and biochemical blood and urine tests. Coagulogram parameters: ACTV- 27c, INR-0.98, PTI – 98%, fibrinogen – 2.9 g / l, TV- 22.1 s. During chest X-ray of additional cervical ribs, narrowing of the costal-subclavian space was not revealed. Ultrasound angioscanning of the venous system of the superior vena cava revealed thrombosis of the right brachial, axillary and subclavian veins to the junction with the brachiocephalic vein.

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About the authors

Boris Semyonovich Sukovatykh

Kursk state medical University

Email: SukovatykhBS@kursksmu.net
ORCID iD: 0000-0003-2197-8756

Ph.D., Professor, head of chair of General surgery 

Russian Federation, K. Marx str., 3 Kursk, 305041, Russian Federation

Nikolay Vladimirovich Bolomatov

Kursk City Clinical Hospital of Emergency Medical Care

Email: n-v-bolomatov@yandex.ru
ORCID iD: 0000-0003-0590-2225

Head of the Department of X-ray surgical methods of Diagnosis and Treatment

Russian Federation, Pirogova str., 14, Kursk, 305035, Russian Federation

Maxim Yurievich Gordov

Kursk City Clinical Hospital of Emergency Medical Care

Email: gordov@mail.ru
ORCID iD: 0000-0001-9618-1923

Head of the Vascular Surgery Department 

Russian Federation, Pirogova str., 14, Kursk, 305035, Russian Federation

nna Valeryevna Larina

Kursk City Clinical Hospital of Emergency Medical Care

Author for correspondence.
Email: larina.inna.30@yandex.ru
ORCID iD: 0009-0001-1232-0824

vascular department surgeon

Russian Federation, Pirogova str., 14, Kursk, 305035, Russian Federation

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