
==== Front
Intern Med
Intern Med
Internal Medicine
0918-2918
1349-7235
The Japanese Society of Internal Medicine

38220192
10.2169/internalmedicine.2990-23
Case Report
Use of the Superficialized Brachial Artery as Vascular Access for a Patient with Myasthenia Gravis with a Frequent Need for Plasmapheresis
Kitano Fumiya 1
Marui Yuhji 2
Sakurai Kenzo 3
Shibagaki Yugo 1
Sakurada Tsutomu 1
Kojima Shigeki 1
1 Division of Nephrology and Hypertension, Department of Internal Medicine, St. Marianna University School of Medicine, Japan
2 Department of Urology, St. Marianna University School of Medicine, Japan
3 Division of Neurology, Department of Internal Medicine, St. Marianna University School of Medicine, Japan
Correspondence to Dr.　Shigeki Kojima, s2kojima@marianna-u.ac.jp

13 1 2024
15 8 2024
63 16 23072310
2 10 2023
19 11 2023
Copyright © 2024 by The Japanese Society of Internal Medicine
https://creativecommons.org/licenses/by-nc-nd/4.0/ The Internal Medicine is an Open Access journal distributed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License. To view the details of this license, please visit (https://creativecommons.org/licenses/by-nc-nd/4.0/).
A 41-year-old woman diagnosed with seronegative myasthenia gravis struggled to maintain remission for a decade, facing crises every 3 months for several years. After repeated apheresis using a non-tunneled non-cuffed central venous dialysis catheter (NTNCC), complications such as catheter-related thrombus in the internal jugular veins and morbid obesity from steroids made the insertion of NTNCC increasingly difficult, leading to consideration of an alternative permanent vascular access (VA) approach. Thus, we created a subcutaneously superficialized brachial artery as the VA, which allowed the patient to undergo safe and uninterrupted apheresis therapy.

blood purification therapy
myasthenia gravis
subcutaneously fixed superficial brachial artery
permanent vascular access
==== Body
pmcIntroduction

Apheresis therapy, including plasma exchange and immunoadsorption, is an important therapeutic modality, especially in the treatment of acute neuroimmune diseases. However, Apheresis requires vascular access (VA) to retrieve a sufficient amount of blood. The VA mainly used for apheresis in acute/intermittent diseases is a non-tunneled non-cuffed central venous dialysis catheter (NTNCC) and superficial veins, which are often associated with problems, such as venous thrombosis, catheter-related bloodstream infection, and poor blood flow (1-3).

We herein report a patient with myasthenia gravis (MG) who required frequent apheresis but in whom inserting an NTNCC was difficult, for which a subcutaneously superficialized brachial artery (SFSBA) was created as an alternative VA, leading to safe and uninterrupted apheresis without complications.

Case Report

A woman in her 40s who had been diagnosed with seronegative MG [negative anti-acetylcholine receptor antibody (anti-AChR antibody) and anti-muscle-specific thyroxinase antibody (anti-MuSK antibody)] and undergone extended total thymectomy had difficulty maintaining remission despite treatment with prednisolone, tacrolimus, pyridostigmine, and intravenous immunoglobulin. She underwent apheresis therapy for frequent recurrence of MG crises using an NTNCC. However, insertion of the NTNCC gradually became difficult due to thrombotic occlusion of the right internal jugular vein and morbid obesity caused by chronic corticosteroid therapy (Fig. 1).

Figure 1. Clinical course of non-tunneled non-cuffed central venous dialysis catheter (NTNCC) malfunction. In mid-October, X-1 year, an NTNCC was inserted through the right femoral vein. Insufficient blood flow occurred frequently because the tip was bent in the blood vessel (A). In early November, X-1 year, based on the progress of the previous right femoral approach, a left internal jugular approach was performed, but the NTNCC tip could only reach the left brachiocephalic vein, and insufficient blood flow occurred frequently (B). In early July, X year, a left internal jugular approach was performed, but NTNCC aberration into the right brachiocephalic vein occurred (C); therefore, femoral vein approaches were performed the next day under fluoroscopic guidance. Insertion of the guidewire revealed that the bilateral femoral veins ran in a loop (D), and insertion through the thigh was abandoned. Finally, under fluoroscopic guidance, the NTNCC was inserted from the left internal jugular vein, and the tip of the catheter was guided and implanted closer to the right atrium than ever before, but NTNCC malfunction frequently occurred (E, F).

She was hospitalized again for a recurrent MG crisis. On admission, her vital signs were stable, but she had a body mass index (BMI) of 39.7, and she had been on home oxygen therapy at 2 L/min but still felt dyspneic, so the volume was increased to 3 L/min, and oxygen saturation was maintained at 98%. Results of blood tests performed at admission, including blood cell counts and coagulation system, which are important for apheresis, were normal. The results of a physical examination on admission were unremarkable and manual muscle test scores for the upper and lower extremities were 4 (good) for both right and left extremities.

Ultrasound cardiography revealed a normal cardiac function. The patient was treated with tacrolimus 3 mg daily, pyridostigmine 180 mg daily, and prednisolone 17.5 mg daily. Because of the extreme difficulty in NTNCC insertion, superficialization of the brachial artery was considered as an alternative VA approach. The left brachial artery (BA) was chosen as the non-dominant limb; however, since the BA ran quite deep, and we expected prolonged wound healing, we designed the superficializing area to be shorter than usual. The BA was exposed to 12 cm in length and raised onto the fascia of the biceps muscle and superficial fascia. Approximately 6 cm of the BA with a diameter of 3 mm was then superficialized straight in shape in a pocket created in the superficial subcutaneous layer. Intraoperatively, the skin and subcutaneous tissue were very fragile and bruised easily, so wound healing took longer than usual, and an open wound was allowed until complete healing was achieved with the use of trafermin spray (Fig. 2A).

Figure 2. Wound site on the left upper arm in November X year (A). The left upper arm was debrided from the superficial artery and returned to the ulnar vein of the forearm for plasma exchange (B). Wound site in September X+2 year (C).

Two months post-surgery, the patient was readmitted because of the recurrence of the crisis, and simple plasma exchange using an SFSBA was initiated. Ultrasound-guided puncture was used to ensure puncture of the forearm basilic vein, which has thick subcutaneous fatty tissue, but there were no puncture difficulties, and apheresis was performed safely and without complications. (Fig. 2B). After two more years, the wound had healed completely (Fig. 2C), and apheresis was conducted repeatedly and successfully without complications or the need for insertion of an NTNCC.

Discussion

MG is an autoimmune disease that targets AChR and MuSK receptors at the neuromuscular junction (4). It sometimes causes muscle weakness, leading to rapid respiratory failure, called a myasthenic crisis. Less than 10% of MG patients attain complete remission after initiation of treatment (5,6), necessitating long-term steroids, immunosuppressants, and possibly frequent apheresis.

There have been several reports of frequent apheresis in MG (7) and only one case of SFSBA as far as we know (8), but the types of VA and troubleshooting have not yet been fully discussed. Sakuma et al. reported that over 34 apheresis procedures were performed at SFSBA over a 5-year period (8). A case of refractory MG needed apheresis twice a week for 2 weeks at regular intervals of approximately 40 days (9), but which type of VA was used is unclear. Regarding cases other than MG, long-term and frequent therapeutic apheresis (TA) for lipoprotein up to 291 apheresis cycles over 23 years with tunneled cuffed dialysis catheters (TCC), autologous veins, and intravascular shunts has been reported (10,11). Few cases in which apheresis was performed mentioned the type of access. We suggest that SFSBA can be stood long-term apheresis, with a reported 87% patency rate at 3 years and 58% patency rate at 5 years, even in maintenance hemodialysis (HD) patients who use SFSBA 3 times a week (12).

Insertion of a catheter for dialysis can negatively and directly affect a patient's daily life and introduce medical complications. If the blood flow is insufficient, the treatment duration can be extended. Therefore, the selection of a safe and reliable VA with relatively few problems is critical in cases of long-term frequent apheresis.

Four types of permanent VAs are used in blood purification therapy: an autologous arteriovenous fistula (AVF), arteriovenous graft (AVG), SFSBA, and TCC. Ideally, VA should provide adequate blood flow, ease of connection, minimal circulatory system strain, low complication risk, and no interference with daily activities. The blood flow rate required for plasma exchange therapy is approximately 100-200 mL/min (13,14), and that for plasma adsorption is approximately 120 mL/min. However, it is difficult to draw blood from a normal superficial vein. SFSBA is a technique developed in Japan in which the brachial artery, which is located under the fascia, is freed and moved subcutaneously. AVF is the first choice for permanent use in maintenance HD patients due to its functional advantages despite concerns about increased preload. However, for patients without a fixed TA schedule, invasive surgeries for VA that cause unnecessary cardiac burden should be avoided. TCCs are not a priority because of the risk of infection and thrombus formation, as well as the complexity of routine self-care. Therefore, we considered SFSBA, which does not affect circulation and does not require device implantation, to be the most appropriate permanent VA for patients requiring frequent apheresis.

While the risk of complications, such as aneurysm formation, may be low because the frequency of punctures to the VA for apheresis treatment is expected to be less than that for maintenance HD, the risk of bleeding after arterial puncture may be increased compared to HD because plasma exchange with albumin preparation decreases coagulation factors. In addition, due to the moderate doses of steroid usage in this case, we predicted the increased risk of skin necrosis and prolonged wound healing (15). There were several points of the SFSBA technique during the procedure. First, when forming the pocket for the superficial artery, we meticulously adjusted the thickness of the skin flap, making it thin enough to identify the superficial artery from the surface while still maintaining adequate blood flow to subcutaneous tissue of the skin flap. Second, as subcutaneous bleeding may cause delayed wound healing and infection, we sutured the subcutaneous tissue of the skin flap to the fascia to secure the superficial artery in place and eliminate dead space. Third, the wound was closed with mattress sutures using monofilament nylon thread, which has a low propensity for infection.

Although there is hope for new drugs, such as eculizumab and efgartigimod alfa, in MG, we anticipate that cases requiring apheresis will continue to be experienced, and awareness of VA options will be essential. In the present case, repeated shared decision-making by the patient, neurologist, and nephrologist allowed for patient-reported outcome-focused treatment selection. Close collaboration among various departments is also an important factor for the success of apheresis therapy.

The authors state that they have no Conflict of Interest (COI).

Acknowledgement

I would like to express my sincere gratitude to the people described here for their expertise and support in all aspects of the research and for their help in the preparation of the manuscript.
==== Refs
1. Rajasekhar A , Streiff MB . 30 - Use of vena cava filters and venous access devices. In: Consultative Hemostasis and Thrombosis. 4th Ed. Elsevier, Amsterdam, 2019: 594-635.
2. Santoro D , Benedetto F , Mondello P , et al . Vascular access for hemodialysis: current perspectives. Int J Nephrol Renovasc 7 : 281-294, 2014.
3. Okafor C , Kalantarinia K . Vascular access considerations for therapeutic apheresis procedures. Semin Dial 25 : 140-144, 2012.22176495
4. Motomura M . Recent treatment of myasthenia gravis. Nihon Naika Gakkai Zasshi (J Jpn Soc Inter Med) 104 : 1953-1958, 2015.
5. Murai H , Masuda M , Utsugisawa K , et al . Clinical features and treatment status of adult myasthenia gravis in Japan. Clin Exp Neuroimmunol 5 : 84-91, 2014.
6. Utsugisawa K , Suzuki S , Nagane Y , et al . Health-related quality-of-life and treatment targets in myasthenia gravis. Muscle Nerve 50 : 493-500, 2014.24536040
7. Tsugawa J , Tsuboi Y , Inoue S , et al . Long-term outcomes of intermittent immunoadsorption therapy for myasthenia gravis. Rinsho Zasshi Naika (Internal Medicine) 103 : 595-598, 2009.
8. Sakuma K , Utsumi N , Akiyama M , et al . A case of plasma exchange for myasthenia gravis refractory to vascular access. Jin To Toseki (Kidney and Dialysis) 65 （Supplemental Volume Access 2008 ）: 190-192, 2008.
9. Nakagawa J , Muramatsu T , Ohta K , et al . A case of refractory myasthenia gravis positive for anti MuSK antibody that was successfully treated with double filtration plasmapheresis. Nihon Toseki Igakkai Zasshi (J Jpn Soc Dial Ther) 40 : 531-535, 2007.
10. Ishikawa T , Siba M , Ikewaki K , et al . A case of long-term LDL apheresis therapy for familial hypercholesterolemia. Prog Med 30 : 2699-2707, 2010.
11. Gülle S , Bak M , Serdaroglu E , Can D , Karabay O . Low-density lipoprotein apheresis by membrane differential filtration (cascade filtration) via arteriovenous fistula performed in children with familial hypercholesterolemia. Ther Apher Dial 14 : 87-92, 2010.20438523
12. Yasunaga C , Nakamoto M , Fukuda K , Goya T . Superficial repositioning of the artery for chronic hemodialysis: indications and prognosis. Am J Kidney Dis 26 : 602-606, 1995.7573014
13. Ipe TS , Marques MB . Vascular access for therapeutic plasma exchange. Transfusion 58 (Suppl 1) : 580-589, 2018.29443413
14. Kalantari K . The choice of vascular access for therapeutic apheresis. J Clin Apher 27 : 153-159, 2012.22535654
15. Wicke C , Halliday B , Allen D , et al . Effects of steroids and retinoids on wound healing. Arch Surg 135 : 1265-1270, 2000.11074878
