Abstract
A vascular access with good function for drug delivery is the basis of chemotherapy. If there is any congenital or acquired vascular abnormality, procedurally related and late complications such as vessel rupture, malposition, and dysfunction of the catheter with ensuing thrombosis may occur, especially when it is undiagnosed or ignored. We describe a case of implantable central venous catheter (CVC) malposition and subsequent insertion of a Hickman catheter for stem cell transplantation after the diagnosis of persistent left superior vena cava (PLSVC) by radiologic image studies. The case is about a 60-year-old male who suffered from mantle cell lymphoma. He complained of discomfort when chemotherapeutic drugs were delivered through an implanted subcutaneous port system. Malposition of the CVC with aberrant path venous catheter, which led to its migration to the right internal jugular vein (RIJV) was noted on the chest X-ray. In addition, results of ultrasound imaging revealed total occlusion of the RIJV, and a subsequent three-dimensional (3D) computed tomography (CT) reconstruction image revealed a PLSVC with an atretic right SVC. Ultrasound-guided venous puncture of the left internal jugular vein and intraoperative fluoroscopy for confirming the correct guide-wire path were used for successful insertion of Hickman catheter without any complication. When unexpected occurrence of migration or malposition of the long-term CVC is detected, early removal of the catheter is vital for preventing further complications. Proper and advanced image studies including ultrasound, contrast-enhanced venography, CT, and magnetic resonance imaging may be necessary for understanding the potential vascular abnormality and guiding the following treatment.
Keywords
catheterization; central venous; Hickman catheter; left superior vena cava; ultrasonography;
1. Introduction
Central venous catheters (CVCs) are frequently used for delivering drugs in chemotherapy and transplantation in oncology. The implanted venous-access devices such as subcutaneous port infusion system and Hickman catheter are alternatives to CVCs for patients requiring frequent use of intravenous-access devices.
In this report, we present a case wherein we were consulted for managing a malposition of a malfunctioning catheter and revision of long-term CVC. After the delicate preoperative assessment, history taking, and proper image examination, the double superior vena cava (SVC) [an atretic right SVC with a persistent left SVC (PLSVC), which drains through the coronary sinus (CS) into the right atrium (RA)] was diagnosed. Ultrasound-guided venipuncture of the left internal jugular vein (LIJV) was performed to implant a Hickman catheter under fluoroscopy successfully.
2. Case report
A 60-year-old man (height: 170 cm; weight: 72 kg) with a history of mantle cell lymphoma stage IV came to our hospital with complaints of intractable discomfort and dysfunctional subcutaneous implantable port.
Initially, he presented with a rubbery lymphadenopathy that was fixed to the left supraclavicular area. He also lost 4 kg in 1 month. Results of a chest and abdominal computed tomography (CT) revealed multiple enlarged lymph nodes in the left supraclavicular fossa, mediastinum, paraaortic area, and bilateral inguinal region. A biopsy of the right inguinal lymph nodes revealed diffuse-type lymphoma (mixed and diffuse small cleaved B cell). A subcutaneous implantable port infusion system was set up, through which he started to receive chemotherapy at a regional hospital with a regimen of rituximab, cyclophosphamide, hydroxydaunorubicin, oncovin, and prednisolone at first, which was then followed by the regimen of rituximab, etoposide, prednisolone, oncovin, cyclophosphamide, and hydroxydaunorubicin. However, complete remission was not achieved after a total of 16 courses of chemotherapy. He was referred to our institute for further treatment.
Subsequent bone marrow biopsy results confirmed the diagnosis of mantle cell lymphoma, and abdominal and neck CT images revealed multiple lymphadenopathies. The bendamustine trial was suggested, of which he received six courses through the established infusion system. In order to evaluate further allogeneic peripheral blood stem cell transplantation (allo-PBSCT), he was admitted to the oncology ward.
During hospitalization, infusion through the port system catheter was not fluent. We were consulted for placement of a Hickman catheter for substitution. There was no remarkable discomfort except for the right temporomandibular joint pain. The follow-up chest radiograph (chest X-ray or CXR) revealed malposition of the catheter tip, which entered the right brachiocephalic vein first, and then ran an unusual retrograde course into the right internal jugular vein (RIJV) (Fig. 1). During the preprocedure visit, the patient complained of pain, heat, flush, and swelling over the right neck region in each course of chemotherapy. In addition, ultrasound examination of the neck was performed, which revealed total occlusion of the occlusive RIJV, but patency of the LIJV (Fig. 2). The three-dimensional (3D) reconstruction images illustrated a PLSVC that drained through the CS into the RA and an atretic right SVC (RSVC) with connection to the azygos vein (Figs. 3 and 4).
Download full-size image
Download full-size image
Download full-size image
Download full-size image
After detailed impartation to the patient about the possible complications due to an anatomic alteration variation, we performed the revision of the subcutaneous implantable port in the anesthesia induction room. We used a single-lumen 9.6 French Hickman catheter (Bard Access Systems, Salt Lake City, UT, USA) for the implantation, during which the patient was given oxygen and continuously monitored with a pulse oximeter, blood pressure monitor, and electrocardiography. Prior to the procedure, the patient was placed in the Trendelenburg position with the neck in extension and turning right. The neck and chest were strictly sterilely prepared. The ultrasound probe was also prepared in the same manner. The patient was then sedated with intravenous alfentanil (500 μg) and midazolam (2 mg), so that we could continue the procedure.
We performed the LIJV venipuncture at the site 1.5 cm above the left clavicle with an 18-gauge hollow needle under ultrasound guidance. Once the vein was targeted, the guide wire was inserted and the trocar needle was removed. With real-time intraoperative fluoroscopic guidance, the position of the guide-wire tip was confirmed to anchor in the PLSVC (Fig. 5). A transverse incision of 0.5 cm in length was made and dissected inward at the skin puncture site. The exit site was created by the left deltopectoral groove approach with a 0.5-cm transverse skin incision. The rest of the procedure, such as creation of subcutaneous tunnel and insertion of the catheter using a peel-apart sheath, was performed by percutaneous implantation. The fluoroscopy was also used to confirm the position of the catheter tip. After assuring the free aspiration of blood from the device and successful flushing of heparinized saline (1000 U/10 mL), the incision wounds were sutured. The nonfunctional previous subcutaneous port was removed. The patient was then sent to the radiology department for postoperative CXR to confirm the final catheter position and the course of the subcutaneous tunnel.
Download full-size image
There were no procedure-related complications, such as pneumothorax or hematoma. Results of the postoperative CXR revealed that the catheter tip was in the PLSVC with smooth subcutaneous tunnel curve (Fig. 5). The patient received allo-PBSCT uneventfully. The transplantated Hickman catheter was removed prior to discharge.
3. Discussion
Dudrick1 suggested the use of CVCs in clinical practice, and thus externalization and tunneling of the CVCs equipped with Dacron cuffs and subcutaneously implanting the infusion ports became an important part of management for chronic disease.1, 2, 3 Currently, cuffed catheters and implanted ports are popular for durable central venous access, especially for many oncology patients requiring long-term central venous access for administering drugs in chemotherapy and transplantation. There are upon-the-spot procedural and late complications related to long-term central venous catheterization, such as pneumothorax, hemothorax, arterial puncture, hematoma, infection, fibrin sheath formation, venous thrombosis, and catheter thrombosis.4, 5 Furthermore, the complication rate is even higher in catheter migration or malposition.
In this case report, we describe a late complication as a result of the malposition of the implantable port catheter. By careful assessment, the PLSVC with an atretic RSVC was diagnosed, and an unusual cause that led to the malposition of the implantable port catheter was discovered. Therefore, we chose the LIJV and adjusted the methods in the placement of a Hickman catheter after preoperative detailed evaluation.
Most frequently, the cephalic vein cut-down technique is used for subcutaneous implantable port insertion by surgeons in Taiwan.6 Intraoperative fluoroscopy is required to confirm the catheter tip in the proper position without malposition or kinking. In cases with the right cephalic vein for insertion of the implantable port, the catheter passes through the right subclavian vein (RSCV) and the right brachiocephalic vein into the SVC. However, malposition of catheter might occur in all insertion techniques. In our practice, we saw several cases of malposition of CVC, which was inserted through the cephalic vein and RSCV. All malpositioned catheters passed through the RSCV and entered the RIJV retrogradely. In the other words, the catheters turned cephalad into the RIJV at the junction of RSCV and RIJV.
In this case, the path of the catheter was abnormal on the CXR image. The catheter passed downstream into the right brachiocephalic vein then turned aberrantly upward to the RIJV, and the catheter tip was positioned within the low RIJV. This unusual radiologic finding aroused our suspicion that there might be anatomic anomalies in this case. By further image studies, the PLSVC was diagnosed. We advocate that if there is any unusual or suspicious finding on initial image study, then detailed survey is crucial. Venography, contrast-enhanced CT, and magnetic resonance imaging (MRI) are useful in diagnosing and confirmation vascular anomalies.7, 8 We chose contrast-enhanced CT combined with 3D reconstruction in this case for rapid diagnosis.
PLSVC is a rare anatomic variation that occurs in 0.3–0.5% of the general population and 4% of patients with congenital heart disease.9, 10, 11 It results from the persistence of the left anterior cardinal vein, which normally obliterates in the 8th week of fetal life. Approximately 50–70% of these patients are at risk of paradoxical embolism because of accompanying lesions (atrial septal defect, unroofed CS, or direct communication of the vein to the left atrium).12, 13, 14, 15, 16
According to the position of a CVC on the CXR, Schummer et al12 suggested a classification system: type I, normal anatomy; type II, only PLSVC; type IIIa, right and left SVC with connection; and type IIIb, right and left SVC without connection. The most common PLSVC variation presented in 82.2% reports was the presence of left- and right-sided SVC. In most cases, the PLSVC drains into the RA through the orifice of an enlarged CS, rendering the patient asymptomatic. However, in 8% of cases, the absence of the CS with drainage directly into the left atrium can cause right to left shunting, leading to cyanosis, systemic embolism, or thrombus, and in catheterization some complications may be induced by guide-wire advance, such as arrhythmia, hypotension, angina, and even cardiac arrest.17 Our case was found to have a variant type IIIb SVC, which was confirmed through 3D-reconstructed CT that showed a PLSVC draining into the RA through the dilated CS in the presence of an atretic right-side SVC. However, some may question about how the venous blood of his right head and right upper extremity drained into the RA in this condition. Although the RSVC was atresic below the turning point of the inserted catheter, it had connection with the azygos vein above the atresia as shown in Fig. 3. In general, the azygos vein has connection to the inferior vena cava (IVC) and the hemiazygos vein drains into the left innominate vein. For this reason, the venous blood of the right head and right upper extremity in this case could drain into the RA through the IVC or PLSVC.
The presence of a PLSVC should be also considered when the central venous catheterization is difficult, as technical difficulties may result in serious complications during catheter placement. Perforation of the right brachiocephalic (innominate) vein in the absence of a right SVC might be caused during catheter advancement. Malposition of the catheter and subsequent late complications are also troublesome. In this case, the catheter tip in the RIJV resulted in the occlusion of the RIJV. The possible mechanisms are the flow direction from catheter being opposite to the RIJV and the direct effects of the chemotherapy agents.
We advocate that the catheter with migration into or malposition in the IJV should not be used. In this case, the surgeon should check the catheter position immediately after the insertion of the port system. Revision should be done without delay to prevent any early complication. By contrast, the clinician should review the CXR prior to using the port system. If any abnormality was noted, we must remove it as soon as possible to reduce the late complications.
Although removal of the catheter is essential to prevent the complication, it is more important to find the underlying cause that leads to the catheter malposition. Revision is necessary in this condition, but the selection of the target vessel is a more important issue. Understanding the underlying cause is essential for our surgical plan.
Consequently, it is important to utilize bedside ultrasound to examine the anatomy of the target vessel preoperatively.18, 19, 20 If we still maintain the revision through the right SVC, the result may be the same or even worse because it creates high-risk complications, such as vessel rupture.5 There are other choices for LIJV, such as the femoral veins. However, we still chose LIJV because of the higher infection rate of femoral central venous catheterization.4 Our patient did not complain of discomfort after PBSCT through the new CVC. Therefore, the avoidance of RIJV approach in this condition is important.
Ultrasound-guided LIJV venipuncture could not be overemphasized to prevent early complications, such as pneumothorax or hematoma.18, 19 After the guide-wire insertion, we used the intraoperative fluoroscopy to check for the guide-wire direction and position. We could also adjust and measure the length of the guide wire under the guidance of fluoroscopy. Finally, CXR was arranged to check the final position of the catheter and any complication after surgery.
Catheter placement with exceptional caution cannot be overemphasized, because catheter tip manipulation in the CS may cause angina, arrhythmias, cardiac arrest, or CS thrombosis.
In summary, when we note the unexpected occurrence of the long-term CVC migration or malposition, we should remove it as soon as possible to prevent the occurrence of long-term or late complications, such as venous rupture or thrombosis. The utilization of proper image studies including ultrasound, contrast-enhanced venography, CT, and MRI may provide us with some clues as to the source of the problem and guide us in appropriate treatment.