Fig. 31.1
Physical exam findings of chest collaterals in a patient with complete bilateral central venous occlusion. Note the numerous central venous catheter scars on the chest wall
Access will have a pulsatile quality from the outflow obstruction.
Increased venous pressures during dialysis.
Decreased flow during hemodialysis.
Prolonged bleeding after access decannulation.
Venous duplex ultrasound of the access and the effected extremity can rule out DVT, postoperative hematoma, and any non-central venous stenosis within the access. Central venous stenosis and occlusions are difficult to diagnose by duplex, although central obstruction is suggested by diminished respiratory variation. The diagnostic study of choice to assess central vasculature is a venogram or fistulagram. This allows delineation of the entire venous outflow, central and peripheral. Stenosis and occlusion are easily identified, with the added benefit of a potential therapeutic treatment with endovascular techniques.
The severity of venous hypertension is classically as follows [7]:
Grade 0: None
Grade 1: Mild symptoms such as mild swelling
Grade 2: Intermittent discomfort with severe swelling
Grade 3: Constant discomfort with late changes such as venous ulceration
Grade 1 can be managed with conservative therapy, while surgical treatment is reserved for patients with grades 2 and 3.
Treatment: General Considerations
The goal of treatment in patients with central venous stenosis and occlusion is improvement in symptoms (i.e., swelling) to a point that is tolerable to the patient with maintenance of the AV access. Patient education about the disease process and conservative measures to decrease arm swelling is the first step. This includes arm elevation and gentle compression of the affected extremity to decrease the symptoms of venous hypertension. Discussion should include an assessment of the patient’s activity level and the use of the arm and the fact that arm swelling is not dangerous. Compression garments can be used to reduce the swelling from severe to moderate, which may be adequate in the less-active patients. The decision whether to compress the area of the fistula must be individualized, as sometimes large, high-flow fistulas may be able to tolerate compression. Aggressive fluid removal during dialysis can also help alleviate symptoms on dialysis days. Large, high-flow fistulas can be plicated near the anastomosis to decrease flow volumes while still remaining functional.
Should these conservative measures fail, there are generally three options to address the effects of central venous stenosis: endovascular treatment, open surgical treatment, and access ligation if there are other possible means of dialysis. The next several pages will highlight the different therapies in detail.
Endovascular Treatment for Central Venous Stenosis
Over the past decade, endovascular techniques have supplanted open procedures because of the large decrease in procedural morbidity, ease of use, and ability of many specialists to perform these procedures. Today, endovascular interventions are the preferred method for diagnosis and treatment of central venous stenosis. The frustration with endovascular therapy is the poor durability of the repairs and need for frequent reinterventions, with some interventions compromising further surgical therapy.
Endovascular interventions can be performed in any facility with suitable fluoroscopic imaging and the ability to safely perform moderate sedation. General anesthesia is not necessary. The procedure starts through venous access, using the existing AV access. Venography is performed from the access anastomosis to the central veins draining to the right atrium. Guiding catheter placement into the central veins may be necessary to adequately visualize any significant stenosis or occlusions. Central stenosis in dialysis patients, unlike anatomic compression in other areas of the venous anatomy, is usually obvious. Confirmatory techniques such as intravascular ultrasounds or pull-through pressures are rarely needed.
Once a central stenosis is identified, the first-line treatment is percutaneous transluminal angioplasty (PTA) (Fig. 31.2). Large-size balloons (10–14 mm) are needed with inflation times of at least 2 min. Due to the fibrous nature of central venous stenosis, it often requires high-pressure, noncompliant balloons. The immediate success of initial PTA alone, without stenting, has been reported up to 70 %. Primary patency results drop down to a sobering 29 % at 1 year [8], and symptom relief at 1 year is equally poor [9]. Due to this poor durability, PTA requires frequent reinterventions to maintain patency. With frequent interventions, patency can be maintained in up to 86 % at 1 year [9].
Fig. 31.2
First panel: The nearly occluded left subclavian vein with significant collaterals. Second panel: The lesion was crossed and a percutaneous angioplasty was performed with a significant waist. Third panel: Venogram after PTA shows a patent subclavian vein with no filling of the previously seen collaterals. Notice subtle irregularity of previously stenotic segment, indicating endothelial injury
Primary stenting also has a very poor performance, with primary patency at 1 year to be only 21 % [8]. The high failure rate of central venous stents is not fully understood, but it is clear that intimal hyperplasia is aggressive in the central veins. Stents are reserved for lesions that recur at intervals less than 3 months, perforations or residual stenosis greater than 50 %. Stents should be oversized by at least 30 % to prevent migration, and care should be taken to not cover critical venous branches (the internal jugular (IJ) or contralateral brachiocephalic vein). Stent placement should be avoided at the costoclavicular junction, where the subclavian vein enters the thoracic outlet. Technology in endovascular surgery is quickly changing, and it is important to recognize that many available studies have used first-generation stainless steel wall stents (Fig. 31.3). More recently, the use of covered stents in the central veins has gained popularity, in part, because small early studies have indicated decreases in intimal hyperplasia and stent failure [10–12]. Outcomes are promising with primary patency at 1 year of 67 % and assisted patency at 2 years of 75 % [12]. Evolving technology with drug-coated balloons and stents, targeted at halting intimal hyperplasia, may further change the endovascular management of central vein stenosis in the future.
Fig. 31.3
Intimal hyperplasia seen on venogram months after a left subclavian stent with a stainless steel Wall Stent (Boston Scientific)
Endovascular Treatment for Central Venous Occlusion
Central venous occlusions are more challenging to treat than central venous stenosis. Crossing occlusions is often performed using hydrophilic wires with guiding catheters and usually requires support with long sheaths. Patience is required for these difficult lesions and establishing access from the arm, the ipsilateral neck (internal jugular), and the contralateral IJ as well. If unable to cross from the upper extremity, attempting to cross from the lower extremity is occasionally helpful. Once the total occlusion has been crossed, some authors advocate for mechanical or catheter-directed thrombolysis to unmask the true stenotic lesion and remove any thrombus. We find this is not typically necessary as the occlusions are normally chronic and more fibrous, and so we will treat with PTA and/or stent as highlighted above and have not experienced embolic complications from this technique.
Endovascular Treatment Complications
It is important to highlight the potential complications associated with endovascular treatment. Access site complications are most common and include bleeding, cellulitis/AV access infections, and access thrombosis. Procedures should be performed under sterile conditions and the extremity adequately prepped. When accessing a prosthetic AV graft, it is our routine to give preoperative antibiotics covering common skin organisms. Complications specific to central vein stenosis/occlusion are potentially life threatening. Venous perforation, with high flow from a distal AV access, can lead to significant bleeding in the mediastinum and pleural or pericardial spaces. Early recognition is essential, and often times the bleeding can be controlled with local balloon inflation or deployment of a stent graft across the tear though open repair may be indicated and requires an emergent surgical consultation.