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CASE REPORT
Year : 2022 | Volume
: 9
| Issue : 5 | Page : 393-396
Obscure presentation of postthrombotic syndrome
Jayesh Patel, Manisha Rajesh Asrani, Arya Patel, Gazal Patel
Vascular Surgery, Pramukh Swami Medical College, Anand, Gujarat, India
Correspondence Address:
Dr. Jayesh Patel
Vascular Surgery, Pramukh Swami Medical College, Anand, Gujarat
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/ijves.ijves_99_22
Postthrombotic syndrome (PTS) will develop in 20% to 50% of patients within 1 to 2 years of symptomatic deep venous thrombosis (DVT). Limbs afflicted with PTS have more than three times the odds of having combined reflux and obstruction compared with limbs without PTS. Here, we present a case of an involvement of the right lower limb with classical features of PTS. Due to its low prevalence and similar presentations as those of chronic venous insufficiency lead to a missed diagnosis and prolonged follow up and complications of PTS. This can be prevented by employing CT venography as an early investigation modality. Here we treated the patient with open method as endovascular procedure was not successful.
Keywords: Chronic venous insufficiency, computed tomography venography, nonthrombotic iliac vein lesion, postthrombotic syndrome
Postthrombotic syndrome (PTS) previously also known as postphlebitic syndrome is a significant chronic complication of deep vein thrombosis (DVT). Post deep venous thrombosis, reflux and obstruction are common cause of development of postthrombotic syndrome. PTS presents as a spectrum of severity ranging from mild-to-severe debilitating chronic leg pain, edema, and ulcers, which often leads to patients seeking medical counseling for this debilitating condition. Limbs with multisegment venous disease with iliac involvement are more likely to develop PTS. This compression results in chronic venous stasis resulting in DVT.[1],[2] Chronic venous insufficiency (CVI) in 20%–50% of patients post-DVT manifests as PTS characterized by ambulatory venous hypertension, which may be brought on by valvular injury, residual venous blockage, or both. Leg pain, edema, and itching along with trophic alterations ranging from hyperpigmentation to venous ulceration are the common presentations.[2],[3]
The goal of this case report is to emphasize the diagnostic and interventional challenges encountered by clinicians when PTS is present and how to resolve them.
Case ReportA 37-year-old obese female presented with dilated tortuous veins over her right leg for the past four years. She also has had complaints of bleed twice within two years due to the dilated veins over her right medial malleolus for which she often presented to the emergency department. The veins were accompanied by the presence of a nonhealing ulcer over the lower 1/3rd aspect of her leg as well. She had no significant history of DVT or a miscarriage and had two live children with an unremarkable family history.
Chronic venous insufficiency lead to non healing ulcer with serous discharge, ulcer was 8 x 6 cm in size with irregular margins and pigmentation in surrounding skin [Figure 1]. It resulted in difficulty in walking and severe burning pain in her leg.
Her difficulty in walking and excruciating burning pain warranted treatment with compression dressings and symptomatic relief with an unexpected increase in the size of the ulcer and progressive worsening of symptoms after a month. She additionally developed swelling in her right leg with venous claudication.
Following her symptoms, a duplex scan examination was undertaken that highlighted an incompetent right saphenous-femoral junction with above ankle perforator incompetence whereas the deep veins of the leg were normal/intact. Visibility of the iliac veins and the inferior vena cava (IVC) was hindered due to obesity.
With a possible diagnosis of PTS, a computed tomography venography was ordered to confirm our suspicions given its reliability to rule out DVT.[4] On computed tomography (CT) venography showed a normal IVC, multiple parametrial–pelvic and abdominal wall venous collaterals. Thrombotic occlusions were found in the right common iliac vein (CIV), external iliac vein (EIV), and internal iliac vein. Deep veins of the right leg were patent with a narrow caliber. On the left side, thrombotic occlusion of CIV extends to the terminal IVC near the bifurcation with multiple collaterals draining into left EIVs. Left EIV and rest of the deep veins of the left leg were normal [Figure 2].
Figure 2: Right CIV, EIV, left EIV thrombotic occlusion. Right CFV narrowed, multiple collaterals. EIV: External iliac vein, CIV: Common iliac veinIn view of her symptoms we decided to do for intervention. First plan both iliac vein angioplasty, second plan if right side not able to cross the lesion in view of long segment occlusion then left iliac vein stenting followed by left to right femoro–femoral cross over (Palma procedure) with synthetic graft with right side endophlebectomy.
We tried from both common femoral veins, right superficial femoral vein and right internal jugular vein with 014,018,032,035 multiples wire but could not negotiate the lesion from anywhere [Figure 3] and [Figure 4].
Relatives were counselled for an open method and we did a right common femoral vein endophlebectomy with midline laparotomy, right visceral rotation and femoro-caval bypass with a 7-mm dacron graft [Figure 5] and an arterio venous fistula [Figure 6] with vein graft from superficial femoral artery to distal part of hood of graft.
Figure 6: SFA to graft hood AVF. AVF: Arteriovenous fistula, SFA: Superficial femoral arterySurgical interventions were successfully followed by infusion of anticoagulation by heparin 500 IU/h and started for 3 days, followed by apixaban 5 mg OD for 14 days. Antibiotics were given for two weeks. On subsequent follow-up, her wound has healthy granulation tissue and she was posted for split thickness skin grafting. On follow up she had resolution of venous claudication, swelling of the limb with healed ulcer [Figure 7]. She is currently on apixaban 2.5 mg and is advised for ankle exercise, manual lymphatic drainage, and Class 2 compression stockings.
DiscussionThe pathophysiology of PTS is a complex set of events involving inflammation, thrombosis, resolution of thrombus, and vein wall damage, which result in outflow obstruction, valvular insufficiency, and calf muscle pump dysfunction. The resultant venous hypertension and subsequent signs and symptoms of PTS present as chronic venous insufficiency, swelling of the limb, varicose veins, pigmentation, and/or chronic nonhealing ulcers.[1],[2]
On presentation, the patient has varicosities with a history of bleeding arising twice from the varicosity along with a large venous ulcer. She was treated locally with crepe bandage, dressing, and analgesics.
We suspected the presence of a prior undiagnosed and untreated DVT in the past which presented as a flare up in the present in the form of PTS after no intervention.
Diagnostic modalities such as laboratory investigations, imaging, or functional tests have no significant role in determining PTS; the diagnosis is mainly guided by clinical signs and symptoms. It should be kept in mind that the acute phase of DVT (initial 3–6 months) mimics PTS lieu to the similar symptoms demonstrated during that period; hence, the diagnosis of PTS should be deferred until the resolution of the acute phase of DVT.[3]
A delayed diagnosis was established in our case due to the fact that the patient hails from a low socioeconomic status with inadequate access to health care, which was coupled with low awareness regarding the severity of the condition and the importance of timely medical intervention for better long-term prognosis. The incidence of PTS is 20 to 50% after proximal DVT.
Nonmodifiable risk factors for DVT include old age, preexisting CVI, and gender. Other factors are Obesity, immobilisation especially after surgeries or extended period of travel, thrombophilia commonly seen in antiphospholipid antibody syndrome, nephrotic syndrome, pregnancy and oral contraceptive pills. Damage to the venous endothelium via trauma, chemotherapy or vasculitis is also a significant cause. Post DVT is highly probable that it will be followed by a presentation of PTS.[5],[6]
PTS is a clinical diagnosis with no specific laboratory, imaging, or functional tests to ascertain it. However, The Villalta-Prandoni scale adopted by the International Society on Thrombosis and Haemostasis (ISTH) can be used to diagnose and grade the severity of PTS.[3],[7]
High-resolution ultrasonography as well as a color and duplex pulsed Doppler are among the early inquiry modalities used in our case. While these are sufficient for detecting reflux and DVT.
The best course of treatment is endovascular management, but due to the long-segment blockage, an attempt at endovascular method through femoral vein and internal jugular vein was unsuccessful followed by a successful endophlebectomy with right femorocaval bypass, AV fistula, and anticoagulation.
Lifestyle modification to relief symptoms in patients with PTS include exercise to improve calf muscle pump function, reduction of weight help to reduce venous hypertension. Limb elevation reduces hydrostatic pressure and increased microcirculatory flow with reduced venous pooling. Venous ulcers can be prevented using moisturizing creams.[8]
Dealing with such chronic cases, we should anticipate the disease extent, its etiology, and different investigation tools to treat it in the best possible way. What we found useful was also having a multi-pronged approach to management in order to provide the best possible care as PTS and reduce healthcare costs. To ensure absence of recurrence need regular follow up and investigation in form of duplex imaging to detect primary patency of graft, timely intervention and proper counselling of patient are key factors for good prognosis.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
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