Abstract
Case history
A 60-year-old male, new to our practice, with a past medical history of systemic lupus erythematosus (SLE) and basal cell cancer, was given Augmentin® for cellulitis. He subsequently developed facial swelling. He was treated for possible penicillin allergy in the emergency department. The patient was not known to have a penicillin allergy.
After a few weeks, his facial swelling did not subside. He had engorged neck veins, facial swelling and flushing typical of superior vena cava syndrome. On examination a right-sided inactive port-a-cath was noted; this had been inserted for SLE treatment 34 years ago.
Computed tomography (CT) of the chest and neck was requested. The CT chest revealed the superior vena cava was narrowed inferiorly near to the cavo-arterial junction and had progressed from the previous CT. The radiologist advised that the narrowing of the superior vena cava was most probably a secondary stricture associated with the longstanding port-a-cath. A CT of the neck was normal.
Discussion with rheumatologist revealed a history of a severe form of SLE treated with very high doses of intravenous cyclophosphamide through the port-a-cath more than 30 years ago. Once the patient’s SLE was in remission, oral hydroxychloroquine was started. It was decided that the port-a-cath should be removed as this was the cause of his symptoms.
The patient was referred to the surgeon who had originally inserted the port-a-cath. The surgeon managed to remove only part of the port-a-cath as it remained fused to the superior vena cava and the stricture. The patient’s symptoms resolved after partial removal of the port-a-cath. The risks of further surgery to achieve complete removal were felt to be too great.
Discussion
SLE is an autoimmune disease and typical disease manifestations include nephritis, arthritis, pleuritis, pericarditis and vasculitis. Before treatment with immunosuppressants SLE was a potentially life-threatening disease (Frostegård, 2005).
Traditionally, SLE has been treated with non-specific anti-inflammatories or immunosuppressants. Non-steroidal anti-inflammatory drugs and the hydroxychloroquine (HCQ), an immunomodulator agent, are used for mild disease. Where major organs are involved, stronger immunosuppressants, such as azathioprine, mycophenolate or cyclophosphamide, are the recommended treatment options. For disease flare ups, corticosteroids can be used. However, these are often continued on a long-term basis (Oon et al., 2016).
Superior vena cana (SVC) syndrome is caused by obstruction or occlusion of the SVC. It is associated with significant morbidity and mortality. Malignancy is a cause in 70% of cases. The increased use of central venous catheters and pacemakers is contributing to an increased incidence of non-malignant SVC syndrome (Azizi et al., 2020).
The clinical presentation of SVC syndrome varies, depending on the severity and location of the obstruction as well as the rapidity of onset and presence of collateral veins. SVC syndrome can present with facial or neck swelling, engorged neck or chest veins, headache, blurred vision and swelling of upper extremities. In rare cases, it can cause oesophageal varices.
The diagnosis of SVC syndrome is based on clinical presentation and imaging, which includes chest X-ray, CT with contrast and magnetic resonance venography. Contrast CT can clarify the extent of blockage and differentiate between extrinsic and intrinsic causes (Azizi et al., 2020).
Treatment depends on the aetiology of SVC syndrome. If malignancy is a cause, a multidisciplinary team (MDT) approach is the norm. The MDT usually includes oncologist, surgeon, respiratory physician and an interventional radiologist. Indwelling catheters or malignancy can cause thrombus formation, caused by stagnant blood flow and hypercoagulability leading to SVC syndrome. In these cases, thrombus removal will be the priority before other interventions, to prevent pulmonary embolism (Azizi et al., 2020).
In this case report, the patient had a long-standing port-a-cath, one of the benign causes of SVC syndrome. SVC syndrome can have a variety of presentations depending on the severity of the obstruction. A careful history, examination and appropriate investigations helped diagnosis and targeted management.
Patient consent
The patient gave written consent for submission and publication of this article.
