CARDIOVASCULAR JOURNAL OF AFRICA • Volume 34, No 1, January–April 2023 46 AFRICA Currently, surgical treatment is the first clinical option for IVL, especially for stage IV disease.6,7 Individually, the surgical procedure, radical or staged surgery, should be estimated and confirmed based on the physical status of the patient at baseline, the size of the IVL, the patient’s physiological reserve function and other personal medical information. However, for patients who receive only staged surgery, it is critical that, postoperatively, regular and periodic follow up is given to detect any further growth and assess the clinical risk of the residual IVL.8 Our patient was diagnosed with stage IV IVL involving the right heart system, in accordance with the evidence from pre-operative images and postoperative pathology. Taking her menopausal and normal basic physiological as well as physical functions into consideration, and after multidisciplinary assessment, radical surgery via a thoracic–abdominal incision was decided on and performed. The tumour body within both the left ovarian vein and uterus was removed in the abdominal procedure. Then, in a thoracic procedure, under assisted cardiopulmonary bypass without perfusion of cardioplegia (deep hypothermic circulatory arrest), once the adhesions between the tumour body and wall of the vessel as well as the atrium were established and any risk was overcome, the tumour body within the renal vein, inferior vena cava and right atrium was separated completely and removed via the orifice of the inferior vena cava. Lastly, without any adverse events such as rupturing or damaging the tumour body, both primary and extended extrauterine parts of the IVL were completely removed. The period of assisted cardiopulmonary bypass was relatively short, significantly lowering the occurrence of cardiopulmonary bypass-related injuries and postoperative complications. Considering the enlarged tumour body within the right atrium, removal of the IVL was done from top to bottom via the right atrium. Surgical removal can be adapted, depending on the findings during the procedure. It has also been demonstrated that for patients with IVL involving the heart system, once any adhesions with the heart and vena cava are determined, removal of the IVL can take place from bottom to top via the ovarian vein.9 Clinically, based on our experience with the surgical treatment of this patient, we have shown that radical surgery of the IVL through a combined abdominal–thoracic incision is safe and reliable. Furthermore, although ultrasonography is useful, hypoechoic lesions can be difficult to distinguish from myxoma or venous thrombosis. If necessary, contrast-enhanced ultrasound should then be performed.10 In our pre- and intra-operative strategy, echocardiography played an important role in the surgical procedure and decision making with regard to detection of the residual and occult tumour body. CT scanning is more effective in multiple tumour evaluations. Moreover, assisted cardiopulmonary bypass is essential to prevent rupture or damage to the IVL. An IVL involving the inferior vena cava or right heart system is closely associated with venous thrombosis. Therefore regular and systematic anticoagulation should be administered during the pre-operative period. It has been suggested the anticoagulant course should last for at least three months after surgery.11 Most importantly, as a rare gynaecological tumour, there should be multidisciplinary co-operation on the peri-operative management of the IVL. Even after surgical removal, there is a need for strict, conservative medical treatment, so that the risk of the IVL recurring is avoided.12 Our patient had multidisciplinary consultation and the surgical procedure was based on consensus between all participants. This promoted a positive postoperative outcome. Fig. 4. In postoperative pathology of the IVL, a spindle cell was found in histological samples of the tumour. Fig. 3. The removed tumour body of the IVL.
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