Anesthetic management for removal of adrenocortical carcinoma with thrombus in the inferior vena cava extending to the right atrium.

Sir,

Adrenocortical carcinoma is a rare, rapid growing tumor which tends to metastasize to the liver, lungs, kidney, renal veins and inferior vena cava. Rarely tumor thrombus may extend through inferior vena cava (IVC) up to the right atrium (RA). Surgical removal poses a challenge to both surgeons and anesthesiologist because of the complications involving surgical access, bleeding, massive blood transfusion, coagulation defect, pulmonary embolism, large fluid shifts, and significant post-operative complications. We report the anesthetic management of a patient with such an involvement.

A 10 kg, one-year-old child was scheduled for inferior vena cava balloon tamponade followed by laparotomy, right adrenal tumor excision and IVC tumor thrombectomy. Investigations showed an elevated testosterone and dehydroepiandrosterone sulfate levels and computerized tomography scan revealed a 5 × 5 cm right adrenal tumor with tumor thrombus extending up to the IVC-RA junction. Echocardiography did not show thrombus extension in the RA and right ventricular function was normal.

The child was premedicated with oral trichloryl 750 mg 1 h before surgery. He was anesthetized with air, oxygen and sevoflurane, under standard monitoring in the cardiac catheterization suite, for positioning of the IVC balloon under image intensifier guidance. A 24G intravenous cannula was placed and trachea intubated with a 5 mm ID uncuffed oral endotracheal tube after neuromuscular blockade with atracurium. Anesthesia was maintained with a 50% mixture of air and oxygen, isoflurane, fentanyl and intermittent boluses of atracurium. A thrombus blocking the IVC up to the IVC-RA junction with collateral flow in the azygos vein was noted on injecting a contrast medium through the femoral vein. The IVC balloon was placed via the right femoral vein at the IVC-RA junction and the balloon catheter was inflated with physiological saline solution for endoluminal occlusion of the free IVC near RA junction under image intensifier guidance. Following balloon occlusion of the IVC, child was transferred to the operating room where anesthesia was maintained with air, O2, isoflurane, morphine and atracurium. Invasive monitoring was established with right IJV cannulation to monitor the central venous pressure, right radial artery cannulation for invasive blood pressure, and a temperature probe was inserted. The urinary bladder was catheterized to monitor urine output. Intraoperatively, after the tumor resection IVC was exposed and thrombus was removed. The inflated RA balloon was pulled out through the same incision to ensure removal of residual thrombus. During this maneuver, there was excessive bleeding. Child was resuscitated with fluid, blood and blood products. Adrenaline infusion (0.1 mcg/kg/min) was started to maintain vitals. 3 ml of 10% calcium gluconate and 20 ml of 7.5% sodium bicarbonate were also infused. Central venous pressure, which was maintained at 6-8 cm of H2O, fell to –2 cm H2O with the acute bleeding.

Surgical re-exploration was done immediately as the patient became hypotensive and the abdomen had distended. Hemoperitoneum due to right phrenic artery bleed was found and the artery ligated. The total blood loss was around 800 ml in 4 h of surgery duration. About 200 ml of fresh frozen plasma, 100 ml of platelet concentrate, 350 ml of whole blood and 550 ml of rejuvenated red cell concentrate were given. He was ventilated in the ICU for about 24 h. He was discharged on the 11th postoperative day in good health with normal testosterone and dehydroepiandrosterone sulfate levels.

Malignancy of the adrenal cortex is rare and causes excessive hormone production which presents as precocious puberty. Elevation of 17 urinary ketosteroids is the most sensitive tumor marker.[1] Four stages of progression have been proposed for adrenocortical carcinoma. Patients in stages I and II of the disease have best chances of cure, whereas those in stages III and IV have a poor prognosis.[2] Surgery is the treatment of choice, even in patients with extensive metastasis.

Anesthetic management for surgical removal of adrenocortical carcinoma with thrombus in the IVC extending to RA is challenging due to high incidence of tumor thrombus migration which may result in pulmonary embolism/cardiovascular collapse. The common methods used to prevent pulmonary embolism are veno-venous bypass or cardiopulmonary bypass with deep hypothermic circulatory arrest. These techniques are ideal as the surgery is done in a bloodless field but their major drawback is bleeding diathesis, which is seen in about 5% of cases.[34] Veno-venous bypass cannot be used in children of <10–15 kg because of the difficulty in maintaining adequate flow through the small cannulas.[5] Other methods used, for complete tumor excision and to avoid tumor seeding and pulmonary embolization, are liver mobilization,[4] IVC filter and endoluminal occlusion cranial to tumor thrombus.[67] The endoluminal occlusion catheter can be placed through a jugular or subclavian route but there is risk of balloon displacement into the RA during surgery. Filter placement is a simple and minimally invasive surgical procedure, but its use is controversial because filter removal may make surgery difficult.[78] In liver mobilization technique, there is need for extensive caval mobilization which carries an even higher risk of embolization.[9]

Keeping in mind the age of the child and merits/demerits of various approaches, we chose the technique of endoluminal occlusion cranial to tumor thrombus. We placed a balloon at distal end of tumor thrombus at the junction of IVC and RA and it was kept inflated till the removal of the thrombus to prevent its migration. This method is less invasive and eliminates the need for cardiopulmonary bypass but there can be excessive bleeding following removal of thrombus.

Very few reports describe the anesthetic management of patients with renal cell carcinoma with thrombus extension till RA. Careful anesthetic planning and preparation, anticipating possible major blood loss and transfusion are necessary for a successful outcome. There is a possibility of pulmonary embolism despite balloon occlusion of IVC and one should take precautions to diagnose and treat it, if necessary. TEE is useful in the preoperative evaluation of thrombus extent, intraoperative confirmation of complete thrombus removal and diagnosis of pulmonary embolism.[910]