TEE-guided one-stage excision of intravenous leiomyomatosis with cardiac extension through an abdominal approach.

NTRAVENOUS LEIOMYOMA is a rare benign tumor of the uterus extending into the right side of the heart through the inferior vena cava (IVC) in 10% of patients. It commonly presents in postmenopausal parous women. 1 This tumor manifests itself clinically with menstrual alteration, dysmenorrhea, ascites, Budd Chiari syndrome, pelvic masses, right heart failure, syncope, and sometimes sudden death, caused by obstruction of the IVC or of the tricuspid valve. The common differential diagnoses for this type of tumor include cardiac tumor or thrombus in transit. 2 In this case, the authors believe that the transesophageal echocardiogram (TEE) examination and the finding of no intracardiac tethering of the tumor allowed the surgery to be performed as a 1-stage procedure and permitted the surgeons to use only a laparotomy incision. Intraoperative TEE is a powerful tool to help plan the surgical approach and evaluate the completeness of surgical excision in patients with intracavocardiac leiomyomatosis. CASE REPORT A 56-year-old woman presented with progressive fatigue for several weeks and difficulty in breathing for 2 weeks. Her past medical history was significant for postmenopausal uterine bleeding 1 year before with negative ultrasound imaging and without a palpable mass on physical examination. She had a history of hypertension, asthma, and nasal polyps. She had a 40 pack per year history of smoking and no history of alcohol use. An ultrasound of the gallbladder suggested a clot in the IVC. A subsequent computed tomography scan of the abdomen and chest revealed a pelvic mass and possible clot extension into the IVC. She was referred from the outside hospital for further care and management. Her initial examination revealed no acute distress. She was 165 cm tall and weighed 125 kg. Her vital signs were a heart rate of 82 beat/min, blood pressure of 150/70 mmHg, a respiratory rate of 14 breaths/min, and 95% oxygen saturation while on oxygen by nasal cannula at 2 L/min, and a room air saturation of 92%. Her laboratory results revealed elevated liver enzymes (alanine aminotransferase 165 U, alanine aminotransferase 149 U), a normal bilirubin level, electrolytes, and hematocrit. She was admitted to the intensive care unit (ICU) and placed on continuous hemodynamic monitoring, oxygen therapy and continuous intravenous heparin. A chest x-ray and electrocardiogram were normal. The abdominal computed tomography scan, chest x-ray, and magnetic resonance imaging showed a uterine mass extending through the right gonadal vein into the IVC to the level of the IVC/right atrium (RA) junction as well as extension into the proximal left renal vein. The initial TEE performed in the ICU showed a polycystic mass measuring 1.8 cm at its greatest visible diameter at the junction of the IVC and RA. The mass protruded slightly into the RA from the IVC with turbulent flow seen around the mass. All the chambers were of normal size with no interseptal defects and normal-appearing valves with mild mitral regurgitation. Biventricular systolic function was normal. There was no pericardial effusion. She was scheduled for exploratory laparotomy, with preparation for median sternotomy, cardiopulmonary bypass, and total circulatory arrest, if necessary. In the holding area, a large-bore peripheral intravenous catheter and a radial arterial catheter were started. After instituting the standard American Society of Anesthesiology monitors and arterial pressure monitoring, she was anesthetized with propofol and endotracheal intubation was facilitated with pancuronium. Anesthesia was maintained with isoflurane in 100% oxygen and intermittent fentanyl for analgesia. After suctioning her stomach with an orogastric tube, the TEE probe was introduced and used to visualize the wire for left internal jugular cannulation, taking care not to dislodge the tumor in the RA. An 8F introducer was placed through her left internal jugular. TEE confirmed the presence of a polycystic, serpentine elongated, nonhomogenous echogenic tumor extending from the hepatic and suprahepatic IVC into the RA with no apparent atrial or IVC attachments (Fig 1). Her hepatic veins, right ventricle, and pulmonary arteries did not reveal any tumor. Her chest, abdomen, and groin were sterilely prepared for the surgery. The cardiac surgical team, along with a cardiopulmonary bypass machine, were available on standby. The initial portion of the procedure involved a lower midline laparotomy and dissection of the tumor by the gynecologic oncologists and vascular surgeons. The vena cava was mobilized so that a proximal clamp could be placed. During this mobilization, the intracardiac portion of the tumor was continuously monitored using TEE, and the authors noted that the tumor moved back into the hepatic IVC by 5 cm. This movement suggested that the tumor could be removed through the IVC (Fig 2). The dissection of the uterus and gonadal vein was completed. The cava was then further mobilized with a clamp on the IVC and with hand control of the vena cava above the gonadal vein. A venotomy was made on the anterior wall of the gonadal vein, to which the tumor was attached, extending to the vena cava, and the tumor was resected free circumferentially at the entrance to the vena cava. Beyond that, the tumor was not attached to the wall of the vena cava, and, with TEE guidance, it was possible to remove the entire tumor through the venotomy in the abdomen. The patient was volume resuscitated during this period. The IVC was noted to be completely clear of tumor, and there was no residual turbulent flow in the IVC. Radical abdominal hysterectomy with bilateral salpingo-oophorectomy was completed. The frozen section on the tumor indicated that it was benign. Further pathology revealed a small endometrial cancer in addition to the intravascular tumor. The patient was left intubated overnight and monitored in the ICU. She had an uneventful recovery and was discharged home on the fourth postoperative day.