P022: WHO NEEDS AN IVC ANYWAY? LESSONS LEARNED FROM RESECTION OF A LARGE INVASIVE ABDOMINAL NEURAL TUMOR IN A YOUNG CHILD.
Nicole McCoy, MD1, Katrin Post-Martens, MD2; 1Mayo Clinic/ Nemours, 2Nemours
A previously healthy 2 year old presented to the children’s hospital for a newly palpated abdominal mass. She underwent a CT scan as well as IR guided biopsy revealing an 8.9cm TRV*9.4cm AP*9.3cm SI ‘neoplastic tumor of neural origin’. She was started on Lovenox for extensive tumor burden in the inferior vena cava.
Surgical resection was coordinated with the Pediatric General Surgeon, Pediatric Cardiothoracic surgeon, Pediatric Cardiac Anesthesiologist, Oncologist, Pathologist and Perfusionist as a multidisciplinary approach was paramount to a successful operative approach. The proposed plan was to begin with an exploratory laparotomy before determining if and when the patient would need to be placed on cardiopulmonary bypass for full excision of the mass creating some uncertainly to the overall OR course from the onset of the operation.
The patient underwent an uneventful intravenous induction. Additional IVs and a radial Aline were placed. She then underwent placement of a left internal jugular central venous catheter under ultrasound guidance in Trendelenburg position. Immediately after the lumens were flushed with heparinized saline, the patient had an abrupt cessation in ETCO2 with resultant desaturation and finally bradycardia. Hand ventilation was difficult. The ETT was removed and replaced, while 1 mcg/kg of epinephrine, a fluid volume bolus was given, and the patient was returned to level position. There was return of ETCO2 and improvement in her oxygen saturation and heart rate. At that time, it was decided she was undergo cannulation onto cardiopulmonary bypass at the start of the case given her hemodynamic fragility.
The surgery proceeded uneventfully with total OR time approximately nine hours. She was able to separate from bypass. The radical resection of the retroperitoneal tumor included removal of the right kidney, segmental portion of the vena cava, surrounding iliopsoas muscle on the right and portions of the bilateral iliac veins. The patient had developed significant collateral vessels to survive with an entirely occluded IVC and this was reassuring that she could potentially continue to have reasonable venous drainage from her lower extremities by way.
The tumor pathology ultimately returned as “neural-like low grade sarcoma with NTRK1 rearrangement”. She is followed closely by pediatric surgery and pediatric oncology and will have regular surveillance scans to evaluation for recurrence and proper lower extremity venous blood flow. She is currently a happy, active playful child, despite complete lack of an inferior vena cava.
This is a difficult case for many reasons. First the tumor burden in her small body was a surgical challenge requiring coordination between multiple specialties to ensure a smooth operating room course. Second, the hemodynamically significant event that occurred following CVL placement was unanticipated and difficult to explain—the differential includes venous air embolism, embolic event, bronchospasm, positioning leading to decreased venous return/decreased cardiac output, or dislodged ETT. Finally, little is known as to life going forward without an IVC. Various case reports state there is an increased right of venous stasis and thrombosis despite adequate collaterals in patients with congenital absence of an IVC.