First-Reported Emergency Pediatric Surgery of its Kind: 5-Year-Old Girl Has Massive Tumor Removed from Abdomen and Heart
Doctors at NewYork-Presbyterian/Morgan Stanley Children's Hospital Successfully Removed Tumor Blocking Blood Flow From Nearly Every Vital Organ and Invading the Heart
Jul 11, 2012
A 5-year-old New York girl is recovering at home and undergoing outpatient treatment after an emergency operation involving the removal and partial-re-implantation of her liver and her right kidney along with open heart surgery to resect an otherwise inoperable tumor in February. The 10-hour operation, the first reported emergency pediatric surgery of its kind, was led by Dr. Tomoaki Kato and Dr. Emile Bacha at NewYork-Presbyterian/Morgan Stanley Children's Hospital. The procedure to remove an extensive cancerous pediatric tumor included several medical teams specializing in abdominal, cardiothoracic, pediatric surgery and anesthesia. The tumor, a neuroblastoma, was shaped like an uneven set of barbells connected by a thick shaft. Tethered to the top of the right kidney, the tumor had worked its way into the liver and ballooned into much of the right atrium of the heart by muscling through a large connecting vein (the largest vein in the body), the inferior vena cava (IVC). "At the time of surgery, the IVC extending from the liver to the heart was completely blocked, creating a backup of blood from the legs and abdominal organs below the diaphragm. The tumor was also inside the heart, pressing against another major vein and partially blocking blood flow from the lungs and the upper body," says Dr. Kato, surgical director of liver and intestinal transplantation at NewYork-Presbyterian Hospital/Columbia University Medical Center and chief of Abdominal Organ Transplantation and professor of surgery at Columbia University College of Physicians and Surgeons.
As a first step, Dr. Kato and his team opened the abdominal cavity to mobilize the kidney and parts of the tumor and prepare abdominal blood flow to the bypass machine. Next, Dr. Bacha's team opened the chest and put the patient on cardiopulmonary bypass. This set the stage for another procedure, deep hypothermic circulatory arrest (DHPA), which cools the body to 18 degrees centigrade to safely stop the patient's blood flow.
As the cardiothoracic team waited for the patient's body to cool, the abdominal team isolated the tumor. When the patient's body reached the optimum temperature, Dr. Bacha's team opened the heart and pulled the top of the tumor from the right atrium. Simultaneously, the liver and the remainder of the tumor were taken out of the abdomen. The liver was repaired and re-implanted while the patient remained on bypass. The patient was then re-warmed and blood circulation was restarted. After blood flow to the liver was re-established, the patient was taken off bypass.
Although the patient's initial symptoms appeared to be relatively mild, seemingly overnight they had gotten much worse. By the time she was rushed to NewYork-Presbyterian/Morgan Stanley Children's Hospital on President's Day, all of her symptoms pointed to a complete blockage of blood flow from the liver, notes Dr. Kato. "Without immediate intervention, it's unlikely she would have lived another 24 to 48 hours."
"We know this type of complex surgery carries a lot of risks and any number of potential side effects, especially since we had to put the patient on an anticoagulant, which helps prevent clotting but increases the likelihood of much more bleeding," says Dr. Bacha, chief of the division of Cardiothoracic Surgery, director of Congenital and Pediatric Cardiac Surgery at NewYork-Presbyterian/Morgan Stanley Children's Hospital and the Calvin F. Barber professor of surgery at Columbia University College of Physicians and Surgeons.
What added to this high-stakes surgery was a race against time. While almost every major operation is fraught with these types of challenges, the cardiothoracic team's window to resect the tumor and repair the IVC was just 30 minutes, to guard against irreparable damage during the cooling process. What's more, both teams had to work in lockstep, timing their actions seamlessly. "Like a delicately choreographed dance, we had to work in synchrony, sometimes together, sometimes apart," says Dr. Bacha.
While no longer in immediate danger, the patient's prognosis will depend on how she responds to a rigorous treatment regimen involving chemotherapy, a bone marrow transplant, radiation, and finally, immunotherapy. "At this point, we are guardedly optimistic about her long-term prospects," says Dr. Darrell Yamashiro, pediatric oncologist at NewYork-Presbyterian/Morgan Stanley Children's Hospital and associate professor of pediatrics, pathology and cell biology at the Columbia University College of Physicians and Surgeons.
Dr. Kato performed a somewhat similar surgery involving the removal and partial implantation of six organs on a 7-year-old just three years ago. To read the story about this young patient, go to http://nyp.org/news/hospital/First-Reported-Case.html
NewYork-Presbyterian/Morgan Stanley Children's Hospital
NewYork-Presbyterian/Morgan Stanley Children's Hospital, located in New York City, offers the best available care in every area of pediatrics - including the most complex neonatal and critical care, and all areas of pediatric subspecialties - in a family-friendly and technologically advanced setting. Building a reputation for more than a century as one of the nation's premier children's hospitals, Morgan Stanley Children's Hospital is affiliated with the Department of Pediatrics at Columbia University College of Physicians and Surgeons, and is Manhattan's only hospital dedicated solely to the care of children and one of the largest providers of children's health services in the tri-state area with a long-standing commitment to its community. It is also a major international referral center, meeting the special needs of children from infancy through adolescence worldwide. NewYork-Presbyterian Hospital also comprises NewYork-Presbyterian Hospital/Columbia University Medical Center, NewYork-Presbyterian Hospital/Weill Cornell Medical Center, NewYork-Presbyterian Hospital/Westchester Division and NewYork-Presbyterian/The Allen Hospital. NewYork-Presbyterian is the #1 hospital in the New York metropolitan area and is consistently ranked among the best academic medical institutions in the nation, according to U.S.News & World Report.
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