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Ganglioneuroma: Multiple Surgeons Take on Multiple Tumors

March 31, 2016

The removal of ganglioneuromas, rare benign tumors of the nervous system, requires the expertise of several disciplines of surgery.

Alyssa Parian, MD

At Julian’s follow-up appointment one month after his surgery, pediatric surgeon Eric Jelin checks his range of motion. “His recovery from surgery has been excellent,” Jelin says. “In terms of post-operative complications, I think we’re out of the woods.”
For six-year-old Julian Colon from Syracuse, NY, a second opinion made all the difference. The tumors on his neck and spine were bigger and deadlier than the cardiologist in New York had indicated. Most importantly, the boy’s deviated airway convinced pediatric surgeon Eric Jelin that he needed to operate quickly. Jelin didn’t venture into the operating room alone, however.

To construct an effective team for the complex operations Julian needed, Jelin turned to neurosurgeon Allan Belzberg and pediatric cardiac Surgeon Luca Vricella, who agreed to assist in the surgeries on short notice. Other specialists in pediatric radiology, neuro-monitoring, pediatric pathology and pediatric cardiac anesthesiology joined the team as well.
“Seeing his deviated airway on the first CT scan got us moving,” Jelin says. “Fortunately, we were able to assemble a team quickly and could begin operative planning almost immediately.”
Ganglioneuroma can grow nearly anywhere in the body, rendering each operation unique to the patient’s anatomy. During the first surgery, Jelin performed a thoracotomy, or incision in the Julian’s chest, to remove the tumors from his spine and heart. Risks for the incision and resection included nerve injury, paralysis and airway collapse from mediastinal mass. Vricella helped dissect the tumor off Julian’s heart, the most delicate segment of the first surgery due to possible complications such as cardiac injury, lung injury and bleeding from the aorta. Fortunately the surgeons avoided the complications and resected the targeted tumors.
During the second surgery, Belzberg removed the tumor from Julian’s neck that Jelin exposed while protecting other structures. Possibilities for complications remained abundant, with the surgeons monitoring closely for signs of stroke, airway injury and bleeding from the carotid artery and jugular vein. The surgeons also avoided damage to the brachial plexus, which would have resulted in arm dysfunction. Each of the surgeons’ roles proved vital in both operations and evasion of these complications.
The team resected 95 percent of the tumors, leaving only 5 percent of harmless tissue encasing a nerve, which could compromise the function of the patient’s left arm if damaged. “To me,” says Vricella, “the immediate reward was to see the left radial pulse right after the first surgery.”
Jelin attributes the success of these complex and difficult surgeries to the team’s ability to collaborate.
“Two weeks after Julian walked through the door, we were fully engaged and prepared for the operation,” Jelin said. “That’s what made the difference here.”
The multidisciplinary approach didn’t end when the surgical gloves came off, either. Julian returned to the Johns Hopkins Children’s Center in mid-November for follow-up imaging and check-ups with Jelin and Belzberg, who work together regularly. Belzberg conducted a full examination and concluded that no damage occurred during surgery. The team plans to continue monitoring Julian’s progress every few months to ensure his good health. Belzberg confirmed that Julian will continue outpatient physical therapy at home for pain in his side and back, though he has already recovered at a fast rate.
“This is a great stride for Hopkins,” says Belzberg. “The multidisciplinary approach helped us complete a difficult surgery and got Julian back on his feet.” 

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