I was reviewing my emails late in the day when I found a message flagged “Important” in the subject line. It was from Kelsey, one of our hospital’s new pediatric surgeons. “Consulted regarding a 16-month-old with a middle mediastinal mass,” her message read. “Compression of trachea. Would love your thoughts.”
The mass Kelsey referred to in her email was in an area where a lot of things can go wrong, what we call the mediastinum—the middle of the chest between the lungs, where several important organs, such as the heart, trachea, and esophagus, reside.
As a thoracic surgeon, I specialize in operating on organs in this area and often review cases with colleagues.
I responded that I’d be happy to speak with her, and in less than 15 minutes she was tapping on my door. Kelsey was obviously very concerned about this one, so I quickly pulled the CT scan of the mass up on the computer as she relayed the details of the case. The patient was a 16-month-old boy who was developing normally but had recently been diagnosed with asthma.
He had been admitted to the hospital because of increasing stridor, a high-pitched sound made during inhalation. Stridor indicates a narrowing somewhere in the main, or proximal, airways—the area of the respiratory tract between the vocal cords high in the neck and where the trachea, or windpipe, branches to meet the two lungs. Stridor is often thought to indicate asthma, but it usually doesn’t. Asthmatics make a different sound: a wheeze. Wheezes occur during exhalation and imply obstruction of the smaller airways that are in the lungs themselves.
The boy’s name was Ian, and it was clear that his breathing problems were not caused by asthma. The CT images showed a five-centimeter mass—about the size of a lemon—narrowing his trachea by more than half and encasing the adjacent esophagus, which carries food from the mouth to the stomach. It looked as if malevolent bees had built a rounded, ill-formed hive in Ian’s chest.
I took in a deep breath: “That looks bad, Kelsey.”
“Yeah,” she replied. “It may be malignant. I’m worried about a sarcoma.”
We knew that about half of mediastinal tumors in children are malignant—aggressive cancers that grow into surrounding organs. Such tumors can take various forms. Sarcomas are malignant tumors of connective tissues such as muscle and bone. Tumors can also form in the lymph glands, the small organs of the immune system that filter bacteria from the bloodstream. Mediastinal tumors are rare, however. A total of 10,000 children are diagnosed with cancer every year in the United States, and mediastinal tumors account for about 100 of those cases.
“If not a tumor, I guess this could be a fistula,” Kelsey said, “with a chicken bone or something lodged there and causing an infection.”
A fistula is an abnormal connection of tissue between two organs. There are a group of congenital fistulas that can connect the trachea and esophagus, which grow from the same embryonic tissue. If a child swallows an object that lodges in the fistula, it can trigger an infection that may result in an inflamed mass. But Kelsey said that Ian’s white blood cell count and other tests that would indicate an infection were normal.
I considered whether we should get other tests, such as a biopsy or an MRI. “We could,” Kelsey replied. “But if the mass enlarges, it will block the trachea completely. I think we just have to go for it.”
We discussed the challenges of removing Ian’s mass surgically. If it was attached to the esophagus extensively, we would have to remove much of the organ and replace it with a section of the stomach. The tracheal part of the procedure could get even more complex. The trachea does not heal as reliably as the esophagus, and only a small amount of tissue can be removed from it for reconstruction, giving us a narrower margin of error in the event of damage and subsequent repair. The complexities and risks were so great that Kelsey and I sought the input of other doctors as well. Kelsey called her mentor at the pediatric surgical program where she had trained, and I called a pediatric surgeon who had trained with me years earlier. These colleagues felt surgery was unavoidable.
