Chylothorax and Thoracic Duct Embolization: Ayla's Story

It was the time when most new parents begin to relax a little. But as tiny Ayla Bowman reached seven weeks of age, her mom and dad had just the opposite experience.

Born in October 2010, Ayla gave every appearance of being a normal, healthy infant. But one morning, just short of two months old, the baby girl had difficulty breathing.

ayla Tests at the Bowmans’ home hospital in Canada soon revealed the cause: chylothorax, a rare and potentially fatal condition.

A baby’s body normally produces about a quart of tissue fluid each day, which is moved through the body by the lymphatic system. In chylothorax, a defect in the system’s thoracic duct allows fluid to leak into the chest cavity and build up to dangerous levels. In Ayla’s case, the fluid had created so much pressure on her tiny lungs that she could barely breathe.

Treatment for chylothorax may involve medication or two surgical procedures — thoracic duct ligation or pleurodesis. Ayla’s medical team tried all three options, without success. Although doctors could drain the fluid from Ayla’s chest, eventually the leaking thoracic duct would cause her to lose too much lymphatic fluid, which in turn would compromise her immunity. Unless they could find some new type of intervention, Ayla would eventually die.

Thoracic duct embolization

A pulmonologist caring for the baby knew of a procedure being used at Children’s Hospital of Philadelphia: thoracic duct embolization. The procedure involves locating the malfunctioning thoracic duct, and then placing a platinum microcoil along with glue to block the leak. By performing the extremely delicate procedure in the Interventional Radiology suite, doctors are able to accurately visualize the thoracic duct using contrast dye and then implant the coil without the need for open surgery. Patients face fewer risks and recover more quickly.

First used in adults in the 1980s and '90s, thoracic duct embolization was further refined by Max Itkin, MD, at the University of Pennsylvania, who has performed the procedure in more than 200 patients. During this time, Marc Keller, MD, a pediatric interventional radiologist at CHOP, became interested in how embolization might help children like Ayla. Together, Keller and Itkin adapted the procedure for pediatric patients.

CHOP’s Interventional Radiology program is among the largest and most advanced pediatric services in the world. The Hospital has three state-of-the-art interventional radiology suites and a multidisciplinary team dedicated to the care of its patients. The CHOP team has performed more than 20 thoracic duct embolizations in children, the highest volume in the world.

Hope and healing

Ayla’s doctors contacted Keller at CHOP and arranged a video conference that included both medical teams and the parents.

it was nice to see everyone talking face to face,” says Ayla's father, Cameron.

For the first time, the Bowmans felt a glimmer of hope. Shortly thereafter, the family traveled from Canada to Philadelphia, where Ayla had the embolization. She came through the procedure beautifully and although she will need help to reach developmental milestones like feeding, her prognosis is excellent.

The Bowmans are grateful to have found a doctor, a team and a hospital that were prepared to deal with Ayla’s rare situation.

“When we looked for information [on the condition], it just wasn’t out there,” says Ayla’s mother Katrina.

That is, until they knew where to look.

Originally posted: October 2011

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