Lymphatic Microsurgery: Daniel’s Story

Imaging performed in the emergency room showed Daniel’s lungs were filled with fluid and his oxygen saturation levels were low. Shortly after arriving at the hospital, Daniel was transferred to the PICU, intubated and placed on a ventilator as his team worked diligently to determine the cause of his symptoms.

“The hardest part was that we didn’t know what was going on,” says Carrie. “We were afraid of what we didn’t know.”

Chest tubes were placed in Daniel’s right and left pleural spaces, between his lungs and chest wall, to drain the fluid. Samples of this fluid revealed elevated lymphocytes (white blood cells) and triglycerides (fat cells) — consistent with a diagnosis of chylothorax, a rare condition in which lymphatic fluid leaks into the space between the lungs and the chest wall. As fluid builds up, it can cause a cough, chest pain and difficulty breathing.

The team recommended the family transfer to Children’s Hospital of Philadelphia (CHOP). Led by Yoav Dori, MD, PhD, the team at the Jill and Mark Fishman Center for Lymphatic Disorders leads the world in the diagnosis and treatment of lymphatic conditions like chylothorax.

“They said, ‘if anyone can fix him, CHOP can,’” says Mike.

Daniel’s doctors arranged a transfer, and he and his dad were taken by ambulance to CHOP’s NICU. By then, Daniel had a large amount of fluid in both of his lungs, and his condition was critical. 

Finding answers at CHOP

Within a few days of Daniel’s arrival, he underwent a dynamic contrast magnetic resonance lymphangiogram (DCMRL), a CHOP-developed imaging technique used to pinpoint the exact source of lymphatic leaks. During this procedure, an MRI contrast agent (a safe, injectable dye) is injected directly into the lymphatic system and tracked by MRI. For Daniel, a lipiodol (special oil) injection was used to locally embolize the leaking areas in his lungs.

While this initial procedure reduced the flow of lymphatic fluid, the fluid continued to re-accumulate. The team knew that Daniel would need surgery, and they began to prepare Daniel’s parents. “The doctors explained that Daniel needed surgery and walked us through what that would involve,” says Mike. “They also discussed the surgery in detail the night before. During the procedure, we got hourly updates. We were told exactly what was going on, and where they were in the process.”

Daniel underwent a lymphovenous anastomosis (LVA) surgery for thoracic duct outlet obstruction with Pablo Laje, MD. In this complex surgery, the surgeon connects the thoracic duct (the main lymphatic vessel) to a nearby vein to recreate the main lymphovenous connection that was not open and restore the flow of lymphatic fluid back to the venous system.

Approximately nine hours start to finish, the surgery was challenging but ultimately successful. Daniel’s chest tube output reduced little by little every day, and he was eventually able to wean off of the ventilator. Within a month, Daniel was breathing comfortably without support and chest x-rays showed no sign of fluid in his lungs. His chest tubes were then removed, and he was able to return home with his family.

Now nearly eight months, Daniel follows up with the Lymphatics team every three months. Says Carrie, “Daniel was very, very sick. It was any parent’s nightmare. But we came out the other end.”

She adds:

“From the transport team to Lymphatics to the NICU doctors, nurses and staff — we cannot speak highly enough about CHOP. CHOP saved Daniel’s life.”