Fresh Air: Lurie Children’s Surgeon Helps Florida Boy Breathe on His Own

September 17, 2019

Liam’s prekindergarten classmates can barely keep up with him, said his mom, Lexy. The 4-year-old loves gymnastics, swimming and has what Lexy calls a “photographic memory,” reciting parts from his favorite books during story time.

Liam’s energy and smarts delight his family because just two years earlier, he couldn’t breathe without supplemental oxygen as he faced a daunting diagnosis.

When Liam was two, he was diagnosed with a congenital portosystemic shunt, a rare condition called the Abernethy malformation. A portosystemic shunt causes diversion of venous blood flow originating in the intestines away from the liver, rather than through it.

Some patients with the condition remain asymptomatic until they are older, but others like Liam are seriously ill at a young age.

The Abernethy malformation in rare instances can lead to hepatopulmonary syndrome with abnormal shunts in the lungs, like Liam had, requiring oxygen therapy. When the condition progresses, it can cause deficits in brain function, pulmonary hypertension and, ultimately, heart failure and death.

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The CT scan on the left shows Liam’s liver with no obvious portal vein due to the Abernethy malformation, or a congenital portosystemic venous shunt. On the CT scan to the right, a yellow arrow points to the portal vein that Dr. Superina enlarged by closing the shunt during two surgeries. The portal vein is essential for blood flow through the liver.

Finding Treatment Across State Lines

Lexy said her family, who lives in St. Petersburg, Florida, had never heard of Ann & Robert H. Lurie Children’s Hospital of Chicago. But it is home to one of the few pediatric surgeons in the world, Dr. Riccardo Superina, who could perform the surgery Liam would need.

Fortunately, Lexy’s coworker has a relative who works at Lurie Children’s and helped connect the family with Dr. Saeed Mohammad, medical director of hepatology and liver transplantation at the hospital, who referred them to Dr. Superina.

“On the day I confirmed with my friend that I would like to talk with the physician at Lurie Children’s who could help my son, I found myself receiving a phone call from him!” Lexy said.

A month after the phone call, the family was traveling more than 1,000 miles from Florida to Chicago for surgery.

When Liam’s parents met with Dr. Superina for the first time, they were stunned by his approachability and demeanor. “He is simply an amazing doctor and person,” said Lexy. “He patiently listens to your concerns and answers questions in plain terms. He never makes you feel ‘small’ for asking a question. We think the world of him.”

Liam underwent two surgeries scheduled five days apart in which Dr. Superina corrected the circulation problem around Liam’s liver by closing the shunt in a staged manner. He closed the shunt halfway during the first surgery, diverting blood flow toward Liam’s dysfunctional and smaller-than-normal portal vein.

The five days in between the surgeries allowed time for his portal vein to naturally expand, so that when Dr. Superina closed the shunt completely during the second surgery, Liam’s blood was forced to flow through the liver through the portal vein, as it was meant to.

Ideal Recovery

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Liam no longer needs supplemental oxygen thanks to Dr. Superina and two surgeries that corrected Liam’s congenital portosystemic venous shunt.

Liam remained intubated for a week following surgery so his liver could heal. After five long days for his parents, Liam was removed from oxygen and Lexy and husband Ryan could finally hold their son. He was able to go home soon after, and about a month later, his pulmonologist, or lung doctor, canceled the order for medical oxygen. Liam no longer needed it.

“We were overjoyed,” Lexy said. “He exceeded our expectations.”

Now, Liam returns to Lurie Children’s just once a year for checkups, which are often a joyful time with Dr. Superina and staff. Liam spends most of his time at school and playing his favorite sports with friends.

“He couldn’t be doing any better,” said Lexy. “It’s just incredible.”

Learn More About Lurie Children's Siragusa Transplantation Center