Saving Luigi

Family journey from Brazil to Chicago highlights need for cultivating surgical expertise in portal vein thrombosis 

Clad in matching camo pants and jacket, clutching a toy under one arm, Luigi Valentini sits quietly on his mother’s lap in one of Lurie Children’s phlebotomy labs. His big green eyes closely follow the nurse’s every move as she scrubs his forearm with rubbing alcohol, then pulls a tourniquet, a tube and a needle. As the needle plunges deep into his vein, Luigi, not quite 4 years old, is the embodiment of calm. 

The improbable stoicism is the likely result of Luigi’s repeated encounters with doctors and nurses since his diagnosis two-and-a-half years ago with portal vein thrombosis (PVT). 

Luigi was born a perfectly healthy baby, so when his pediatrician detected a slightly enlarged spleen during the 1-year check-up, his parents, Bernardo and Ana Paula, were not particularly alarmed. But an ultrasound a few days later brought an unsettling revelation: The portal vein, which feeds blood and nutrients from the intestines into the liver, was clogged by a massive clot that caused it to swell and throb with high pressure. 

In children, PVT can stem from blood-clotting disorders, intraabdominal abscesses or as a complication from cardiopulmonary bypass. Most of the time, it is idiopathic. 

A pediatric gastroenterologist in Brazil told the Valentini family a sit-and-wait approach was their best — indeed, their only — choice. But six months later, the boy developed esophageal varices, a common complication of PVT and the portal vein hypertension that it invariably causes. As the family sought second, third and fourth opinion, one esteemed physician after another told them nothing could be done. Then Luigi’s mother went online. One name kept popping up over and over again: Riccardo Superina, a pediatric transplant surgeon at Lurie Children’s and a world renowned authority on pediatric liver diseases. For nearly two decades now, Superina has performed the meso-rex bypass, a procedure to rebuild hepatopetal blood flow. Done properly, in a carefully selected subset of children, the approach can be curative.

The Valentinis’ experience illustrates an all too common scenario, Superina says. Families are often told their only option, is to follow the condition closely to reduce risk for complications. There’s also the misconception that kids may outgrow PVT. They don’t, Superina says. The sit-and-wait approach involves frequent blood draws, periodic endoscopies, variceal ligation and possible spleen removal. This strategy, Superina says, relegates a child to a medicalized existence and does nothing to avert the long-term metabolic and physiologic sequelae of liver disease.

Without treatment, children with PVT progress to portal vein hypertension. The spleen continues to enlarge. The liver — starved of blood and nutrients — shrivels. Some patients develop pulmonary hypertension, which, in turn, stresses the cardiac muscle and can lead to ventricular hypertrophy. Some patients progress to hepatic encephalopathy, marked by learning and behavioral problems. 

“Restoring blood supply into the liver can reverse the metabolic and physiologic consequences of portal vein hypertension, including pulmonary hypertension,” Superina says.

But the meso-rex bypass is not suitable for everyone. Its success depends on having well preserved veins in and around the liver, particularly a robust intrahepatic portal vein. Superina estimates that 80 percent of children with PVT are good candidates for the meso-rex bypass. The rest can undergo a spleno-renal shunt, in which the splenic vein is detached from the portal vein and attached to the left renal vein. A venogram performed by a trained pediatric interventional radiologist can pick out the less than ideal candidates with nearly 100-percent predictive accuracy. 

On Feb. 29, 2016, Luigi underwent a six-hour operation. The procedure, Superina says, worked beautifully. He anticipates that Luigi’s spleen will shrink back to normal size and his liver will regain its function. 

“This is not a trivial disorder and kids don’t just outgrow it,” Superina says. “With good pre-op assessment and careful patient selection, surgery procedure can be curative nearly 100 percent of the time.”

About the Rex Shunt

The meso-rex bypass, often called the Rex shunt, was developed in the late 1990s in Europe by Belgian surgeon Jean de Ville de Goyet, a colleague and friend of Superina’s. Captivated by the idea, Superina asked de Goyet to send him a sketch of the technique. Superina performed his first meso-rex bypass following a hand-drawn diagram that de Goyet had emailed him. It worked and so the Rex shunt made its debut in North America. 

Since 1997, Superina has performed more than 200 such procedures at Lurie Children’s, making this the largest pediatric case series in the country and, possibly, the world. 

It’s a delicate procedure, tricky to perform even for experienced adult surgeons who venture to operate on children. 

“The liver is temperamental organ, highly vascular, so the margin of error is even narrower in children than it is in adults,” Superina says. 

This is also why, only a handful of pediatric surgeons in the United States dare foray into it, he says. 

To ensure that pediatric cases are treated by pediatric surgeons in pediatric centers with multi-disciplinary expertise, Superina is grooming the next generation of homegrown specialists at Lurie Children’s. 

“Philosophically, as pediatric surgeons, we owe it to our patients to treat this disease,” Superina says. 

To help spread his expertise beyond borders, Superina makes frequent visits abroad to perform cases in countries including Israel, China, Nepal and Uzbekistan.

Superina is also collaborating with fellow scientists from nearby Northwestern University’s Feinberg School of Medicine as part of a group working to create lab-grown veins using patients’ own stem cells. These “designer” veins, Superina says, could be used to reconstruct the vessels around the liver, eliminating the need to use portions of the jugular vein.​