Article from: Summer - 2019



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    By Holly Edmiston

    Making Mandy Well

    Amanda’s hospital stay

    Above: Amanda’s hospital stay left the Smith family with countless mementos — photo albums, gifts from visitors and handwritten notes from loved ones.

    Amanda Smith’s recollections of a surgery she had at age three are what one might expect from a child — a long stay in the hospital over Christmas, her mom at her side, a small stuffed reindeer with a red bow.

    For her parents, Terry and Kathy Smith, the memories are more fraught. Their usually talkative, active toddler had suddenly become subdued and listless. They spent a week believing she had cancer, were relieved to find out she did not, and then held their collective breath as Mandy underwent an untested and innovative surgery.

    Focal Spot’s summer 1988 issue

    Above: Amanda’s puzzling illness and revolutionary treatment landed her on the cover of Focal Spot’s summer 1988 issue.

    A few years after events unfolded, Amanda’s diagnosis, treatment and recovery were chronicled in a Focal Spot cover story:

    “Amanda Smith, then just three years old, came to St. Louis Children’s Hospital jaundiced and obviously very ill. Amanda’s pediatrician suspected hepatitis — a diagnosis that early blood work failed to support.

    The real trouble was neither so obvious nor so commonplace. By the time Mandy finally left the hospital 35 days later, she had toughed her way through two major surgeries and had made medical history.”

    Summer 1988
    Focal Spot

    Her first ultrasound exam at Children’s Hospital was conducted by Gary D. Shackelford, MD, now professor emeritus of radiology at MIR. Shackelford recalls Amanda’s case as one of the most memorable of his career.

    “It was the coming together of Mandy, as she was known then, being a delightful child, the complexity of her case, along with the thrill of working closely with Jessie Ternberg,” he says.

    Jessie L. Ternberg, MD, who died in 2016, was a renowned Washington University School of Medicine pediatric surgeon, respected by her colleagues and beloved by her patients. After ultrasound scans identified an enlargement on the head of Amanda’s pancreas, Ternberg performed an exploratory surgery. The intent was to locate what pathology had determined was likely a malignant tumor and then perform a Whipple procedure — an operation that removes the head of the pancreas, the first part of the duodenum, the gallbladder and the bile duct.

    Ternberg Calls an Audible

    Instead, based on what she felt was “a lack of certainty” in the pathologist’s report, Ternberg made the “courageous and correct” decision, as Shackelford terms it, to end that first surgery after removing only a tissue sample of the mass for further study.

    Amanda’s ultrasound

    Above: An early clue in unraveling the mystery of Amanda’s illness was this ultrasound image of her liver.

    Over the next eight days, more tests and additional stains were done on that tissue. It was a long week for the Smiths, who believed that their young daughter had cancer.

    By this time, Amanda was a bit of star at the hospital. In what ended up being a 34-day stay, a record at the time, she had amassed quite the roster of local celebrity visitors. St. Louis Cardinals outfielder Andy Van Slyke and legendary shortstop Ozzie Smith stopped by, though Amanda was most excited to meet team mascot Fredbird. St. Louis Blues player Brian Sutter gave her a signed puck, and even Santa Claus made an appearance.

    “There I was, holding Mandy in a rocking chair, with six tubes coming out of her, and along comes Brian Sutter and one of his brothers,” says Terry Smith, who notes those visits were bright spots in some dark days. “We were very down, and then several days later Dr. Ternberg said things were looking a little better; they weren’t 100% sure the tumor was malignant.”

    Finally, the family received the good news they’d been waiting for: the tumor was benign. The enlargement, as it turned out, was the result of bile ducts in the liver not draining properly.

    In addition to ultrasound, Shackelford and his radiology colleagues had used computed tomography (CT) to confirm the enlarged head of Mandy’s pancreas. “We did not have MRI at the time,” he says. “We identified this discrete mass, but it was not a ‘slam dunk’ cancer.”

    Amanda and Family

    Above Amanda’s parents, Terry and Kathy Smith, were by her side during her 34-day stay at St. Louis Children’s Hospital. These days the family bonds over vintage Chevrolet Corvettes and antiques.

    An Unprecendented Procedure

    Eventually the mass was classed as a hemangio-endothelioma, an extremely rare, vasoactive tumor about which little was known. Once Ternberg knew for certain the tumor was benign, she was ready to go in again and “reroute Mandy’s plumbing” in a way that had never been done.

    Ternberg undertook a complicated operation that included two procedures that had not previously been combined in a single patient. First, she reestablished flow from the gallbladder by opening its tip directly into the duodenum, bypassing the duct that was blocked by the mass. Then, to drain the pancreatic enzymes, Ternberg resected the tail of the pancreas and hooked it into a loop of the small bowel. The pancreas then drained in a reverse fashion in what’s known as a Roux-en-Y anastomosis.

    None of Amanda’s physicians knew what to expect of the tumor that had been left undisturbed during the surgery. Some suspected it might have been growing slowly since birth and worried that it would continue. To monitor it, she returned to Children’s Hospital for regular imaging until she reached her 21st birthday.

    Although the tumor did not grow and eventually dissipated in the years following the surgery, Amanda’s singular anatomy, courtesy of Ternberg, remained of interest. “Other doctors always wanted to see it,” she says, “because it was so unusual.”

    Gary D. Shackelford, MD

    Above: Gary D. Shackelford, MD, professor emeritus of radiology at MIR, recalls Amanda’s case as one of the most memorable of his career.

    Shackelford continued to conduct follow-up ultrasound exams on Amanda for several years after her surgery, but he eventually fell out of touch with her and her family. “To learn that she is now a happy and thriving adult really warms my heart,” he says. “I’m thrilled that Mandy is doing well.”

    Another thing Shackelford remembers fondly is the close relationship that existed at the time between other clinicians and radiologists. It was a normal occurrence for a clinician to come to the radiology reading room to discuss a case, he says.

    Although interaction among physicians may happen differently today, Shackelford says the biggest change in approaching a case like Amanda’s would likely be the use of MRI, which was not available at the time of her diagnosis. Back then, even CT was only about 10 years old as a mainstream imaging technology, he says, and to have had MRI would have been game-changing.

    Vincent M. Mellnick, MD, associate professor of radiology and chief of abdominal imaging at MIR, agrees with both of those assessments. There’s no doubt things being digitized with PACS (picture archiving and communication system) has decreased the number of daily conversations with surgeons in the reading room, he says. However, he adds that subspecialization actually has led to more consultation, just in a different form.

    “A case like Mandy’s would be discussed in a weekly multidisciplinary conference today,” Mellnick says. “I think surgeons still find our opinions valuable; it’s just more formalized.”

    As for MRI, even when it became popular in the 1990s, it was mostly used for the brain and not as widely for the liver and bile ducts, Mellnick says. “Today, body MRI has definitely become the main modality for imaging bile ducts and pancreas,” he says. “And not only have other modalities, such as PET and CT, advanced — with more sophisticated machines and greatly increased speeds —but combined PET/MRI allows us to combine two modalities that give a very accurate picture of both the anatomy and function of the area being scanned.” And all of these modalities, he notes, use tracers to clearly show the difference between healthy and diseased tissue.

    Jessie L. Ternberg, MD

    Above: Instead of performing the scheduled Whipple on Amanda, pediatric surgeon Jessie L. Ternberg, MD, (below, circa 1988) decided to take a tissue sample of the mass for further study.

    Depending on the symptoms and the age of the patient, diagnosis today for a child like Amanda would still likely start with an ultrasound scan to examine the bile ducts, says Mellnick, because ultrasound doesn’t use radiation and can easily be redone if the child moves during the scan.

    After the source was identified, MRI or CT would often be used. And, as in Amanda’s case, a biopsy would likely be performed. But instead of a full-on exploratory surgery, this would be accomplished with imaging guidance in a noninvasive way. If the mass were found to be benign, next steps would be different for children and adults.

    In an adult patient, the duct might be stented and then repeatedly imaged to monitor any growth. Eventually, if it was needed to relieve symptoms, a surgeon might go in to resect the mass and create a new conduit for bile to flow into the bowel.

    With children, it might be handled a little differently. “We are not as knowledgeable about what would happen long-term with these types of tumors,” says Mellnick. “To resect them is more technically difficult in a child, and they would have to live with the altered anatomy so much longer than an older patient. That means there would be a higher likelihood of complications and the need for additional procedures down the road.”

    Happy and Healthy 30 Years Later

    It’s fair to say Amanda has been lucky. In the more than 30 years since her second surgery, she has experienced no adverse symptoms or complications, even during her self-described “tomboy” years when she was encouraged to slow down and not overexert, lest she damage her restructured internal organs. But she’s aware that she could at some point experience a complication.

    “I do worry sometimes that it might come back,” she says, “but I feel fine.” In fact, Amanda has no recollection of her surgeries, and the only pain she remembers is the IV in her hand and when nurses removed the tape from her staples, leaving a scar she and her family have long referred to as her “upside-down frown.”

    “We can’t thank the hospital and Dr. Ternberg and MIR enough for all of the expertise they had,” says Terry. “We did not know what was going on, and they didn’t either for the longest time. But no one gave up.”

    Gary D. Shackelford, MD

    Above: Shackelford (second from right) fondly remembers the close relationship between radiologists and other clinicians.

    Vincent M. Mellnick, MD

    Above “Subspecialization actually has led to more consultation, just in a different form,” says Vincent M. Mellnick, MD, chief of abdominal imaging at MIR.