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05.23.03
A STEP ahead
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sounds like a scene from a movie, but Heung
Bae Kim, MD, assistant in Surgery, really did sketch out the
idea for an entirely new surgical procedure on the back of a napkin.
The procedure, which lengthens the bowels of children with short
bowel syndrome (SBS), was something Kim had been thinking about and
sharing with colleagues since he was in medical school a decade ago. But
it wasn’t until he had a conversation with Tom
Jaksic, MD, PhD, associate in Surgery, that the idea became
reality.
SBS is a deadly disorder caused by surgical removal of part of the small
bowel or several diseases, the most common of which are necrotizing enterocolitis,
an infectious inflammatory disease of premature newborns; intestinal atresia,
a failure of development of a portion of the intestine; and intestinal
volvulus, which occurs when the bowel gets twisted and the blood supply
is impaired.
Regardless of the cause, the result is that the remaining bowel grows
wider, creating more surface area to absorb whatever nutrients it can,
slowing down food digestion, breeding bacteria and potentially causing
life-threatening infections. In addition, patients with SBS must get their
nutrients delivered directly into the stomach or small intestine via a
tube (a process known as enteral nutrition). In some cases this is not
possible and patients must get nutrition intravenously, which can cause
liver failure and the need for a liver transplant.
A surgery called the Bianchi Procedure, in which the bowel is cut in half
and one end is sewn to the other, has been used to treat the disease,
but the bowel often re-dilates, leaving patients in the same condition
as when they started.
The procedure that Kim and Jaksic developed, called serial transverse enteroplasty, or STEP, is based on the idea that stapling v-shapes into alternating sides of the bowel will decrease its width and increase its length. "I thought it would work because it fulfills all of the requirements of an ideal' bowel lengthening procedure, in that it lengthens the bowel, it tapers the dilated portions of the bowel, and it doesn't require removal of any bowel," says Kim. "It's also a simple concept, and simplicity of design is important in surgery."
After testing the procedure in the lab, the surgeons were ready to try
it on a patient in need of immediate help, and Alex Malo was just such
a patient. Born with a condition called gastroschisis—where a small hole
in his stomach caused Alex to be born with four feet of intestines outside
of his body—Alex underwent an unsuccessful Bianchi procedure in January
of last year, but still had to get all of his nutrients intravenously.
Jaksic and Kim knew they would need to act quickly so his liver wouldn’t
become irreparably damaged, so they approached Alex’s family with the
possibility of trying the STEP procedure.
The family eagerly agreed, and last February, Alex became the first person
to have the STEP procedure. He has done very well since. “Before the surgery
Alex was unable to get any nutrition enterally,” says Jaksic. “But now
he gets 60 percent that way and his liver is in perfect condition.” Post-surgical
studies have shown that food moves more quickly through the small bowel
and the nutrients are absorbed more completely, both of which lead to
less bacterial growth and healthier children.
“One of the great things about this surgery is that it can easily be taught
to other surgeons,” says Jaksic. After presenting the surgery at the Surgical
Section of the American Academy of Pediatrics, Jaksic and Kim have been
asked by surgeons from other hospitals in the United States and Canada
how to perform the operation.
Children’s surgeons have performed the operation on three patients since
Alex. And while the Children’s patients who have undergone the procedure
still receive much of their nutrition enterally, Jaksic says the goal
is that one day they will all be able to eat food normally for the first
time in their lives.—MC