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STEP procedure for short bowel syndrome
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
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
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