John Heller, Post-Gazette Briana Neenan with her daughter, Ana, at Children's Hospital, where doctors performed a surgery, the first in Western Pennsylvania, that allows her abnormal small intestine to digest food better. Click photo for larger image. Graphic: New surgical procedure

A girl who was born with a small intestine less than a tenth of the normal length had a novel surgery last month that has enabled her to gain weight and giggle at her mom.

It was the first time the operation has been done in Western Pennsylvania, said her surgeon, Dr. Ed Barksdale, co-director of the intestinal care center at Children's Hospital of Pittsburgh.

Briana Neenan, of Austin, Texas, found out when she was four months pregnant that the baby she carried had a gastroschisis, meaning the intestines protruded through the belly because of a hole in the abdominal wall.

"I was a nervous wreck," she said.

Ana was born six months ago, a month before doctors intended to do a cesarean section and more than six weeks before her official due date. Only about an inch of her bowel was exposed. Her mom was elated.

"No problem, woo hoo!" she figured. "Just cut that [belly] open, stick that sucker back in, let's go home."

But the surgeon discovered that her small intestine was not connected to her stomach nor to her large intestine, and although repairs were made, Ana's doctors were pessimistic about her chances.

The baby was soon receiving nourishment primarily through an intravenous line in a technique called total parenteral nutrition, or TPN, because she cannot get what she needs by eating. But TPN damages the liver with long-term use.

Last month, the Neenans went to Children's so that Ana could be evaluated for small intestine and liver transplantation.

"And Dr. Barksdale came up to me and said there's this new procedure he had and he wanted to try it on Ana," Miss Neenan recalled. "If there's anything I can do to make her better, whether it's 50 billion surgeries or not, I'm going to do it."

In gastroschisis, the intestine gets kinked and trapped in the abdominal defect, stunting its development, Dr. Barksdale explained. Babies are typically born with about 100 inches of small bowel; Ana's was 6 inches long.

The segment she had was enlarged and floppy, creating a "cesspool" as the surgeon put it, or bacterial breeding ground. Sometimes that germ overgrowth caused infections of Ana's intravenous lines. The abnormal bowel also hampered proper absorption of nutrients.

On July 14, Dr. Barksdale performed a serial transverse enteroplasty, or STEP operation, which was developed a few years ago by surgeons at Boston's Children's Hospital. Fewer than 50 have been done in the country.

With a surgical stapler, V-shapes are made in alternating sides of the intestine at regular intervals, creating a narrower, zig-zag pathway.

It has slowed the passage of food through Ana's small intestine, so she has fewer bowel movements, and she also has fewer infections. Her gut absorbs glucose better, in part because there are fewer bacteria to compete for it, he said.

Ana now gets about 70 percent of her calories through TPN. If that can be reduced to 50 percent, with the remainder going through the feeding tube in her stomach, then "we will have done well," Dr. Barksdale said.

"She's gaining weight on the same amount of calories she was on before so we think she's getting more out of her nutrition," he added.

Miss Neenan says her daughter is "doing awesome." The baby is eating more than she ever has through her feeding tube. Jaundice, which made her skin orange, has subsided.

"She has gone from just sleepy and tired to playful," the delighted mom said. "Now she giggles continuously. We can say, 'Where's Mama?' And she'll look at me and start laughing."

Ana has such a short small intestine, and her liver is already so damaged, that she will likely still need organ transplants, Dr. Barksdale said.

"We'll need to get her to about a year of age," he said. "This allows us a bridge to get there. Many kids with this short an intestine don't make it that far that they're candidates for transplantation."

Some children may be able to avoid a transplant with the STEP procedure, the surgeon said. The hospital's intestinal care center saw 47 children with short bowel syndrome last year, and six or seven of them might be candidates for the procedure.

He performed the STEP operation on another patient last week, and expected to do two more this week.

"In most centers in the country, they would see two to three patients a year who might qualify for this," Dr. Barksdale said. "We have the largest center of patients with short bowel syndrome in the world."

The Neenans went home a few days ago, but they'll be back for checkups every six to eight weeks.