UM Medical Center team separates conjoined twins

THE BALTIMORE SUN

An odyssey that began last fall with the delivery of conjoined twins in a hospital near the border of Uganda and Congo has ended happily with the separation of two healthy girls at the University of Maryland Medical Center.

The girls, Loice and Christine Onziga, sat wriggling and cooing in their parents' arms yesterday as surgeons described the intricacies of an operation that lasted 12 hours and involved 35 medical professionals.

"Today, my wife and I are very happy parents to see that both our daughters are alive and separate," said Gordon Onziga, who farms 6 acres in a village in Uganda.

Surgery on the twins - who were born attached from the breastbone to the navel and fused at the heart, liver and diaphragm - took place April 19 but was kept quiet until yesterday. Doctors said the 6 1/2 -month- old girls were thriving physically and emotionally; they seem happiest when placed in the same crib, where they can playfully paw at each other.

At a news conference yesterday, surgeons described the tense moment when they clamped a blood vessel that connected the sisters' hearts. It was then that doctors observed that the hearts could beat independently and would not require risky reconstructive surgery. Surgeons were able to complete the operation, confident that the sisters could survive apart.

"We expect them to live normal, healthy, productive lives as two individuals," said Dr. Eric Strauch, a pediatric surgeon who directed the operation with Dr. Marcelo Cardarelli.

Though the sisters still arch their backs slightly, a holdover from the days when they strained not to bump heads, doctors expect them to achieve a normal posture and eventually crawl and walk normally.

It could take another year or so for the sisters to catch up with other children their age, though doctors said they saw no reason why they would not develop normally on every level.

Doctors said yesterday that they expect to discharge the girls in several days to the nearby Ronald McDonald House, though they will probably remain in Baltimore for medical care until November.

The Onziga sisters' journey began in October when their mother, Margret, went into labor and prepared to deliver what she thought was a normal baby at her family's home in Congo, formerly Zaire. When she failed to progress, she was taken by bus to a hospital where two fused babies were delivered Oct. 28 by Caesarean section.

They were transferred to a regional hospital just across the border in Uganda and later to Mulago Hospital in the Ugandan capital, Kampala. There, the sisters came to the attention of doctors from the University of Maryland who were participating in an exchange program.

Because Uganda did not have facilities to perform the complex separation, the doctors worked through officials at the University of Maryland to have the twins flown to Baltimore for surgery. The sisters arrived Feb. 28.

On April 19 - in an operating room packed with surgeons, anesthesiologists, nurses, technicians, biomedical engineers and electricians - the girls were separated.

The operation was provided free to the family by the University of Maryland Medical System. Doctors said it would be months before they could tally the cost, which promises to run into the hundreds of thousands of dollars.

It was the second separation of conjoined twins at the University of Maryland and the fifth in Baltimore. Three have been performed at Johns Hopkins Hospital.

Conjoined twins occur about once in every 200,000 live births. The condition is actually more common than that, but most attached fetuses die in the womb. The condition occurs when identical twins fail to separate in the womb. The overall survival rate of conjoined twins is between 5 percent and 25 percent, with about 75 percent of surgical separations resulting in at least one twin surviving.

Though precise statistics aren't kept, some estimates place the number of surgical separations at about 200 worldwide, with most performed since 1950.

In this case, doctors at the Kampala hospital were reassured by results of a crude CT scan that a surgical separation could be successful. This was because each of the girls, though fused along the chest and abdomen, had a full complement of organs - though the connection between their hearts remained mysterious.

Dr. Cindy Howard, a UM pediatrician who visits the Kampala hospital two months a year, said she was intrigued when the hospital's pediatrics chief, Dr. Margaret Nakakeeto, told her about the unusual patients and asked for her help.

"She described them as gorgeous babies who seemed to have a chance," Howard recalled yesterday. "She said, 'These children can do well, as well as we can guess. Can you think about it?'"

Though she made no promises, Howard called Dr. Jay Perman, chief of pediatrics at the University of Maryland Medical Center. Perman, along with hospital officials, eventually arranged to accept the twins there.

With their babies wrapped in a blanket and Howard by their side, the parents made the long trip from Kampala to London and finally to Baltimore.

Once the sisters arrived in Baltimore, doctors performed ultrasound, magnetic resonance imaging and other tests that showed - to a high degree of likelihood - that each heart had four chambers. Though severing the artery presented some risk, surgeons decided it was a risk worth taking.

Aside from obvious social problems if left attached, it was doubtful that the sisters could survive without surgery, Howard said.

Loice's heart was pumping blood into Christine's, a Herculean task that left Loice's heart in danger of failing. Also, if one girl eventually developed an infection, the illness would most likely spread to the other and send the two into a dangerous spiral.

Although technically the dominant sister, Loice was smaller than her sister. At birth, the girls weighed a combined 6 pounds, with Christine about 25 percent larger than Loice. Even now, with a month to catch up after surgery, Loice weighs about 9 pounds to her sister's 11.

Several days before the operation, the surgical team made a practice run in the operating room to make sure all equipment was on hand and functioning properly, and that separate teams were assigned to each girl.

Once in surgery, the twins were placed on their sides. Surgeons cut into the abdomen and continued toward the chest, separating first the liver and later the diaphragm and heart.

When surgeons clamped off the vessel connecting the hearts, they were relieved to find that the two hearts pumped vigorously. If the hearts failed to do so, surgeons were prepared to place the girls on heart-lung machines and begin surgery to reconfigure the delicate organs.

"That would have been a real nightmare," said Dr. Bartley Griffith, chief of cardiac surgery. "The prognosis would have been poor, but we were prepared."

Once the separation was complete, the girls were moved to different tables where physicians and nurses began work to close up their abdomens and chests.

Sheets of synthetic material were used to reconstruct parts of the diaphragm, chest and abdominal walls, and skin was pulled over the surgical wound and secured. For each child, the area of attachment was about 6 inches long and 4 inches wide.

Twelve hours after the twins were wheeled together into the seventh-floor operating room, they were wheeled out on separate gurneys and into separate elevators, and taken two floors down to the intensive care unit.

The girls remained on ventilators for about 10 days and have experienced no serious medical problems, doctors said.

Although the girls, at times, struggled before surgery to move independently and roll over, they now seem to crave contact with each other. Doctors and nurses realized that the twins cried less if placed in the same crib - so for now, they spend most of their time together.

To stimulate the girls and aid in their development, nurses massage and talk to them while making direct eye contact.

Each girl was left with a slight opening in her heart, Howard said. While Christine's will most likely close on its own, Loice's could require medical attention. Doctors would hope to make the repair nonsurgically - through a catheter that is passed from the groin to the heart.

For those who have cared for the girls, the case of Loice and Christine Onziga has been a source of amazement.

"For a lot of physicians and nurses, this has been exceedingly rejuvenating," said Erin Guidice, a pediatric chief resident. "This is why we do what we do."

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