One girl survives separation surgery

Twins: The heart of a 13-month-old who was born joined to her sister at the head, is unable to withstand the rigors of the complicated operation.

September 17, 2004|By Erika Niedowski | Erika Niedowski,SUN STAFF

Working late into the night in a crowded O.R. outfitted with a specially designed rotating operating table, doctors were within an hour or two of separating conjoined twins Lea and Tabea Block when things started to go wrong.

Tabea's heart was beating irregularly, too slow to pump enough blood through her 13-month-old body. The medical team needed to accelerate the pace of their work - or both sisters might die.

"It became clear that we did not have very much time," Dr. Benjamin S. Carson, head of pediatric neurosurgery at the Johns Hopkins Children's Center, said yesterday at a news briefing at the hospital.

By 12:15 a.m. yesterday, the twins, who were born connected at the top of their heads, were separate for the first time.

But as one team of doctors worked to replenish blood that Lea had lost, close her skull and sew up her scalp, another undertook an exhaustive effort to restart her sister's now-stopped heart.

"It was not possible to really resuscitate that heart at that point," said Carson.

"Their anatomy was extremely complicated," he said of the twins. "So we knew that it was going to be an enormous challenge to separate them."

The girls' parents, Nelly and Peter Block, who arrived with their daughters in the United States in May, made no public statements yesterday. But several doctors at the Hopkins briefing, including one who traveled to Baltimore from Germany to offer his support, said they were strong people with a deep religious faith.

"Please tell the team that we appreciate all the efforts the team made for our children," they said, according to Dr. Tilman Polster of Bethel Hospital in Bielefeld, where the twins had received medical care.

Polster said the Blocks saw Lea in the intensive care unit shortly after her surgery ended. Carson said the girl was "quite stable," with a normal heart rate and blood pressure, and she had even tried to open her eyes.

Good chance to recover

Barring complications, which could include infection or brain swelling, she is expected to recover, although she will require several cranial reconstructive surgeries. The first of those could occur as early as next week.

Carson first learned of the twins from their pediatrician, before they were born. Hopkins later assembled a medical team - which included 17 neurosurgeons, 14 anesthesiologists, five plastic surgeons and more than 40 nurses, among others - and spent months preparing for the surgery.

They conducted imaging studies, examined computerized anatomical models of the girls' brains and even performed a surgical dress rehearsal just before Labor Day.

The hope was to separate the twins in a single marathon procedure that began Saturday and was expected to last between 24 and 48 hours.

But, 7 1/2 hours into the surgery, after surgeons had peeled back the thick outer covering of the girls' brains, opened their shared skull and begun separating the major blood vessels that were tangled together, Tabea went into cardiac arrest.

She nearly died that night.

"She suffered a lethal arrythmia that we were able to resuscitate her from," said Dr. Deborah Ann Schwengel, a pediatric anesthesiologist at the Children's Center.

But the setback meant halting the separation, at least temporarily. Working quickly, the surgical team closed the incisions in the twins' scalps and moved the girls into the pediatric intensive care unit.

Doctors found that Tabea's heart still wasn't contracting properly and, for a time, Carson said, they thought they might have to perform an emergency separation to save Lea.

Finally, the little girl's condition stabilized.

Waiting, watching

For several days, the twins remained in the ICU, and doctors monitored their condition to determine when they would be strong enough for surgery. By Wednesday, the team decided, they were.

Surgeons worked throughout the day and into the night to tease apart brain tissue, millimeter by millimeter, and separate the dural venous complex, a mass of blood vessels at the back of their heads.

The separation was nearly complete when Tabea's heart problems began. She died about an hour after the final separation cut was made.

Carson - who, characteristically, resumed his regular operating schedule yesterday - said he would do one thing differently if he could do it again: He would perform a so-called cranial expansion on the girls, to create more room for their brains, and then wait several weeks or even months before the separation. That could have lessened the adhesion between the brains.

"Their brains were very, very pressed together," he said.

Odds were against them

Twins connected at the head - known as craniopagus - are the rarest kind of conjoined twins, occurring in one out of every 2 million live births. One of the oldest known living pairs, Lori and Reba Schappell of Pennsylvania, who share about a third of their brain tissue, are in their early 40s.

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