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Separation Ordeal

How Maryland Helps
Conjoined Twins Win Independence

Christine and Loice Onziga did not have an easy entrance into this world. When their mother, Margret, went into labor, unlike a normal delivery, the head didn’t crown. Instead, three feet appeared. Not only were these babies breech, they were also conjoined.

On October 28, 2001, in an African hospital in the Democratic Republic of the Congo, Christine and Loice were delivered by Caesarean section. Connected from the chest to the navel, what the future would hold for these girls no one knew.

Almost six months later, on April 19, 2002, far from Africa, an extraordinary team at the University of Maryland Medical Center successfully separated the pair, giving them a future full of possibilities. Sixteen years had passed since a set of conjoined twins had been separated at the University of Maryland.

Cindy Howard, MD, clinical assistant professor of pediatrics at the medical school, along with then senior pediatric residents Sherri Shubin, MD and Sue Rhee, MD, were the first Maryland physicians to meet the Onziga twins. The doctors were in Uganda’s capital city of Kampala at Mulago Hospital as part of an exchange program between Maryland and Makerere University Medical School.

The Onziga Family At this same time, the newborn twins were in the Ugandan hospital’s neonatal intensive care unit. They had been brought to Kampala shortly after they were born. They appeared healthy, and with the technology available, the doctors in Africa determined that Loice and Christine had their own hearts, a good indication that the girls could be separated. The physicians, however, could not assess whether they had separate livers.

Drs. Howard, Shubin, and Rhee quickly bonded with the girls. Their only hope for a long and healthy life was to be separated, I says Dr. Howard, who led the campaign to bring the twins to Maryland for surgery.
Almost four months after the girls were born and after the medical center and the school agreed to care for them at no charge, Christine and Loice Onziga began the long journey from Kampala to Baltimore. They had already traveled hundreds of miles in Africa. Now, along with Dr. Howard, Heather Symons, MD, another senior pediatric resident, and their parents, Margret and Gordon, they would travel thousands of miles more to Maryland.

Assembling the Team
Shortly after arriving in Baltimore, the family settled into the Ronald McDonald House and quickly learned the route they would repeatedly walk to the hospital. Their very first appointment at the hospital, just two days after coming to Baltimore, was with pediatric surgeon Eric Strauch, ’88, assistant professor of surgery. When I saw them, I thought: ‘Wow, the girls are here, and we really are going to do this!’ says Dr. Strauch.

In the weeks leading up to the surgery, the girls would be seen by many more physicians, nurses, medical students, residents, therapists, and other staff members at the medical center.

The growth and nutrition team, led by Maureen Black, PhD, professor of pediatrics, ensured that the twins were gaining weight. Everyone agreed Loice looked tiny compared to her sister. In mid-March, their combined weight was 7.68 kilograms or 16.9 pounds. Although they were progressively gaining weight, Loice needed to put on more weight before she would be ready for surgery. Just before the operation, the girls weighed a total of 9.0 kilograms or 19.8 pounds.

The radiology team, led by George Gross, MD, professor of diagnostic radiology, used MRI, scans, and the most advanced technology to determine what the girls looked like on the inside.

Loice and Christine were joined from the breastbone to the navel. This type of connection is classified as
thoracopagus and is the most common connection in conjoined twins. Each girl appeared to have her own spleen, kidneys, and gallbladder. They did, however, share a diaphragm, sternum, chest wall, and abdominal wall. They had a fused liver, and their hearts were connected by a muscular tube. Doctors were concerned about the heart because Loice and Christine did not have separate heart beats.

"None of the imaging tests we performed before surgery could tell us exactly what we would find. It was only after we got inside that we learned that the girls shared a large vein in the heart," says Marcelo Cardarelli, MD, assistant professor of surgery.

On the day of the surgery, it took more than six hours for pediatric anesthesiologists Anne Savarese, MD and Monique Bellefleur, MD, both assistant professors of anesthesiology, to prepare the babies for the separation. "It was an exceedingly challenging surgery since we needed to care for two patients sharing some of the same internal systems," says Dr. Savarese, director of pediatric anesthesiology at the University of Maryland Hospital for Children.

By early afternoon, the girls were anesthetized. Dr. Strauch and the team were then able to make the first incision through their common abdominal wall. They then started separating the girls’ liver. They continued upward to the chest. The hearts and the connecting vessel were separated last.

"We first had to clamp the vein connecting the hearts as a test to see if their blood pressure and oxygen levels would remain steady and assure that both hearts would continue at normal rates and rhythms. Those little hearts continued beating as if it was their inherent right," says Bartley Griffith, MD, chief of cardiac surgery and professor and head of the medical school’s division of cardiac surgery.

Less than four hours after the first incision, the girls were separated. Loice and Christine were transferred to separate operating tables where the physicians and nurses began the process of closing their abdomens and chests. Plastic surgeon, Bradley Robertson, MD, assistant professor of surgery, used a synthetic material to reduce the remaining tension, allowing him to close each girl’s diaphragm, chest, and abdominal walls.

"Thank goodness we had a dress rehearsal for the surgery. There were so many people in that operating room, but it was well choreographed. Everyone knew exactly what to do, and it never felt like a crowd," says Dr. Cardarelli.
There were roughly 35 people in the operating room at all times. All persons, including the physicians, nurses, technicians, biomedical engineers, security personnel, and facility engineers, offered their expertise during the surgery at no cost to the patients. "It was very impressive to see the skillfulness and enthusiasm of everyone involved," says Jay Perman, MD, chief of the University of Maryland Hospital for Children and professor and chair of pediatrics at the medical school.

The Surgery

Once the surgery was over, the girls were taken to the pediatric intensive care unit where, initially, they were under the watchful eye of Courtney Robertson, MD, assistant professor of pediatrics. They remained in the PICU and the intensive care’s step-down unit for over a month. They were then transferred to the B Wing of the Hospital.
Dr. Strauch worried more about the recovery than the surgery. "We needed to watch the patients closely for infection. Those first 72 hours after the surgery were the most critical," he says.

Second Time Around
Loice and Christine are the second set of conjoined twins to be separated at the medical center. In June 1986, a 24-member team led by head professor of pediatric surgery, J. Laurance Hill, MD, successfully separated two month-old Baltimore girls, Ciera and Tiera Bennett. These sisters were also thoracopagus-type twins. Just like Christine and Loice, they shared a liver, diaphragm, chest wall, heart sac, and a vessel.

"The most exciting part of the surgery was making that first incision, but the most vivid memory of the surgery was the heart beating outside the chest wall," remembers Dr. Hill. Because of the difference in size and oblique position of their hearts, one of the girl’s hearts protruded out of her chest.

"Every time I tried to push it back in the chest, the heart would fail," adds Dr. Hill. Using ingenuity, he was able to position a sheet of plastic material in a tent shape over the heart and gradually fold the tent pushing the heart back into the chest.

Tiera and Ciera’s surgery was videotaped and proved to be a good teaching tool for the team separating Christine and Loice. Both surgical teams helped prepare for surgery with baby dolls sewn together at the chest. The dolls aided the doctors in determining how many intravenous lines were needed and where they should be placed.

The Road to Recovery
Ten weeks after the surgery, Christine and Loice returned to the Ronald McDonald House, but they continued to make frequent visits to the medical center throughout the summer and fall. It was not unusual to see Gordon pushing the double stroller with the girls and their many toys through the revolving door at the hospital.

Loice and Christine saw specialists from pulmonology and gastroenterology. They also saw the pediatric residents in the pediatric ambulatory center for their well child check-ups.

Soon after the girls were on the road to recovery, they began working with physical therapist, Elizabeth Cross, MSW, PT. She would have two major challenges: the girls’ backs were hyper-extended, and they could not sit up. Both of these conditions interfered with normal gross motor development.

Ms. Cross fitted the girls with back braces to be worn at night. During the day, at least three times a week, she worked with the girls, stretching tight muscles and strengthening the weak ones. "My ultimate goal was to have Loice and Christine walk into their village, holding the hands of their mother and father," says Ms. Cross. "I remember seeing the girls working with Elizabeth, and I was so pleased because they had made such progress," recalls Dr. Strauch.

At the same time the girls were undergoing physical therapy, the growth and nutrition team continued to make certain that the girls were being properly nourished. Loice needed to gain enough weight for pediatric cardiologist, Jon Love, MD, to be able to repair two small defects in her heart.

On the twins’ first birthday, October 28, Loice was taken into the pediatric catheterization lab where Dr. Love, an assistant professor of pediatrics, would attempt to close an atrial septal defect (ASD) and a patent ductus arteriosus (PDA). Loice was just too small for Dr. Love to be able to repair the ASD, a hole in the upper chamber of her heart; but using a coil, the cardiology team was able to close the PDA, an open duct between the aorta and pulmonary artery.

Five days later, on November 2, 2002, Christine and Loice returned to Uganda accompanied by Margret, Gordon, Drs. Howard and Love, and Ms. Cross. Nine months earlier, Christine and Loice had arrived in Maryland with an uncertain future. Now, the healthy girls headed home just days away from taking their first independent steps. They were developmentally right on target.

Maryland’s medical center and school spent the better part of a year caring for the twins. The physicians, nurses, and therapists who watched the girls grow and thrive felt proud of their progress and knew that it was time to relinquish their care and development to another community that would raise them in Africa.

"There was a great esprit de corps during the nine months that the girls were here," recalls Dr. Howard. "On more than one occasion, someone said to me that they were proud to be part of the University of Maryland and the team that cared for Loice and Christine."

Ajonye Christine and Senema Loice are well on their way to leading normal lives and fulfilling the meanings of their African names, "the most beautiful" and "the most special."

By Alexandra Bessent

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