Tuesday, April 23, 2013
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Six-month-old conjoined twins from Franklin, Va., were surgically separated at Children’s Hospital of Richmond at VCU in a 14-hour operation on Monday that completed a series of procedures begun last year, including the division of their liver.
A’zhari and A’zhiah Jones are in stable condition in the Neonatal Intensive Care Unit, where they spent the first six months of their lives.
“We’re very optimistic that the twins will have a full and complete recovery,” said David Lanning, M.D., Ph.D., surgeon-in-chief at the Children’s Hospital of Richmond at VCU. “At this point we don’t anticipate any future operations or need for any long term medications. I see the girls living full happy lives as individuals.”
The complex, multidisciplinary procedure commenced Monday at 6:45 a.m. and was the second separation of conjoined twins at Children’s Hospital of Richmond at VCU in the last 18 months. In November 2011, Lanning and a large team of specialists successfully separated 19-month-old conjoined twins, Maria and Teresa Tapia of the Dominican Republic, in a complex 20-hour procedure.
The Jones twins, classified as thoracopagus, were joined at the chest and abdomen and shared a liver and the pericardium, the double-walled sac containing the heart and the roots of the great vessels.
Led by Lanning, the care team included nearly 40 physicians, surgeons, pediatric subspecialists, nurses, surgical technicians, occupational and physical therapists and ancillary staff members.
“While A’zhari and A’zhiah look similar to the Tapia twins from the outside, we were faced with a different set of challenges that we had to overcome as a team,” Lanning said. “Despite these differences, our experience with the Tapia twins has been vitally helpful with caring for the Jones twins.”
According to Lanning, while phased-separation surgeries of twins conjoined at the head and of twins conjoined at the hips have been successful, there are no published reports of successful, phased-separation surgeries of twins sharing vital organs and conjoined at the abdomen and heart like the Jones girls.
Nachell Jones was surprised at 12 weeks gestation to learn that she was having twins, and she said she was shocked at 13 weeks when she discovered through advanced sonography that her twins were conjoined. Jones was admitted to VCU Medical Center at 35 weeks for observation. On Oct. 10, at 36 weeks, Jones gave gave birth through a planned cesarean section to 10-pound (combined weight), 17-inch conjoined twins.
Five days after birth, CT scans were taken of the twins’ chests, abdomens and pelvises and reviewed by Laura Carucci, M.D., associate professor in the Department of Radiology and director of computed tomography at VCU Medical Center. Carucci was an integral part of the Tapia twins’ imaging studies in 2011. The scans revealed a conjoined liver and a shared pericardium.
The first stage of the phased separation began on Oct. 25 when surgeons separated the conjoined liver and closed the girls’ abdomens.
“As the girls became critically ill over the second week of their lives, we had to urgently separate their conjoined liver as this was the source of their uncompensated cross circulation. However, complete separation at that time would almost assuredly have resulted in their deaths as A’zhari was in renal failure and A’zhiah had severe cardiac hypertrophy. A phased surgery was the optimal plan,” Lanning said.
The planning and support extended beyond the medical community as several individuals and groups within VCU offered the twins their expertise. VCU’s Department of Fashion Design and Merchandising created dresses for the Jones twins. Additionally, VCU Department of Sculpture and Extended Media graduates and students worked to craft a cast model of the twins that plastic surgeons studied to ensure the appropriate size skin expanders were used to achieve closure after separation.
The girls’ health and development were closely monitored over the following weeks and months by numerous pediatric specialties including cardiology, neonatal medicine, nephrology, gastroenterology, radiology, surgery and transplant surgery, nursing, and physical and occupational therapy. Four months later on Feb. 14, surgeons placed tissue expanders in the twins’ abdomens. The balloon-like expanders enabled the growth of excess skin to be used for closure and reconstruction following surgery.
In the months leading up to the final separation, the surgical team carefully planned the step-by-step operating timetable, which included reviewing imaging tests and setting specific times for the various aspects of the surgery. The team also designated separate colors for the bandages and tubing that would belong to each child. A’zhari was given pink and A’zhiah blue.
“We made the decision to proceed with the final separation once the girls gained weight and their renal and heart problems resolved,” said Lanning. The risks of not separating the girls included difficulty feeding and possible aspiration.
A team of seven anesthesiologists monitored the girls’ vitals and managed pain control throughout the surgery. Following separation, A’zhari was moved to another operating room and teams of surgeons began the final reconstruction of the girls’ abdomens. Following reconstruction, the girls were transported to separate rooms in the Neonatal Intensive Care Unit, where they will recover with the help of the same nurses who have cared for and watched them grow over the past six months. The girls eventually will be reunited in one room.
“The nurses are very caring and like my family,” said their mother, Nachell Jones. “They are very supportive of me and the girls. It’s like they are their own kids.”
“I am humbled by the unwavering dedication and collaboration of our surgeons, physicians, nurses and entire medical team,” said Leslie G. Wyatt, R.N., vice president of children’s services and executive director of Children’s Hospital of Richmond at VCU. “This complex, multifaceted and phased separation surgery speaks volumes to the depth and breadth of services and expertise available at CHoR. A mother can now breathe a sigh of relief that her daughters will live full, happy and healthy lives.”
“It has been one hard journey, like a roller-coaster,” Jones said. “Some days it’s great and other days it’s hard. Today is one of the great days. They are my little miracles.”
About Children’s Hospital of Richmond at VCU
Children's Hospital of Richmond at VCU (CHoR) is Central Virginia's only comprehensive, full-service hospital dedicated to the care of children. CHoR is ranked among the nation's top children's hospitals by U.S. News and World Report and is Virginia's only Level 1 pediatric trauma center verified by the American College of Surgeons. With more than 15 locations across Central Virginia, CHoR provides pediatric inpatient and outpatient services that cover nearly all children's health-related needs. As part of VCU Health, CHoR is committed to ensuring access to care for all children, training future pediatric caregivers and making new discoveries that improve understanding and treatment of childhood diseases. For more, see chrichmond.org