Liver transplantation
Mother-to-infant surgery highlights Liver Program
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Heung
Bae Kim, MD,
pedriatic general surgeon.
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When 6-month-old Adara Lucia Henriquez and her family arrived
at Children's Hospital Boston from Panama, she was too small and
too fragile to have the liver transplant she desperately needed.
Under the direction of Maureen
Jonas, MD, medical director of Children's Liver
Transplant Program, nurses and physicians worked diligently
for three months to improve Adara's condition enough so she could
endure the procedure. Adara needed the liver transplant because
she suffered from biliary atresia, and toxins were building up
and damaging her liver. She subsequently developed cirrhosis in
her liver. Although she had surgery shortly after birth in Panama,
the operations did not fix her problems, and her postoperative
course was complicated by infections and malnutrition.
[see
video clip]
Adara's family raised sufficient funds to travel to the United
States for advanced medical treatment, but at less than 10 pounds,
she faced a common problem in pediatric patients: the limited
availability of appropriate size-matched donor organs. Due to
infrequent organ availability, particularly for infants, Children's
Liver Transplant Program has often turned to alternative donor
strategies, including split liver and living-donor liver transplantation.
A collaboration with the Lahey
Clinic allows Children's to offer standard whole organ, segmental
graft, split liver and living-related liver transplants to infants,
children and adolescents. Children's Liver Transplant Program
is part of its larger Solid
Organ Transplant program which also encompasses kidney, lung
and heart transplantation.
The Liver Program offers diagnostic and therapeutic capabilities
including Endoscopic Retrograde Cholangio-Pancreatography (ERCP),
Magnetic Resonance Cholangio-Pancreatography (MRCP), endoscopic
ultrasound and endoscopic therapy of varices. Specialists also
perform interventional radiologic diagnostics and therapeutics
such as transjugular biopsy, PTC, biliary drainage, and TIPSS
procedures. Non-transplant surgical options, such as biliary diversion
procedures for chronic cholestasis and portosystemic shunt operations,
are available for appropriate patients.
In a nine-hour procedure in March, Elizabeth Pomfret, MD and Roger
Jenkins, MD, of the Lahey Team performed a challenging operation
on Adara's mother, Marta, to prepare an adequate lobe of her liver
for transplantation. Simultaneously, Heung
Bae Kim, MD, and Craig
Lillehei, MD, pediatric transplant
surgeons from Children's, worked with James Pompaselli, MD, a
surgeon from the Lahey Clinic, to remove Adara's damaged liver
and replace it with the lobe extracted from her mother. The entire
team was concerned and cautious due to the minute size of Adara's
blood vessels. "Adara weighed only 10 pounds when we operated,
which is one of the smallest babies we've ever transplanted,"
says Dr. Kim. "She had very small blood vessels compared to her
mother. That's what we were worried about the most. We told Adara's
parents that there was a 30 percent chance she wouldn't live through
the surgery."
Adara not only survived, but left the hospital in early April.
She is doing well, but must remain in the U. S. for several months
of follow-up care. The family hopes to return home to Panama City
in three or four months. According to her proud father, Adara
is smiling and moving around in her crib, things she was unable
to do before her surgery.