By Bijan Eghtesad, MD
When children are diagnosed with biliary atresia, alpha-1-
antitrypsin deficiency or another condition leading to end-stage
liver disease, liver transplantation may eventually be required. This
typically occurs prior to age 2 or during the teenage years.
In 40 to 50 percent of children, biliary atresia is the reason that
liver transplantation is required, usually before age 5 (median: 1 to
2 years). The "ideal" donor is similar in size; yet despite this large
number of potential recipients, few donors are under age 5.
Wait-listing babies a concern
This mismatch produced excessive waiting-list mortality among
smaller children, prompting the development of innovative techniques
like reduced-size, and later on, split-liver transplantation. Yet the waiting time and resulting mortality rate for small children
remained high until increased expertise in split-liver transplantation
led to a successful attempt at live-donor liver transplantation
(LDLT) in 1989. Due to the need for this alternative, especially in
pediatric populations, several centers in this country and around the
world rapidly adopted and advanced LDLT. As a result, the high
mortality rates for smaller children dropped from 25 percent to almost
zero in less than 15 years.
Equal or better outcomes with LDLT
This not only resulted in an increase in the number of pediatric
liver transplants; it also produced long-term post-transplant
outcome data suggesting equal, if not better, survival in children
receiving LDLT versus cadaveric grafts. The one-year patient
survival rate following LDLT is now between 88 and 94 percent. In Cleveland Clinic's Pediatric Liver Transplantation Program, we
pledge to do everything possible to make transplant a reality for such
patients, from infancy through their late teens. We offer every
transplant option imaginable, including whole-organ, split-graft
and live-donor transplants. We use state-of-the-art techniques, and
laboratory and radiologic advances provide us with more information
on our patients than ever before, so that we can better evaluate
them and plan the best treatment strategies.
20 successful transplants in 4 years
Our highly experienced, multidisciplinary team has performed 20
liver transplants in 20 pediatric patients over the last 4 years,
with 95 percent patient survival. This patient population included
6 recipients of live-donor grafts (4 of them under 1 year of age
at transplant) and 2 split-liver transplant recipients. My colleagues in transplant surgery, John Fung, MD, and Charles
Miller, MD, have several decades of cumulative experience in
pediatric and adult liver transplantation, including LDLT. Pediatric
hepatologists Vera Hupertz, MD, a skilled clinician and wonderful
pediatrician, and Kadakkal Radhakrishnan, MD, an expert in
pediatric metabolic liver disease, are key to our team's success.
An institution-wide team
In the Pediatric ICU, our intensivists offer an extraordinarily high
level of coverage pre- and post-transplant, with an experienced board-certified pediatric
intensivist on site 24 hours a day, seven days a week. This translates
into excellent clinical outcomes for children in their care. When
babies require transplantation, our adult anesthesiologists work in
tandem with dedicated Children's Hospital anesthesiologists. Rounding out our Pediatric Liver Transplant team are experienced
pathologists, infectious disease specialists, radiologists and psychiatrists. Through it all, dedicated pediatric transplant coordinators
and social workers ensure prompt communication and a streamlined
transplant process.
We welcome the opportunity to work with you to offer any infant,
child or teen with end-stage liver disease an increased chance for
long-term survival.
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