Pediatric liver transplantation is now an established and successful modality of treatment worldwide for children with acute and chronic end stage liver disease. The advent of new surgical techniques, immunosuppressive drugs, sophisticated peri and postoperative care has tremendously improved the survival rates in pediatric patients over the last four decades. In the current era, pediatric liver transplant has one and ten-year survival rates of 90 and 80% respectively.
Till a few years back, in the absence of liver transplant facilities in India, a patient with liver failure had only two options, certain death or travel abroad for a transplant. In 1998, India´s first successful pediatric liver transplant was performed in our unit. The recipient is now a healthy teenager. The youngest baby in India was successfully transplanted in August 2008 at the age of 7 months. We have now performed 43 pediatric liver transplants. We have recorded 90% survival rates. Our results are now comparable with the best centers in the west. The surgical expertise gained over the last few years combined with the lessons learnt in the medical management have contributed considerably to this achievement.
The indications of pediatric liver transplant include biliary atresia, fulminant hepatic failure, chronic liver failure, metabolic disorders like Crigler-Najjar syndrome type I, primary hyperoxaluria type 1, urea cycle disorders and non-resectable hepatic tumors.
The contraindications to liver transplant are active, uncontrollable and untreatable sepsis or multi-system diseases such as mitochondrial cytopathy or irreversible cardiopulmonary disease, extra hepatic malignancy and active HIV infection unresponsive to highly active retroviral therapy. Appropriate patient selection, immaculate preparation and proper timing of transplantation are equally important as the surgical procedure itself for the final outcome.
In each child, pre transplant assessment includes blood cultures, viral serologies (HIV, HBV, HCV, HAV, CMV, HSV, VZV),ultrasound Doppler, computerized tomographic angiography of abdomen, dental, developmental,psychatric and cardiac evaluation in addition to liver function tests, hemogram and serum biochemistry. Hepatic complications like ascites, fluid retention and bleeding varices are managed as per the unit treatment protocol.
It is essential to make sure that the routine immunization is complete. However, in children undergoing emergency liver transplants, completing the immunization with live vaccines is not possible. The pretransplant nutritional status has an important bearing on the postoperative outcome. Nearly 70% of all children who qualified for a transplant at our center were malnourished. Most of these children have poor oral intake, hence nasojejunal tube placement and overnight feeding is required.
As cadaveric organs are a scarcity in India, living related liver transplantation (LRLT) is the only feasible option in our country till such time that cadaveric donation gains popularity. The usual donor is a parent or a relative of the same blood group with a healthy liver and adequate volume of the left lobe. The donors are assessed for fitness and extensive pre operative workup and counseling is undertaken. A CT angiography of liver for volumetry is obtained in all prospective donors. A psychiatric assessment is performed for all donors. Every transplant is approved by the government appointed authorization committee. Our primary concern is always the safety of the donor and the transplant recipient.
Early postoperative monitoring of the graft function along with maintenance of hemodynamic parameters is of paramount importance. Strict aseptic precautions are followed while caring for transplant patients. Postoperatively patient is returned to ICU, intubated and ventilated. Central venous pressure and arterial blood pressure is continuously monitored. In the early postoperative period, laboratory investigations are performed frequently: complete blood count, arterial blood gas analysis, serum electrolytes, serum bilirubin, creatinine, liver enzymes and PT/INR. Microbiological diagnostic tests are performed as and when required. As transplant recipients are at an increased risk for vascular thrombosis, frequent evaluation with Doppler ultrasound to evaluate flow is the norm for early detection and intervention.
Post transplant, children are put on prophylactic antibiotic therapy. To achieve early extubation, the cessation of sedative drugs is attempted as early as possible. Systemic antifungals are given to all patients with proven fungal infection.the usual immunosuppressive regimen consists of triple regimen - Tacrolimus, Steroids and Mycophenolate Mofetil as per protocol.
All children who have been successfully transplanted regain a normal life and growth in time. The usual follow up after discharge is with the pediatrician. Apart from the fact that they need lifelong immunosuppressive medication to prevent rejection of their new liver, they can expect a life which is quite normal in most respects including physical activity and reproductive function.
There are certain unique hurdles in the success of pediatric liver transplant that need to be overcome in India. Early referral is of utmost importance so that there is enough time to prepare the child and the family for liver transplant. Malnutrition is a major problem and needs to be addressed. Misconceptions about liver transplant need to be removed. The education and counseling of the family and the child is of paramount importance to help the family through the stressful procedure, the prolonged post operative period and the life-long immunosuppressive therapy with its attendant risks and side effects. With a success rate of 90% in the adult and pediatrics liver transplants performed in the last 3 years and a follow up of 11 years, we can say with satisfaction that liver transplant is now well established in India.