Table 1

Medium and late term complications after liver transplantation in children

ComplicationIncidence (approx)Commonly raised LFTsTypical time courseFurther investigationsManagement
Infection
ViralSpecific: up to 20%
Non-specific: very common
ALTAny timeViral serology and PCR studies liver biopsySome specific treatments available; most cases resolve spontaneously; rarely progression to subacute hepatic failure
BacterialCholangitis: up to 20%
Sepsis: < 5%
Bilirubin1 month to years later; often underlying biliary problemSeptic screen, US, cholangiographyAntibiotics ± antifungal treatment; look for predisposing factors: biliary obstruction, hepatic ischaemia, overimmunosuppression
Fungal, parasitic< 5%BilirubinAny timeSeptic screen, US, cholangiographyLook for predisposing factors: biliary obstruction, hepatic ischaemia, overimmunosuppression
Biliary tract obstruction
Intrahepatic stricture(s) Extrahepatic stricture(s)
Bile stones
Up to 20%ALP, GGT, (bilirubin)1 month to years laterUS, cholangiographyPrompt referral to specialist centre; dilatation, stenting, or reconstructive surgery for single strictures; multiple strictures are usually ischaemic in origin and eventually lead to graft failure
Vascular compromise/ischaemia
PV stenosis or occlusion
HA stenosis or occlusion
HV stenosis or occlusion
Up to 10% overallLFTs usually normalUsually present from early post-op. but clinical presentation often late with portal hypertension ± ascitesUS/Doppler, MRA, angiographyPV: ballooning, stenting, or surgical reconstruction
HA: usually asymptomatic but may cause biliary strictures and slow graft failure
HV: ballooning or stenting; eventually retransplantation
Rejection
Late acute rejection5–10%Bilirubin, ALT1 month to years laterLiver biopsy for histologyPrompt referral for biopsy and enhanced immunosuppression; delay in treatment might increase risk of progression to CR
Chronic rejectionUp to 10%Bilirubin3 to 12 monthsLiver biopsy for histologyEarly forms might reverse but more often progress to graft failure
Autoimmune hepatitisUp to 5%ALTMonths to years (median 2 years)IgG, autoantibodies, liver biopsyReferral for liver biopsy; most cases show a good biochemical and histological response to enhanced immunosuppression
Lymphoproliferative disease
Lymphadenopathic
Systemic
Lymphomatous presentation
Up to 10%LFTs usually normalFirst 12 months but up to years later; EBV susceptible children and heavily immunosuppressed most at riskEBV serology and PCR, US, CT, EBV nuclear antigen and immunocytochemistry on tumour tissueReduction or discontinuation of immunosuppression; chemotherapy (see UKCCSG protocol)
Recurrence of original disease (such as autoimmune hepatitis)30–60%LFTs may be normalMonths to years later; recurrence risk increases with time after transplantationBiochemical and/or immunological tests; liver biopsyReferral for liver biopsy and immunosuppressive treatment
  • ALP, alkaline phosphatase; ALT, alanine aminotransferase; CR, chronic rejection; CT, computed tomography; EBV, Epstein Barr virus; GGT, γ-glutamyl transferase; HA, hepatic artery; HV, hepatic vein; LFTs, liver function tests; MRA, magnetic resonance angiography; PCR, polymerase chain reaction; PV, portal vein; UKCCSG, UK Children’s Cancer Study Group; US, ultrasound scan.