Paediatric Liver Transplant Programme Wits Donald Gordon Medical Centre

Transcription

1 Paediatric Liver Transplant Programme Wits Donald Gordon Medical Centre J Loveland, J Botha, R Britz, B Strobele, S Rambarran, A Terblanche, C Kock, P Walabh, M Beretta, M Duncan et al

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3 1817 reveal the presence of 1 st clear English language reference to BA John Burns Regius Professor of Surgery University of Glasgow Burns J. Principles of Midwifery, including the diseases of women and children 1817:602

4 Looking at the problem of biliary atresia from the vantage point of 30 years experience with the lesion, we can say with certainty that the jaundiced baby who has had no extrahepatic bile duct has been the most disappointing patient for the surgeon in the whole realm of lesions theoretically correctable by a surgical procedure.

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6 Scientific fields progress in a linear and continuous way Laborious grind and dog work Predictable solving of puzzles that are left open in a current field of knowledge..a pleasant way to keep busy when one is not up to useful work Coran Hind Grosfeld Millar Davenport Puri Loveland

7 The Structure of Scientific Revolutions Normal science with a paradigm and a dedication to solving puzzles; followed by serious anomalies, which lead to a crisis; and finally resolution of the crisis by a new paradigm Paradigm shift in treatment and outcomes Ian Hacking Introductory Essay The Structure of Scientific Revolutions

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9 Kasai et al. Journal of Paediatric Surgery (6)

10 Alluded to ductal diameter of 150 microns as potentially prognostic for drainage 10 of 14 with ducts > 200μ drained Only 1 of 13 < 150μ Size may be of great significance All cured cases surgery before 4 months of age Not a few cases. Might be curable if portoenterostomy carried out before 4 months of age, preferably within 3 months after birth Kasai et al. Journal of Paediatric Surgery (6)

11 The First 1963 C Henry Kempe presented Bennie Solis to Starzl Department of Paediatrics University of Colorado Infectious Diseases Specialist Coined term Battered Child Syndrome Defender of children Starzl suggested transplant Kempe agreed 1981 when moved to Pittsburgh, firmly supported moving program forward The Puzzle People

12 Complementary role of KP and LT now accepted standard treatment BA Vacanti et al. Journal of Pediatric Surgery : Sequential employment significantly improved prognosis Otte et al. Hepatology (Suppl):41S-48S

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17 Loveland et al. S Afr Med J 2012;102(4):

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19 SO WHERE ARE WE TODAY 134 transplants 8 re-transplants 36.57% LDLT (49/134) 34.33% whole (46/134) 18.66% splits (25/134) 10.45% Reduced size (14/134) 12 fulminant hepatic failure 12 CLKT

20 (1st 6m) Number of transplants UNIT GROWTH Year of transplant

21 DEMOGRAPHIC OF AETIOLOGY % of recpients with chronic liver failure (n=117) biliary atresia budd chiari - veno-occlusive disease oxalisis auto-immune disease chronic rejection of liver graft alpha-1 antitrypsin deficiency alagille syndrome (biliary hypoplasia) wilson's disease polycystic kidney disease and hepatic fibrosis biliary agenesis hepatoblastoma hepatocellular carcinoma cryptogenic maple syrup urine disease PFIC - progressive familial intrahepatic cholestasis other

22 % of recipients (n=115) IMMUNOSUPPRESSION steroids tacrolimus MMF other Immunosuppression at time of transplant

23 GRAFT UTILIZATION Entire Series 134 Era 1 31 Era Whole Grafts (58%) 28 (27.18%) Reduced Size Grafts (32%) 4 (3.88%) Split Grafts 25 3 (10%) 22 (21.6%) Living Donor Grafts (47.57%)

24 GRAFT TYPE BY ERA Whole Reduced Split Era 1: Era 2: Present LDLT Loveland et al. S Afr Med J (11)

25 RECIPIENT AGE

26 Entire Series Era 1 Era 2 Age Range (months) Mean Age Median Age Weight Range (kilograms) Mean Weight Median Weight Loveland et al. S Afr Med J (11)

27 RECIPIENT WEIGHT

28 Entire Series Era 1 Era 2 Age Range (months) Mean Age Median Age Weight Range (kilograms) Mean Weight Median Weight Loveland et al. S Afr Med J (11)

29 Patient Survival Percent survival Time

30 HEPATIC ARTERY THORMBOSIS 4.5% incidence (6/134) 1 Reduced = HAT Day 1 = Died before ReTx 1 Whole = ReTx = Died 1 Whole = Revised D1 = NAD 1 Whole = HAT Day 9 = ReTx Day 22 = NAD 1 Split = HAT Day 10 = ReTx Day 13 = Died 1 Whole = HAT Day 21 = Good function to date 1 Whole = Rupture D11 = Revised

31 VASCULAR COMPLICATIONS 7 PVT and 1 PVS 1 PVT revised with venous interposition 1 PVS required angioplasty and stent 1 PVT required splenorenal shunt Another planned 2 patients venoplasty caval anastomosis

32 BILIARY COMPLICATIONS Increased incidence after split and LDLT Cut surface leaks Long term increase in strictures Ischaemia Attention to detail: LDLT harvest Back table split Biliary reconstruction during implant Meticulous arterial reconstruction

33 LARGE FOR SIZE SYNDROME

34 IMPACT OF LDLT Access greater organ pool Significant ethical issues Benefits Decreasing waiting list death elective transplant Well matched organ may lead to better long-term graft and patient survival Donor operation places well individual at risk: morbidity overall average 35% Decision made without coercion Psychosocial evaluation Anatomical assessment

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36 % of patients aged <=5y THE IMPACT OF Weight Height MUAC

37 ANOTHER SIGNIFICANT HAZARD 3.5 (95% CI ) for patient survival 3.2 (95% CI ) for liver graft survival

38 Number of infections NOSOCOMIAL INFECTION % % 7.1% 0 Bacterial Viral Fungal / yeast

39 INTERVENTION BILIARY ATRESIA Identification Early referral Surgery Centralization Follow up Early referral Transplant assessment

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42 INTERVENTION TRANSPLANT Nutritional support Pre-transplant Infection control Venous access and care Antibiotic stewardship Long Term outcomes Immunosuppression

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