AT THE CHRIS HANI BARAGWANATH ACADEMIC HOSPITAL DR RR MOREKE - PowerPoint PPT Presentation

LATE PRESENTATION BILIARY ATRESIA AT THE CHRIS HANI BARAGWANATH ACADEMIC HOSPITAL DR RR MOREKE (MBCHB) 11 TH NOVEMBER 2017

INTRODUCTION  Biliary atresia(BA) is a destructive inflammatory obliterative cholangiopathy of neonates  Affects varying lengths of both intra-hepatic and extra-hepatic bile ducts  If untreated, progressive liver cirrhosis leads to death by age 2 years  Incidence: Taiwan 1 in 5000 o UK & France 1 in 17000-19000 o RSA (Soweto) 1 in 2500-8000 (1993-6)  There’s no primary medical treatment relevant in the management of BA  Surgical intervention is the only modality available for definitive diagnosis (intra- operative cholangiogram) and therapy(Kasai porto-enterostomy (KPE))

INTRODUCTION CONT…  Several factors affect success of surgery including: o Age at surgery: better if patients aged <60 days o Extend of liver damage/fibrosis o Experience of the Medical Centre/centralization of care etc  Many patients are still presenting to hospital >3 months of age and prognosis of KPE in these, is generally poor  In our resource scarce setting, where liver transplantation is only available in a minority of patients, its imperative that we diagnose BA and refer our patients for KPE early

OBJECTIVES  Determine the total number of patients with BA seen at CHBAH from Jan 2010 - Dec 2015  Determine the number of late presentations  Identify factors contributing to late referral and/or presentation  Document management of late presenters  Document outcome of the late presenters

STUDY DESIGN  Retrospective, descriptive study  Sample population ◦ All patients seen at CHBAH, by Paediatric Gastroenterology Hepatology and Nutrition Unit (PGHNU) between Jan 2010 and Dec 2015 ◦ Data collected from PGHNU database ◦ Medical records were reviewed  Inclusion and exclusion criteria  Data analysis (percentages, median and interquartile ranges, p values calculated for relevant parameters)  Late presentation for this study: defined as age ≥ 90 days at presentation

RESULTS  A total of 122 patients were seen during study period  102 fulfilled the criteria for inclusion  53 patients presented at ≤89 days (52%)  49 presented at ≥90 days ( 48%)

DEMOGRAPHIC DATA Age At Presentation Age (in All patients (102) Early (≤90days) Late (≥90days) days) Median (IQR) Median (IQR) Median (IQR) 82 days 52 days 172 days (51.0 ;166.0) (36.0 ;68.0) (193.5 ;119.5) SEX All patients < 90 days > 90 days P Values M (n) 42 25 17 F (n) 60 28 32 0.28 M:F 1 : 1.43 1:1.12 1: 1.88

Place of residence PROVINCE Total <90 days >90 days P values Gauteng 76 44 32 Other 25 9 16 0.09 province Other 1 0 1 country

FACTORS CONTRIBUTING TO DELAY IN PRESENTATION Number of Factors Reasons for late presentation patients Delay in presentation :normal 41 RHT 1 (admission/transfer/surgery/liver biopsy) Parental Defaulted FU - 2 PHC 4 - 1 DHS - 1 THS Failure to ever present to PHC 6 Ongoing data Preference for traditional medication collection False reassurance “normal” 12 Primary Health Care Clinics Stool/urine documented not checked 3 (Presumed rest not checked either) FU given but defaulted - 1 DHS 3 - 2 PHS Misdiagnosis 3 (sepsis, breast milk jaundice) Failure to refer to hospital 40

FACTORS CONTRIBUTING TO DELAY IN PRESENTATION Number of Factors Reasons for late presentation patients Failure to investigate 1 District hospital Failure to act on blood results 1 services Delay due to unnecessary investigations 0 No FU 1 Misdiagnosis (sepsis) 1 Failure to refer to a THS 2 Liver biopsy inconclusive 0 Tertiary hospital services Delay in surgery (>13 days of admission) -lack of expertise/limited resources(time, operating 2 theaters) No FU 0 Misdiagnosis 1 (UTI) Failure to refer for surgery 1 (UTI)

Education & Social circumstances Maternal education Early Late (highest level achieved) Basic education 8 12 (Grade 12 or less) Basic degree 1 2 Diploma 1 0 Place of residence Early Late Informal (shack) 4 4 RDP 2 2 House 7 11

MANAGEMENT OF LATE PRESENTERS Management Total numbers % of total LP KPE Total 10 * 10/49 -20.4% Functioning 2** (20%) Partially functioning 4 (40%) Non functioning 3 (30%) Demised post op 1 (10%) (biliary leak and sepsis) Lap only, no KPE 2 2/49 - 4.1% 49 49/49 -100% Liver biopsy Referred 6 (no KPE) Transplant 6/49 -12.2% Transplanted LRDT 1 On active list 1 Worked up awaiting to be 1 listed Demised while on active list 3

OUTCOMES OF THE LATE PRESENTERS (up to 31 st January 2017) % of total late Outcomes Total numbers presenters Alive 8 (2 functioning KPE**) 16.3% Demised 17 34.7% Referred to private 2 4.1% Lost to follow up 15 30.6% No FU 7 14.3%

CONCLUSION  A significant number of patients with BA (48%) presented late for management  KPE was offered to only a small number of the late presenters but was in most cases not successful  The majority of late presenters progressed to portal hypertension and ultimately demised  Liver transplantation is only accessible to a small number of patients  In a resource poor society KPE can be used to bridge the gap until transplantation is required

CONCLUSION CONT…  Factors for delay in presentation and diagnosis were identified at all levels of health care  The study emphasizes the importance of educating the community and all health care professionals of the necessity for early identification and referral of a cholestatic child  Parental education about the condition appears to be lacking but due to inadequate data, could not correlate with educational/ social status of parents  Emphasis should be placed on educating staff at PHC clinics- lectures, educational posters, management algorithms or stool colour charts in the RTHB

FUTURE We hope the study will: ◦ Improve awareness of BA ◦ Encourage screening for BA ◦ SASPGHAN- ideal platform to engage with the department of health at national level to implement new strategies for diagnosis and management of BA ◦ Screening ◦ Creating SA BA Registry ◦ Liver transplantation support

THANK YOU: DR HAJINICOLAOU