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) 11TH NOVEMBER 2017

• 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
• UK & France 1 in 17000-19000
• 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-
• perative cholangiogram) and therapy(Kasai porto-enterostomy (KPE))

INTRODUCTION

INTRODUCTION CONT…

• Several factors affect success of surgery including:
• Age at surgery: better if patients aged <60 days
• Extend of liver damage/fibrosis
• Experience of the Medical Centre/centralization of care etc
• Many patients are still presenting to hospital >3 months of age and prognosis
• f 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 days) All patients (102) Median (IQR) Early (≤90days) Median (IQR) Late (≥90days) Median (IQR) 82 days (51.0 ;166.0) 52 days (36.0 ;68.0) 172 days (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 province 25 9 16 0.09 Other country 1 1

FACTORS CONTRIBUTING TO DELAY IN PRESENTATION

Factors Reasons for late presentation Number of patients

Parental Delay in presentation :normal 41 RHT (admission/transfer/surgery/liver biopsy) 1 Defaulted FU

• 2 PHC
• 1 DHS
• 1 THS

4 Failure to ever present to PHC 6 Preference for traditional medication Ongoing data collection Primary Health Care Clinics False reassurance “normal” 12 Stool/urine documented not checked (Presumed rest not checked either) 3 FU given but defaulted

• 1 DHS
• 2 PHS

3 Misdiagnosis (sepsis, breast milk jaundice) 3 Failure to refer to hospital 40

FACTORS CONTRIBUTING TO DELAY IN PRESENTATION

Factors Reasons for late presentation Number of patients

District hospital services Failure to investigate 1 Failure to act on blood results 1 Delay due to unnecessary investigations No FU 1 Misdiagnosis (sepsis) 1 Failure to refer to a THS 2 Tertiary hospital services Liver biopsy inconclusive Delay in surgery (>13 days of admission)

• lack of expertise/limited resources(time, operating

theaters) 2 No FU Misdiagnosis 1 (UTI) Failure to refer for surgery 1 (UTI)

Maternal education (highest level achieved) Early Late

Basic education (Grade 12 or less) 8 12 Basic degree 1 2 Diploma 1

Place of residence Early Late

Informal (shack) 4 4 RDP 2 2 House 7 11

Education & Social circumstances

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 (biliary leak and sepsis) 1 (10%)

Lap only, no KPE

2 2/49 - 4.1%

Liver biopsy

49 49/49 -100%

Transplant

Referred 6 (no KPE) 6/49 -12.2% Transplanted LRDT 1 On active list 1 Worked up awaiting to be listed 1 Demised while on active list 3

OUTCOMES OF THE LATE PRESENTERS (up to 31st January 2017)

Outcomes Total numbers % of total late 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