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

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

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

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 0 1

FACTORS CONTRIBUTING TO DELAY IN PRESENTATION

Factors Reasons for late presentation Number of

patients P

are

nta

l

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

Pri

mar

y H

eal

th C

are

Clin

ics 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

Dis

tric

t h

osp

ital

serv

ice

s

Failure to investigate 1

Failure to act on blood results 1

Delay due to unnecessary investigations 0

No FU 1

Misdiagnosis (sepsis) 1

Failure to refer to a THS 2

Te

rtia

ry h

osp

ital

se

rvic

es Liver biopsy inconclusive 0

Delay in surgery (>13 days of admission)

-lack of expertise/limited resources(time, operating

theaters) 2

No FU 0

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 0

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