late presentation biliary atresia at the chris hani ...paedhpb.org/2017/saturday/late presentation...
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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