prolonged jaundice Flashcards
Case
CC: prolonged jaundice
History of Present Illness
Patient is a 3mo old F, born FT to a 35 year G4P3 (3003) mother at
home assisted by a TBA, apparently normal at birth.
2 months PTA, noted to have progressive jaundice and light-yellow
stools.
3 weeks PTA, noted to have gradual abdominal enlargement,
prompting consult today.
(-) prenatal check-ups
(+) maternal cough and colds during first trimester of pregnancy
(-) previous pregnancies delivered normally
NBS : negative
Ancillary History
Family Medical History: (-) Family history of bronchial asthma or
malignancy, (-) family history of DM and hypertension
Immunization history: given BCG x1, OPV x1, DPT x1 dose only.
Nutritional History: Purely breastfed
Personal/Social History: lives in a rented apartment, father and
mother both employed, 3rd of 4 siblings
PHYSICAL EXAMINATION
General Survey
Awake, not in CP distress
Anthropometrics
Weight =4.9 kg
Vital signs
BP 80/50 HR110 bpm RR 39 bpm T 36.5C O2 sats (room air) =
100%
Skin: No rash, (+) jaundice
Head and Neck: AFSF, pink conjunctivae, icteric sclerae, (-) nasal
congestion, (-) cervical lymphadenopathies
Chest and Lungs: equal chest expansion, (-) retractions, no
wheezes
Cardiac: Adynamic precordium, normal rate and regular rhythm,
no murmurs
Abdomen: Normoactive bowel sounds, (+) distended abdomen,(+)
hepatomegaly,
Extremities: Full and equal pulses, (-) edema/cyanosis/clubbing,
CRT less than 2 sec
LABORATORY RESULT:
CBC Result
WBC 7 x109/L
RBC 3.1x109/L
Hgb 110 g/L
Hct 0.38%
MCV 85fL
MCH 25 pg
Platelets 350x109/L
Neut% 0.7
Lymph% 0.3
Mono% 0.0
Eo% 0.0
Baso% 0.0
HBT USG: (+) cone-shaped echogenic density cranial to the
bifurcation of the portal vein, gall bladder not visualized
HIDA scan: normal uptake, negative excretion into the intestine
Percutaneous liver biopsy: bile ductular proliferation, the
presence of bile plugs, and portal or perilobular edema and
fibrosis, with the basic hepatic lobular architecture intact
AST: elevated
ALT: elevated
Serum Direct bilirubin: elevated
Serum Total bilirubin: elevated
PT PTT: normal
Hepatitis profile: normal
TORCH titers: normal
CXR: normal
What is your primary working impression?
Primary Working Impression: BILIARY ATRESIA
Basis for Diagnosis
History
normal at term birth
(+)maternal infection
Acholic stools
Abdominal enlargement
Physical Examination
(+) hepatomegaly
Jaundice
Distended abdomen
What are your differential diagnosis for this case?
- Choledochal cyst
Rule in: Acholic stools
Jaundice
Presented in infancy
less likely:
USG finding: : (+) cystic dilatation of the extrahepatic ducts
- TORCH
Rule in: Jaundice
(+) maternal infection
less likely: Normal TORCH profile
USG finding unlikely
- Neonatal hepatitis
rule in: Jaundice
less likely: Normal hepatitis profile
USG finding unlikely
Liver biopsy: severe, diffuse
hepatocellular disease, with distortion of lobular architecture, marked infiltration with inflammatory cells,
and focal hepatocellular necrosis; the bile ductules show little alteration
- Alagille syndrome
rule in: Jaundice
less likely:
With unusual facies
Butterfly vertebrae, cardiovascular
abnormalities
Liver biopsy: (+) bile duct paucity
(9) What is your plan of management for this patient?
e. Diagnostic Tests/Labs
Abdominal USG - triangular cord (TC) sign, which
represents a cone-shaped fibrotic mass cranial to the
bifurcation of the portal vein (represents the fibrous
remnants at the porta hepatis)
Hepatobiliary scintigraphy with technetium-labeled
iminodiacetic acid derivatives is used to differentiate
biliary atresia from nonobstructive causes of
cholestasis. The hepatic uptake of the agent is normal
in patients with biliary atresia, but excretion into the
intestine is absent. Although the uptake may be
impaired in neonatal hepatitis, excretion into the
bowel will eventually occur. Obtaining a follow-up
scan after 24 hr is of value to determine the patency
of the biliary tree. The administration of
phenobarbital (5 mg/kg/day) for 5 days before the
scan is recommended because it may enhance biliary
excretion of the isotope; sensitive but not specific
test because it fails to identify other structural
abnormalities of the biliary tree or vascular
anomalies –> less practical and of limited usefulness
Percutaneous liver biopsy
CBC – Normal
f. Management
b.1. Goals of Management:
Pharmacologic Management
Non-pharmacologic management
Hydration
Refer to GI and Ped Surgery
Exploratory laparotomy and direct cholangiography to determine the presence and site of obstruction
Surgical repair: Treatment of choice is hepatoportoenterostomy (Kasai) procedure (minute bile duct remnants, representing residual channels, may be present in the fibrous tissue of the porta hepatis; such channels may be in direct continuity with the intrahepatic ductule system. In such cases, transection of the porta hepatis with anastomosis of bowel to the proximal surface of the transection may allow bile
drainage)
b.2. Possible complications
Portal hypertension; long term plan is liver
transplantation
Anticipatory care
Proper nutrition
Vaccination
Mineral supplementation
Common causes of cholestasis:
1. Biliary atresia
2. Genetic/metabolic – Alagille syndrome, cystic fibrosis,
progressive familial intrahepatic cholestasis, alpha-1
antitrypsin disorders, IEM
3. Endocrine – Thyroid d/os, panhypopituitarism
4. Idiopathic neonatal hepatitis
5. Infections – CMV, syphilis, rubella, toxoplasmosis,
herpes virus
- Viral hepatitis (ABC) do not cause neonatal cholestasis
What is biliary atresia?
Neonatal cholestasis – conjugated hyperbilirubinemia developing
within the 1st 90d of extrauterine life
- DB >1.5-2mg/dL
- DB generally exceeds 20% of TB
Biliary atresia – progressive scarring of bile ducts outside and
inside the liver that leads to complete blockage of bile flow in the 1st 3 MOL. Most frequent identifiable cause of obstructive
jaundice. Leading indication for liver transplantation in pedia
What are the 2 major types of biliary atresia?
2 major types:
1. Cystic disorder – diff choledochal cysts
2. Noncystic form – fetal and perinatal with neonatal
sclerosing cholangitis
MC form is obliteration of entire extrahepatic biliary tree at or
above porta hepatis (85%)
What is the pathophysiology of biliary atresia?
- Congenital malformation of the biliary ductular system
–> progressive inflammatory lesion causing bile obliteration
What are the clinical manifestations?
CM:
1. New-onset/persistent jaundice >8 weeks
2. Other congenital anomalies
a. Complex CHD
b. Situs inversus
c. Polysplenia
d. Intestinal malrotation
3. Acholic stools
4. abN liver site and consistency
5. hepatomegaly
6. features of advanced ds: portal hypertension, growth
failure, moderate to severe splenomegaly, caput
medusa, ascites, umbilical/inguinal hernia, coagulopathy not responsive to VitK
What are the diagnostics of biliary atresia?
Dx
1. CBC – r/o infection
2. Reticulocyte count – r/o hemolytic causes
3. E’, BUN, Crea, Ca, PO4
4. Elev SGOT, SGPT, GGT (>300, nonspecific renal or
hepatic), ALP
5. TB, DB, IB – elev TB and DB (conjugated hyperbili)
6. Total protein, albumin, cholesterol, PT/PTT
7. r/o ID: BCS, UCS, CSF C/S, TORCH titers, VDRL, urine for
CMV, hepatitis B and C serology
8. refer to Ophtha for evaluation
9. metabolic: protein electrophoresis, alpha-1 antitrypsin level and phenotype and sweat chloride (for cystic
fibrosis), Thyroid function test, urine/serum amino acids, NBS, urine reducing substance, urine bile acids
10. abd UTZ – NPO to visualize GB, feeding with exam may
demonstrate GB fxn
- triangular cord sign – triangular or tubular tissue seen
in the porta hepatis
- useful in excluding choledochal cyst or gallstone disease causing extrahepatic bile duct obstruction
11. Hepatobiliary scintigraphy – premedicate with
phenobarbital 5mkd x 3-5d
- Limited specificity precludes the use of HBS scan as a stand alone test in making a definitive dx of Biliary atresia
12. Percutaneous liver biopsy – diagnostic for biliary atresia, gold standard. bile ductular proliferation.
13. Percutaneous transhepatic cholangiography
14. ERCP - Limited specificity of MRCP, ERCP, PTCC
provides a limited role toward dx biliary atresia
15. Histopathological findings in an appropriately timed
liver biopsy - is the most supportive test in the
evaluation of the infant with protracted conjugated
hyperbilirubinemia
16. Evaluation by intraoperative cholangiogram and
histologic examination of the duct remnant - is
considered the gold standard to dx biliary atresia
What is the management?
Mgt
1. Refer to GI and Pedsurg
2. Exploratory laparotomy with intraoperative
cholangiogram/direct cholangiography
3. Kasai procedure – for biliary atresia that is not
surgically correctable with excision of a distal atretic
segment
- Roux-en-Y portoenterostomy
- Golden period = 2 months
o Bile flow re-established in 80-90% if
performed prior to 8-weeks old
o Bile flow re-established in <20% after 12
weeks-old
o Within the first 60 DOL – 70% established
blood flow
o After 90 DOL <25% will have bile flow - Complications: ascending cholangitis and
reobstruction, failure to re-establish bile flow
4. correction of malnutrition – due to malabsorption of
dietary long chain TG à replace/MCT supplements - fat soluble Vitamin malabsorption à Vit.A, alphatocopherol,
25-hydroxycholecalciferol - micronutrient deficiency à MV, Ca, PO4, Zn
- water-soluble vitamin deficiency
- caloric intake – 125% of RDA, breastfeeding, formula
enriched with MCT
5. control portal hypertension – control bleeding, salt
restriction, spironolactone
6. Liver transplantation – biliary atresia is the MC
indication - initial tx when detected late or as salvage procedure
for failed Kasai - survival rate 80% at 1 yr, 70% at 5yrs
- primary indication for transplant evaluation: persistent
cholestasis assoc with severe malnutrition, growth
failure, hepatocellular dysfunction; decompensated
cirrhosis (intractable ascites and hemorrhage)
7. Ursodeoxycholic acid for pruritus
8. Supplementation with fat-soluble vitamins
Vit A 5,000-25,000 IU/d of water miscible prep
Vit D 3-5x RDA for age
3-5 ug/kg/d as 25-hydroxyvitamin D
Vit E 15-25 IU/kg/d
Vit K 2.5mg/twice a week to 5mg/d
Case
General Data:
CC: prolonged jaundice
History of Present Illness
Patient is a 5mo old F, born FT to a 35 year G4P3 (3003) mother at
home assisted by a TBA, apparently normal at birth.
1 month PTA, noted to have progressive jaundice and light-yellow
stools
3 weeks PTA, noted to have gradual abdominal enlargement,
prompting consult today.
(-) prenatal check-ups
(+) maternal cough and colds during first trimester of pregnancy
(-) previous pregnancies delivered normally
NBS : (-)
Ancillary History
Family Medical History: (-) Family history of bronchial asthma or
malignancy, (-) family history of DM and hypertension
Immunization history: given BCG x1, OPV x1, DPT x1 dose only.
Nutritional History: Purely breastfed
PHYSICAL EXAMINATION
General Survey
Awake, not in CP distress
Anthropometrics
Weight =6 kg, length =63 cm, HC = 42, CC=40, AC= 39
Vital signs
BP 80/50 HR110 bpm RR 39 bpm T 36.5C O2 sats (room air) =
100%
Skin
No rash, (+) jaundice
Head and Neck
AFSF, pink conjunctivae, icteric sclerae, (-) nasal congestion, (-)
cervical lymphadenopathies
Chest and Lungs
equal chest expansion, (-) retractions, no wheezes
Cardiac
Adynamic precordium, normal rate and regular rhythm, no
murmurs
Abdomen
Normoactive bowel sounds, (+) distended abdomen,(+)
hepatomegaly,
Extremities
Full and equal pulses, (-) edema/cyanosis/clubbing, CRT less than 2 sec
LABORATORY RESULT:
CBC
Date Normal
WBC 5 x109/L
RBC 3.1x109/L
Hgb 110 g/L
Hct 0.38%
MCV 85fL
MCH 25 pg
Platelets 350x109/L
Neut% 0.7
Lymph% 0.3
Mono% 0.0
Eo% 0.0
Baso% 0.0
HBT USG: (+) cystic dilatation of the extrahepatic ducts
AST: elevated
ALT: elevated
Serum Direct bilirubin: elevated
Serum Total bilirubin: elevated
PT PTT: normal
Hepatitis profile: normal
TORCH titers: normal
(1) What is your primary working impression?
Primary Working Impression
* Choledochal cyst
Basis for Diagnosis
Basis for Diagnosis
(1) History
* normal at term birth
* (+)maternal infection
* Acholic stools
* Abdominal enlargement
(2) Physical Examination
* (+) hepatomegaly
* Jaundice
* Distended abdomen
What are your differential diagnosis for this case?
- Biliary atresia
- RI: acholic stool, jaundice, presented in infancy
- RO: N LFT, USG N - TORCH
- RI: jaundice, maternal infection
- RO: N TORCH profile, USG finding unlikely - Hepatitis
- RI: jaundice
- RO: N hepa profile, USG finding unlikely
