1

SUBMITTED TO :
Dr.(Mrs) Sukhwinder Kaur
Lecturer,NINE

SUBMITTED BY:
Daily Happy Langba
MSc.(N),Pediatrics,2
nd
Year
NINE
2

Identification data
Name :Yogpreet
Age :3 months
Sex :Male
C.R no :1279761
Bed no/ Ward : 8/PGE/Nehru Hospital
Religion :Hindu
Nationality :Indian
Date of admission :2.2.11
Diagnosis : Neonatal Cholestasis with EHBA
Surgery :Plan for Kasai procedure
Consultant :Dr.Thapa
Fathers education : Class 5
th
passed
Fathers occupation :Labourer
Mothers education : Illeterate
Mothers occupation :Housewife
Address :Rajasthan
Informant :Mother
CHIEF COMPLAINTS:-
The child is admitted with C/O hyperbilirubinemia.
HISTORY OF PRESENT ILLNESS: -
The child was born by FTNVD in a government hospital in Rajasthan .There was no problem
during the antenatal period. The child cried immediately after birth , birth weight 2kg & since
birth there was yellow discoloration of eyes, and pale stool. They took him to a government
hospital at Rajasthan , then from where they got reffered to PGIMER for further management.
Here the child is diagnosed as a case of extra hepatic biliary atresia.
HISTORY OF PAST ILLNESS: -
Besides this disease which has been seen from birth, no history of other illness
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PERSONAL HISTORY:-
Antenatal History: There was no complaints during the ante natal period
Birth History: FTNVD at hospital. Cried immediately after birth. There was no incidence of
cord bleeding.
Developmental History:. Normal development but with signs of failure to thrive (wt=2.7kg).
Dietary History: Exclusive breast feeding
Immunization History: Child is not immunized.
FAMILY HISTORY:- No significant family history present .Nuclear family with five
members. The other four child are fully healthy.
Family Tree:






SOCIO-ECONOMIC STATUS:A low class family with limited sanitation facility. They have
access to safe drinking water and an Indian toilet.
GENERAL PHYSICAL EXAMINATION: -
Body built :ectomorphic
Gait :NA
Height :70 cm
Weight :2.7 kg
Mother
,33yrs

Father,
40 yrs


5 yrs 10 yrs
3 yrs
8 yrs
4

Pulse :154/min
RR :48/min

HEAD TO FOOT ASSESSMENT:-
Head :normal head size and shape.Head circumference 35 cm,fontanelles
palpable
Eyes :pupils normal size, reacting to light, yellowish discoloration of eye +
Lips :pink in color
Nose :normal, no abnormal discharge, no DNS
Ear :normal hearing, no discharge, wax or pus formation.
Tongue :pink in color, moist
Teeth :NA
Neck :normal length, no palpable lymph nodes, normal ROM, rashes on the
neck folds and pruritis+
Chest :normal chest movements,chest clear.
Abdomen :bowel sounds normal, no distension
Back :Normal curvature of spine.
Extremities :normal muscle mass,tone,adequate hydrated skin but icteric
Genitalia :clean & healthy, no abnormal discharge.

SYSTEMIC EXAMINATION:-
Respiratory system:-
RR:48/min
Normal respiratory pattern.
Bilateral chest clear, air entry equal.
Cardiovascular system:-
HR: 154 bpm, S
1,
S
2
present
CFT:<3sec.
All peripheral pulses are palpable.
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Central Nervous system:-
No h/o of seizures. Normal Reflexes.
Musculoskeletal system: -
Muscle mass normal
Muscle power tone.
Endocrinal system:-
No endocrinal dysfunction is yet noticed.
Gastrointestinal system:-
Bowel sounds are normal. Child is on exclusive breast feeding
Genitourinary system:-
Child is urinating & defecating normally.
Integumentary system:-
Skin icteric and rashes on the neck folds, pruritis +, normal body
temperature,adequate hydration,personal hygiene maintained.









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CASE IN DETAILS
Liver Disease Development
The liver is a complex organ serving multiple metabolic functions. From a digestive
standpoint, its primary function is that of an exocrine organ producing bile for the emulsification
of fats. Postnatally, the liver receives its blood from two separate sources, approximately 25%
from the hepatic artery and 75% from the portal vein. The portal vein drains the splanchnic bed
and allows the liver the opportunity to regulate and metabolize substances absorbed by the
intestine and hormones produced in the GI tract.
Bile is composed primarily of water. The concentration of solids in the bile are increased
threefold by the gallbladder. The fetal liver is capable of synthesizing bile acids from cholesterol
slowly, and the rate of synthesis increases progressively throughout gestation. The major bile salt
in newborns is taurocholate. Conjugation of bile salts with glycine in preference to taurine
gradually increases, and, by adulthood, most bile salts are conjugated with glycine. The bile acid
pool is very small in the preterm infant, but gradually increases in the newborn and matures
throughout infancy. This relatively small bile acid pool results in reduced bile salt secretion and,
in addition to the relative pancreatic insufficiency, plays a role in the less efficient absorption of
fat in thenewborn infant. Bile acids are reabsorbed in the ileum through an active transport
mechanism. There is some passive transport of bile acids in the jejunum and colon as well. In the
fetus, taurocholate is absorbed passively and active ileal transport appears after birth.

Cholestatic Liver Disease
Cholestasis is not a disease; rather, it is a symptom of many diseases. It is defined as a
pathologic state of reduced bile formation or flow. This definition applies more to the
experimental situation, where the rates of bile formation and flow can be measured, than to
human cholestasis, where neither can be assessed. Therefore, the clinical definition of cholestasis
is any condition in which substances normally excreted into bile are retained.
Most liver diseases in the neonatal period present with cholestasis, or conjugated
hyperbilirubinemia . Although elevation of the amino transferase (i.e., transaminase) enzymes is
considered the hallmark of hepatocellular injury in older children and adults, neonates may have
significant hepatocellular injury even in the presence of normal amino transferase levels. There
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are many causes of prolonged conjugated hyperbilirubinemia in the neonatal period. These can
be subdivided into general categories, including the following: infectious disorders; toxic insults
such as parenteral nutrition and sepsis; metabolic disorders; anatomic disorders, including
congenital hepatic fibrosis and choledochal cyst; and idiopathic infantile cholangiopathies,
including primarily biliary atresia and neonatal hepatitis.

Definition

Neonatal cholestasis refers to conjugated hyperbilirubinemia >1.5 - 2 mg% in a newborn/ infant
with passage of high coloured urine with or without clay stools.
Etiology

Neonatal Cholestasis syndrome
Extra Hepatic Obstruction
Hepatocellular causes
Paucity of intra hepatic ducts
A) Causes of Extra Hepatic Obstruction
Biliary Atresia
Choledochal Cyst
Spontaneous perforation of bile ducts
Biliary stenosis
Inspissation of bile ducts
Mass/peritoneal bands
B) Hepato Cellular: infective, metabolic, miscellaneous and idiopathic.
C)Paucity of Intrahepatic Hepatic Ducts
Syndromic - Alagille's syndrome,Byler's, Aagene's
Non - Syndromic - a 1AT deficiency,idiopathic, Familial

Biliary Atresia Biliary Atresia or Extra hepatic biliary atresia(EHBA)
Biliary atresia is characterized by obliteration or discontinuity of the extrahepatic biliary
system, resulting in obstruction to bile flow. The disorder represents the most common surgically
treatable cause of cholestasis encountered during the newborn period. If not surgically corrected,
secondary biliary cirrhosis invariably results. Patients with biliary atresia can be subdivided into
2 distinct groups: those with isolated biliary atresia (postnatal form), which accounts for 65-90%
of cases, and patients with associated situs inversus or polysplenia/asplenia with or without other
congenital anomalies (fetal/embryonic form), comprising 10-35% of cases.
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EHBA is a progressive inflammatory process that causes both intrahepatic & extrahepatic
bile duct fibrosis,resulting in eventual ductal obstruction.The incidence of biliary atresia is
between 1 in 10,000 to 25,000 live birthsThere does not seem to be a racial or genetic
predisposition,although there is a female predominance of 1.4:1.Associated malformations
include polysplenia,intestinal atresia & malrotation of the intestine.Biliary atresia if untreated
leads to cirrhosis,liver failure & death in the first 2 years of life.

Pathophysiology
The exact cause of biliary atresia is unknown ,although immune mechanisms or viral
injury may be responsible for the progressive process that results in complete obliteration of the
bile ducts .Biliary atresia is acquired late in gestation or in the prenatal period & is manifested a
week after birth.Although congenital infections such as cytomegalovirus,rubella virus,Epstein
Barr virus,rotavirus & reovirus type 3 have been implicated as a cause of hepatocellular damage
leading to biliary atresia.Immune mediated bile duct injury from viral exposure & immaturity of
the neonatal immune system may play a role in the destruction of bile ducts & development of
EHBA.
Early in the course of the disease,the intra hepatic ducts are patent from the intralobular
ductules to the portal hepatis.The size of the structure are variable & is correlated with the age of
the infant.These structures are present in most affected infants under 2 months of age but
gradually disappears & by 4 months are completely replaced by fibrous tissue.
The degree of involvement of the extrahepatic biliary ducts is also variable.Microscopic
examination of the liver tissue reveals cholestasis with absent or diminished bile ducts
proliferations & fibrosis.
Differentiation of idiopathic neonatal hepatitis from biliary atresia
No single biochemical test or imaging procedure is entirely satisfactory.Diagnostic schemas
incorporate clinical,historical,biochemical & radiologic features.Idiopathic neonatal hepatitis has
a familial incidence of approx 20 %,whereas biliary atresia is unlikely to recur within the same
family.A few infants with biliary atresia have an increased incidence of other abnormalities such
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as the polysplenia syndrome with abdominal heterotaxia,malrotation,levocardia & intra
abdominal vascular anomalies.Neonatal hepatitis appears to be more common in premature or
SGA infants.Persistently acholic stools suggest biliary obstruction,but patients wuth severe
idiopathic neonatal hepatitis ay have a transient severe impairment of bile excretion.Consistently
pigmented stools rule against biliary atresia.The findings of bile stained fliud on duodenal
intubation also excludes biliary atresia.Palpataion of the liver may find an abnormal size or
consistency in patients with extrahepatic biliary atresia;this is less common with neonatal
hepataitis.
Signs & symptoms

In book In patient
Jaundice and pruritis
Elevated direct bilirubin
Dark color urine
Acholic or gray colored stool
Hepatomegaly
Elevated serum amino transferase, alkaline
phosphatase,gammaglutamyl transpeptidase
Nil

Diagnostic Evaluation
Early diagnosis is key to the survival of the child with EHBA;the outcome in children
surgically treated before 2 months of age is much better than in patients withy delayed
treatment.The diagnosis of biliary atresia is based on history,physical findings & lab studies.Lab
test includes a complete blood count,serum bilirubin levels & LFT .Additional lan analysis
includes alpha antitrypsin level,TORCH titres,hepatitis serology,alpha feto protein & urine
cytomegalovirus.An abdominal sonogram is usually performed to identify potential causes of
extrahepatic obstruction such as choledochal cyst.

Advances in hepatobiliary imaging using 99mTc scans have made it possible to differentiate
cholestatic from obstructive jaundice with a high degree of accuracy, particularly after
administration of phenobarbital for 5 days before the scan .Hepato biliary scintigraphy with
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technetium labeled iminodiacetic acid derivatives is used.The hepatic uptake of the agent is
normal in patients with biliary atresia but excretion into the intestinal tract is absent.Although the
uptake may be impaired in neonatal hepatitis,excretion into the bowel will eventulally
occur.Obtainig a follow up scan after 24 hr is of value to determine the patency of the biliary
tree.The administration of Phenobarbital is recommended because it may enhance biliary
excretion of the isotope.

Absence of the extrahepatic duct on ultrasound and the finding of extrahepatic cholestasis on
biopsy mandate surgical exploration. An initial exploration is made through a limited right
subcostal incision. If a normal gallbladder is seen, a transcholecystic cholangiogram is obtained.
If the extrahepatic biliary tree appears normal, a liver biopsy is obtained,and the incision is
closed.
If the gallbladder is atretic, or the liver is obviously cirrhotic, suggesting an obstructive process,
the incision is enlarged so that the extrahepatic biliary system can be formally explored. Any
remnant of the gallbladder or extrahepatic biliary duct through which a cholangiogram can be
performed is used.

Liver biopsy,exploratory laparotomy,intraoperative cholangiogram & laproscopic guided
cholangiography are some of the diagnostic modalities.

Investigation done
Name of the investigation Normal values Patient s findings
26.1.11
Hb 12-18 g/dl 11.1 g/dl
TLC 4-11 x 10
3
8000
Blood urea 10-50 mg/dl 28
Serum creatine 0.5-1.2 mg/dl 0.8 mg/dl
Bilirubin Total 0-1 mg% 16
Conjugated 0-0.3 mg% 10.9
Liver Biopsy Enlarged liver with EHBA

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Therapuetic Management
The primary treatment of biliary atresia is hepatic portoenterostomy(Kasai
Procedure).This surgical procedure involves dissection of the porta hepatis to expose an area
through which bile may drain.A roux-en-Y jejuna limb is then anastomosed to the porta
hepatis(a Y shaped anastomosis performed to provide bile drainage without reflux).Tation is that
minute bile duct remants,representing residual channels,may be present in the fibrous tissue of
the porta hepatis,such channels may be in direct continuity with the intrahepatic ductile
system.In such cases,transaction of the porta hepatis with anastomosis of bowel mucosa to the
proximal surface of the transaction may allow bile drainage.If flow is not rapidly established
within the first months of life,progressive obliteration & cirrhosis ensue.If microscopic channels
of patency > 150 m in diameter are found,post operative establishment of bile flow is likely.The
success rate of Kasai procedure is higher if performed before 8 weeks of life.Therefore,early
referral & promt evaluation of infants with suspected biliary atresia is emphasized.There are
several variations in this procedure. When the operation is performed in infants younger than 3
months of age, there is reasonable expectation that bile will drain into the intestine. Children who
fail to drain bile after the portoenterostomy may be rescued by liver transplantation before 1 year
of age, although the complication rate in these infants is higher than in older children with liver
transplants .
In approx 80 to 90% of infants with biliary atresia who are operated on when younger
than 10 weeks of age,bile drainage is achieved.However,progressive cirrhosis still occurs &
many eventually require a liver transplantation.Complications following the portoenterostomy
procedure include ascending cholangitis,cirrhosis,portal hypertension & GI
bleeding.Prophylactic antibiotics are given following Kasai procedure to minimize the risk of
ascending cholangitis.
Orthotopic liver transplantation (OLT) is now the considered to be the definitive therapy
for biliary atresia.The portoenterostomy offers increased survivability upto 5 yrs but fibrosis &
cirrhosis may occur.Further more it increases the chances of finding a suitable donor organ
before the patient reaches end stage renal disease.Complications following liver transplantation
include obstruction & bile leak at the biliary anastomosis,portal
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hypertension,hemorrhage,infection & rejection.Immunosuppresive drugs are required following
transplantation.

Medical management of biliary atresia is supportive.It includes nutritional support with infants
formula that contain medium chain triglycerides & essential fatty acids.Supplementation with
fat soluble,a multivitamin & minerals including iron,zinc & selenium is usually
required.Aggresive nutritional support in the form continous tube feedingts or total parentral
nutrition may be indicated for modeate to severe failure to thrive,the enteral solution should be
low in sodium.Phenobarbital may be prescribed following hepatic portoenterostomy to stimulate
bile flow, & ursodeoxycholic acid may be used to decrease cholestasis & the intense pruritus
from jaundice

Treatment received by Yogpreet
A to Z drops, 5 drops BD
Evion drops 5drops OD
Inj Vitamin K 3mg iv stat
Inj Vitamin D 30000 IV/IM stat
Inj Vitamin A 25000 IU/IM stat X 4days OD
Tad Udiliv 150mg tab bd

Nursing management
Early diagnosis is essential if surgical correction is to be successful.During the time
when the diagnosis is being made,the nurse assists with tests & procedures.If the Kasai
procedure has been performed the nurse must care for the double stoma & collect,measure &
replace the bile by way of the stomal openings.Teach the parents the s/s of cholangitis like
fever,tenderness in the right upper quadrant,decreased bile flow & jaundice.If the flow of bile
decreases after 3 months this may indicate progression of the liver disease.If bile flows
normally.the external conduit may be closed 1 or more years after initial surgery .
Medical management is necessary because fat cannot be absorbed by these infants.It
consists of a diet high in proteins & low in fats.Medium chain triglycerides can be used for older
children.Water soluble vitamins can be given because of malabsorption of the fat soluble
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vitamins.Since steatorrhea impairs Ca absorption the calcium intake is increased.Phenobarbital
may be given to reduce the infants irritability.Cholestryramine which binds intraluminal bile is
used when bile flow is evident.If portal hypertension with ascites is present because of
progressive liver disease & cirrhosis,salt is restricted & diuretics are given.
The nursing care is basically supportive.The afflicted infants become increasingly
irritable & listless as the disease progresses because of the accumulation of the toxic products in
the body.& the nursing management should be planned to provide as much rest & sleep as
possible.They should be cuddled & comforted whenever possible within their limits of tolerance.

Nursing Diagnosis:-
Impaired skin integrity related to pruritis and canulla in situ
Altered skin color r/t accumulation of the bile secondary to fibrosis of the biliary tract
Activity intolerance related to liver dysfunction
Altered nutrition less than body requirement related to effects of liver dysfunction
Risk for infection related to prolonged hospitalistion
Anxiety and knowledge deficit related to disease condition as evidenced by verbalisation
Goals
Short Term goal
To relieve itching
To prevent skin infection
To avoid chances of infection
To reduce anxiety
Long Term Goal
To ensure normal growth and development with minimal complications
Prognosis
Without treatment the average time of the infants with extrahepatic atresia is about 2
yrs.About half of those who are treated may survive for 5 yrs or longer,some possibly into
adolescence but may develop chronic cholangitis or severe portal hypertension.The Kasai
procedure is less successful in infants over 2 months of age.The long term outcome for infants
who have liver transplants is not yet known.

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References
Marlow:Textbook of pediatric nursing,6
th
edition,Elsevier company,696-697
Wong: Nursing care of infants & children,Mosby company,7
th
edition,470-472
Averys neonatalogy pathophysiology & management of the newborn,2005,6
th

edition,Lippincott company,1933-1937
Kliegman and et al; Nelson textbook of Pediatrics; 18
th
edition; Vol.2;472