Case Presentation

On

Extra Hepatic Biliary

Atresia

SUBMITTED TO :

Dr.(Mrs) Sukhwinder Kaur

Lecturer,NINE

SUBMITTED BY:

Daily Happy Langba

1

MSc.(N),Pediatrics,2nd Year

NINE

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

Father’s education : Class 5th passed

Father’s occupation :Labourer

Mothers education : Illeterate

Mother’s 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.

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HISTORY OF PAST ILLNESS: -

Besides this disease which has been seen from birth, no history of other illness

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

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Mother,33yrs

Father,40 yrs

5 yrs10 yrs 3 yrs8 yrs

Height :70 cm

Weight:2.7 kg

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:-

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HR: 154 bpm, S1, S2 present

CFT:<3sec.

All peripheral pulses are palpable.

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.

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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

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 bandsB) Hepato Cellular: infective, metabolic, miscellaneous and idiopathic.

C)Paucity of Intrahepatic Hepatic DuctsSyndromic - Alagille's syndrome,Byler's, Aagene'sNon - 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,

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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.

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

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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

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.

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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

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 103 8000

Blood urea 10-50 mg/dl 28

Serum creatine 0.5-1.2 mg/dl 0.8 mg/dl

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Bilirubin Total 0-1 mg% 16

Conjugated 0-0.3 mg% 10.9

Liver Biopsy Enlarged liver with EHBA

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 µm 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 &

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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

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 30’000 IV/IM stat

Inj Vitamin A 25000 IU/IM stat X 4days OD

Tad Udiliv 150mg ½ 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 .

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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

vitamins.Since steatorrhea impairs Ca absorption the calcium intake is increased.Phenobarbital

may be given to reduce the infant’s 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

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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.

References

Marlow:Textbook of pediatric nursing,6th edition,Elsevier company,696-697

Wong: Nursing care of infants & children,Mosby company,7th edition,470-472

Avery’s neonatalogy pathophysiology & management of the newborn,2005,6th

edition,Lippincott company,1933-1937

Kliegman and et al; Nelson textbook of Pediatrics; 18th edition; Vol.2;472

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