General Objectives:

After this case presentation, the presenters will be able to acquire knowledge, develop skills
and positive attitude towards the care of patients with Biliary Atresia.


Specific Objectives:
1. To be able to define Biliary Atresia .
2. To be able to identify predisposing and precipitating risk factors of BIliary Atresia.
3. To be able to identify the actual and basic symptomalogy for Biliary Atresia.
4. To be able to describe and discuss the anatomy and physiology of bile ducts.
5. To be able to trace the pathophysiology of the chosen case, Biliary Atresia.
6. To be able to make a drug study of all the medications given to a patient with Biliary Atresia
7. To be able to formulate 5 priority Nursing Care Plan for a patient with Biliary Atresia.














Biographical Data:
Name: Bacan, Christian
Age: 7 months old
Sex: Male
Date of birth: Dec. 15, 2013
Address: Asuncion, Maasin City
Mother: Melodina Ramos

Name of Physician: Dr. Jala

Present health history:
A case of C.B, male 7 month old infant from Asuncion, Maasin City came in with a chief
complaint of LBM.

Past health history:
Patients mother verbalizes that she has no problem during her pregnancy until she gave
birth. She also verbalized that her child already received BCG, HEP B, DPT, OPV immunizations.
According to her the childs skin was normal until 1 week old. But this is the first time that the child is
hospitalized.

PHYSICAL ASSESSMENT

General Appearance

-the patient is weak in appearance, very small build for his age.

Eyes, Ears, Nose, Mouth

-icteric sclera
-sunken eyeballs
-no lesions noted
-no discharges noted
-ears are symmetric with no lesions
-nose is at midline, no lesion noted
-nasal membrane is dry, nasal flaring noted
-tongue and lips are dry, no lesion noted






Respiratory

-crackles heard upon auscultation
-tachypneic with RR of 60 cpm

Abdomen

-globular, round in shape
-no lesions and irritation noted

Extremities

-wasted
-no lesion noted

Skin

-deep jaundice down to legs
-no lesion noted























Gordons Functional Pattern

Health perception and health management pattern

-according to his mother, her perception of health is maayong panglawas ug walay sakit. Patient was
fed by breast milk but then transferred to formula milks in small amounts.

Nutritional metabolic pattern

Before admission -according to his mother, she breastfed her baby for only about 3-4 months but after
trying to give him formula milk the baby doesnt want to be breastfed anymore.
During admission-patients is fed by formula milk in small amounts

Elimination pattern

Before admission-mother stated that she changes her babys diaper 2-3 times a day and sometimes uses
lampin.
During admission-patient is experiencing LBM and stool is grayish in color.

Sleep rest pattern

Before admission-patient usually sleeps 10-12 hours sometimes he wakes when hungry.
During admission-patient cannot sleep well and usually wakes up when something or someone touch
him.

Coping Stress tolerance

The patient only expresses his feelings through crying.














DEVELOPMENT TASK

*Erik Erickson

Infancy: Birth to 18 Months
Ego Development Outcome: Trust vs. Mistrust
Basic strength: Drive and Hope
The major emphasis is on the mother's positive and loving care for the child, with a big emphasis on
visual contact and touch. If we pass successfully through this period of life, we will learn to trust that life
is basically okay and have basic confidence in the future. If we fail to experience trust and are constantly
frustrated because our needs are not met, we may end up with a deep-seated feeling of worthlessness
and a mistrust of the world in general.

*Sigmund Freud

Age Range: Birth to 1 Year
Erogenous Zone: Mouth
During the oral stage, the infant's primary source of interaction occurs through the mouth, so the
rooting and sucking reflex is especially important. The mouth is vital for eating, and the infant derives
pleasure from oral stimulation through gratifying activities such as tasting and sucking. Because the
infant is entirely dependent upon caretakers (who are responsible for feeding the child), the infant also
develops a sense of trust and comfort through this oral stimulation.


















COMPLETE DIAGNOSIS OF CASE CHOSEN


Definition:
Biliary atresia is a congenital absence or under-development of one or more of the biliary structures,
causing jaundice and liver damage
- Mosbys Pocket Dictionary of Medicine, Nursing & Health Professions

Biliary atresia: Congenital absence or closure of the major bile ducts, the ducts that drain bile from
the liver.
- www.medterms.com

Biliary atresia is the failure of a fetus to develop an adequate pathway for bile to drain from the liver to
the intestine.
- www.medical-dictionary.thefreedictionary.com
BILIARY ATRESIA
A condition in which the normal extrahepatic biliary ducts is disrupted. This may lead to to fibrosis,
biliary cirrhosis, and eventual failure.
2 types:
Fetal-embryonic (Syndromic)-characterized by cholestasis, appears in the first two weeks of life,
accounts for 10-35% of all cases. Associated with other congenital defects like polysplenia, malrotation,
intestinal atresia, and cardiac anomalies
Perinatal (Acquired)-acouns for the remaning 65-100%, this form is typically found in neonates aged 2-8
weeks. Progressive obliteration of the extrahepatic bile ducts after birth. Not associated with other
congenital anomalies.





ETIOLOGY
Predisposing Precipitating
Race: Asian maternal nutrition
Sex: Female maternal exposure to teratogens
Age: neonate
Immaturity of immune system



SYMPTOMALOGY
Newborns with this condition may appear normal at birth. However, jaundice (a yellow color to
the skin and mucous membranes) develops by the second or third week of life. The infant may gain
weight normally for the first month, but then will lose weight and become irritable, and have worsening
jaundice.
Other symptoms may include: Actual Basis
Tea colored urine
Enlarged spleen
Foul-smelling stools
Pale or clay-colored stools
Only slight weight gain since birth











ANATOMY AND PHYSIOLOGY
















Pathophysiology
Predisposing Precipitating
Race: Asian maternal nutrition
Sex: Female maternal exposure
Age: neonate to teratogens
Immaturity of immune system



































Viral Exposure Immaturity of Neonatal Immune system
Extrahepatic bile duct fibrosis
Ductal Obstruction
Blocks bile (conjugated bilirubin)
flow to the intestine
Lack of bilirubin and
metabolites in intestine
Gray Colored Stool
Bile excessively
accumulate in blood
Icteric
Sclera
Excess amount
deposits to skin
Jaundice
Hyperbilirubinemia
Excess amounts
excretes through urine
Tea Colored
urine

Laboratory Results
Hematology
Leucocytes -11.6 x 10g/dL
Thrombocytes - 18.0 g/L
Hemoglobin - 78 g/dL

Stool Exam
Characteristic: Soft
Color: Dark gray

Urinalysis
Characteristics: Dark Yellow, cloudy
pH: 6.0


Abdominal UTZ
Impressions:
-Mild liver parychema disease
-Acalculous Cholesytitis
-Splenomegaly
-Bilateral Pyelonephratic changes
-Minimal Intraperitoneal fluid collection




















Surgical Management

KASAI PROCEDURE

The Kasai procedure is named after Dr. Morio Kasai, a Japanese surgeon who developed it in 1951. It is
also called Roux-en-Y or hepatoportojejunostomy.

This is the recommended treatment for children with biliary atresia.

PROCEDURE:

- The surgeon first carefully remove the damaged ducts outside the liver
- They use a small segment of the patients own intestine to replace the ducts
at the spot where bile is expected to drain.
- This segment will connect the ducts to the duodenum and the rest of the
intestines.
- A Y-shaped passageway is formed by this procedure which allows the bile to
flow from the liver into the intestine.

After the procedure:

- Recovery time for the child 7-10 days after procedure.
- Patient will be given medications to prevent ascites or fluid build up.
Pre operative Nsg. Responsibility
- Do not give child anything to eat or drink for 4-6 hours before surgery

Post operative Nsg. Responsibility
- Child will need to be fed with breastmilk and/or other formulas. These
provide types of fats that are easier for the liver to absorb
- Child is given supplements that provide Vit. A D E K. These are fat soluble
vitamins that, without bile, the childs body cant absorb normally.
- Child is given antibiotic medications for a few months after surgery. This
helps prevent infection caused by bacteria that travel from the intestines
into the liver and bile ducts. Child may be prone to infections in the liver.
- Child will also be given bile acid replacements medication. This helps
improve bile flow.