1229 Exam 6 Biliary Atresia and more

Question 1

What is Biliary Atresia?

Answer 1

It is a destructive inflammatory process that leads t ofibrosis and obliteration of the biliary tree.

Question 2

Who is more likely to have Biliary Atresia?

Answer 2

It is more commonly seen in girls and preterm infants. It is seen 2x more often in African American and Chinese infants than Caucasians.

Question 3

What causes Biliary Atresia?

Answer 3

It is unknown

Question 4

What are the two known forms of Biliary Atresia?

Answer 4

Postnatal and fetal-embryonic

Question 5

What is Postnatal thought to be the result of?

Answer 5

Infection.

Question 6

What is fetal-embryonic result from?

Answer 6

a congenital absence of biliary ducts.

Question 7

S&S of Biliary Atresia?

Answer 7

Jaundice
pale stools 
dark urine
appears well for the first 2 months of life then infant shows signs of FTT.
abdominal distention
difficult to console/comfort

Question 8

What is the primary symptom of Biliary Atresia?

Answer 8

Jaundice–
it may be visible with a total bilirubin level of 5mg/dl. this jaundice remains after the time that primary physiological jaundice would have subsided.

Question 9

How do I diagnosis Biliary Atresia?

Answer 9

Early diagnosis of biliary atresia is the key to survival.
CBC
electrolytes
bilirubin
liver enzymes
TORCH titer and sweat test may be done.
Sonography will show biliary patency
*Percutaneous liver biopsy is highly reliable
surgical laporotomy is a definitive diagnosis

Question 10

How do I treat Biliary Atresia?

Answer 10

Surgery in the first 2 months of life have an 80% survival rate.
Primary surgery for these infants is the Kasai procedure (a hepatic portoenterostomy) in which a segment of the intestine is anastomosed to the liver to attempt to drain the bile.

Question 11

What is the Prognosis of Biliary Atresia?

Answer 11

Untreated biliary atresia results in progressive cirrhosis and death usually by age two.
The kasai procedure is NOT a cure. It wil lbuy the child some time while a liver is procured.
The major challenge with these children is the shortage of liver donors.

Question 12

Post op Nursing care for Biliary Atresia?

Answer 12

Routine post op abdominal surgery care
care of the NG tube
care of the child with long term TPN
comfort measures
bathe the child in cool or tepid bath water
keep the room cool.
Keep the child’s fingernails trimmed short
refer the family to a local support group

Question 13

What is a Hernia?

Answer 13

the protrusion or projection of an organ or part of an organ through the muscle wall of the cavity that normally contains it.

Question 14

What is a diaphragmatic hernia?

Answer 14

Abdominal contents protrude into the thoracic cavity through an opening. This type of hernia usually results from failure of the pleuroperitoneal canal to close completely during embryonic development.

Question 15

Facts about Diaphragmatic Hernia

Answer 15

• It’s life threatening.
• Severe respiratory distress occurs shortly after birth
• As the infant cries, abdominal organs expand, decreasing the size of the thoracic cavity.
• The infant becomes dyspneic and cyanotic

Question 16

S&S of Diaphragmatic Hernia:

Answer 16

Barrel-shaped chest and sunken abdomen
bowel sounds heard in chest
heart sounds heard over right side of chest

Question 17

How do I diagnosis Diaphragmatic Hernia?

Answer 17

Confirmed by chest xray.

Question 18

How do I do a prenatal diagnosis for Diaphragmatic Hernia?

Answer 18

ultrasound– poly hydramnios, mediastinal shift, and the absence of a stomach bubble.
mediastinal shift to the unaffected side
auscultation may reveal decreased breath sounds on the affected side.

Question 19

How do I treat Diaphragmatic Hernia?

Answer 19

Immediate respiratory support
position the infant with the head and thorax higher than the abdomen to facilitate downward movement of abdominal organs
NG tube for stomach decompression
IV fluids administered through an umbilical artery cath
surgical repair of the defect

Question 20

Post op Management for Diaphragmatic Hernia?

Answer 20

continuation of ventilatory therapy
monitor acid base balance
postion the infant on the affected side to facilitate expansion of teh lung on the unaffected side
continue gastric decompression
monitor for S&S of infection
monitor thermoregulation
maintain adequate cardiac output

Question 21

What’s the prognosis for Diaphragmatic Hernia?

Answer 21

The prognosis for diaphragmatic hernia is poor.
50-80% mortality
Most deaths result from pulmonary hypoplasia– incomplete development of lung tissue
The prognosis depends on the size of the defect and on pulmonary function of the contralateral side.

Question 22

Nursing Alert for Diaphragmatic Hernia:

Answer 22

any newborn with a scaphoid (hollowed) abdomen, moderate to severe respiratory distress, decreased breath sounds unilaterally, and a history of polyhydramnios should be suspected of haing a diaphragmatic hernia. Ventilation should not be given with a bag mask to prevent further intestinal air and subsequent respiratory compromise.

Question 23

What is Omphalocele?

Answer 23

A midline defect of the abdominal wall that results in herniation of the bowel and intra abdominal contents into the umbilical cord. It is usually covered by a membranous sac which protects the viscera. often associated with chromosomal defects.

Question 24

What is Gastroschisis?

Answer 24

A small defect normally located to the right of the umbilicus and not limited by a membrane.

Considered to be sporadic in occurrence and is not associated with chromosomal abnormalities.Other abnormalities are not commonly reported with this defect.

Question 25

How do you diagnose Omphalocele and Gastroschisis?

Answer 25

They can both be made with a prenatal ultrasound.

Question 26

What does the ultrasound of Omphalocele show?

Answer 26

covering sac is present
10-20% are premature
NEC is seen if sac is ruptured
Malabsorption is seen if sac is ruptured
Cardiac anomalies seen in 20% of these infants

Question 27

What does the ultrasound of Gastroschisis show?

Answer 27

No covering sac
50-60% are premature
NEC is seen in approx. 20% of all cases
Malabsorption is common
Cardiac anomalies are rarely seen

Question 28

What is an Imperforate Anus?

Answer 28

a broad term that applies to a number of anorectal defects caused by abnormal development. Clinically, anorectal malformations are classified on the basis of sex, and the relationship of the rectum to the puborectalis muscle

Question 29

What is the patho for Imperforated Anus?

Answer 29

the defect arises from abnormal development and separation of the cloacal membrane.
The level of lesion causing an imperforate anus state corresponds with the level of arrested decent of the urorectal septum.

In affected children with lumbosacral spine abnormalities, a neuropathic bladder may be seen.
The lesion is usually characterized as a rectourethral fistula in males or rectovaginal fistula in females.

Question 30

Uroiogical defects:

Answer 30

renal agenesis, renal dysplasia, malrotated or ectopic kidney, lower urinary tract anomalies

Question 31

Children with imperforate anus lesions are at risk for lower urinary tract anomalies including voiding dysfunction and reflux

Answer 31

:)))

Question 32

What does imperforate Anus look like?

Answer 32

anus may be displaced
anal dimple
opening is stenotic or absent and may be covered by a thin strip of epithelium bulding from meconium in the bowel.
no meconium passage within 24-48 hours after birth. epithelium adjacent to the anal opening is abnormal in appearance
presence of fistula.
S&S of urologic manifestations

Question 33

Diagnostic Evaluation for Imperforate Anus:

Answer 33

Physical Exam
Laboratory findings: Serum BUN
KUB
IVP
Renal bladder ultrasound
cystogram
endoscopic studies

Question 34

Nursing Management for Imperforate Anus:

Answer 34

Directed toward indentification of the malformation, then the healing of the anorectoplasty site.
manage rectal drains
NG tube care
IV care 
Colostomy care
Prevent constipation
Encourage breast feeding

Question 35

What do I teach my patient about Imperforate Anus?

Answer 35

Teach anal dilation

INstruct parents regarding stooling patterns and s$s of stricture.