1229 Exam 6 Biliary Atresia and more Flashcards Preview

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What is Biliary Atresia?

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


Who is more likely to have Biliary Atresia?

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


What causes Biliary Atresia?

It is unknown


What are the two known forms of Biliary Atresia?

Postnatal and fetal-embryonic


What is Postnatal thought to be the result of?



What is fetal-embryonic result from?

a congenital absence of biliary ducts.


S&S of Biliary Atresia?

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


What is the primary symptom of Biliary Atresia?

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.


How do I diagnosis Biliary Atresia?

Early diagnosis of biliary atresia is the key to survival.
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


How do I treat Biliary Atresia?

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.


What is the Prognosis of Biliary Atresia?

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.


Post op Nursing care for Biliary Atresia?

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


What is a Hernia?

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


What is a diaphragmatic hernia?

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.


Facts about Diaphragmatic Hernia

*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


S&S of Diaphragmatic Hernia:

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


How do I diagnosis Diaphragmatic Hernia?

Confirmed by chest xray.


How do I do a prenatal diagnosis for Diaphragmatic Hernia?

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.


How do I treat Diaphragmatic Hernia?

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


Post op Management for Diaphragmatic Hernia?

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


What's the prognosis for Diaphragmatic Hernia?

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.


Nursing Alert for Diaphragmatic Hernia:

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.


What is Omphalocele?

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.


What is Gastroschisis?

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.


How do you diagnose Omphalocele and Gastroschisis?

They can both be made with a prenatal ultrasound.


What does the ultrasound of Omphalocele show?

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


What does the ultrasound of Gastroschisis show?

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


What is an Imperforate Anus?

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


What is the patho for Imperforated Anus?

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.


Uroiogical defects:

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