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NS7920 > Module 5: Jack, Amanda, Jake > Flashcards

Module 5: Jack, Amanda, Jake Flashcards

1
Q

What is Congenital Diaphragmatic Hernia (CDH)?

A

A CDH is a hole in the diaphragm that allows passage of the abdominal viscera into the thorax,
- resulting in severe respiratory and cardiovascular compromise.

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2
Q

What does Congenital Diaphragmatic Hernia (CDH) allow?

A

This can allow the stomach and the intestines to move into the chest cavity and crowd the heart and lungs leading to pulmonary hypoplasia,
- resulting in life threatening respiratory difficulties.

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3
Q

What does an abnormal development of the diaphragm before birth lead to?

A

leads to defects ranging from a thinned area in the diaphragm to its complete absence.

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4
Q

What can an absent or partially formed diaphragm lead to?

A
  • An absent or partially formed diaphragm allows the stomach and intestines to move into the chest cavity and crowd the heart and lungs.
  • This crowding can lead to underdevelopment of the lungs (pulmonary hypoplasia), potentially resulting in life-threatening breathing difficulties that are apparent once the umbilical cord is cut.
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5
Q

What are 3 pathophysiology of congenital diaphragmatic hernia CDH involves?

A

pulmonary hypoplasia
- underdeveloped lung and decreased number of alveoli
- can be unilateral or bilateral
- if a diaphragmatic hernia develops toward the end of pregnancy or after birth, pulmonary hypoplasia does not occur

pulmonary hypertension
- as a result of pulmonary hypoplasia, the ductus arteriosus will remains open in an attempt to maintain fetal circulation

pulmonary immaturity

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6
Q

What is the aim of treating infant with Congenital Diaphragmatic Hernia (CDH)?

A

Treatment of an infant with CDH is aimed at stabilizing the infant to allow for surgical repair of the diaphragm.

**aimed at managing the effects of pulmonary hypoplasia and pulmonary hypertension:

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7
Q

What are 8 management for Congenital Diaphragmatic Hernia (CDH)?

A

Intubation
- avoid bag mask ventilation: can cause abdominal distention and increases difficulty if ventilation

Gastric
- gastric decompression via sump tube

ventilation:
- HFOV or jet ventilation is usually used as they provide smaller pressures and minimize overdistention of underdeveloped alveoli.

oxygenation:
- supplemental oxygen is provided to help with end organ perfusion.

cardiac function:
- Inotrope or vasopressor therapy (dopamine, dobutamine, milrinone) is provided to help improve cardiac function.

paralysis:
- Rocuronium is provided to optimize ventilation and oxygenation.

sedation:
- Sedation keeps the infant comfortable and helps to stabilize gases.

iNO:
- Inhaled nitric oxide facilitates pulmonary vasodilation when used in conjunction with adequate ventilation.

ECMO (Extracorporeal Membrane Oxygenation):
- When adequate oxygenation is not being achieved, ECMO can be used both as a rescue therapy in those with severe hypoxia after surgical repair and in the stabilization of infants before and during surgery.

surgery:
- The defect is either closed by primary repair or, in the case of a large defect, set with a prosthetic patch. It is important to note that even though the abdominal contents are no longer in the chest cavity, the pulmonary hypoplasia still exists, which can require long-term ventilation, nitric oxide, sildenafil, and a long NICU stay.
- surgical repair when hemodynamically stable

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8
Q

What is the long term outlook for infants who have a Congenital Diaphragmatic Hernia (CDH) related to (3)?

A

The long-term outlook for infants who have a CDH is related to the degree of pulmonary hypoplasia.
- They may have bronchopulmonary dysplasia related to barotrauma and volutrauma from mechanical ventilation as well as growth and nutritional disorders (feeding intolerance, oral aversion, vomiting).

  • resp insufficiency
  • developmental delay
  • feeding issues
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9
Q

What is a risk when using ECMO to treat Congenital Diaphragmatic Hernia CDH (2)?

A

The use of ECMO to treat CDH places the infants at
- risk for sensorineural hearing loss as well as
- neurodevelopmental abnormalities related to hyperventilation and ototoxic medications.

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10
Q

What does infants with abdominal wall defects have?

A

Infants with abdominal wall defects have abnormal openings along the abdomen so that intestines and other organs grow outside the infant’s body

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11
Q

What are the 5 abdominal wall defects (AWD) include?

A
  • gastroschisis,
  • omphalocele,
  • bladder extrophy,
  • cloacal extrophy, and
  • body-stalk syndrome.
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12
Q

What are the two most common types of abdominal wall defects (AWD)?

A
  • gastroschisis and
  • omphalocele.
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13
Q

What is Gastroschisis?

A
  • A full abdominal wall thickness defect that occurs to the right of the umbilicus and exposes the bowel to amniotic fluid.
  • Usually delivered vaginally
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14
Q

What is Omphalocele?

A
  • Involves a herniation of the abdominal viscera through the umbilical ring ( (sometimes including the liver, stomach, & spleen).
  • the herniated contents are covered by the umbilical cord.
  • If the omphalocele is small and does not involve the liver, a vaginal delivery might be possible.
  • A giant omphalocele usually requires a cesarean delivery to avoid membrane rupture and liver trauma.
  • The sac can be intact or ruptured
  • Infants with an omphalocele have a higher risk for other anomalies as opposed to infants with gastroschisis.
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15
Q

Based on available data, what are your priorities of care for Amanda (gastroschisis)?

A
  • Connect the replogle to low continuous suction to help with gastric decompression.
  • Insert an IV for intravenous fluids and prepare for PICC line insertion if your site provides these.
    -Start antibiotics.
  • Protection of the intestine:
    = If not in a silo, support bowel with towels and so on to prevent constriction of blood flow to the intestines due to pulling.
    = If in the silo, the silo provides some protection and helps to decrease the rate of insensible water loss and hypothermia.
  • Monitor all visible areas of the bowel for perfusion.
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16
Q

Umbilical lines can be placed in infants who have a gastroschisis or omphalocele.
True
False

A
  • Umbilical lines are generally contraindicated in infants with an omphalocele and rarely seen in infants with gastroschisis.
  • Peripheral IV’s and PICC line insertion is more common. Infants with AWDs are often on long-term parental nutrition and, therefore, if a PICC line is not inserted pre-operatively, one will usually be inserted post-operatively.
17
Q

Which abdominal wall defects (AWD) has a higher incidence of associated anomalies?

A

Omphalocele

18
Q

Which defect has less evaporated fluid losses? Why?

A

omphalocele because the abdominal contents are covered.

19
Q

What is the surgical goal for abdominal wall defect AWD?

A

The surgical goal is to return the viscera to the abdominal cavity while minimizing the risk of damage to the bowel.

20
Q

What does the repair of gastroschisis involves?

A

The repair of gastroschisis involves
- returning the extra-abdominal contents back into the abdominal cavity,
- followed by abdominal wall closure.

21
Q

What are the two ways gastroschisis repair can be done?

A
  • be performed with an immediate primary gastroschisis repair or,
  • more commonly, a repair done in a series of steps (staged),

**depending upon postnatal assessment of the condition of the exposed intestine.

22
Q

What is Primary Gastroschisis Repair

A
  • The baby undergoes surgical repair immediately upon admission to the NICU.
  • The intestine is returned to the abdominal cavity and the abdominal wall is closed during one procedure.
    **This is usually done with a small defect.
23
Q

What is Staged Gastroschisis Repair?

A
  • Prenatal exposure of the fetal intestines to the amniotic fluid can be associated with bowel dilation and inflammation, thus making primary repair unfeasible.
  • If the amount of bowel outside the abdomen is especially large or dilated or if the baby’s condition is unstable, a staged approach will need to be performed over an average of 5– 10 days.
  • Upon admission to the NICU, a silastic pouch, commonly referred to as a “silo,” is placed around the herniated bowel.
  • The bowel is then slowly and gently pushed back down into the abdomen over a period of 5–10 days.
  • The infant is then taken to the operating room where the silo is removed, and the opening in the abdomen is closed surgically.
24
Q

What are the two types of Omphalocele Repair done?

A
  • Primary Omphalocele Repair
  • Staged Omphalocele Repair
25
Q

What is the Primary Omphalocele Repair?

A

Similarly to a gastroschisis, a small omphalocele can be surgically repaired immediately
- where the exposed organs are returned to the abdominal cavity and
- the herniation is closed.

26
Q

What is the Staged Omphalocele Repair?

A
  • Similarly to the gastroschisis staged repair, a silastic pouch is used to allow the herniation to be slowly pushed back into the abdomen.
  • Once the organs are returned to the abdomen, the omphalocele is closed surgically.
  • However, in the event of a giant omphalocele, surgery to close the abdominal wall defect may be postponed some 6– 12 months to allow the abdominal cavity to enlarge as the baby grows.
27
Q

What is the outcome of patients who have gastroschisis?

A

depends largely on the condition of the vulnerable bowel

28
Q

What is the outcome of patients who have omphalocele?

A

depends largely on the associated anomalies and medical conditions.

29
Q

What is Hydrops fetalis?

A
  • Hydrops fetalis is a serious condition of the fetus or newborn where abnormal amounts of fluid build up in two or more fetal compartments,
    = causing edema, pleural and pericardial effusions, and ascites.
  • The excess fluid can interfere with organ function and can result in the death of the infant before or after delivery.
    **Hydrops is a very serious condition that can interfere with organ function and can result in death.
30
Q

What are the two types of hydrops fetalis:

A
  • Immune Hydrops Fetalis
  • Non-Immune Hydrops Fetalis
31
Q

What is Immune Hydrops Fetalis

A
  • A complication of a severe form of Rh incompatibility.
  • Rh compatibility causes massive red blood cell destruction, which leads to several problems, including total body swelling.
  • Severe edema can interfere with how the body organs work.
  • The number of babies who develop immune hydrops fetalis has dramatically decreased since the introduction of RhoGAM.
32
Q

What is Non-Immune Hydrops Fetalis

A
  • Occurs when a disease or medical condition disrupts the body’s ability to manage fluid.
  • There are three main causes for this type:
    = heart or lung problems
    = severe anemia
    = genetic defects
33
Q

What is the basic mechanism for the formation of fetal hydrops?

A
  • is an imbalance of interstitial fluid production and lymphatic return.
  • Fluid accumulation in the fetus can result from
    = congestive heart failure,
    = obstructed lymphatic flow, or
    = decreased plasma osmotic pressure
34
Q

What is 3 Hydrops Management in the delivery room?

A

Multiple personnel will need to be present to stabilize the infant:

  • Intubation: respiratory distress due to excessive edema
  • Needle aspiration: to remove excess fluid to assist with ventilation and cardiac output
  • Umbilical line: for emergency medications
35
Q

What are the Hydrops Management in the NICU?

A
  • Respiratory support
    ventilatory support and supplemental oxygen
    can be complicated by pulmonary hypoplasia, pulmonary edema, or reaccumulation of ascites or pleural effusions
  • Management of edema
    continued paracentesis and thoracentesis to remove excess fluid
    multiple long-term chest tubes may be required
  • Frequent blood gases
    to monitor for acidosis
  • Strict intake
    fluid volume and medication dosages will be based on “dry” weight for infant instead of actual weight
  • Strict output
    urinary catheter
  • Medications
    vasopressors and inotropes to improve cardiac function
    diuretics to assist with edema
    rocuronium to maximize ventilation
    pain medication
    antibiotics
  • Blood product administration
  • Arterial blood pressure monitoring
  • Central venous pressure monitoring
36
Q

What are the 3 main causes for non-immune hydrops fetalis?

A

There are three main causes for this type:
= heart or lung problems
= severe anemia
= genetic defects

37
Q

What are the two major types if congenital diaphragmatic hernia CDH?

A

Bochdalek
Morgagni

38
Q

What are some therapeutic goal/treatment for baby with gastroschisis in NICU (3)?

A
  • maintenance of physiological homeostasis
  • IVF
  • resp support if needed
  • thermoregulation
  • bowel protection
  • vascular access (avoid umbilical access)
  • NG tube for intestinal decompression
39
Q

What are 3 things hydrop fetalis causes?

A
  • edema,
  • pleural and pericardial effusions, and
  • ascites.

NS7920 (5 decks)

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