Module 5: Jack, Amanda, Jake Flashcards
What is Congenital Diaphragmatic Hernia (CDH)?
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.
What does Congenital Diaphragmatic Hernia (CDH) allow?
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.
What does an abnormal development of the diaphragm before birth lead to?
leads to defects ranging from a thinned area in the diaphragm to its complete absence.
What can an absent or partially formed diaphragm lead to?
- 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.
What are 3 pathophysiology of congenital diaphragmatic hernia CDH involves?
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
What is the aim of treating infant with Congenital Diaphragmatic Hernia (CDH)?
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:
What are 8 management for Congenital Diaphragmatic Hernia (CDH)?
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
What is the long term outlook for infants who have a Congenital Diaphragmatic Hernia (CDH) related to (3)?
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
What is a risk when using ECMO to treat Congenital Diaphragmatic Hernia CDH (2)?
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.
What does infants with abdominal wall defects have?
Infants with abdominal wall defects have abnormal openings along the abdomen so that intestines and other organs grow outside the infant’s body
What are the 5 abdominal wall defects (AWD) include?
- gastroschisis,
- omphalocele,
- bladder extrophy,
- cloacal extrophy, and
- body-stalk syndrome.
What are the two most common types of abdominal wall defects (AWD)?
- gastroschisis and
- omphalocele.
What is Gastroschisis?
- A full abdominal wall thickness defect that occurs to the right of the umbilicus and exposes the bowel to amniotic fluid.
- Usually delivered vaginally
What is Omphalocele?
- 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.
Based on available data, what are your priorities of care for Amanda (gastroschisis)?
- 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.
Umbilical lines can be placed in infants who have a gastroschisis or omphalocele.
True
False
- 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.
Which abdominal wall defects (AWD) has a higher incidence of associated anomalies?
Omphalocele
Which defect has less evaporated fluid losses? Why?
omphalocele because the abdominal contents are covered.
What is the surgical goal for abdominal wall defect AWD?
The surgical goal is to return the viscera to the abdominal cavity while minimizing the risk of damage to the bowel.
What does the repair of gastroschisis involves?
The repair of gastroschisis involves
- returning the extra-abdominal contents back into the abdominal cavity,
- followed by abdominal wall closure.
What are the two ways gastroschisis repair can be done?
- 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.
What is Primary Gastroschisis Repair
- 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.
What is Staged Gastroschisis Repair?
- 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.
What are the two types of Omphalocele Repair done?
- Primary Omphalocele Repair
- Staged Omphalocele Repair
What is the Primary Omphalocele Repair?
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.
What is the Staged Omphalocele Repair?
- 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.
What is the outcome of patients who have gastroschisis?
depends largely on the condition of the vulnerable bowel
What is the outcome of patients who have omphalocele?
depends largely on the associated anomalies and medical conditions.
What is Hydrops fetalis?
- 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.
What are the two types of hydrops fetalis:
- Immune Hydrops Fetalis
- Non-Immune Hydrops Fetalis
What is Immune Hydrops Fetalis
- 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.
What is Non-Immune Hydrops Fetalis
- 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
What is the basic mechanism for the formation of fetal hydrops?
- 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
What is 3 Hydrops Management in the delivery room?
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
What are the Hydrops Management in the NICU?
- 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
What are the 3 main causes for non-immune hydrops fetalis?
There are three main causes for this type:
= heart or lung problems
= severe anemia
= genetic defects
What are the two major types if congenital diaphragmatic hernia CDH?
Bochdalek
Morgagni
What are some therapeutic goal/treatment for baby with gastroschisis in NICU (3)?
- maintenance of physiological homeostasis
- IVF
- resp support if needed
- thermoregulation
- bowel protection
- vascular access (avoid umbilical access)
- NG tube for intestinal decompression
What are 3 things hydrop fetalis causes?
- edema,
- pleural and pericardial effusions, and
- ascites.
