Perinatal Diseases Flashcards
1
Q
A
2
Q
Retinopathy of Prematurity
Crucial Factors
A
Birth weight
Gestational age
Number of days O2 administered
3
Q
Retinopathy of Prematurity (ROP)
(Retrolental Fibroplasia)
A
- Abnormal eye condition
- Occurs in premature/low birth weight infants that receive supplemental O2
4
Q
Retinopathy of Prematurity (ROP)
Pathophysiology
A
•Excessive fiO2
retinal vasoconstriction
necrosis of blood vessels
new vessels form and increase in number
hemmhorage of delicate new vessels cause scarring
scarring can lead to retinal detachment and blindness
5
Q
Other Factors Associated with ROP:
A
Hypercapnia, hypocapnia, IVH, Infection, Lactic Acidosis, mineral deficiencies,
6
Q
Retinopathy of Prematurity (ROP)
Clinical Manifestations
(Retrolental Fibroplasia)
A
Poor eye tone, loss of tracking.
7
Q
Retinopathy of Prematurity (ROP)
Laboratory Findings:
(Retrolental Fibroplasia)
A
Ultrasounds will identify retinal damage.
8
Q
Retinopathy of Prematurity (ROP)
Modes of Treatment
(Retrolental Fibroplasia)
A
Prevention
Decrease FiO2’s as low as possible
Cryotherapy and laser therapy can be performed based on severity
9
Q
Retinopathy of Prematurity (ROP)
Prognosis
(Retrolental Fibroplasia)
A
Varying degrees of sight loss and potential blindness
10
Q
IVH - Intraventricular Hemorrhage:
A
- Very common in low birth weight babies (premature infants)
- Incidence ranges from 20% –80%
- About 40% of IVH are associated with development of hydrocephalus
11
Q
IVH - Intraventricular Hemorrhage:
Risk Factors
A
Prematurity
Birth asphyxia
Hypoxia,
Hypercapnia,
acidosis,
RDS,
pneumothorax,
changes in hemodynamic status,
rapid blood volume expansion,
PDA
12
Q
IVH - Intraventricular Hemorrhage:
Pathophysiology
A
- Bleeding starts in the germinal matrix (a highly vascular network that matures and gets smaller with gestational age)
- Immature vasculature in premature infants
- blood fills the ventricles
- compression of brain parenchyma
- Any factor that increases or decreases cerebral blood flow will cause rupture
- Patient condition will deteriorate depending on size of bleed.
13
Q
Intraventricular Hemorrhage
Clinical Manifestations
A
- subtle to catastrophic
- Range from asymptomatic to bulging fontanels, sudden drop in hematocrit, apnea, hypotension, hypotonia, bradycardia, acidosis, seizures, and loss of consciousness.
14
Q
Intraventricular Hemorrhage
Diagnosis
A
- Head Circumference-Will be done daily to see if there is swelling
- Ultrasound
15
Q
Intraventricular Hemorrhage
Management
A
- best treatment is prevention
- Supportive treatment – maintain CPP, suppress seizures, manage respiratory status
- Ventricular drains and serial lumbar punctures can drain excess CSF
- Complications are more severe with the higher grade bleeds – can range from developmental delay to overt vegetation or death.
16
Q
Congenital Diaphragmatic Hernia
A
- CDH is a severe disease that manifest as severe respiratory distress
- Characterized by abnormal opening due to closing failure or membrane hypoplasia that leaves an opening between the peritoneal and pleural spaces
- So the abdominal content will move up and take over
17
Q
What are the 2 types of congenital diaphragmatic hernia
A
-
Bochdalek Hernia
- Lateral and posterior defect
- Usually will occur in the left hemidiaphragm
- Most common
- Occurs 90% of the time
-
Morgagni Hernia
- Medial and anterior
- Occurs on both sides
- Liver will prevent some up the upwards movement
18
Q
Congenital Diaphragmatic Hernia
Pathophysiology
A
- Lung Hypoplasia
- Decrease Alveolar Count
- Decreased pulmonary vasculature
- Pulmonary hypertension
- Unusual anatomy of inferior vena cava
- Left lung does not have enough space to develop properly
19
Q
Congenital Diaphragmatic Hernia
Chest X Ray
A
Loop and air in the thoracic cavity
Mediastinum will be pushed to the right
20
Q
Congenital Diaphragmatic Hernia
Clinical Manifestations
A
- Significant respiratory distress at birth with severe hypoxia and acidosis
- Scaphoid Abdomen
- Depressed Triagular shape
- Heart displaced to the oppsoite side of hernia
- Barrel Chest
- Increased PVR with a persistent shunt
- Worsening right to left shunt
21
Q
Congenital Diaphragmatic Hernia
Diagnosis
A
In utero with an ultrasound
CXR
22
Q
Congenital Diaphragmatic Hernia
Management
A
-
Immediate intubation and mechanical ventilation
- We have to intubate immediately as BMV will push air into the intestines
- Paralysis
- OG/NG
-
HVOV
- Low volume, low pressure, and high frequency
- This is the 1st line of defense
- Inhaled iNO for the pulmonary hypertension
- ECMO
- Not routinely use and Edmonton and Vancouver are the only centres in Canada with pediatric ECMO
- Surgery
- Can help, but it will not reverse the hypoplastic lung
- Remember that these problem tend to occur along with other problems so surgery may not be an option right away
23
Q
Congenital Diaphragmatic Hernia
Prognosis
A
50% mortality
24
Q
Periventricular Leukomalacia (PVL)
A
Area of infarct due to hypoxic ischemic encephalopathy.
The most common ischemia brain injury in premature babies, but is also seen in term infants
Often occurs concomitantly with IVH
Caused by asphyxia either in utero, during labour, or in the postnatal phase.
25
Periventricular Leukomalacia (PVL)
Pathophysiology
Occurs in the white matter adjacent to the lateral ventricles
Injury occurs in response to hypotension, ischemia, and necrosis due to asphyxia.
Can also results from increased fluid or hemorrhagecompressing arterioles in the white matter.
26
Periventricular Leukomalacia (PVL)
Clinical Manifestation
* Decreased tone
* Apneas and bradycardias
* Irritability
* Seizures
27
Periventricular Leukomalacia (PVL)
Lab Findings
•Densities or cysts on cranial US.
28
Periventricular Leukomalacia (PVL)
Management
* No treatment is currently available.
* Increased risk of developing Cerebral Palsy, developmental delays and Epilepsy
29
Hyperbilirubinemia
Bilirubin is created when hemoglobin breaks down
After birth, the babe needs to be fed early to establish normal GI flora to initiate the babe’s mechanism of breaking down the biliinto the conjugated form. If this is not possible or there are other problems, the biliwill not be broken down and will start building up unconjugated bili.
30
**Neonatal Jaundice**
* Occurs in 50% of normal births
* Can occur due to delayed feeding, hemolytic disease, liver/gut defects.
* Kernicterus is severe hyperbilirubinemia that toxifies the baby and leads to CNS impairment, seizures, motor dysfunction.
* Treatment for Jaundice:
* Phototherapy (blue light) helps to form conjugated bilirubin to allow excretion, and exchange transfusion.
31
Necrotizing Enterocolitis (NEC)
Usually occurs in premature infants
Inflammatory destructive bowel disease
Characterized by necrosis of the colon and ileum caused by intestinal ischemia
Necrosis will cause abdominal distension, sepsis, hypoxemia, respiratory failure, intestinal perforation
Gangrene can also occur
Related to asphyxia in utero
Mortality depends on severity of disease and increases with low birth weight
32
_Omphalocele_
* A protrusion of variable amounts of abdominal organs from an opening below the umbilicus Gastroschisis.
* Protrusion of the abdominal organs from an opening beside the umbilicus, R side.
33
**_Trisomies_**
* Chromosomal disorders with variable defects
* Trisomy 21 is Down’s Syndrome – maternally derived extra chromosome 21.
34
Spina Bifida
* Variable degrees of failure of the vertebral column to close fully.
* Can be fully exposed or partially.
Can include meninges, spinal cord, or both.
35
Tracheoesophageal Fistula
Defect with atresia of the upper esophagus with communication to the trachea
Results from a utero malformation
The reflux of saliva and gastic acid into the trachea results in aspiration pneumonitis
Crying of the infant often results in the distension of the stomach with air and the elevation of the diaphragm contributing to respiratory distress
36
Types of TR Fistula
1. Esophageal atresia with a distal TF (most common)
2. Isolated esophageal atresia with a long gap of missing esophagus between proximal and distal esophageal pounches
3. TEF without esophageal atresia
4. Esophageal atresia with proximal fistula
5. Esophageal atresia with both proximal and distal TEFs
37
Tracheoesophageal Fistula
Clinical Manifestation
* Excessive oral secretions
* Bubbling and frothing at the mouth
* Drooling may be associated with chocking or gasping
* Sporadic and/or continuous respiratory distress with cyanosis-Especially during feeding
* Repeated regurgitation
* Distended abdomen with bagging
* Babies with this will have infection problems
38
Tracheoesophageal Fistula
Diagnosis
Inability to pass to the stomach
Chest and abdominal x-ray
39
Tracheoesophageal Fistula
Management
Intubation and ventilation
Distended abdomen (aerophagia)
Keep HOB upright at 30
Frequent oral suctioning
Broad spectrum antibiotics
Surgical repairs
40
Tracheoesophageal Fistula
Prognosis
90% survival with surgical repair
41
Pierre-Robin Syndrome
* Congenital anomaly that presents itself with a **small mandible and oropharynx that causes the tongue to occlude the airway**
* Defect characterized by (all will result in obstruction)
* Mandibular hypoplasia
* Posterior/downward placement of tongue
* Micrognathia
* Cleft palate
42
Pierre-Robin Syndrome
Clinical Manifestation
Symptoms vary with degree of severity of obstruction from mild to severe respiratory distress
Respiratory distress is not relieved through crying
Very difficult to BMV and intubate
43
Pierre-Robin Syndrome
Management
* Prone position
* Allows the tongue to fall forward and open airway
* Nasotracheal Intubation
* Temporary insertion of OPA and NPA
* A nasotracheal tube can help maintain a patent airway
* Trach and surgical repair
44
Pierre-Robin Syndrome
Prognosis
Good prognosis may or may not require surgical repair
45
Choanal Atersia
* **A blockage or absence of the posterior nares due to congenital malformation**
* Can be membranous or bony in origin
* Obstruction can be unilateral or bilateral
* Occurs twice as often in females
* Occurs in 1/700 births
46
Choanal Atersia
Clinical Manifestation
·Respiratory distress with cycle of distress and cyanosis with momentary relief of obstruction
·Clinical improvements when the neonates cries
·Significant respiratory distress at birth because babies are naturally nose breathers which is why when in respiratory distress they open their mouths to cry
47
Choanal Atresia
Diagnosis
The inability to pass a catheter through the nares into the oropharynx
48
Choanal Atresia
Management
Stimulation so infant breathes through mouth
Oral airway
Intubation
49
Choanal Atresia
Prognosis
Good with reconstruction
50
Tracheomalacia
Flaccidity of supporting tracheal cartilage
Widening of posterior membranous wall
Reduced A-P airway caliber
Can occur from high pressure ventilation or a difficult intubation suction
51
Tracheal Stenosis
Typical around a vascular ring
Narrowing of eth trachea due to a birth defect
Can also be caused by a traumatic/chronic intubation
Hardening and narrowing
52
Vascular Ring
A vascular ring is a congenital abnormality
There can be a vascular ring are the trachea and esophagus
53
Tracheomalacia/Tracheal Stenosis
Pathophysiology
Both result in a decreased radius on inspiration
Recurring pneumonia
Hyperinflation
54
Tracheomalacia/Tracheal Stenosis
Clinical Manifestations
Chronic wheezing which worsens with increased WOB
Hypoxemia
Hyperinflation
55
Tracheomalacia/Tracheal Stenosis
Lab Findings
Viewed with a flexible bronchoscopy
56
Tracheomalacia/Tracheal Stenosis
Management
Observation
Balloon dilation (stenosis)
Tracheal resection
Tracheostomy tube
CPAP
57
Tracheomalacia/Tracheal Stenosis
Prognosis
Good with surgical care
58
Choanal Atersia
Clinical Manifestation
·Respiratory distress with cycle of distress and cyanosis with momentary relief of obstruction
·Clinical improvements when the neonates cries
·Significant respiratory distress at birth because babies are naturally nose breathers which is why when in respiratory distress they open their mouths to cry
59
Tracheomalacia/Tracheal Stenosis
Prognosis
Good with surgical care
60
Tracheomalacia/Tracheal Stenosis
Management
Observation
Balloon dilation (stenosis)
Tracheal resection
Tracheostomy tube
CPAP
61
Tracheomalacia/Tracheal Stenosis
Lab Findings
Viewed with a flexible bronchoscopy
62
Tracheomalacia/Tracheal Stenosis
Clinical Manifestations
Chronic wheezing which worsens with increased WOB
Hypoxemia
Hyperinflation
63
Tracheomalacia/Tracheal Stenosis
Pathophysiology
Both result in a decreased radius on inspiration
Recurring pneumonia
Hyperinflation
64
Vascular Ring
A vascular ring is a congenital abnormality
There can be a vascular ring are the trachea and esophagus
65
Tracheal Stenosis
Typical around a vascular ring
Narrowing of eth trachea due to a birth defect
Can also be caused by a traumatic/chronic intubation
Hardening and narrowing
66
Tracheomalacia
Flaccidity of supporting tracheal cartilage
Widening of posterior membranous wall
Reduced A-P airway caliber
Can occur from high pressure ventilation or a difficult intubation suction
67
Choanal Atresia
Prognosis
Good with reconstruction
68
Choanal Atresia
Management
Stimulation so infant breathes through mouth
Oral airway
Intubation
69
Choanal Atresia
Diagnosis
The inability to pass a catheter through the nares into the oropharynx
70
Choanal Atersia
* **A blockage or absence of the posterior nares due to congenital malformation**
* Can be membranous or bony in origin
* Obstruction can be unilateral or bilateral
* Occurs twice as often in females
* Occurs in 1/700 births
71
Pierre-Robin Syndrome
Prognosis
Good prognosis may or may not require surgical repair
72
Pierre-Robin Syndrome
Management
* Prone position
* Allows the tongue to fall forward and open airway
* Nasotracheal Intubation
* Temporary insertion of OPA and NPA
* A nasotracheal tube can help maintain a patent airway
* Trach and surgical repair
73
Pierre-Robin Syndrome
Clinical Manifestation
Symptoms vary with degree of severity of obstruction from mild to severe respiratory distress
Respiratory distress is not relieved through crying
Very difficult to BMV and intubate
74
Pierre-Robin Syndrome
* Congenital anomaly that presents itself with a **small mandible and oropharynx that causes the tongue to occlude the airway**
* Defect characterized by (all will result in obstruction)
* Mandibular hypoplasia
* Posterior/downward placement of tongue
* Micrognathia
* Cleft palate
75
Tracheoesophageal Fistula
Prognosis
90% survival with surgical repair
76
Tracheoesophageal Fistula
Management
Intubation and ventilation
Distended abdomen (aerophagia)
Keep HOB upright at 30
Frequent oral suctioning
Broad spectrum antibiotics
Surgical repairs
77
Tracheoesophageal Fistula
Diagnosis
Inability to pass to the stomach
Chest and abdominal x-ray
78
Tracheoesophageal Fistula
Clinical Manifestation
* Excessive oral secretions
* Bubbling and frothing at the mouth
* Drooling may be associated with chocking or gasping
* Sporadic and/or continuous respiratory distress with cyanosis-Especially during feeding
* Repeated regurgitation
* Distended abdomen with bagging
* Babies with this will have infection problems
79
Types of TR Fistula
1. Esophageal atresia with a distal TF (most common)
2. Isolated esophageal atresia with a long gap of missing esophagus between proximal and distal esophageal pounches
3. TEF without esophageal atresia
4. Esophageal atresia with proximal fistula
5. Esophageal atresia with both proximal and distal TEFs
80
Tracheoesophageal Fistula
Defect with atresia of the upper esophagus with communication to the trachea
Results from a utero malformation
The reflux of saliva and gastic acid into the trachea results in aspiration pneumonitis
Crying of the infant often results in the distension of the stomach with air and the elevation of the diaphragm contributing to respiratory distress
