Download From Apgar to Z

From Apgar to Z-plasty
Pediatric Medical Terms
But first….
• ABG – Arterial blood gases
– Blood test using blood from an artery (usually
radial artery)
– Used to determine gas exchange levels in the
blood related to lung (respiratory) function
– Tests pH, and CO2 and O2 levels
One more….
• ABP – Arterial blood pressure
– Blood pressure (force exerted by circulating blood
on walls of blood vessels) measured through an
arterial line
– Often used in ICU
Apgar Score
• Virginia Apgar, U.S. anesthesiologist, 1909-1974)
– System of scoring infant’s physical condition one
minute and five minutes after birth.
– Heart rate, respiration, muscle tone, response to
stimuli and color
– Each rated 0, 1, or 2 with maximum score of ’10’
– Low scores = immediate attention
– A low score at one minute is a sign of asphyxia
– A low score at five minutes is an index of the
possibility of death
Apgar Score
Score
Sign
0
1
2
Heart Rate
Absent
Slow (less than 100) Greater than 100
Respiratory Effort
Absent
Slow, irregular
Good; crying
Muscle tone
Limp
Some flexion of
extremities
Active motion
Reflex irritability
No response
Grimace
Cough or sneeze
Color
Blue, pale
Body pink;
extremities blue
Completely pink
Atrial Septal Defect (ASD)
• In developing fetus, the interatrial septum
develops to eventually separate the left and
right atria
• ASD is a congenital heart defect involving the
interatrial septum that enables blood to flow
between the left and right atria
• Results in improper mixing of low oxygen
venous blood (right side)with high oxygen
arterial blood (left side)
ASD
• This mixture of blood is called a ‘shunt.’ A
‘right to left’ shunt typically poses more
danger for the patient.
• Results in cyanosis, pulmonary hypertension,
right-sided heart failure, stroke
Patent Foramen Ovale
• The foramen ovale (foraymen ovalee) also
remains open during fetal development but
after birth it should close completely
• In approximately 25% of people, the foramen
ovale does not entirely seal. Elevation in the
pulmonary circulatory system (i.e., pulmonary
hypertension -- chronic or transient,like when
you cough) can cause the foramen ovale to
remain open.
Patent foramen ovale
• This is a PFO – It is a small channel with little
hemodynamic consequence. Clinically, it is linked to
decompression sickness, paradoxical embolism and
migraine. Investigation is underway about the role of
PFO in stroke and TIAs in the absence of other
problems.
Bronchopulmonary Dysplasia
• BPD
• <34 weeks’ gestation and <4.5 pounds
• Associated with infant respiratory distress
syndrome (IRDS)
• Damage to lung tissue
due to prolonged
mechanical ventilation
BPD
• Often a high amount of pressure and a higher
oxygen concentration are necessary due to
stiff, underdeveloped lungs
• Over time, pressure from the ventilator and
excess oxygen intake can injure the lungs
leading to IRDS.
• If IRDS persists, then dx of BPD is given if baby
needs oxygen after 28 days or past 36 weeks’
postconceptual age.
BPD
• Also caused by trauma, pneumonia and other
infections
• Associated with inflamation and scarring of
lung tissues
• Tx with bronchodilators (albuterol) and
diuretics to reduce buildup of fluid in lungs
• Severe cases – steroids (see Cushingoid faces)
Congenital Diaphragmatic Hernia
• CDH
• Applied to a variety of congenital birth defects
that involve abnormal development of the
diaphragm
CDH
CDH
• Three major defects:
– Failure of diaphragm to close
– Herniation of abdominal contents into the chest
– Pulmonary hypoplasia (decreased lung volume)
• Majority occur on left side, some on right side,
small fraction bilateral
• Leads to severe, life-threatening respiratory
distress
• Treatment is often……
Extracorporeal Membrane
Oxygenation
ECMO
• Provides both cardiac and respiratory support
when heart and lungs can no longer serve
their function
• ~75% effective in saving a newborn’s life
• Cannot be <4.5 pounds so rules out most
premature and/or small BW (birth weight)
infants
ECMO machine
ECMO Machine
ECMO
• Time limit is usually around 21 days
• Tubes are placed via a large vein at the base of
the neck (right side) with a cannula leading to
the right atrium of the heart
• Another cannula is placed in a large artery
(carotid)
• Cannulas are connected to tubing of the
ECMO machine
Infant in ICU on ECMO
ECMO
• Blood drains from right side of neck through
tubing
• Blood is oxygenated, rewarmed and returned
to the body through the arterial cannula
• As heart/lungs improve, amount of blood flow
through the circuit can be decreased
Free and Appropriate Public
Education
• FAPE
•
•
•
•
Mandated by IDEA
Defines the rights of students with disabilities
Looks different for each child
Driven by the IEP process
Least Restrictive Environment
• LRE
– The location in which FAPE is provided
– Can vary based on severity of disability
– We are required to evaluate what type of services
a child needs and create an environment that
meets those needs
Apnea and Bradycardia
• ABC
– Apnea is a pause in breathing with one or more of
the following characteristics:
• > 15-20 sec
• Associated with color change (pale, purplish or blue)
• Associated with bradycardia
– Bradycardia
• Slowing of the heart rate, usually <80 bpm
• May be due to reflex (e.g., NGT or stooling)
ABC
• Common causes:
– Prematurity, low blood sugar, infection, sz, PDA,
BI, high or low body temp, insufficient oxygen
• Common treatment:
– Caffeine, CPAP, mechanical ventilation, periodic
stimulation
Clostridium difficile
• C. diff
– Most serious cause of antibiotic associated
diarrhea (AAD) and can lead to colitis, a severe
infection of the colon, often resulting from
eradication of the normal gut flora by abx
(antibiotics)
– Bacteria naturally residing in the body becomes
overgrown, bacterium release toxins causing
bloating, constipation, diarrhea with abdominal
pain
C. diff
• Human transmission by the fecal-oral route
• Residence in hospital or nursing home is a risk
factor
• Rate of acquisition is estimated to be 13% in
patients with hospital stays of up to 2 weeks
and 50% in those longer than 4 weeks
• Vancomycin is treatment of choice
• Wear gloves!! Wash with soap and water;
alcohol-based hand rubs are ineffective!!
CHARGE syndrome
• A recognizable (genetic) pattern of birth
defects
• 1:9-10,000 births
• CHARGE (was a way to refer to the cluster of
features seen)
– Coloboma of the eye (a ‘cleft’ of the iris, retina,
choroid, macula or disc)
– Heart defects
– Atresia of the choanae
– Retardation of growth and/or development
– Genital and/or urinary abnormalities
– Ear abnormalities and deafness
CHARGE
• These features are no longer used in making a
dx of CHARGE syndrome, but the name stays
• Clinical dx is made using a combination of
Major and Minor features
– Coloboma, choanal atresia, CN abnormality, outer,
middle and/or inner ear, heart defects, cleft
lip/palate, TE fistula, kidney abnormalities, genital
abnormalities, growth deficiences, “typical face,”
palm crease, behavior, limb/skeletal, etc.
Necrotizing enterocolitis
• NEC
• Typically seen in preemies
• Timing of onset is generally inversely
proportional to gestational age at birth (e.g.,
the earlier a child is born, the later the signs of
NEC are seen)
• Portions of the bowel undergo necrosis (tissue
death)
NEC
NEC
• Tx includes providing bowel rest by stopping
enteral feeds, gastric decompression with
intermittent suction, fluid replacement,
support for BP, parenteral nutrition, and abx
therapy
• Emergency surgery resection of necrotic
bowel may be required
• Colostomy may be required (reversed later)
NEC
•Warning –
Picture of resection is next
NEC
NEC
• Almost never seen before oral feedings are
introduced
• Formula feeding increases risk of NEC by
tenfold compared to infants who are breastfed
alone.
• Breastmilk (even expressed BM) –
antiinfective effect, immunoglobulin agents,
rapid digestion
Methicillin-resistant
Staphylococcus aureus
• MRSA
• A strain of staph that is resistant to broad
spectrum antibiotics
• Can be fatal
• Most MRSA infections occur in hospitals or
other health care settings (HA-MRSA = health
care associated MRSA)
MRSA
• Recently another type has been found among
otherwise healthy individuals in the
community (CA-MRSA = Community
associated MRSA)
– Serious skin and soft tissue infections
– Serious form of pneumonia
MRSA
• Staph bacteria normally found on skin and in
nose of about 1/3 of the population
• If you have staph on your skin or in your nose
and aren’t sick, you are said to be ‘colonized’
but not infected.
• You can pass the bacteria on to others,
though.
MRSA
• MRSA infections start out as small red bumps
that can quickly turn into painful abscesses
Kate…don’t look
MRSA
• Staph are generally harmless unless they enter
the body through a cut or other wound
• Risk factors:
– Young age (not fully developed immune system)
– Contact sports
– Sharing towels or athletic equipment
– Weakened immune system
– Crowed, unsanitary living conditions
– Health care
Kate…don’t look, again
MRSA
• Prevention
– Wash you hands/use alcohol sanitizer
– Wash you hands often
– Don’t share personal items
– Shower after games/practices
– Sit out games/practices if you have an infection
– Sanitize linens (towels, sheets, etc.)
– Get tested
– Use antibiotics appropriately
Tracheoesophageal Fistula
• TEF
• Abnormal connection (fistula) between the
esophagus and trachea.
• Common congenital abnormality
• In adults, usually sequela of surgical
procedure such a laryngectomy
TEF
Congenital TEF
• Type A – proximal and distal esophageal buds
(esophageal agenesis = EA)
• Type B – Proximal esophageal termination in
the lower trachea with distal esophageal bud
(EA + TEF)
• Type C – Proximal esophageal atresia
(esophagus ending in a blind loop) with distal
esophagus arising from the lower trachea or
carina. (EA + TEF) 90% of cases
Congenital TEF
• Type D – Proximal esophageal termination on
the lower trach or carina with distal esoph
arising from carina (EA + TEF)
• Type E (or Type H) – Variant of type D; if the
two segments of esoph communicate, has a
resemblance to the letter ‘H.’ (TEF without
EA)
Congenital TEF
Congenital TEF
• Failed fusion of the tracheoesophageal ridges
during the third week of embryological
development
• Unable to feed safely
• Prompt surgery is required
• May develop feeding difficulties and chest
problems post-operatively
Congenital TEF
• Clinical presentation
– Copious salivation
– Choking, coughing, cyanosis with the onset of
feeding
• Treatment
– Resection of fistula and anastomosis of
discontinuous segments
– Complications
• Structure, leak at point of anastomosis, recurrence
Congenital TEF
• Some babies with TEF also have other
abnormalities
• Most commonly associated with VACTERL
association
VACTERL or VATER Association
• Acronym used to describe a series of
characteristics
– V – vertebrae
– A – imperforate anus or anal atresia
– C – cardiac anomalies
– TE – TEF
– R – Renal or kidney anomalies
– L – limb anomalies (radial agenesis)
VACTERL Association
VACTERL
• There is a wide range of manifestation so the
exact incidence is unknown
• Babies usually have at least three or more
individual anomalies
• 75% have heart disease (VSD, ASD, TOF)
Human respiratory syncytial virus
• RSV
– The major cause of lower respiratory tract
infection and hospital visits during infancy and
childhood
– In US, 60% of infants infected during their first RSV
season (usually winter months)
– Nearly all children have been infected by age 2-3
– Severe RSV infections have been increasingly
found among elderly patients
RSV
• Produces only mild symptoms in most people
• Causes bronchiolitis leading to severe
respiratory illness requiring hospitalization
and sometimes death (rare)
• Predisposes children to asthma/RAD
RSV
RSV
• No known immunization
• Infection does not induce protective immunity
so you can get it again
• Synagis (palivizumab) – a moderately effective
prophylactic drug used for high risk infants
(preemies, immuno suppressed, etc.)
Patent Ductus Arteriosus
• PDA
• Before birth the two major arteries (aorta and
pulmonary artery) are connected by a blood
vessel called the DA
• This vessel is an essential part of fetal blood
circulation.
• DA mostly closes within 12-24 hours after
birth and is completely sealed after three
weeks
PDA
• If DA stays open (patent), it allows oxygen-rich
blood from aorta to mix with oxygen-poor
blood from the pulmonary artery.
• If uncorrected it may lead to congestive heart
failure
•
PDA
PDA
• Typically found in preemies, congenital rubella
syndrome and chromosomal abnormalities
such as Down Syndrome
• Relatively common in US ~8:1000 births
(preemies)
• In full-term (~2:1000 births)
• Twice as common in girls
PDA
• Symptoms
– Increased WOB, poor weight gain
• Treatment
– Ligation – DA manually tied shut, plugs or coils
– Indomethacin (inhibitor of prostaglandin)
• Usually used with preemies
Ventricular Septal Defect
• VSD
• Defect in the ventricular septum, the wall
dividing the left and right ventricles of the
heart; a “hole” in the heart
• Causes a murmur that is audible to
auscultation
VSD
• A large amount of oxygen-rich blood from
heart’s left side is forced through the defect in
to the right side. Then it’s pumped back to
the lungs, even though it’s already been
refreshed with oxygen.
• The already over-oxygenated blood displaces
blood that needs oxygen. So the heart must
pump more blood and may enlarge from all
the work.
VSD
VSD
• Causes volume overload on the left ventricle
leading to hypertension
• Usually manifests a few weeks after birth
• Often closes on its own as heart grows
(childhood and adolescence)
• Symptoms:
– Breathlessness
– Poor feeding
– FTT
VSD
• If the opening is large, closing the hole in the
first two years of life (patch or sewn together)
is recommended to prevent serious problems
later.
• Collectively, the most common congenital
heart defect (2-6:100,000 births)
• 30-60% of all NB with a congenital heart
defect
Tetralogy of Fallot
• TOF
• Four key features
– Pulmonary stenosis
– Overriding aorta
– VSD
– Right ventricular hypertrophy
• An ASD or PFO is called a pentalogy of Fallot
TOF
TOF
• 400 per million live births
• Males > females
• ‘Tet spells’
– Sudden marked increase in cyanosis followed by
syncope; may result in hypoxic brain injury and
death
– Older children squat, which cuts off circulation to
the legs and improves blood flow to brain and
vital organs
TOF
• Emergency management of tet spells
– Beta blockers (propanolol)
– Morphine
– Ephinephrine
• Palliative surgery (shunt) then total surgical
repair when older as required
TOF
• 90% of patients with total repair as infant
develop progressively leaky pulm valve as
heart grows to adult size but valve does not
• At risk for sudden cardiac death or heart
failure.
Z-plasty
• Plastic surgery technique
• Improves functional and cosmetic appearance
of scars
• Can elongate a contracted scar or rotate the
scar tension line
• Used in repair of cleft lip, palate and
ankyloglossia
Z-plasty
Z-plasty
That’s all folks!