From Apgar to Z-plasty

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From Apgar to Z-plasty

• Pediatric Medical Terms

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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

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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

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Apgar Score

• Virginia Apgar, U.S. anesthesiologist, 1909-1974)
• System of scoring infants 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

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Apgar Score
Score Score 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
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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)

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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

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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.

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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.

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Bronchopulmonary Dysplasia

• BPD
• lt34 weeks gestation and lt4.5 pounds
• Associated with infant respiratory distress
  syndrome (IRDS)
• Damage to lung tissue
• due to prolonged
• mechanical ventilation

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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.

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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)

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Congenital Diaphragmatic Hernia

• CDH
• Applied to a variety of congenital birth defects
  that involve abnormal development of the diaphragm

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CDH
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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

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Extracorporeal Membrane Oxygenation
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ECMO

• Provides both cardiac and respiratory support
  when heart and lungs can no longer serve their
  function
• 75 effective in saving a newborns life
• Cannot be lt4.5 pounds so rules out most premature
  and/or small BW (birth weight) infants

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ECMO machine
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ECMO Machine
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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

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Infant in ICU on ECMO
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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

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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

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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

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Apnea and Bradycardia

• ABC
• Apnea is a pause in breathing with one or more of
  the following characteristics
• gt 15-20 sec
• Associated with color change (pale, purplish or
  blue)
• Associated with bradycardia
• Bradycardia
• Slowing of the heart rate, usually lt80 bpm
• May be due to reflex (e.g., NGT or stooling)

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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

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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

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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!!

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CHARGE syndrome

• A recognizable (genetic) pattern of birth defects
• 19-10,000 births

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• 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

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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.

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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)

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NEC
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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)

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NEC

• Warning
• Picture of resection is next

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NEC
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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

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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)

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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

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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 arent sick, you are said to be colonized
  but not infected.
• You can pass the bacteria on to others, though.

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MRSA

• MRSA infections start out as small red bumps that
  can quickly turn into painful abscesses

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Katedont look
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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

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Katedont look, again
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MRSA

• Prevention
• Wash you hands/use alcohol sanitizer
• Wash you hands often
• Dont 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

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Tracheoesophageal Fistula

• TEF
• Abnormal connection (fistula) between the
  esophagus and trachea.
• Common congenital abnormality
• In adults, usually sequela of surgical procedure
  such a laryngectomy

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TEF
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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

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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)

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Congenital TEF
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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

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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

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Congenital TEF

• Some babies with TEF also have other
  abnormalities
• Most commonly associated with VACTERL association

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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)

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VACTERL Association
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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)

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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

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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

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RSV
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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.)

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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

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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

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PDA
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PDA

• Typically found in preemies, congenital rubella
  syndrome and chromosomal abnormalities such as
  Down Syndrome
• Relatively common in US 81000 births (preemies)
• In full-term (21000 births)
• Twice as common in girls

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PDA

• Symptoms
• Increased WOB, poor weight gain
• Treatment
• Ligation DA manually tied shut, plugs or coils
• Indomethacin (inhibitor of prostaglandin)
• Usually used with preemies

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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

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VSD

• A large amount of oxygen-rich blood from hearts
  left side is forced through the defect in to the
  right side. Then its pumped back to the lungs,
  even though its 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.

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VSD
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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

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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 (2-6100,000 births)
• 30-60 of all NB with a congenital heart defect

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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

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TOF
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TOF

• 400 per million live births
• Males gt 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

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TOF

• Emergency management of tet spells
• Beta blockers (propanolol)
• Morphine
• Ephinephrine
• Palliative surgery (shunt) then total surgical
  repair when older as required

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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.

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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

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Z-plasty
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Z-plasty
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Thats all folks!