From Apgar to Z-plasty

1. From Apgar to Z-plasty Pediatric Medical Terms

2. 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

3. 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

4. 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

5. Apgar Score

6. 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)

7. 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

8. 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.

9. 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.

10. 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

11. 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.

12. 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)

13. Congenital Diaphragmatic Hernia • CDH • Applied to a variety of congenital birth defects that involve abnormal development of the diaphragm

14. CDH

15. 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……

16. Extracorporeal Membrane Oxygenation

17. 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

18. ECMO machine

19. ECMO Machine

20. 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

21. Infant in ICU on ECMO

22. 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

23. 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

24. 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

25. 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)

26. 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

27. 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

28. 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!!

29. CHARGE syndrome • A recognizable (genetic) pattern of birth defects • 1:9-10,000 births

31. 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

32. 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.

33. 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)

34. NEC

35. 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)

36. NEC • Warning – Picture of resection is next

37. NEC

38. 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

39. 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)

40. 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

41. 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.

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

43. Kate…don’t look

44. 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

45. Kate…don’t look, again

46. 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

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

48. TEF

49. 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

50. 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)