Tips for doing well in neonatology section of Pediatric Boards

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Tips for doing well in neonatology section of
Pediatric Boards

• Shantanu Rastogi, MD, FAAP
• Neonatologist, Maimonides Medical Center
• Assistant Professor of Pediatrics
• Mount Sinai School of Medicine

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Some general points

• There are no negative markings and hence no
  questions SHOULD be left unanswered
• When the answers are not clear in the first
  reading then try the method of exclusion to get
  to the best possible answer

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Rastogis Rule

• Common presentations of common diseases
• Rare presentations of common diseases
• Common presentations of rare diseases
• Rare presentations of rare diseases

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FETAL WELL BEING
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Biophysical profile

• 5 categories with score of 0 or 2
• NST, fetal body movements, breathing, fetal tone,
  amniotic fluid volume
• 10 is well fetus, 2 is certain fetal asphyxia, 4
  or 6 needs frequent reevaluation for delivery

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Electronic Fetal Heart Rate Monitoring

• Normal FHRv of 6-15bpm, basis of nonstress
  test-reactive positive test is normal
• Abnormal patterns
• Tachycardia, gt160, infection
• Bradycardia, lt110, head compression
• Loss of FHRv, hypoxia
• Decelerations
• Early, mirror image of uterine contractions, head
  compression
• Variable, irregular, umbilical cord compression
• Late , occur 10sec after uterine contraction and
  last longer, uterine placental insufficiency

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RESUSCITATION
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Resuscitation-recent changes

• No intrapartum meconium suctioning
• Oxygen use
• Can use lt100 but if no improvement in
  saturations, increase to 100
• If lt32w to use pulse oximeter keeping saturations
  between 90-95
• Epinephrine IV is preferred but if using ET use
  x10 dose
• Special use of laryngeal mask, CO2 monitor, and
  careful temp. control-use of clear plastic bags

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Case

• 28 w baby is delivered vaginally has HR of 90/m
  and irregular respiration, cyanosis, hypotonia
  with no reflexes
• WHAT IS THE APGAR SCORE?
• WHAT IS THE FIRST STEP?
• WHAT IS THE SUBSEQUENT STEP?

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Case continued

• After 1 min of IPPV the HR is 60/m
• WHAT IS THE NEXT STEP?

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INFECTIONS
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GBS

• The total number of cases of sepsis has gone down
  due to the decrease in GBS sepsis, but that from
  gram negative remain the same
• CDC guideline-recent changes
• To screen all women at 35-37 wks of gestation
• If GBS positive prior preg. does not mean it is
  positive in present pregnancy unless there was
  invasive neonatal infection
• No prophylaxis required for elective c-section if
  GBS positive and with no ROM/labor
• Adequate prophylaxis is by completion of 2 doses
  of penicillin

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GBS

• Risk Factors
• Intrapartum Prophylaxis to mother -if GBS
  positive OR unknown with lt35 weeks GA, ROM 18h,
  maternal fever 100.4F OR GBS bacteriuria OR
  previous invasive neonatal GBS
• Rx to baby-Symptomatic OR if mother for IP and
  baby less than 35 wks/ROM gt18h/duration of IP
  less than 4h.
• Clinical presentation
• Early-lt7DOL, pneumoniagtsepsisgtCNS, ascending
  infection
• Late onset-gt7DOL, less mortality but more CNS
  involvement and sequelae, deep infection as
  cellulitis, arthritis, osteomyelitis

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Other bacterial infections

• E.coli/Klebsiella sp. Absolute numbers have not
  decreased and hence the proportion of the
  neonatal sepsis cases from these organism have
  increased. Typically progress thru 3 stages of
  shock
• Listeria- gram rod, in unpasteurized milk,
  cheese, raw vegetables and uncooked meat
• Early onset lt7d, transplacental, chocolate
  colored amniotic fluid, preterm deliveries,
  sepsis/pneumonia
• Late onset gt7d, nosocomial, meningitis with
  mononuclear cells

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Other congenital infections
Clinical presentation CMV Rubella Toxoplasmosis
LBW
Liver/Spleen
Jaundice
Petechiae
CHD
Cataract
Retinopathy
Cerebral calcification Periventricular cortical
Microcephaly
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Hepatitis B positive mother
Mat. status Newborn gt2kg Newborn gt2kg Newborn lt2kg Newborn lt2kg
HBV HBIG HBV HBIG
3 doses, 1stlt12h 1 dose, lt12h 4doses, 1stlt12h 1dose, lt12h
unknown 3 doses, 1stlt12h 1 dose, lt7d 4doses, 1stlt12h 1dose, lt12h
_ 3 doses, 1st at1-2M None 3 doses, 1st at1-2M None
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Congenital Syphilis
VDRL
Inference
FTA
Mother Baby Mother Baby
- - - - No or prozone
- - False
/- Mother/B disease
- - Treated disease
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Conjunctivitis

• Onset will give the clue
• Hours-chemical-silver nitrate
• Day 2-5-Nisseria gonorrheae-purulent, emergency,
  needs IV antibiotics
• Day 5-14-Chlamydia-bilateral, cough

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CHROMOSOMAL ABN.
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Trisomy 21

• Recurrence risk- If no translocation- 1 risk
  till mat.age of 37y, if mat. translocation-10-15,
  if pat. translocation-5
• Types- 94 non-disjunction,3-5 translocation, 2
  mosaic. Commonest cause in both old and young
  mothers is non-disjunction

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Trisomy 21

• Defects
• Cardiac (40-50)-Endocardial cushion defect, VSD
• Extremities-single palmer crease, 5th finger has
  hypoplastic middle phalange and clinodactyly
• Face-slanting palpebral fissure, Brushfield
  spots, epicanthic folds, short neck, flat occiput
• GI- duedenal atresia, Hirschsprung Disease
• Neurology- hypotonia, MR,
• Other hypothyroidism, leukemia, hip dysplasia

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Other chromosomal anomalies

• Trisomy 13 (Patau-MIDLINE deformities)
• Holoprosencephaly,cleft lip/palate,coloboma,
  sloping forehead, cutis aplasia, VSD,
  polydactyly, hyperconvex nails, persistence of
  fetal Hb
• Trisomy 18 (Edward)
• Cardiac (common, VSD, PDA, PS),clenched hand,
  overlap of 2nd over 3rd and 5th over 4th finger,
  rocker bottom feet, small mouth/eyes/palpebral
  fissures, short sternum, hernia, cryptorchidism

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PULMONARY
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Case

• 28 wk preterm baby was delivered and intubated
  and placed on settings of RR 40, PIP of 25, PEEP
  of 5 and O2 of 100. Decision was made to give
  surfactant.
• WHAT IS THE NEXT EXPECTED CHANGE IN THE
  VENT.SETTINGS?
• If surfactant was not given as the O2 requirement
  decreased to 50 and was clinically observed.
• WHAT CHANGE WILL NEED TO BE FOLLOWED FOR
  PREDICTING IMPROVEMENT?

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RDS-Surfactant Def.

• Clinical course Peak-1 to 3 d and recovery
  starts with diuresis
• Risk Factors Low GA, male, Mat. DM, perinatal
  depression
• RDS in term SPB def, Mat. DM, Beckwith Weideman
  syndrome, congenital syphilis
• Pathology Hyaline membrane (cellular debris in
  fibrinous matrix)
• Treatment-surfactant replacement,
  fluid/electrolyte and respiratory management
• Complications pneumothorax as sudden
  decompensation
• DD for reticulogranular CXR- GBS pneumonia, PAPVR
  
  

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Congenital malformations

• CCAM
• cyst of lung tissue with small communication with
  bronchial tree, blood supply from pulm.
  circulation
• Can present IU (hydrops, polyhydramnios, pulm.
  hypoplasia) or neonatal ( resp. distress)
• 3 types
• I - large cyst, good prognosis
• II- medium size cyst, 50 associated anomalies,
  prognosis ??
• III- large lesion with multiple small cyst,
  usually present IU, poor prognosis

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Congenital malformations

• Congenital emphysema
• Commonest lesion, commonest in left upper lobe
• Over distention of lobe due to loss of cartilage
  in large airways
• Variable clinical usually mild respiratory
  distress
• Sequestered lung
• Non functioning lung tissue with systemic b.s.
• 2 types
• Intralobar - mostly left lower, few other
  anomalies
• Extralobar - mostly between LL and diaphragm,
  many associated anomalies
• Often asymptomatic, rarely can present in utero

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Case

• FT BB delivered by stat c/s for bradycardia
  required resuscitation by IPPV. Improved but soon
  developed progressive respiratory distress and
  poor perfusion. ABG showed 7.1/72/42/-5/18 on
  100 oxygen.
• WHAT IS THE PROBABLE CAUSE?
• WHAT NEEDS TO BE DONE NOW?

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Air leak syndromes

• Pneumothorax
• Air between parietal and visceral pleura
• Risk Factors-Aspiration synd.,lung diseases (RDS,
  MAS), ventilation (high PIP)
• Presentation can be with tension (severe RD,
  bradycardia, apnea, hypotension with mediastinal
  shift), large leak or small leak.
• Complication IVH by dec. venous return, SIADH
• Diagnosis asymmetrical air entry,
  transillumination
• Rx EMERGENCY if tension-needle aspiration
  followed by chest tube, supportive

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Airleak Syndromes

• Pneumopericardium- air in pericardial sac
• Usually associated with other airleak syndromes
• If large- muffled HS, venous congestion,
  decreased CO
• Rx if symptomatic-pericardial aspiration, high
  mortality
• Pneumomediastinum- air in mediastinum
• Usually after IPPV or difficult intubation, high
  PIP
• Muffled heart sounds, CXR-sail sign
• Usually need supportive treatment
• Pulmonary Interstitial Emphysema-air in
  interstitial space
• Usually preterm with RDS and on ventilation
• Rx decrease MAP, if unilateral-selective
  intubation/blocking of bronchus

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Chronic lung disease Vs BPD

• BPD- 36 w of GA with oxygen requirement
• Mechanical trauma to susceptible lungs (preterm
  lungs) leading to inflammation, injury is
  increased with decreased antiproteases and
  antioxidants in the preterm
• Poor compliance, increased WOB, pulmonary
  hypertension, RVH
• Radiographic classification I to IV
• Rx- nonspecific as supportive care, good
  nutrition (120-150cal/kg/day), diuretics,
  bronchodilators, steroids

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Apnea

• Cessation of air flow for gt20s with
  cyanosis/bradycardia
• Types Central (no effort, no air flow),
  Obstructive (no airflow despite effort), Mixed
• Cause prematurity (usually after 12h of life),
  infection, maternal med.(narcotics, magnesium),
  infant med.(Indomethacin), CNS disorders (IVH)
• Treatment Treat underlying disease,
  methylxanthines (caffeine) , CPAP, ventilation

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Transient Tachypnea of Newborn

• It is a diagnosis of exclusion
• Cause delayed clearing of lung fluid
• Risk factors elective c/s, maternal DM,
  perinatal depression, precipitous delivery
• Usually resolves 2-3 days
• Treatment oxygen/ CPAP

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Meconium aspiration syndrome

• Definition MSAFRDCXR changes
• Clinical usually post-term, severe respiratory
  distress
• Complicationspulmonary hypertension, airleak
  syndromes
• CXRsnow storm appearance
• Prevention ??intrapartum suction of meconium
• Rx respiratory support, correcting acidosis,
  antibiotics, surfactant

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Case

• FT AGA BB was delivered to mother with GDMA2 not
  well controlled. AS were 8,9 developed
  respiratory distress with cyanosis within few
  hours of being in the WBN. Vitals are stable with
  tachypnea associated with saturation of 70 in the
  right hand and 45 in the left leg. CXR shows
  lungs with decreased blood flow.
• HOW TO CONFIRM THE DIAGNOSIS?
• WHAT IS THE DRUG OF CHOICE?HOW IS ITS TOXICITY
  MONITORED?

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Pulmonary Hypertension

• Cause
• Maladaptation- normal vasculature but
  vasoconstriction (hypoxia, hypothermia,
  polycythemia, pneumonia)
• Maldevelopment-abnormal structure of pulmonary
  vascular bed (chronic intrauterine hypoxia,
  pulmonary hypoplasia)
• Rx- respiratory support (no hyperventilation),
  correcting acidosis, NO, ECMO

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Congenital diaphragmatic hernia

• Types-Posterolateral thru Foramen of Bochdalek
  (LgtgtR) and central thru Foramen of Morgagni
• In-utero as hydrops, after birth as RD due to
  lung hypoplasia, scaphoid abdomen
• Complications-related to lung hypoplasia and
  pulmonary hypertension (PH)
• Treatment strategy-IMMEDIATE intubation,
  stabilizing the PH and delayed surgical repair
• Usually delivered in ECMO centers as may be
  needed for treating PH

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Hypoplastic lungs (Potters sequence)

• Causes
• Intrathoracic compression (CDH, CCAM)
• Thoracic (neurological ds. as Werdnig Hoffman
  syndrome)
• Extrathoracic (common, causes of oligohydramnios
  specially kidney diseases)
• Clinical- related to primary cause but usually
  present with severe respiratory distress
• Rx respiratory support and treatment of the cause

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Neurological ds. causing RD

• Werdnig Hoffman- usually in neonatal period as
  hypoplastic lungs/hypoventilation
• Brachial plexus injury- with h/o shoulder
  dystocia and LGA, can be associated with Horner's
  Syndrome, chest fluoroscopy -eventration of the
  diaphragm
• Injury after cardiac surgery-recurrent laryngeal
  nerve injury mild distress with stridor, direct
  laryngoscopy will show ipsilateral vc in
  cadaveric position with no movement with crying.

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CARDIOLOGY
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Congenital Heart Disease-Some Facts

• Incidence 8/1000 live births (excluding PDA in PT
  newborns) with 25 with other associated
  abnormalities
• VSD- commonest CHD
• TOF- commonest cyanotic HD beyond neonatal period
• TGA-commonest cyanotic HD in first week of life
• HLHS- 2nd commonest cyanotic HD in first week of
  life and commonest cause of cardiac mortality
  during that period

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Congenital cardiac disease

• When to suspect-murmur, cyanosis with minimal
  respiratory distress
• IMMEDIATE need for ECHO, consider starting
  PROSTAGLANDIN. 3-5 babies with PG have apnea-
  might require intubation

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Presentation of CCHD

• 5Ts, DO, ESP- TGA, TOF, TAPVR, Tricuspid
  atresia, Truncus arteriosus, DORV, Ebsteins
  Anomaly, Single ventricle and Pulmonary atresia
• HLHS Vs sepsis usually HLHS presents after the
  duct is closed by 48-72 h and baby presents with
  cardiac failure to the ER with no murmur. If no
  high risk factors for infection always consider
  the diagnosis of HLHS

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PDA

• Normal course-physiological closure 12-15h ,
  anatomic closure several months, about 4 of
  term, 10 of 30-37 wk and 50 of lt30 wk do not
  close by 72 h and considered PDA
• S/S-
• Term-asymptomatic, machinery murmur, if large
  have bounding pulse, CHF
• Preterm can also have decreased bf to the
  gut-NEC, Pulmonary hage. and prolonged
  intubation
• Treatment-Fluid restriction, maintaining
  hematocrit, ibuprofen (indomethacin), surgical
  correction

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Maternal conditions and CHD

• Maternal drugs
• Aspirin/Indomethacin-PH/PDA closure,
  Lithium-Ebsteins anomaly, Ethanol-VSD
• Maternal diseases
• Lupus-Cong. Heart block (anti Ro, anti La Ab),
  Diabetes (VSD-commonest, TGA, ventricular
  hypertrophy-most specific)

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NEUROLOGY
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SCaLP Injuries

• In SubCutaneous tissue- caput succedaneum, soft,
  crossed midline/sutures, usually with molding,
  resolves over several days
• Beneath Galea Aponeurotica in Loose areolar
  tissue- subgaleal, can move to neck and behind
  ear, can cause anemia, hypotension, jaundice,
  resolves in 2-4 wk
• SubPeriosteal- cephalhematoma, confined to suture
  lines, firm, 10 have skull fracture, jaundice,
  resolve in weeks to months

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Intraventricular Hemorrhage

• From where-germinal matrix, usually involutes by
  34 wk
• Incidence increases with PT e.g. lt1kg 30,
  1-1.25kg 15, 1.25-1.5kg 8
• Timing- 50 in 24h and 90 in 72h present as S/S
  of anemia CNS involvement.
• Grading- I thru IV
• Prognosis- Poor in grade III/ IV
• Complication- hydrocephalous/PVL

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Periventricular Leukomalacia

• Where- periventricular white matter usually focal
• Risk factors-prematurity, hypotension, IVH
• US shows bilateral periventricular echodensities
• Outcome usually as spastic diplegia with
  associated cognitive and visual defects

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Birth Asphyxia

• Definition
• pH lt7, ASlt3 at 5min, neurological sequelae (HIE),
  multiple organ dysfunction
• HIE staging ( Sarnat Stages)
• Stage 1-usually hyperactive CNS with
  sympathomimetic activity, 100 normal
• Stage 2-decreased CNS activity, lost reflexes,
  parasympathomimetic activity, seizures, 80
  normal
• Stage 3- variable presentation, seizures rare,
  burst suppression EEG, 100 severe sequelae

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Cerebral palsy

• Non progressive neurological deficit
• Incidence2-5/1000, increase with dec. GA
• Clinicopathological correlation
• Selective neuronal necrosis-common after HIE,
  diffuse damage, Quadriplegia, MR, seizures
• Parasagittal cerebral injury-due to dec.
  perfusion, necrosis in watershed areas of
  carotids, weakness of proximal muscles UgtL
• Focal or multifocal ischemia- usually in FT,
  meningitis, trauma, thrombotic syndromes, as
  hemiplegias, seizures, cognitive defects
• Status Marmoratus- kernicterus, HIE, basal
  ganglia, spasticity, choreoform movements

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Brachial plexus injury
Erb-Duchenne Klumpke
Roots C5-7 C8-T1
Incidence Common Rare
Typical S/S Waiters tip Ape hand
Differentiate Palmer grasp -
Associated C4/5 (phrenic nerve), C7 (scapular winging) T1( Horners syndrome)
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Neural Tube Defect

• Incidence variable geographically, more with
  folic acid def., maternal diabetes, valproate
  intake
• Intrauterine diagnosis- inc. alpha fetoprotein
  and cholinesterase in amniotic fluid
• Prognosis-level of involvement e.g. no ambulation
  with cervical, thoracolumbar involvement and with
  sacral can ambulate without braces

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Hydrocephalous

• 2 types
• Obstructive- common, commonest cause is post
  hemorrhagic HC, others are aqueductal stenosis,
  Dandy-Walker Syndrome (cystic dilatation of 4th
  ventricle, 70 have other abnormalities), rarely
  masses
• Communicating-usually after bleeds, infections,
  NTD, Arnold Chiari malformation

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Case

• Mother is rushed in for stat c/s for abruption.
  Apgars are 2, 3, 8- improving after IPPV, chest
  compression and fluid resuscitation. Improves and
  admitted to NICU. Mother had uneventful antenatal
  course. Baby develops generalized tonic clonic
  seizures and bradycardia at 12h of life-
  controlled by phenobarb.
• WHAT IS THE CAUSE OF THE SEIZURES

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Siezures

• Types
• Subtle-most frequent, oral, facial ocular
  activity, may be associated with changes in HR,
  resp. BP and sats.
• Multifocal clonic-one limb migrating to another
• Focal clonic- may represent focal disease
• Tonic-change in posture, more in preterm
• Myoclonic
• Many causes- usual asphyxia, metabolic (Ca,
  glucose etc), infection, trauma, malformation
• Initial drug of choice is phenobarb

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METABOLIC/ENDOCRINE
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Inborn errors of metabolism

• When to suspect just about any S/S specially if
  the initial usual diagnosis e.g. sepsis is not
  responding to the usual forms of treatment
  e.g.antibiotics.
• Specific smells
• Sweaty feet-Isovaleric acidemia/Glutaric aciduria
• Male cat urine-Glycinuria
• Maple syrup odor-Branched chain aa(MSUD)
• Musty odor-PKU

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Flow chart of IEM-with hyperammonemia

• Acidosisketonuria-MSUD, Lactic acidemia,
  glutaric aciduria, glycinuria
• Acidosis without ketonuria-Fatty acid oxidation
  problem
• Without acidosis or ketonuria-urea cycle defects,
  check citrulline
• Very high-Argininosuccinic Acid (ASA) Synth. Def.
• Absent- check urine orotic acid
• High-Ornithine transcarboxylase def.
• Low/Normal-Carbamyl phosphatase synthetase def.,
  N-acetyl glutamate def.
• Normal or slight increase-check ASA
• Present-AS acid lysase def
• Absent-check arginine, if inc.-arginase def., if
  normal or low-transient neonatal hyperammonemia

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Galactosemia

• AR, galactokinase or Galactose-1-PO4ase
  uridyltransferase def.
• Presents when feeds are introduced as lethargy,
  hepatomegaly, liver failure, renal tubular
  acidosis
• Can have cataract at birth
• Increased risk of infection specially E.coli
• Lab-elevated LFTs, galactose in urine (reducing
  substance positive with negative glucose oxidase
  test )
• Rx- elimination of all galactose and lactose in
  diet

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PKU and Homocystinuria

• Classic PKU-AR, def. of phenylalanine
  hydroxylase, mousy or musty urine odor, severe MR
  and siezures if untreated, diagnosed by NBS, Rx
  by low phenylalanine diet
• Homocytinuria-AR, commonly by def. of
  cystathianine synthetase, usually asymptomatic in
  neonatal period, has downward dislocated lens
  (D/D Marfans), myopia, osteoporosis, scoliosis,
  arachnodactyly, dec.joint mobility (D\D Marfans),
  MR seizures, thrombotic episodes, Rx Dec.
  methionine, supplement cytiene, folate, pyridoxine

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Glycogen storage disease

• 8 types, 1,2 3 are common
• Type1 von Gierke, Glucose 6 PO4ase def., has
  lactic acidosis, hepatomegaly, diarrhoea,
  bleeding disorder, poor prognosis
• Type 2-Pompes, lysosomal glucosidase def., muscle
  weakness, cardiomegaly, CHF, poor prognosis
• Type 3- Forbes, low glucose, hepatomegaly, muscle
  fatigue, usually after neonatal period, good
  prognosis

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MPS and Lipidoses

• MPS- dysostosis multiplex, AlderRielly bodies in
  WBC and urine MPS
• Hurler-iduronidase def., cloudy cornea, HSM,
  coarse features, short stature, kyphosis
• Hunters-iduronidase sulfatase def., Xlinked, only
  MPS with retinal abn.
• Lipidoses
• Gauchers-glucocerebrosidase def., Gaucher cell in
  bone marrow, normal retina, type I-normal CNS,
  type II, profound CNS loss
• Niemann Pick-sphignomyelinase def., foam cells in
  bone marrow, Type A cherry red spot, profound CNS
  loss, Type B normal retina, normal CNS

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Temperature Regulation

• Neonates more prone to heat loss as
• Dec. skin thickness-radiantconductive loss
• Dec. subcut. Fat
• Dec. peripheral vasoconstriction leading to dec.
  heat conservation
• Immature autonomic nervous system
• Increased BSA to wt.-radiant heat loss
• Convective Incubators-large radiant loss(dec by
  double wall) small evaporative loss(dec by inc.
  humidity) and small conductive heat loss (dec by
  rubber mattress)
• Radiant Warmer-large convective and evaporative
  loss, reduced by plastic sheet

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Hypothyroidism

• Commonest cause-thyroid dysgenesis
• Early presentations-prolonged jaundice, large
  post. fontanelle
• Others-umbilical hernia, macroglossia, hypotonia,
  goiter
• Diagnosis- by newborn screening-low T4 and high
  TSH
• Rx-levothyroxine sodium

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Case

• Baby delivered after difficult vaginal delivery
  to a mother with gestational diabetes poorly
  controlled by insulin. Baby was 4300g and was
  send to WBN where he developed tachypnea with
  occasional jitteriness.
• WHAT IS THE D/D FOR TACHYPNEA IN THIS BABY?

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Hypoglycemia- IDM

• Commonest presentation of IDM and can primarily
  present as tachypnea (Other causes are RDS, TTN,
  CHD (VSD) , birth asphyxia birth trauma and
  hypocalcemia)
• Other common presentations are hypocalcemia,
  polycythemia and jaundice
• Specific malformations-Hypertrophic Obstructive
  Cardiomyopathy d/t asymmetrical ventricular
  septal hypertrophy and caudal agenesis syndrome

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Hypocalcemia

• Types
• Early (till 72h) maternal causes (DM,
  hyperparathyroidism), perinatal causes
  (prematurity, asphyxia, infections)
• Late (after 72h) hypoparathyroidism,
  hypomagnesemia, vitamin D def.
• S/S-If symptomatic as jitteriness, high pitched
  cry, Chvostek/Trousseau sign, siezures, prolonged
  QTc
• Rx- Underlying cause, Ca, Vit. D, low PO4

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Congenital Adrenal Hyperplasia

• Commonest cause-21 hydroxylase def. (followed by
  11beta hydroxylase def.)
• S/S with 21OH-salt wasting in 2nd week with
  inc.K, dec.Na and hypotension with
  pseudohermaphroditism in females and males may
  have precocious puberty.
• Diagnosis-elevated 17OHP in amniotic fluid or
  serum
• Rx-Antenatal-maternal glucocorticoid,
  Postnatal-replacement of GC/MC

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HEMATOLOGY
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Cord compression

• Usually presents as variable decelerations
• Most common presentation is anemia due to mild to
  moderate compression compresses the umbilical
  vein leading to pooling of the blood in the
  placenta leading to anemia usually seen in CBC
  done 4-6h of life.

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Twin to twin transfusion(TTT)

• Discordant twins when the weight of the twins
  differ by 15-25
• One of the causes of DT is TTT
• Usually TTT takes place in monochorionic, mono or
  di-amniotic twins, mortality can be 50
• Donor twin is anemic and develops hypovolemia and
  oligohydramnios
• Recipient twin is polycythemic and may develop
  hydrops
• When donor dies- blood flows from recipient
  leading to its demise soon after the donors hence
  need for STAT c/s

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Isoimmunization-Rhesus factor

• Decreased incidence due to use of Rhogam
• Mother is Rh- i.e. dd (Rhesus An has 3 components
  C, D, E with D as the major component). Baby is
  Rh i.e.DD or Dd.
• Initial pregnancy usually induces IgM which do
  not cross placenta, but repeat exposure induces
  IgG which crosses placenta easily causing
  hemolysis of fetal RBC.
• Prevented by giving Rhogam to Rh-mother at 28 wk
  GA and at birth of Rh baby.

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Isoimmunization-ABO

• Incidence same in first or subsequent pregnancies
• Mothers with group A or B produce IgM antibodies
  and that of O produce IgG which easily crosses
  placenta
• Usually milder than Rh as the antigen is on all
  the tissues and they capture the antibodies
  transferred from the mother
• Usually indirect Coombs is positive
• Has spherocytosis with B-O incomp.

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AAP guidelines for bili management

• To measure bilirubin in hours of life
• Aggressive phototherapy and specific follow up
  depending on the zone in the hourly bilirubin
  charts

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Phototherapy

• Mechanism of action
• Photo-isomerization-configurational 4Z15Z to
  4Z15E
• Photo-isomerization-structural-lumibilirubin
• Photo-oxidation
• Blue light- effective wavelength (710-780nm) and
  penetrates skin well.
• If phototherapy given to baby with high direct
  bilirubin -bronze baby syndrome

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Jaundice related to breast feeding Vs Breast
feeding jaundice

• Jaundice related to BF
• Usually exaggerated physiological jaundice due to
  decreased intake
• BF jaundice
• Prolonged with peak of 20-30mg/dl by 2 wk and
  than normalize over 4-12 wk
• Rapid decrease after cessation of breast feeding
  and rises 2-4mg/dl after resuming BF
• Can cause kernicterus

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Thrombocytopenia

• Sick Vs Well baby
• Commonest for well babies is Alloimmune and
  Autoimmune, and for sick babies is sepsis/DIC
• Autoimmune
• Transference of antiplatelet antibodies as that
  of lupus, ITP
• Maternal and newborns platelets are low
• Alloimmune
• Transplacental transference of maternal
  antibodies ( like Rh disease), with normal
  maternal platelets
• Severe, can have IC bleed, death in 20

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Case

• BB delivered at home with precipitous delivery
  was admitted to WBN and discharged with mother.
  Was exclusively breast fed. Found to have fresh
  bleeding per rectum on DOL 4. There is no other
  site of bleeding or pertinent history. Clinical
  examination is normal.
• WHAT NEEDS TO BE DONE IMMEDIATELY?

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Hemorrhagic Disease of Newborn (Vit.K def.)

• Vitamin K needed for carboxylation reaction which
  activates the clotting factors
• Newborns are predisposed as have no bacterial
  flora intestine to produce Vit.K and also has
  liver immaturity
• Decreased placental transference if maternal
  intake of anticonvulsants, warfarin and ATT or
  when exclusively breast fed.
• Types
• Early-less than 24h, maternal drugs
• Classic- 2-7days, exclusive breast feeding
• Late-2w-6m, hepatobiliary disease, inc.ICT

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GASTROENTEROLOGY
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Esophageal atresia

• 30-40 with associated abnormalities specially
  as VACTERL
• 4 types-commonest upper end atresia and lower
  end with fistula to the trachea
• H type is rare but commonest one for the exams-
  S/S as cough during feeding and recurrent
  aspirations
• Rx-surgical-primary or delayed as in stages.

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Case

• Baby is delivered to mother with history of
  polyhydramnios. Mother had irregular antenatal
  care. Baby did not tolerate feeds and started to
  have non bilious vomiting. OG tube could be
  passed to the stomach. AXR was ordered.
• WHAT WOULD BE THE LIKELY AXR PICTURE

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Double bubble sign

• Associated with duodenal atresia
• High rate of association with trisomy 21, other
  malrotation and CHD.

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Case

• 24 week PT AGA BG has had relatively uneventful
  course in NICU. At 8 weeks of life when
  tolerating full feeds developed abdominal
  distention. Feeds were stopped and AXR showed
  pneumotosis intestinalis. Antibiotics were given
  and no surgical intervention was required. Baby
  recovered.
• WHAT IS THE COMMONEST SEQUALAE THAT NEEDS TO BE
  WATCHED FOR?

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Necrotising Enterocolitis

• 10 of those less than 1500g
• Predisposing factors-prematurity, feeds,
  infection
• AXR- pneumotosis intestinalis
• Outcome-high mortality, morbidity-small gut
  syndrome if surgery is done, strictures if
  medically treated

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Congenital Hyperplastic Pyloric Stenosis

• 3/1000 births, male x5
• Related to decreased NO production
• Hypochloremic, hypokalemic, metabolic alkalosis
• Barium-string sign, US-bulls eye sign
• Rx-Pyloromyotomy

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Case

• 2D old FT Baby in WBN develops distention and
  has not passed meconium since birth. PMD orders
  an AXR which shows large dilated stacked loops
  with absence of air in the recto-sigmoid region.
• WHAT IS THE NEXT STEP?

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Case continued

• If it reveals gradual narrowing of the sigmoid
• WHAT IS THE LIKELY DIAGNOSIS AND HOW TO CONFIRM
  IT?

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Hirschsprungs Disease

• 15000, usually male, 80 rectosig. only
• Associated with trisomy 21
• Failure of cranial to caudal migration of neural
  crest cell-dec. parasymp. innervation
• Diagnosis-XR, biopsy-absent ganglion cells
• Complication-Acute bacterial colitis
• Rx- single stage pull through or initial
  colostomy followed by correction

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Meconium Plug vs. ileus vs. peritonitis

• Plug
• benign variation of Hirschsprungs disease
• Delayed passage of meconium
• Usually has small colon, IDM
• Ileus
• 90 have CF
• bilious vomiting, obstruction, AXR-bubbles in the
  intestinal lumen
• enema successful in 60
• Peritonitis
• In utero perforation
• Secondary to ileus, atresia, volvulus,
  gastroschisis
• Usually seal spontaneously or can require surgery

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Omphalocele vs. Gastroschises
Omphaloc. Gastroch.
Incidence Common Rare
Chrom. Abn. Common Rare
Midline Yes No (usu.Rt)
Covering Yes No
Umb.cord Involved Normal
Assoc.Abn. More Less-intest.abn
M/M More Less
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DERMATOLOGY
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ERYTHEMA TOXICUM

• Most common 30-70
• Onset DOL 2-3
• 1-3mm erythematous macule/papule-pustule
• Fades in 5-7 days
• May reoccur
• Benign, has eosinophils

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PUSTULAR MELANOSIS

• More in dark skinned
• 3 stages-non-inflammatory pustule, ruptured
  pustule with scale, hyperpigmented macule (up to
  3m)
• Benign, has neutrophils
• No Rx

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Mystery Case

• Baby boy born with oligohydramnios, Potters
  Sequence, and hypoplastic lungs requiring ECMO.
  Had B/L hydronephrosis diagnosed antenatally and
  the voiding cystourethrogram shows-

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