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
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
Rastogi’s Rule • Common presentations of common diseases • Rare presentations of common diseases • Common presentations of rare diseases • Rare presentations of rare diseases
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
Electronic Fetal Heart Rate Monitoring • Normal – FHRv of 6-15bpm, basis of nonstress test-reactive positive test is normal • Abnormal patterns • Tachycardia, >160, infection • Bradycardia, <110, 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
Resuscitation-recent changes • No intrapartum meconium suctioning • Oxygen use • Can use <100% but if no improvement in saturations, increase to 100% • If <32w 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
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?
Case continued • After 1 min of IPPV the HR is 60/m • WHAT IS THE NEXT STEP?
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
GBS • Risk Factors: • Intrapartum Prophylaxis to mother -if GBS positive OR unknown with <35 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 >18h/duration of IP less than 4h. • Clinical presentation • Early-<7DOL, pneumonia>sepsis>CNS, ascending infection • Late onset->7DOL, less mortality but more CNS involvement and sequelae, deep infection as cellulitis, arthritis, osteomyelitis
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: <7d, transplacental, chocolate colored amniotic fluid, preterm deliveries, sepsis/pneumonia • Late onset: >7d, nosocomial, meningitis with mononuclear cells
Congenital Syphilis VDRL FTA Inference
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
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
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
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
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?
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
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
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
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?
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
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
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
Apnea • Cessation of air flow for >20s 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
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
Meconium aspiration syndrome • Definition: MSAF+RD+CXR changes • Clinical: usually post-term, severe respiratory distress • Complications:pulmonary hypertension, airleak syndromes • CXR:snow storm appearance • Prevention: ??intrapartum suction of meconium • Rx: respiratory support, correcting acidosis, antibiotics, surfactant
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?
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
Congenital diaphragmatic hernia • Types-Posterolateral thru Foramen of Bochdalek (L>>R) 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
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
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.
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
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
Presentation of CCHD • 5T’s, DO, ESP- TGA, TOF, TAPVR, Tricuspid atresia, Truncus arteriosus, DORV, Ebstein’s 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