NEONATAL EMERGENCIES

Rebecca Starr, D.O.Pediatric Emergency Medicine FellowMarch 20, 2014

OBJECTIVES

Define the neonatal period and a helpful mnemonic for neonatal emergencies

Review case-based emergencies associated with the neonate

Discuss common infections in the neonate Differentiate between infectious, cardiac,

GI, metabolic, and endocrine emergencies Gain confidence in dealing with neonatal

patients

CASE PRESENTATION

7 day old M presents to the ED with a 1 day history of poor feeding, lethargy, and increased work of breathing.

Pre and postnatal history are unremarkable

What is your next step?

FREAK OUT!!!!

SO REALLY…. WHAT’S SHOULD I BE THINKING?

“THE MISFITTS” Trauma/Abuse (NAT) Heart and Lung Endocrine Metabolic disturbances Inborn errors of metabolism Sepsis Formula issues Intestinal Toxins Trisomies Seizures

CASE PRESENTATION #1

7 day old M presents to the ED with a 1 day history of poor feeding, lethargy, and increased work of breathing. At PMD today, a temperature of 101.5 noted rectally.

Pre and postnatal history are unremarkable

What is your next step?

RULE OUT SEPSIS WORK UP?

What do you want to order?

RULE OUT SEPSIS WORK UP

Blood culture CBC CMP Urinalysis Urine culture CSF studies CSF culture HSV PCR CXR (+/-) RVP (+/-) NS bolus (+/-) Antibiotics- Ampicillin and Gentamicin or 3rd

generation cephalosporin (0-28 days)

FEVER IN THE NEONATE

Neonate: 0-28 days Fever: 38 C or 100.4 F

Also consider hypothermia Difficult to evaluate clinically Increased susceptibility to infection >10% of infants with fever will have a serious bacterial infection

UTI- 30% Meningitis- 20% Bacteremia/septicemia- 15%

NEONATAL FEVER

Peripheral WBC alone not an accurate screen for SBI

Consider concomitant viral illness with SBI

All febrile neonates should have a full sepsis evaluation and be admitted for IV antibiotics

STUDY ON NEONATAL FEVER IN THE PEDS ED

2253 neonates ( 0-28 days old) 16% discharged, 84% admitted

Jain et al, Pediatrics, 2014

INFECTIONS IN THE NEONATE

Group B Streptococcus E. coli Listeria S. aureus H. influenza S. pneumonia N. meningitis Viral

RSV HSV Enterovirus

GROUP B STREPTOCOCCUS

Gram positive cocci Most common infection of the newborn Cause of neonatal pneumonia,

bacteremia, and meningitis Up to 1/3 of women are colonized Early and late-onset infections Tx: Ampicillin Fatality rates 2-15%

EARLY AND LATE ONSET

Early onset: 1 hour to 7 days Bacteremia 45% Pneumonia 40% Meningitis <10% Higher fatality rate

Late onset: 7 days to 3 months (27 day median) Bacteremia 45% Meningitis 40%

ESCHERICHIA COLI

Gram negative rod Most frequent cause of infection in the

first 7 days of life Most common cause of meningitis in

neonates Significant cause of UTI’s and urosepsis Tx: Gentamicin or 3rd generation

cephalosporin

LISTERIA MONOCYTOGENES

Gram positive rod Can mimic diphtheroids on gm stain

Highest incidence in patients < 1 month old Infected from colonized mothers

Meconium staining, PROM, transplacentally

Tx: Ampicillin Resistant to cephalosporins

Fatality rate 15%

CASE PRESENTATION #2

7 day old M presents to the ED with a 1 day history of poor feeding, lethargy, increased work of breathing and poor color. No history of fever and afebrile on presentation. Cap refill 4 seconds on exam and no palpable femoral pulses.

Pre and postnatal history are unremarkable

What diagnosis is concerning for this patient?

CONGENITAL HEART DISEASE

1/125 births Usually ductal dependent

Closes by 72 hours Symptoms include:

Tachypnea Cyanosis Pallor Lethargy FTT Sweating with feeds

Hypoxia and cyanosis usually unresponsive to oxygen Left and right sided heart lesions

CONGENITAL HEART DISEASE

Left sided: systemic blood flow is dependent on ductal patency Coarctation of the aorta Hypoplastic left heart

Right sided: pulmonary blood flow is dependent on ductal patency (Cyanotic Lesions) Truncus Arteriosus Transposition of the great vessels Tricuspid atresia Tetralogy of Fallot TAPVR

CLINICAL

Shock Poor/absent distal pulses Poor perfusion/color Cap refill >3 sec Tachypnea

Cardiac Failure Hepatomegaly Large heart Gallop Harsh murmur

CASE PRESENTATION #2

7 day old M presents to the ED with a 1 day history of poor feeding, lethargy, increased work of breathing and poor color. No history of fever and afebrile on presentation. Poor perfusion on exam.

Pre and postnatal history are unremarkable

What medication do you want to give?

WHAT TO DO?

Prostaglandin E1!!!!! 0.05mcg/kg/min Response within 15 minutes Watch for:

Hypotension, flushing, APNEA!

Pressure support Fluids Echo Cardiology consult

NAME THAT CARDIAC ABNORMALITY

TETRALOGY OF FALLOT

Boot-shaped heart

TETRALOGY OF FALLOT

Four criteria1. Pulmonary atresia/stenosis2. RV hypertrophy3. VSD4. Over-riding aorta

NAME THAT CARDIAC ABNORMALITY

TRANSPOSITION OF THE GREAT ARTERIES Egg on a string

TRANSPOSITION OF THE GREAT ARTERIES Most common cyanotic lesion

presenting in the first week of life To be compatible with life, mixing must

occur via an ASD, VSD, or PDA

NAME THAT CARDIAC ABNORMALITY

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

Snowman sign

TOTAL ANOMALOUS PULMONARY VENOUS RETURN

All four pulmonary veins fail to make their normal connection to the left atrium

CASE PRESENTATION #3

7 day old M presents to the ED with a 1 day history of poor feeding, irritability, very jittery, and mild respiratory distress. No fevers but clammy/ wet skin. PE reveals a tachycardic infant with microcephaly and triangular faces.

What do you want to know about Mom?

Maternal History of Grave’s Disease!

GRAVES DISEASE AND THE NEONATE

1-5% of infants from moms with Graves Results from the transplacental

passage of maternal stimulatory TSHR-Ab

Can be seen in mom’s with active Graves or ones previously treated with thyroidectomy or radioactive iodine

GRAVES DISEASE AND THE NEONATE

At birth, infants can be Hypothyroid with a goiter Euthyroid due to maternal PTU Hyperthyroid due to maternal TSHR-Ab

Neonatal screening Self- limiting Resolution by 12 weeks

NEONATAL THYROTOXICOSIS

Essentially “thyroid storm” picture Irritability Respiratory distress Tachycardic Hyperthermic Shock Cardiac Failure

NEONATAL THYROTOXICOSIS

Treatment includes: Beta-blockade

Propanolol 0.1mg/kg IV Blocking thyroxine production

PTU 5-10mg/kg PO Blocking thyroxine release

Potassium-iodide 1-4 drop PO Decreasing T4 T3 conversion

Dexamethasone 0.1mg/kg IV

CASE PRESENTATION #4

7 day old F presents to the ED with a 1 day history of poor feeding, vomiting, poor tone, and lethargy. No history of fever.

BP 50/32 with a cap refill of 4 seconds

It was a home birth and neonatal screening wasn’t performed

CASE PRESENTATION #4

Physical exams reveals

What is the diagnosis?

CONGENITAL ADRENAL HYPERPLASIA Autosomal recessive, variable penetrance Involve a defect in the adrenal production

of cortisol, mineralocorticoid, or both Salt-wasting or non-salt-wasting

21-hydroxylase deficiency: >90% of all cases

Functioning 21-hydroxylase Converts 17-hydroxyprogesterone into cortisol Converts progesterone to aldosterone

CONGENITAL ADRENAL HYPERPLASIA Lack of 21-hydroxylase causes:

Build-up of 17-hydroxyprogesterone Converted into androgens

CAH PRESENTATION

Cortisol deficiency hypoglycemia, hypotension, and shock

Aldosterone deficiency hyponatremia, hyperkalemia, and dehydration

Androgen excess virilization of female genitalia, less common in males Males will have normal genitalia at birth and

will present in salt-losing adrenal crisis

WORK UP

Blood work: CMP Accucheck 17-hydroxyprogesterone levels Cortisol levels Aldosterone and renin levels

CAH TREATMENT

NS bolus Treat any electrolyte abnormalities

If hypoglycemia given dextrose Na+ K+

Stress dose hydrocortisone 50-100mg/m2 IV Glucocorticoid and mineralocorticoid

activity

CASE PRESENTATION #5

7 day old F presents to the ED with a 1 day history of poor feeding, vomiting green material, poor tone, and lethargy. No history of fever.

BP 57/42 with a cap refill of 4 seconds What intestinal emergency are you

concerned for?

MALROTATION AND VOLVULUS

Congenital anomaly during intestinal development Small bowel predominantly on the right

side Cecum is displaced into the epigastrium Ladd’s bands course over the horizontal

part of the duodenum Small intestine mesentery has an

unusually narrow base Midgut is prone to volvulus

MALROTATION AND VOLVULUS

DIAGNOSIS

Abd xray May show duodenal obstruction “Double bubble sign”

Upper GI- gold standard Concern for malrotation if the duodenal

C-loop doesn’t cross midline and the duodenojejunal junction isn’t the left of the spine

MALROTATION ON UPPER GI

“Whirlpool sign” indicates volvulus

TREATMENT

ABC’s Fluid resuscitation NPO NG tube to suction Pediatric Surgery consult!

PUTTING IT ALL TOGETHER

History is key! (prenatal, birth, maternal) ABC’s IV access with appropriate blood work Fluids Antibiotics Imaging? Remember the differential

THE MISFITTS RESPECT THE NEONATE

THE MISFITTS

Trauma/Abuse (NAT) Heart and Lung Endocrine Metabolic disturbances Inborn errors of metabolism Sepsis Formula issues Intestinal Toxins Trisomies Seizures

REFERENCES

Jain S, Cheng J, Alpern E, et al. Management of febrile neonates in US pediatric emergency departments. Pediatrics. 2014;133:187-195.

Menrke DP, Nieman LK, Martin KA, et al. Diagnosis of classic congential adrenal hyperplasia due to 21-hydroxylase deficiency. In: UpToDate. March 2014.

Menrke DP, Nieman LK, Martin KA, et al. Genetics and clinical presentation of classic congenital adrenal hyperplasia due to 21-hydroxylase deficiency. In: UpToDate. April 2013.

Batra CM. Fetal and neonatal thyrotoxicosis. Indian Journal of Endocrinology and Metabolism.2013.17:50-54.

Questions?