Pediatric Emergency Conference Guanzon Guerrero Guerzon Guevarra Guinto Gutierrez Hermoso Icasas Ignacio

Pediatric Emergency Conference

Guanzon Guerrero Guerzon Guevarra Guinto Gutierrez Hermoso Icasas Ignacio

General DataMichael 3 week old male

Chief complaint: seizures

History of Present Illness:

Gestational and Birth History: 25 year old primigravidUnremarkable prenatal and birth historyCS - due to CPDBW: 2.8 kgSpontaneous cry and respirationNursery Stay: 3 daysNewborn screening:

Positive for congenital adrenal hyperplasia- Advised confirmatory test and further work-up but was

not able to comply

History of Present Illness:2 days PTC:

(+) 5x water stools(+) 3x vomiting of previously

ingested foodFew hours PTC:

- drowsy- generalized tonic-clonic

seizuresRushed to the ER

Initial assessment at the ER:

Was in active seizures

afebrileCR: 180 bpmRR: 50-60 cpmBluish lips

Initial management at the ER:Oxygen per mask was

administeredDiazepam 0.3 mg/kg/dose per IV

at 15 mins. Interval for 3 doses

(Seizure persisted)

Phenobarbital – loading dose

Secondary assessment:Unremarkable chest and

abdomen(+) hypospadiasPenile length: 1.5 cmDarkly pigmented scrotal sac

Pulses were full and equalGood capillary refill

Laboratory results:Lab tests Result Reference Value

Na 120

K 6.0

Hgt low

Salient Features

Differential DiagnosisPresenting manifestation: seizure

in a neonateSeizure: paroxysmal, time limited

change in motor activity and/or behavior that results from abnormal activity in the brain

Common in pediatric age groupNeonates are at particular risk for

the development of seizureNelson’s textbook of Pediatrics 17th edition

Manifestations of a seizure:• Vocal: Cry or gasp, slurring of words, garbled

speech• Motor: Head or eye turning, eye deviation,

posturing, jerking (rhythmic), stiffening, automatisms (purposeless

repetitive movements such as picking at clothing, lip smacking); generalized or focal movements

• Respiration: Change in breathing pattern, cessation of breathing, cyanosis

• Autonomic: Pupillary dilatation, drooling, change in respiratory or heart rate, incontinence, pallor, vomiting

• Loss of consciousness or inability to understand or speak

Practice parameter: Evaluating a first nonfebrile seizure in childrenReport of the Quality Standards Subcommittee of the American Academy of Neurology, the Child Neurology Society, and the American Epilepsy SocietyD. Hirtz, MD; S. Ashwal, MD; A. Berg, PhD; D. Bettis, MD; C. Camfield, MD; P. Camfield, MD; P. Crumrine, MD; R. Elterman, MD; S. Schneider, MD; and S. Shinnar, MD, PhD

Seizure

Infectious

causes:

Bacterial

Viral

Fungal

AfebrileFebrile

Seizure – like Events

•Benign Paroxysmal Torticollis of Infancy•Breath holding spells: - cyanotic spells - pallid spells•Shuddering attacks•Night terrors

• Metabolic disorders• Electrolyte

imbalance• Hypoglycem

ia• Hypoxia• Drug related

Non-febrile seizure

Things to consider:Metabolic:

Congenital Adrenal HyperplasiaElectrolyte Imbalance:

diarrhea(5X) + vomiting (3x)Hypoxia:

bluish lips

Seizuresresult from rapid abnormal electrical

discharges from cerebral neuronspresents clinically as involuntary alterations

of consciousness or motor activityConsumption of oxygen, glucose, and

energy substrates (e.g, ATP, phosphocreatine) is significantly increased in cerebral tissue during seizures.

Optimal delivery of these metabolic substrates to cerebral tissue requires adequate cardiac output and intravascular fluid volume.

Pediatric, Status Epilepticus; emedicine 2008

Factors that lower Seizure ThresholdSleep deprivationHyperventilationPhotic stimulationInfectionMetabolic disturbancesHead traumaCerebral ischemiaKindling

Handbook of Neurosurgery by Greenberg

Drugs which can cause seizures

Antibiotics◦Penicillins◦Isoniazid◦Metronidazole

Anesthetics, narcotics◦Halothane,

enflurane◦Cocaine,

fentanyl◦Ketamine

Psychopharmaceuticals◦Antihistamines◦Antidepressants◦Antipsychotics◦Phencyclidine◦Tricyclic

antidepressants

Important points in the History The course of current seizure activity

◦ Time and nature of onset of seizure activity◦ Involvement of extremities or other body parts◦ Nature of movements (eg, eye movements, flexion,

extension, stiffening of extremities), including any focal movements and details of postictal neurologic deficit

◦ Incontinence◦ Cyanosis (perioral or facial)◦ Duration of seizure activity prior to medical attention◦ Mental status after cessation of seizure activity

Fever or intercurrent illnesses Prior history of seizures - If present, specify

medications, anticonvulsant use, and compliance.


Important points in the HistoryHead injury (recent and remote)CNS infection or disease (eg, meningitis,

neurocutaneous syndrome)Intoxication or toxic exposureOther CNS abnormality (eg, ventricular-

peritoneal shunt, prior CNS trauma)Birth history and developmental delay (eg,

anoxic encephalopathy, cerebral palsy)Other medical history (eg, acquired

immunodeficiency syndrome, systemic lupus erythematosus, type 1 diabetes mellitus)


Important points in the PE Signs of sepsis or meningitis

◦ Temperature more than 38.5°C; in patients younger than 2-3 months, more than 38.0°C

◦ Respiratory distress◦ Cyanosis◦ Poor peripheral perfusion◦ Bulging fontanelles in infant◦ Meningismus (in children >12-18 mo)◦ Presence of petechiae or purpura, herpetic vesicles

Evidence of head or other CNS injury◦ Bradycardia, tachypnea, and hypertension (Cushing triad for signs of

increased intracranial pressure)◦ Poor pupillary response◦ Asymmetry on neurologic examination◦ Abnormal posturing◦ Gross deformity or soft tissue injury to head

Hallmarks of neurocutaneous syndromes (e.g., port wine stain)


Monitoring of Vital FunctionsRespiratory rate, blood pressure,

cardiac rateObservation of seizure activitySkin bruises, petechiae or needle

marksPapilledema, retinal hemorrhagesOrganomegaly and abdominal

tenderness

Status EpilepticusContinuous clinical or

electroencephalographic seizures lasting for at least 30 minutes or recurrent seizures without return of consciousness during interictal period: the series lasting for 30 minutes or more. It is a medical emergency.

Handbook of Medical & Surgical Emergencies, 6th edition

Clinical Classification of Status Epilepticus

Overt generalized convulsive status

epileptus

Subtle generalized convulsive

status epilepticus

Simple status epilepticus

Nonconvulsive status

epilepticus

Continuous convulsive activity and intermittent convulsive activity without regaining full consciousness

•Convulsive (tonic-clonic)•Tonic•Clonic•Myoclonic

Coma following generalized convulsive status epilepticus with or without motor activity

Consciousness preserved

•Simple motor status epilepticus•Sensory status epilepticus•Aphasic status epilepticus

Consciousness impaired; twilight or fugue state

•Petit mal status (absence status)•Complex partial status epilepticus

Handbook of Medical & Surgical Emergencies, 6th edition

EtiologiesFebrile seizuresCerebrovascular accidentsCNS infection IdiopathicEpilepsySubtherapeutic antiepileptic drugElectrolyte imbalanceDrug intoxicationAlcohol withdrawalTraumatic brain injuryAnoxiaTumor


Neonates (first month of life)

Early childhood (<6 y)

Children and adolescents (>6 y)

Birth injury (eg, anoxia, hemorrhage) and congenital abnormalities

Birth injury Birth injury

Metabolic disorders (eg., hypoglycemia, hypocalcemia, hyponatremia) and inborn errors of metabolism (eg., lipidoses, amino acidurias)

Febrile convulsions (3 mo to 6 y) Trauma

Infection (eg, meningitis) Infection Infection

Metabolic disorders Epilepsy with inadequate drug levels

Trauma Cerebral degenerative disease

Neurocutaneous syndromes Tumor

Cerebral degenerative diseases Toxins

Tumors Idiopathic

Idiopathic


DIFFERENTIAL DIAGNOSIS

In children < 1year age75% acute cause

30% electrolyte disorders28% secondary to CNS

infection19% associated with fever


Prolonged seizures are associated with cerebral hypoxia, hypoglycemia, and hypercarbia and with concurrent and progressive lactic and respiratory acidosis.

When cerebral metabolic needs exceed available oxygen, glucose, and metabolic substrates (especially during status epilepticus), neuronal destruction can occur and may be irreversible.

Hypoxia, hypercarbia, hyperthermia, tachycardia, hypertension, hyperglycemia, hyperkalemia, and lactic acidosis result from massive sympathetic discharge.


Prolonged seizures

Duration of seizure

Life threatening

systemicchanges

DeathTemporary

systemicchanges

Werner, MD; GTC SE in Children; University of Kentucky Hospital

Respiratory

Hypoxia and hypercarbia◦ Ventilation (chest rigidity from muscle spasm)

◦Hypermetabolism ( O2 consumption, CO2

production)

◦Poor handling of secretions

Werner, MD; GTC SE in Children; University of Kentucky Hospital

HypoxiaHypoxia/anoxia markedly

increase the risk of mortality in SE

Seizures (without hypoxia) are much less dangerous than seizures and hypoxia

Towne AR. Epilepsia 1994;35(1):27-34

Neurogenic Pulmonary Edema

Rare complication of SE in children

Likely occurs as consequence of marked increase of pulmonary vascular pressure during SE

Johnston SC. Postictal pulmonary edema requires pulmonary vascular pressure increases. Epilepsia 1996;37(5):428-32

Johnston SC. Postictal pulmonary edema requires pulmonary vascular pressure increases. Epilepsia 1996;37(5):428-32

Acidosis

RespiratoryLactic

◦ Impaired tissue oxygenation

◦ Increased energy expenditure

Hemodynamics

Sympathetic overdrive ◦ Massive catecholamine /

autonomic discharge◦ Hypertension◦ Tachycardia◦ High CVP

Exhaustion Hypotension Hypoperfusi

on

Exhaustion Hypotension Hypoperfusi

on

0 min 60 min

Cerebral blood flow - Cerebral O2 requirement

Blood pressure

Blood flow

O2 requirement

Seizure duration

Hyperdynamic Exhaustion

Lothman E. Neurology 1990;40(5 Suppl 2):13-23.

Hyperdynamic phase ◦ CBF meets CMRO2

Exhaustion phase◦ CBF drops as

hypotension sets in

◦ Autoregulation exhausted

◦ Neuronal damage ensues

GlucoseG

luco

se

Seizure duration

30 min

SE

SE + hypoxia

Lothman E. Neurology 1990;40(5 Suppl 2):13-23.

Hyperdynamic phase ◦ Hyperglycemia

Exhaustion phase◦ Hypoglycemia

develops◦ Hypoglycemia

appears earlier in presence of hypoxia

◦ Neuronal damage ensues

MortalityThe primary determinant of mortality

and morbidity of SE in children is its etiology

The greatest mortality and highest rate of neurological deficits occurs when SE is caused by an acute neurological condition (infection, trauma, stroke)

Mitchell WG. J Child Neurol 2002;17 Suppl 1:S36-43.

Mean duration of SE in patients without neurologic sequelae is 1.5hours.

Mortality is lowest among children (~6%)subtherapeutic AEDsunprovoked SE

Highest Mortalityelderly patientsSE due to anoxia or CVA


MANAGEMENT

Management

1. Control the seizure.2. Correct the electrolyte

imbalance3. Work-up and treatment of

Congenital Adrenal Hyperplasia4. Anticipatory Guidance

General Initial Management of Seizure1. Assessment and control of the airways and

of ventilation, 2. ABG, ECG and blood pressure monitoring.3. Other measures include:

◦ i.v. glucose and thiamine as required,◦ emergency measurement of antiepileptic

drug levels, electrolytes and magnesium,

◦ a full haematological screen, ◦ measures of hepatic and renal function.The cause of the status should be identified urgently and may require treatment in its own right.

Management: First line medication: BZPs. Second line medication:

Phenobarb > phenytoin (in patients under the age of 1-2 yrs.)

Management: Other investigations and

management strategies include:

Stat ABG/VBG (with lytes, Hb, & lactate).

Full set of labwork including LFT’s, ammonia, urine and blood cultures.

Empiric antibiotics (if needed). Head U/S or CT

Management of Hyponatremia

Acute Hyponatremic Hypovolemia with Neurologic Symptoms

Treatment of choice is 3% hypertonic saline at 100 ml/h.◦ For each 100 ml of 3% hypertonic saline, serum sodium

concentration increase s by approximately 2 mmol/l. ◦ Duration of hypertonic saline treatment is based on the

improvement in the patient’s symptoms and signs.

Patients with acute hyponatremia◦ may be lethargic, disoriented, agitated, and have anorexia

and nausea . ◦ Physical findings: abnormal sensorium, pathological

reflexes, Cheyne-Stokes respiration, hypothermia, and seizures.

Severe symptoms: seizures, obtundation, and coma, ◦ 3% sodium may be infused at 4–6 ml/kg/h.

Acute Hyponatremic Hypovolemia with Neurologic SymptomsThe immediate treatment of hyponatremic

seizures in neonates:◦ provide enough sodium in a 10-minute period to

elevate serum sodium level to 125 mEq/L by using 3% normal saline solution.

The amount of sodium required : (125 -?) x (0.6) x (wt kg) = X

mEq

?= patient’s serum sodium, 0.6 is the dilution constant, X = number of mEq to correct sodium level to 125

mEq.

Treatment of CAH Patients suspected of 21-

hydroxylase deficiency should have the following bloodwork sent:

1. Electrolytes2. Glucose3. 17-hydroxyprogesterone levels4. Cortisol levels5. Aldosterone and renin levels.

After drawing appropriate bloodwork:

1. Patients with dehydration, hyponatremia, or hyperkalemia should receive a bolus of isotonic crystalloid to restore volume.

2. Hypoglycemic patients should receive a dextrose bolus infusion.

3. Patients suspected of adrenal insufficiency should be treated with steroids empirically (i.e. rather than waiting for the results of confirmatory studies).

Treatment of CAH

Treatment of CAH When administering steroids:

◦ Use an initial dose of HC 1-2 mg/kg IV (followed by q6h dosing) The disadvantage of

hydrocortisone is that it will confound any ACTH-Stim testing.

The advantage of hydrocortisone is that it is a complete steroid—with both glucocorticoid and mineralocorticoid activity.

Glucocorticoids: hydrocortisone is the glucocorticoid of choice during childhood.◦ Longer-acting glucocorticoids, such as

prednisolone and dexamethasone, can be used in adults, but they are generally avoided in children because of concerns about growth suppression.

◦ Hydrocortisone is recommended in the pediatric population because of its lower potency, which permits easier titration of appropriate doses.

Mineralocorticoids: to control electrolytes and plasma renin activity. ◦ Mineralocorticoid replacement is achieved with

fludrocortisone.

Treatment of CAH

Infants with salt-losing CAH often need sodium chloride supplementation. ◦ Routine salt supplementation is not usually needed

after the first 6-12 months of life. ◦ Additional salt intake may be needed with exposure to

hot weather or with intense exercise.

Treatment during physical stress, e.g. febrile illness, surgery, trauma:◦ Patients with classic CAH need increased, e.g.

doubling or tripling, doses of hydrocortisone.◦ Intravenous hydration may be required.◦ Hypoglycaemia may occur with exercise, illness or

fasting. Intake of carbohydrates and glucose should be increased.

Treatment of CAH

All patients should wear or carry medical alert

identification specifying adrenal insufficiency.

Etiopathogenesis

Speiser, et al. New England Journal of Medicine, 2003

Adrenal Crisis

Primary

Congenital Adrenal Hypoplasia/Aplasia

Congenital Adrenal Hyperplasia

Adrenal Hemorrhage

Adrenocorticotrophic Hormone unresponsiveness

Acute Illness

Adrenoleukodystrophy

Secondary

Differential Diagnosis

Congenital Adrenal Hypoplasia

Acutely in the neonatal periodPrimarily affects boysMutation of DAX1Hypogonadotropichypogonadism

= cryptorchidism

Adrenocorticotrophic Hormone UnresponsivenessNo salt losing manifestationsHypoglycemia, seizures and

hyperpigmentation in first decade of life

Marked adrenocortical atrophy with relative sparing of the zonaglomerulosa

Both sexes equally affected, autosomal recessive

Mutation in the gene for ACTH receptor

Adrenal HemorrhageNeonatal period due to difficult

laborPresenting signs: abdominal

mass, anemia, unexplained jaundice or scrotal hematoma

May be due to an infection (ex. Tuberculosis, meningococcemia)

AdrenoleukodystrophyAdrenocortical deficiency is

associated with demyelination in the CNS

Impaired β-oxidation in peroxisomes = levels of very long chain fatty acids found in tissues and body fluids

Rare autosomal recessive disorder in neonates

Reserved slides

Types of Status EpilepticusGeneralized Status

◦ Convulsive: generalized convulsive tonic-clonic status epilepticus (SE) is the most frequent type

◦ Absence◦ Secondarily generalized: accounts for ~75% of

generalized SE◦ Myoclonic◦ Atonic (drop attack): especially in Lennox-Gastaut

syndromePartial Status (usually related to anatomic

abnormality)◦ Simple (Epilepsy Partialis continuans)◦ Complex◦ Secondarily generalized


Documents

Pediatric Emergency Conference Guanzon Guerrero Guerzon Guevarra Guinto Gutierrez Hermoso Icasas Ignacio