Pediatric Emergency Conference

Pediatric Emergency ConferenceGuanzon Guerrero Guerzon Guevarra Guinto Gutierrez Hermoso Icasas IgnacioGeneral DataMichael 3 week old male

Chief complaint: seizuresHistory 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 complyHistory of Present Illness:2 days PTC:(+) 5x water stools(+) 3x vomiting of previously ingested foodFew hours PTC:- drowsy- generalized tonic-clonic seizuresRushed to the ERInitial assessment at the ER:Was in active seizuresafebrileCR: 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 doseSecondary assessment:Unremarkable chest and abdomen(+) hypospadiasPenile length: 1.5 cmDarkly pigmented scrotal sac

Pulses were full and equalGood capillary refillLaboratory results:Lab testsResultReference ValueNa120K6.0HgtlowSalient FeaturesDifferential DiagnosisPresenting manifestation: seizure in a neonateSeizure: paroxysmal, time limited change in motor activity and/or behavior that results from abnormal activity in the brainCommon in pediatric age groupNeonates are at particular risk for the development of seizure

Nelsons textbook of Pediatrics 17th editionMetabolic, toxic, structural and infectious dieases are more likely to be manifested during this time10Manifestations of a seizure:Vocal: Cry or gasp, slurring of words, garbled speechMotor: Head or eye turning, eye deviation, posturing, jerking (rhythmic), stiffening, automatisms (purposeless repetitive movements such as picking at clothing, lip smacking); generalized or focal movementsRespiration: Change in breathing pattern, cessation of breathing, cyanosisAutonomic: Pupillary dilatation, drooling, change in respiratory or heart rate, incontinence, pallor, vomitingLoss of consciousness or inability to understand or speakPractice 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, PhDSeizureInfectious causes:BacterialViral FungalAfebrileFebrileSeizure like EventsBenign Paroxysmal Torticollis of InfancyBreath holding spells: - cyanotic spells - pallid spellsShuddering attacksNight terrors

Metabolic disordersElectrolyte imbalanceHypoglycemiaHypoxiaDrug relatedBirth trauma or intrauterine malposition is also considered to cause damage to the sternocleidomastoid muscle in the neck.[citation needed] This results in a shortening or excessive contraction of the sternocleidomastoid muscle, often with limited range of motion in both rotation and lateral bending. The head is typically tilted in lateral bending toward the affected muscle and rotated toward the opposite side, 2-4 weeks12Non-febrile seizureThings 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 2008Factors that lower Seizure ThresholdSleep deprivationHyperventilationPhotic stimulationInfectionMetabolic disturbancesHead traumaCerebral ischemiaKindlingHandbook of Neurosurgery by GreenbergDrugs which can cause seizuresAntibioticsPenicillinsIsoniazidMetronidazoleAnesthetics, narcoticsHalothane, enfluraneCocaine, fentanylKetaminePsychopharmaceuticalsAntihistaminesAntidepressantsAntipsychoticsPhencyclidineTricyclic antidepressants16Important points in the HistoryThe course of current seizure activityTime and nature of onset of seizure activityInvolvement of extremities or other body partsNature of movements (eg, eye movements, flexion, extension, stiffening of extremities), including any focal movements and details of postictal neurologic deficitIncontinenceCyanosis (perioral or facial)Duration of seizure activity prior to medical attentionMental status after cessation of seizure activityFever or intercurrent illnessesPrior history of seizures - If present, specify medications, anticonvulsant use, and compliance.

Pediatric, Status Epilepticus; emedicine 2008Important 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)

Pediatric, Status Epilepticus; emedicine 2008Important points in the PESigns of sepsis or meningitisTemperature more than 38.5C; in patients younger than 2-3 months, more than 38.0CRespiratory distressCyanosisPoor peripheral perfusionBulging fontanelles in infantMeningismus (in children >12-18 mo)Presence of petechiae or purpura, herpetic vesiclesEvidence of head or other CNS injuryBradycardia, tachypnea, and hypertension (Cushing triad for signs of increased intracranial pressure)Poor pupillary responseAsymmetry on neurologic examinationAbnormal posturingGross deformity or soft tissue injury to headHallmarks of neurocutaneous syndromes (e.g., port wine stain)Pediatric, Status Epilepticus; emedicine 2008Monitoring 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 editionClinical Classification of Status EpilepticusOvert generalized convulsive status epileptusSubtle generalized convulsive status epilepticusSimple status epilepticusNonconvulsive status epilepticusContinuous convulsive activity and intermittent convulsive activity without regaining full consciousness

Convulsive (tonic-clonic)TonicClonicMyoclonicComa following generalized convulsive status epilepticus with or without motor activityConsciousness preserved

Simple motor status epilepticusSensory status epilepticusAphasic status epilepticusConsciousness impaired; twilight or fugue state

Petit mal status (absence status)Complex partial status epilepticusHandbook of Medical & Surgical Emergencies, 6th editionEtiologiesFebrile seizuresCerebrovascular accidentsCNS infectionIdiopathicEpilepsySubtherapeutic antiepileptic drugElectrolyte imbalanceDrug intoxicationAlcohol withdrawalTraumatic brain injuryAnoxiaTumorHandbook of Neurosurgery by GreenbergNeonates (first month of life)Early childhood (6 y)Birth injury (eg, anoxia, hemorrhage) and congenital abnormalitiesBirth 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

Pediatric, Status Epilepticus; emedicine 2008DIFFERENTIAL DIAGNOSISToxins and medicationsTopical anesthetics (eg, lidocaine)Anticonvulsant overdoseCamphorHypoglycemic agents (eg, insulin, ethanol)Carbon monoxideCyanideHeavy metals (eg, lead)Pesticides (eg, organophosphate)CocainePhencyclidineBelladonna alkaloidsNicotineSympathomimetics (eg, amphetamines, phenylpropanolamine [recalled from US market])Tricyclic antidepressants

24In children < 1year age75% acute cause30% electrolyte disorders28% secondary to CNS infection19% associated with feverHandbook of Neurosurgery by GreenbergProlonged 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.Pediatric, Status Epilepticus; emedicine 2008Prolonged seizuresDuration of seizureLife threateningsystemicchangesDeathTemporarysystemicchangesWerner, MD; GTC SE in Children; University of Kentucky Hospital27Nicely reviewed in :Fountain NB. Status epilepticus: risk factors and complications. Epilepsia 2000;41 Suppl 2:S23-30

Marked systemic and neurologic changes occur after about 30-60 minutes of seizure activity (see Fountain) and also: DeLorenzo RJ, Towne AR, Pellock JM, et al. Status epilepticus in children, adults, and the elderly. Epilepsia 1992;33 Suppl 4:S15-25Bassin S, Smith TL, Bleck TP. Clinical review: status epilepticus. Crit Care 2002;6(2):137-42Neurologic injury is likely to occur after 60 minutes of SE ( reviewed in: DeLorenzo.)There is also a nice graph with probable time relationship in:Haafiz A, Kissoon N. Status epilepticus: current concepts. Pediatr Emerg Care 1999;15(2):119-29RespiratoryHypoxia and hypercarbia Ventilation (chest rigidity from muscle spasm)Hypermetabolism ( O2 consumption, CO2 production)Poor handling of secretionsWerner, MD; GTC SE in Children; University of Kentucky Hospital28HypoxiaHypoxia/anoxia markedly increase the risk of mortality in SESeizures (without hypoxia) are much less dangerous than seizures and hypoxia

Towne AR. Epilepsia 1994;35(1):27-3429Neurogenic Pulmonary EdemaRare complication of SE in childrenLikely 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):428-3230AcidosisRespiratoryLacticImpaired tissue oxygenationIncreased energy expenditure31HemodynamicsSympathetic overdrive Massive catecholamine / autonomic dischargeHypertensionTachycardiaHigh CVPExhaustionHypotensionHypoperfusion0 min60 min32Cerebral blood flow - Cerebral O2 requirementBlood pressureBlood flowO2 requirementSeizure durationHyperdynamicExhaustionLothman E. Neurology 1990;40(5 Suppl 2):13-23.Hyperdynamic phase CBF meets CMRO2Exhaustion phaseCBF drops as hypotension sets inAutoregulation exhaustedNeuronal damage ensues33GlucoseGlucoseSeizure duration30 minSESE + hypoxiaLothman E. Neurology 1990;40(5 Suppl 2):13-23.Hyperdynamic phase HyperglycemiaExhaustion phaseHypoglycemia developsHypoglycemia appears earlier in presence of hypoxiaNeuronal damage ensues34MortalityThe 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:S36-43.35The fact that infants with SE have a higher mortality is likely due to the different etiologies of SE in infants, when compared to older children.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 CVAHandbook of Neurosurgery by GreenbergMANAGEMENTManagementControl the siezure.Correct the electrolyte imbalance (Hyponatremia).Treatment of Congenital Adrenal Hyperplasia

General Initial Management of SeizureAssessment and control of the airways and of ventilation, ABG, ECG and blood pressure monitoring.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.ABG to see if there is metabolic acidosis and hypoxia requiring immediate treatment through airway management and supplemental oxygen39

Management:First line medication: BZPs.Second line medication:Phenobarb > phenytoin (in patients under the age of 1-2 yrs.)

Phenytoin has a myocardial depressant effect and unpredictable metabolism in the neonate.

41Management:Other investigations and management strategies include:

Stat ABG/VBG (with lytes, Hb, & lactate).Full set of labwork including LFTs, ammonia, urine and blood cultures.Empiric antibiotics (if needed).Head U/S or CT

Management of Hyponatremia

Acute Hyponatremic Hypovolemia with Neurologic SymptomsTreatment 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 patients symptoms and signs.

Patients with acute hyponatremiamay 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 46 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

?= patients serum sodium, 0.6 is the dilution constant, X = number of mEq to correct sodium level to 125 mEq.

Treatment of CAHPatients suspected of 21-hydroxylase deficiency should have the following bloodwork sent:ElectrolytesGlucose17-hydroxyprogesterone levelsCortisol levelsAldosterone and renin levels.Treatment of CAHAfter drawing appropriate bloodwork:Patients with dehydration, hyponatremia, or hyperkalemia should receive a bolus of isotonic crystalloid to restore volume.Hypoglycemic patients should receive a dextrose bolus infusion.Patients suspected of adrenal insufficiency should be treated with steroids empirically (i.e. rather than waiting for the results of confirmatory studies).Treatment of CAHWhen 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 steroidwith both glucocorticoid and mineralocorticoid activity.Also advantageous as no parenteral form of mineralocorticoid is available in the USA.

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 CAHInfants 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 CAHAll patients should wear or carry medical alert identification specifying adrenal insufficiency.

Reserved slidesEtiopathogenesis

Speiser, et al. New England Journal of Medicine, 2003Differential DiagnosisCongenital Adrenal HypoplasiaAcutely in the neonatal periodPrimarily affects boysMutation of DAX1Hypogonadotropichypogonadism = cryptorchidismAdrenocorticotrophic Hormone UnresponsivenessNo salt losing manifestationsHypoglycemia, seizures and hyperpigmentation in first decade of lifeMarked adrenocortical atrophy with relative sparing of the zonaglomerulosaBoth sexes equally affected, autosomal recessiveMutation in the gene for ACTH receptorAdrenal HemorrhageNeonatal period due to difficult laborPresenting signs: abdominal mass, anemia, unexplained jaundice or scrotal hematomaMay be due to an infection (ex. Tuberculosis, meningococcemia)AdrenoleukodystrophyAdrenocortical deficiency is associated with demyelination in the CNSImpaired -oxidation in peroxisomes = levels of very long chain fatty acids found in tissues and body fluidsRare autosomal recessive disorder in neonatesTypes of Status EpilepticusGeneralized StatusConvulsive: generalized convulsive tonic-clonic status epilepticus (SE) is the most frequent typeAbsenceSecondarily generalized: accounts for ~75% of generalized SEMyoclonicAtonic (drop attack): especially in Lennox-Gastaut syndromePartial Status (usually related to anatomic abnormality)Simple (Epilepsy Partialis continuans)ComplexSecondarily generalizedHandbook of Neurosurgery by Greenberg