Paediatric Emergency

1
Paediatric Emergency

• Dr Mark Lee
• Staff Specialist, Paediatric Emergency Medicine
• Dr Alison Martin
• Paediatric Respiratory Registrar
• Three Interesting cases

2
Case 1

• Six week old male presents with respiratory
  distress.

3
History

• Previously well child.
• One day history of vomiting.
• Lethargic.
• Noted to have increased work of breathing.
• Also noted to be blue around mouth and lips.
• No fever.
• No diarrhea.
• No history of trauma.

4
Background history

• First born.
• Non-consanguineous marriage.
• Parents of Persian descent and have lived in
  Australia for past two years.
• No family history of respiratory or cardiac
  disease, musculoskeletal disease,SUD.

5
Perinatal History

• Uncomplicated pregnancy.
• No maternal fever.
• No history varicella or HSV contact.
• Term baby, LUCS for failure to progress.
• Apgar 71 and 105
• Birth weight 3.25Kg (
• Head circumference 35cm (50 th).
• Breast fed and IMI vitamin K given.

6
Examination ( on arrival-ED)

• Reduced level of consciousness(LOC).
• Responding to pain( no focal posturing).
• Mottled limbs and pale.
• Central cyanosis.
• No rash.
• Blood glucose 7.8mmol/L.
• Temperature 35.6c( rectal).
• Not Dysmorphic.

7
Examination ( Respiratory)

• Respiratory rate 60.
• Cyanosed.
• Saturations unobtainable.
• Overt respiratory distress.
• Grunting expiratory noise.
• Bilateral chest wall movement.
• Good air entry bilaterally.

8
Examination ( Cardiac)

• Heart rate (HR) 180-200 and regular.
• Blood pressure(B.P)- unrecordable.
• Pulses- weak central pulses-upper limb equals
  lower limb- LR.
• Refill time 5 seconds.
• No murmur.
• Apex not palpated.

9
Examination- Neurological

• No abnormal posturing.
• Tone reduce generally.
• Symmetrical movements.
• Fontanelle down.
• Equal eye movements.
• PEARL.

10
Examination -Abdominal

• Abdominal examination reviewed a 2 cm liver edge
  regular edge.
• The rest of exam was unremarkable.

11
Provisional Diagnosis

• Shock .
• Respiratory extremis.

12
Immediate treatment

• Airway-100 oxygen by mask.
• Circulation- access obtained by intraosseous
  needle to tibia.
• Blood taken for FBC,Culture,UEC, LFT, glucose.
• Venous blood gas.

13
Secondary survey

• No additional findings, except blister to dorsum
  of left hand.
• Weight 5.4 KG(75th centile).
• Head circumference 39cm (50th centile).
• Length (50 centile).
• Nil dysmorphic features.
• Nil neurocutanteous stigmata.

14
Results

• Blood gas(venous)
• pH - 6.79
• PaC02 -56mmHg
• Pa02 --
• Be -24

15
What now?
16
Airway and breathing

• Rapid sequence induction.
• Given Thiopentone 20mg and Suxamethonium 15mg
  -I/0.
• Child intubated with 4.0mm ETT via Left nostril
  tied at 13cm.
• Ventilated CMV- Fi02 50, PIP/PEEP 22/5 ,rate
  25min.
• Vecuronium 0.5mg.
• Morphine and Midazolam infusion at 4ml/hr.

17
Circulation

• N/saline bolus 20/ml kilogram( x2)
• Sodium Bicarbonate 1mmol/kg ( x3)
• Antibiotics cefotaxime(50mg/kg),ampicillin
  (50mg/kg)
• Antiviral-acyclovir (30mg/kg)
• Right radial arterial line inserted.

18
Treatment(Cont)

• Orogastric tube -6 fr.
• IDC.
• Mobile chest x-r.
• Over head warmer.
• Continuous infusion of 5 dextrose with N/4
  saline .

19
ECG
20
ECG

• Sinus tachycardia 160-200.
• Normal voltage complexes.
• No evidence of infarction.

21
Results-1

• Bicarbonate 10mmol/L
• Potassium 7.4 mmol/L,
• Sodium 140mmol/L
• Chloride 103mmol/L
• Lactate 5.2 mmol/L
• LFT mild elevation
• CK 55U/L
• Creatinine 58umol/L

22
Results-2

• WCC18.0 x109/L
  
• Neutrophils 15.6
• Band count 6.8
• Hb 98g/L
• Platelets 203 x109/L

23
Summary- up to now

• Shocked.
• Profound metabolic acidosis with an increased
  anion gap.
• Cardiomegaly.
• Non diagnostic ECG.
• Abnormal white cell count.
• Initial resuscitation and stabilisation complete.

24
Causes of shock

• Cardiogenic- Arrhythmias,cardiomyopathy,structural
  problem myocardial infarction, myocardial
  contusion.
• Hypovolaemic- Haemorrhage,intussusception,gastroen
  teritis, peritonitis.

25
Causes of shock

• Distributive- septicemia,anaphylaxis,spinal cord
  injury.
• Obstructive- tension pneumothorax,cardiac
  tamponade, pulmonary embolism.

26
Possible causes

• Cardiogenic.
• Hypovolemia-Intussusceptions.
• Sepsis.
• Inborn error(organic acidemia).
• Poisoning- saliclates, iron.

27
Abdominal ultrasound(bedside)

• Normal

28
Cardiac echo

• Dilated and globally hypo kinetic left ventricle
• Normal structure and connections
• LV shortening 6 (25-40)
• Diagnosis-Severe dilated cardiomyopathy.
• Possible non compaction syndrome

29
Subsequent Progress

• Improved quickly with ventilatory support and
  fluid expansion

• pH _at_1hr 6.86,3hr 7.26, 6hr 7.44
• Commenced on antifailure medication
  lasix,digoxin,captopril .
• Thiamine (ivi)added.
• Extubated to CPAP at 48 hours.

30
Progress

• He required no inotropic support.
• Throughout resuscitation he was in sinus
  tachycardia with no periods of a systole.
• He was discharged from ICU to the ward on day 4
• His Ejection fraction improved from 6 to 13 by
  day 6.
• He was discharged home on day 6 ,breast feeding.
• Discharged with antifailure medications and
  thiamine.

31
Serology

• Paired titres in child and mother ,sample from
  blister on hand taken .
• Mycoplasma.
• HSV.
• Enteroviral.
• Adenovirus,Echovirus,Coxsackie.
• EBV .
• Parvo.
• All negative.

32
Other Results

• Urine no growth.
• CSF no growth ,PCR for HSV-negative.
• Head ultrasound normal.

33
Metabolic Screen

• Ammonia level normal.
• Serum Carnitine level normal.
• Repeat blood lactate normal.
• Urine metabolic screen NAD.
• Skin biopsy for acylcarnitine profile
  normal-excludes MCAD,SCAD,LCAD,VLCAD, CPT2
• Thiamine level low (TTP effect 21).
• Normal LFT.

34
Provisional Diagnosis

• Severe dilated cardiomyopathy
• Why?

35
Dilated Cardiomyopathy(DCM)

• Onset usually insidious, but acute in up to 25
• 50 have preceding viral illness.
• Family history of cardiomyopathy found in up to
  20 .

36
Dilated Cardiomyopathy(DCM)

• Incidence 2.6-36 per 100,000 children.
• Genetic causes account for 30 .
• 1/3 die ,1/3chronic heart failure, 1/3 improve.
• Causes of death , heart failure, malignant
  arrhythmias,transplant complications.
• 50 of patients present less than2 years old.

37
DCM - Causes

• Viral- Coxsackie B (20 ),adenovirus
  Echovirus,rubella, varicella, EBV,polio, HIV,
  Parvovirus,influenza.
• Bacterial- mycoplasma,sepsis.
• Parasites- Toxoplasma.
• Fungi- Histoplasma, Actinomyces

38
DCM - Causes

• Neuromuscular- Duchene muscular dystrophy,
  Friedreich ataxia.
• Coronary artery disease- Anomalous left coronary
  artery.
• Endocrine-Hypo/hyperthyroidism,hypoglycemia.

39
DCM - Causes

• Drugs-Adriamycin,cyclophosphamide.
• Collagen vascular disease-Rheumatic fever,
  Rheumatoid arthritis, Kawasaki disease
• Nutritional- Kwashiorkor, thiamine, selenium
  deficiency.
• Haematological- sickle cell, iron deficiency.

40
DCM - Causes

• Metabolic- Glycogen storage disease, carnitine
  deficency, fatty oxidation defects,
  mucopolysaccharidoses,organic acidaemias,CDG.
• Genetic- ( 30 ) AD(1p,3p) XL(Xp21, xq28 Barth
  syndrome), polygenic
• Non compaction syndrome.
• Idiopathic.

41
Thiamine responsive Cardiomyopathy

• Several case reports.
• Dramatic response to parental thiamine.
• Defective cellular thiamine uptake.
• Thiamine uptake studies not readily available.

42
Non compaction syndrome

• Is a rare cause of congenital cardiomyopathy.
• Altered structure of the left ventricular
  myocardium- extremely thickened hypo kinetic
  segments , non compacted endocardial layer.
• Multiple prominent trabeculations in the
  myocardium.

43
Non compaction syndrome

• Arrest of the normal compaction of the loose
  interwoven mesh of myocardial fibers in embryo.
• Diagnosis by distinctive echocardiogram findings.

44
Review Circulation (2003) 36 cases

• Most present with CCF at median age 90 days.
• Males equal females.
• 78 survival ( death from VT, CCF),
• 6 gene mutation found,(Xq28)
• 20 family history DCM.

45
Review Circulation (2003)- 36 cases,

• 4 cases underwent transplant.
• 9 who showed initial improvement deteriorated at
  median interval 6 years.
• Majority on long term anti-failure medications.

46
Progress

• Repeat cardiac echo _at_ 5 months post presentation
  return to normal function
• Medications ceased.
• Now _at_ almost 2 years old normal development, off
  all medication, passed hearing test.

47
Summary

• 6 week old male presented with profound metabolic
  acidosis and cardiogenic shock, secondary to
  dilated cardiomyopathy.

48
Summary

• The cause of the cardiomyopathy is unknown
  however likely possibilities are
• Idiopathic.
• Viral.
• Non- compaction syndrome .
• Thiamine deficiency.

49
Paediatric Retrievals
50
Paediatric Retrievals- The Problems

• Croup from Maitland (No ED room).
• Singleton retrieval (No ICU beds).
• Direct to ICU( no Paediatrician).
• Sending hospital given little or no
  advice/support.
• Ruptured appendix from Maitland not resuscitated
• What about the non retrieved children?

51
What needs to be done

• Direct link with all treating consultants before
  being transferred.
• Provide advice to sending hospital and the
  retrieval team .
• Provide correct placement and ongoing management
  of child on arrival to JHH.

52
(No Transcript)