Paeds Flashcards
(50 cards)
Syndromes:
Apert
AD
cardiac / renal
“C’s”
premature closure of cranial sutures
midface hypoplasia
choanal atresia
cleft palate
C5/6 fusion
syndactyly
II due to megalocephaly/hypoplasia of white matter/corpsum collosum agenesis
Key
1. OSA 50% –> UAW –> routine maneouver
craig sims
craniosynostosis - usual surgery
Syndomes:
Beckwith-Wiedemann
Macroglossia
Visceromegaly - big heart etc
* Hypoglycaemia
Omphalocoele
Key
1. Glucose when fasting
Beckwith-Wiedemann syndrome (BWS) presents unique perioperative challenges due to its hallmark features. Here’s a mnemonic to simplify key considerations, focusing on airway management, tumor risks, and pharmacological adjustments:
B.E.C.K.W.I.T.H
Big tongue (macroglossia): Anticipate difficult airway; use video laryngoscopy or fiberoptic intubation[1][5].
Euglycemia: Monitor glucose pre-/intraoperatively due to neonatal hypoglycemia risk[1][3].
Cancer surveillance: Check for Wilms tumor (abdominal ultrasound) and hepatoblastoma (AFP levels)[3][9].
Kidney function: Avoid renally excreted neuromuscular blockers (e.g., pancuronium); use cisatracurium (Hoffman degradation)[3][6].
Watch hepatic metabolism: Prefer sevoflurane over hepatotoxic volatiles (e.g., halothane) due to hepatoblastoma risk[3][9].
Inspect for organomegaly: Gentle handling during abdominal procedures to avoid injury[1][5].
Think asymmetry (hemihyperplasia): Adjust positioning and IV access sites[1][5].
Hemodynamic stability: Assess for cardiomyopathy preoperatively; avoid myocardial depressants[9].
This mnemonic emphasizes proactive management of airway challenges, tumor-related precautions, and tailored pharmacologic choices to mitigate risks associated with BWS.
Citations:
[1] https://medlineplus.gov/genetics/condition/beckwith-wiedemann-syndrome/
[2] https://en.wikipedia.org/wiki/Beckwith%E2%80%93Wiedemann_syndrome
[3] https://www.eviq.org.au/cancer-genetics/paediatric/risk-management/3700-beckwith-wiedemann-syndrome-risk-management
[4] https://pmc.ncbi.nlm.nih.gov/articles/PMC2987155/
[5] https://www.chop.edu/conditions-diseases/beckwith-wiedemann-syndrome
[6] https://www.ncbi.nlm.nih.gov/books/NBK1394/
[7] https://www.childrenshospital.org/conditions/beckwith-wiedemann-syndrome
[8] https://rarediseases.org/rare-diseases/beckwith-wiedemann-syndrome/
[9] https://my.clevelandclinic.org/health/diseases/21993-beckwith-wiedemann-syndrome
Answer from Perplexity: pplx.ai/share
Syndromes:
CHARGE
Colobama of eye
Heart defects
choAnal atresia
R - develop delay
G - GU abnor
E - ear abnor
Key
1. Cardiac defect
2. Poor resp reserve
Syndrome:
DiGeorge
1:4000 microdeletion 22q11.2 chromosome deletion
~ cardiac disease
Key
1. Micrognathia
2. Cleft palate
3.
Syndrome
Cri-du-Chat
C - C
Cat cry / cardiac defect
Severe II
Hypotonia
Key
1. Micrognathia
2. Abn larynx
3. Cardiac defect
Syndrome
Prader-Willi
Chr 15
PWC OOHH - cardiac/obesity/OSA/hypot/hypog
Hypotonia
Hypogonad
II
erratic
short
MO***
Key
1. OSA
2. Cardiac
3. Difficult PIVC
4. Difficult to fast
Syndrome
Treacher Collins Syndrome
1st 2nd branchial arches
AB C - b arches collins
Key
1. Very diff airway
2. Abnom funnel shape larynx
3. LMA effective
4. AW more diff with age - mandible does not grow
- ## require trache
Conditions associated with airway problems
Pierre Robin
Treacher Collins
Goldenhar
Micrognathia
Retrognathia
goldenhar - Congenital heart disease - asymmetry treacher collins
PRS - cleft palate, tongue immobile due to small mandible
AW mx - nasopharyngeal (suture - tricky if cleft palate), jaw thrust, LMA, prone/lateral (displace tongue)
Syndrome
Down’s
Commonest - 1.6/1000
3/3/3/3
CNS
Impaired development
Atlantoaxial instability
Epilepsy
CVS
Congential cardiac defects - AVSD/TOF
Eisenmenger’s syndrome
*Bradycardia (autonomic dys)
Resp
Recurrent RTI –> O2 + PT
hypoventilation
Endo
Obesity
Hypothyroidism
GORD
Ex:
Large tongue
crowding mid facial feactures
narrow palate
micrognathia
short/broad neck
CVS/resp EX + IX
Hypotonia
Key:
1. Difficult BMV
2. Bradycardia —> consider pre-induction atropine + early attach ECG + 6% sevo/dial back sooner
3. Difficult PIVC
4. Avoid a2 agonist maybe - bradycardia (2microg/kg) - but faster
5. If intubate - CMAC
Pyloric stenosis
PAEDIATRICS – GI/Gen - Pyloromyotomy
[16A08] A six-week-old term baby weighing 4.0 kg requires pyloromyotomy for pyloric stenosis.
How would you assess the baby’s hydration status? (50%)
Detail and justify your resuscitation regimen. (50%)
1:350
80% male
4-6 weeks of age
hypochloraemic
alkalosis
dehydrate
never urgent –> resus
PERIOP:
- risk of asp due to GOO
- NGT
- RSI+cric if NG loss >2mL/kg/h
rectus sheath block if laparoscopic
remove NGT end of surg
Extubate awake
Risk of apnoea (worsened by alkalosis)
Resus and replace NG loss with NS 0.9%
a) Hydration status
5/10/15% body weight loss
5% - skin turg/CRT
10% (shock) - fontanelle/cool skin/oliguria
15% - ALOC/sbp down
b) Resus
- replace/resus - 10mL/kg
- maintain - 4/2/1
- ongoing loss - add K+
- K+ = 3mmol/kg/24h OR add 20mmol to 1L (20mmol/L KCl)
c) ongoing loss PRN
Think
1. NGT
2. Add K
3. Cl > 95, HCO3 <300, K>3.5
4. UO 1-2ml/kg/hr
Shock - give resus fluid stat
Dehydr - loss given over 24-48h
SSU + SAQ
Emergency drug doses
- Sux (IV/IM)
- Atropine
- Adrenaline (anaphylaxis/arrest)
- Adenosine
- Amiodarone
Shock
Cardioversion
Fasting
sux IM 4mg/kg
sux IV 1.5-2mg/kg
atropine 20microg/kg
adrenaline
1. anaphylaxis 10microg/kg
2. arrest 10/kg
3.*reduce to 1microg/kg in LAST
+ IM
150microg = <6yo
300 microg = 6-12yo
500 microg = 12yo+
q5min PRN lateral thigh
adenosine
100–>200–>300microg/kg
amiodarone
5mg/kg
Shock
arrest 4J/kg
other 1–>2–> 4Jg
1hr = CF
4hr = BM
6hr = Everything else - FM
https://www.anzca.edu.au/safety-advocacy/standards-of-practice/perioperative-anaphylaxis-management-guidel
Pros and cons of T piece
Pros:
1. Low resistance
2. valveless
3. lightweight
4. expiratory limb > Vt - prevent emtrainment of room air during SV
5. assess Vt
6. PEEP/CPAP by occluding bag
7. potential of assisted/controlled ventilation
8. Lung compliance
9.
Cons:
1. Up to 20kg, inefficient beyond
2. Scavenging is limited
3. FGF min 3L for most
4. FGF dependent of RR - high RR = high FGF
CMT
prejx
muscle atrophy - spinal/limb —> kypho
Key
1. RLD
2. Neuropathic pain
3. Avoid sux
4. inc sens to NDMB
cvs/resp/drugs/triggers
Charcot-Marie-Tooth (CMT) disease presents specific perioperative considerations, particularly regarding neuromuscular blockers and volatile agents. Here’s a mnemonic to remember key points: C.M.T. PAUSE
C - Check Respiratory Function
- Assess for respiratory muscle weakness, especially in advanced cases[4][7].
- Monitor for scoliosis-related restrictive lung disease[2][6].
M - Muscle Relaxant Caution
- Avoid succinylcholine due to risk of hyperkalemia from muscle denervation[7].
- Use reduced doses of non-depolarizing agents (e.g., rocuronium) as patients may be sensitive[7].
T - Temperature & Positioning
- Maintain normothermia (cold extremities common due to muscle atrophy)[2].
- Pad pressure points to prevent ulcers (sensory loss in feet/hands)[2][8].
P - Pain Management
- Avoid excessive opioids (neuropathic pain may require adjuncts like gabapentin)[4][8].
- Consider regional anesthesia cautiously (weigh risks of nerve injury)[8].
A - Anesthetic Choice
- Prefer TIVA (total intravenous anesthesia) over volatile agents to avoid theoretical exacerbation of neuropathy[7].
- If using volatiles, monitor for prolonged sedation (muscle weakness)[7].
U - Unusual Anatomy
- Anticipate foot/hand deformities (e.g., pes cavus, hammer toes) for IV access and positioning[1][4].
- Use soft padding and neutral positioning to prevent joint injury[2][6].
S - Sensory Precautions
- Inspect extremities post-op for unnoticed injuries (reduced pain/temperature sensation)[2][5].
E - Evaluate Post-Op Weakness
- Monitor for delayed respiratory depression or prolonged neuromuscular blockade[7].
This mnemonic highlights critical perioperative steps to mitigate risks in CMT patients, emphasizing neuromuscular blocker sensitivity, respiratory vigilance, and tailored anesthesia plans.
Citations:
[1] https://www.mayoclinic.org/diseases-conditions/charcot-marie-tooth-disease/symptoms-causes/syc-20350517
[2] https://www.mda.org/disease/charcot-marie-tooth/signs-and-symptoms
[3] https://www.sydney.edu.au/news-opinion/news/2017/07/17/first-effective-treatment-of-children-with-charcot-marie-tooth-d.html
[4] https://www.hopkinsmedicine.org/health/conditions-and-diseases/charcotmarietooth-disease
[5] https://medlineplus.gov/genetics/condition/charcot-marie-tooth-disease/
[6] https://www.mda.org/disease/charcot-marie-tooth
[7] https://www.ninds.nih.gov/health-information/disorders/charcot-marie-tooth-disease
[8] https://www.healthdirect.gov.au/charcot-marie-tooth-disease
[9] https://www.nhs.uk/conditions/charcot-marie-tooth-disease/symptoms/
[10] https://www.mayoclinic.org/diseases-conditions/charcot-marie-tooth-disease/diagnosis-treatment/drc-20350522
[11] https://www.cmtausa.org/understanding-cmt/what-is-cmt/
[12] https://www.nhs.uk/conditions/charcot-marie-tooth-disease/treatment/
[13] https://www.rch.org.au/neurology/patient_information/Charcot-Marie-Tooth_disease/
[14] https://www.mda.org/disease/charcot-marie-tooth/medical-management
[15] https://www.cmtausa.org/living-with-cmt/managing-cmt/medications/
[16] https://www.betterhealth.vic.gov.au/health/conditionsandtreatments/charcot-marie-tooth-disease-cmt
[17] https://www.ncbi.nlm.nih.gov/books/NBK562163/
[18] https://my.clevelandclinic.org/health/diseases/6009-charcot-marie-tooth-disease-cmt
[19] https://www.nhs.uk/conditions/charcot-marie-tooth-disease/
Answer from Perplexity: pplx.ai/share
Friedreich’s
AR
Skeletal muscle weakness
Key
1. Risk of RF
2. cardiac involvement
3. avoid sux - debility —> hyperK
4. inc sens to NMDB
Duchenne
Beckers
X-linked recessive
- lack of dystrophin (duchenne)
- partial lack (beckers)
Prox muscle wasting
CVS/RESP failure
Can be normal at birth, weakness before 8, WC by adolescence
Key:
1. DCM - negative inotropes…
2. RLD
3. risk of AIR (anaes induced rhabdo) - HALOGENATED
- absent
Myotonic dystrophy
Type 1: DMPK gene - birth
Type 2: CNBP - mild
CNS: bulbar —> aspiration
Key:
- Aspiration —>
Lung protective strategies
F: fluid mx
I: Intubate
P: pain
P: PT - pre/post
V: ventilation strategy
- avoid sux - generalised contractures from fasciculations
-
Myotonia congenita
AD chr 17
muscle hypertrophy
- resp - asspiration
Hyperkalamic periodic paralysis
Hypokalaemic periodic paralysis
Metabolic myopathies
Mitochondrial myopathies
loss of ATP product
Hurler’s
Muccopolysacchrides
Deposits - airway, muscle, skin, cardiac
Difficult intubate
LMA effective
Consider PICU if intubated
Syndrome
Williams
Sudden death
Supravalvular AS - tertiary only if elective
Marfans
DCM
Arrhythmmias AOurtic root dialtion