Paediatric anaesthesia12 / 17

Giant omphalocele with Beckwith-Wiedemann syndromeTerm newborn girl, day 2, 3.9 kg, with a giant omphalocele holding liver and bowel, for silo placement and staged repair

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Know this first

Omphalocele has a sac; over half have other anomalies, about 20% cardiac.
Beckwith-Wiedemann: large tongue, hyperinsulinism and hypoglycaemia.
Neonatal hypoglycaemia: 10% dextrose 2 mL/kg, then 8 mg/kg/min, keeping glucose above 40–50 mg/dL.
No primary closure if airway pressure exceeds 25–30, or bladder or gastric pressure exceeds 20 cmH2O.

The patient

Term newborn girl, day 2, 3.9 kg, with a giant omphalocele holding liver and bowel, for silo placement and staged repair. Beckwith-Wiedemann features: large protruding tongue, large for dates. Echo booked, not yet done. On 10% dextrose; two glucose readings of 38 mg/dL overnight. Vitals: HR 150, BP 64/38, SpO2 96% on air, RR 50, temperature 36.6 °C.

The examiner asks

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Consider
What are your considerations?
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- A 2-day-old with Beckwith-Wiedemann and a giant omphalocele, for silo and staged repair. Patient
- As a newborn she loses heat and water fast and needs glucose 6–8 mg/kg/min.
- Omphalocele brings other anomalies in over half, heart lesions in about 20%. Disease
- Hyperinsulinism in Beckwith-Wiedemann explains her low glucose.
- Her large tongue can make mask ventilation and intubation difficult. Airway
- Surgery isn't urgent: there's time to stabilise her and image her heart. Surgery
Prepare
How do you prepare her?
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- Echo before theatre to define any heart lesion. Disease
- Frequent glucose checks; her dextrose infusion never stops, including in transfer.
- Sac kept covered; theatre warmed and forced-air warming ready. Patient
- Videolaryngoscope, infant SGA, nasal airway and a second senior anaesthetist. Airway
Plan
How do you conduct the anaesthetic?
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- Gas induction, keeping her breathing until I know I can ventilate past her tongue. Airway
- Full relaxation for reduction; no nitrous oxide to distend her bowel. Surgery
- Airway pressure watched as viscera go in; over 25–30 cmH2O, reduction stops.
- Bladder or gastric pressure over 20 cmH2O means a silo, not closure.
- Ventilated in the neonatal unit afterwards while staged reduction continues. After surgery
Crisis
After induction, mask ventilation fails: no chest rise, SpO2 85% and falling. What do you do?
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- Difficult airway algorithm: can't mask-ventilate; call for help now. (Crisis)
- Two-person mask with jaw thrust and a nasal airway to lift her tongue.
- Still failing: an SGA to oxygenate her.
- Intubate by videolaryngoscope, no more than two attempts.
- Can't oxygenate: needle cricothyroidotomy without delay, or ENT rigid bronchoscopy or tracheostomy.
- HR under 60 despite effective ventilation: start chest compressions.
Crisis
Two hours in, a point-of-care glucose reads 28 mg/dL. What now?
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- Hypoglycaemia in Beckwith-Wiedemann; anaesthesia hides the signs. Crisis
- Bolus 10% dextrose 2 mL/kg IV.
- Then 10% dextrose at 8 mg/kg/min, keeping glucose above 40–50 mg/dL.
- Recheck glucose soon after, then frequently.
- Neonatology to manage her hyperinsulinism after theatre. After surgery

Fatal mistakes

No glucose monitoring in Beckwith-Wiedemann.
Forcing primary closure as airway pressures climb.
No difficult airway plan for her large tongue.

Checked against: Stoelting 8e · Barash 9e

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