Anaesthesia and Beckwith-Weideman syndrome

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2003

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Southern African Journal of Anaesthesia and Analgesia

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University of Cape Town

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Abstract
Synopsis of patient: A 15 hour old, 4.2 kg male presents for closure of a large exomphalos. He was delivered by C-section for foetal distress after a prolonged labour at a peripheral hospital. Apgars were recorded as 6 and 8. His mother was an unmarried primigravida who attended antenatal clinic on one occasion. Meconium aspiration was suspected at birth. Preoperative assessment revealed a large term baby with features of Beckwith-Weideman syndrome - a large tongue; a faint naevus on the forehead; and a skin crease on the ear lobe. Assessment of the liver and spleen was difficult in view of the large omphalocoele (5x6cm). The exomphalos was stained by the meconium in utero. He was tachypnoeic but the chest was clear. There was a 2/6 ejection systolic murmur at the left sternal border. Chest xray was normal apart from mild cardiomegaly. Blood sugar on admission was 1.2 mmol.l ; electrolytes were within normal limits. Haemoglobin was 17gm. (Hct 55).
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