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The Surgery of Conjoined Twins

The Surgery of Conjoined Twins. Edward Kiely Great Ormond Street Hospital London. Conjoined Twins. always existed always fascinated. > 6000 B.C. ‘Double goddess’ Sisters of Catathoyuk. Anatolian Civilisation Museum Ankara. 80 B.C.

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The Surgery of Conjoined Twins

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  1. The Surgery of Conjoined Twins Edward Kiely Great Ormond Street Hospital London

  2. Conjoined Twins always existed always fascinated

  3. >6000 B.C. ‘Double goddess’ Sisters of Catathoyuk Anatolian Civilisation Museum Ankara

  4. 80 B.C. Ischiopagus twins: FisoleMuseo San Marco, Florence

  5. ~940 AD Male ischiopagus twins Kappadokia, Armenia lived together for 30 years – one died surgeons tried to save the surviving twin by separation – died 3 days later first recorded separation

  6. Twins 1689 Elizabeth, Catherine Meyerin(Basel) omphalopagus Johannes Fatio applied transfixion ligature fell off day 9 – both survived reported by Koenig

  7. 1811 Chang & Eng

  8. Chang&Eng Portrait: RCS

  9. Twins Chang, Eng Bunker omphalopagus travelled, exhibited widely became wealthy landowners married sisters 21 children died aged 63 years

  10. Twins incidence about 1:50,000 pregnancies 60% stillborn female preponderance 3:1 natural history altered by antenatal u/s

  11. Twins aetiology probable fusion of embryonic discs in third week of gestation

  12. Twin types more common thoracopagus(17%) omphalopagus(14%) ischiopagus(12%) parapagus(24%)

  13. Twins types less common pygopagus(4%) craniopagus(4%) cephalopagus(11%) rachipagus(2%)

  14. Twins prenatal diagnosis common frequently advised to terminate

  15. Twins postnatally is separation desirable? possible? mandatory? if so, when?

  16. Twins separation always possible but what will each have? can each survive? is conjoined life so terrible?

  17. Twins who should do this? standard surgical techniques but approach is unusual anatomy complicated some structures absent

  18. Twins thoracopagus conj. livers 100% conj. hearts100% conj. gi tracts50%

  19. Twins ischio/para/pygo-pagus complicated urological anatomy may have single set of genitalia if genitals not divisible, what then?

  20. Twins investigations dictated by site of union cardiac evaluation essential cross sectional imaging essential gi contrast, angio studies unhelpful

  21. Twins final decision to proceed death without separation conjoined life intolerable two survivors likely

  22. Twins when to operate? given a choice – at about 3 months

  23. Twins planning meeting surgery anaesthesia theatre staff picu staff labs ward staff radiology psychology chaplainpress office

  24. Twins pre-operative planning plan initial stages plan major separations do not plan the order of events options for closure planned in detail

  25. Twins for the procedure two anaesthetic teams one surgical team initially other surgical specialties as needed later two surgical teams two operating theatres

  26. Twins male twins twin 1 ileostomy, rectum twin 2sigmoid colostomy

  27. Twins male twins post-operation twin 1stable twin 2unstable (needed low CVP) prostheses plicated as tolerated twin 1 closed 12 d.; twin 2 closed 16 d.

  28. Twins 1985 – 2010 33 sets 2 sets left for operation elsewhere 31 sets managed by GOS

  29. Twin types of union thoracopagus 13 (41%) omphalopagus 6 (18%) parapagus 6 (18%) pygopagus 3 (9%) ischiopagus 3 (9%) craniopagus 1 (3%)

  30. Twins other problems abn. duod. bile ductsimperforate anus cardiacabn./ insuff. intestinal atresia absent hepatic vs. ruptured liver crossed uretershypoplasticlungs ureters not crossed bladder extrophy

  31. Twins no operation 8 sets conjoined hearts 7 sets died

  32. Twins operated emergency separation 9 sets 4 survivors (22%) elective separation 12 sets 22 survivors (91%)

  33. Twins emergency separation of the 14 who died already dead 2 uncorrectable hearts5 cot death 1

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