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

Conjoined Twins always existed always fascinated

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

80 B.C. Ischiopagus twins: Fisole Museo San Marco, Florence

~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

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

Chang & Eng 1811

Chang & Eng Portrait: RCS

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

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

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

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

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

Twins prenatal diagnosis common frequently advised to terminate

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

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

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

Twins thoracopagus conj. livers 100% conj. hearts100% conj. gi tracts 50%

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

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

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

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

Twins planning meeting surgery anaesthesia theatre staff picu staff labs ward staff radiology psychology press office chaplain press office

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

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

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

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.

Twins 1985 – sets 2 sets left for operation elsewhere 31 sets managed by GOS

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

Twins other problems abn. duod. bile ducts imperforate anus cardiac abn./ insuff. intestinal atresia absent hepatic vs. ruptured liver crossed ureters hypoplastic lungs ureters not crossed bladder extrophy

Twins no operation 8 sets conjoined hearts 7 sets died

Twins operated emergency separation 9 sets4 survivors (22%) elective separation 12 sets22 survivors (91%)

Twins emergency separation of the 14 who died already dead 2 uncorrectable hearts 5 cot death 1

Twins elective separation the 2 who died cardiac insuff. 1 aspiration 1

Twins we recommend pre-natal consultation delivery by CS delivery close to surgeons expect the unexpected