The Surgery of Conjoined Twins

The Surgery of Conjoined Twins

Edward KielyGreat Ormond Street Hospital

London

Conjoined Twins

always existed

always fascinated

‘Double goddess’

Sisters of Catathoyuk

>6000 B.C.

Anatolian Civilisation MuseumAnkara

80 B.C. Ischiopagus twins: Fisole

Museo San Marco, Florence

~940 AD

Male ischiopagus twins Kappadokia, Armenia

lived together for 30 years – one diedsurgeons tried to save the surviving twin by separation – died 3 days later

first recorded separation

Twins

1689Elizabeth, Catherine Meyerin

(Basel)

omphalopagusJohannes 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 widelybecame wealthy

landowners married sisters

21 children died aged 63 years

Twins

incidence about

1:50,000 pregnancies

60% stillbornfemale

preponderance 3:1

natural history altered by antenatal u/s

Twins

aetiology

probable fusion of embryonic discs

in third week of gestation

Twin

typesmore common

thoracopagus (17%)

omphalopagus (14%)

ischiopagus (12%)

parapagus (24%)

Twins

types

less common

pygopagus (4%)

craniopagus (4%)

cephalopagus (11%)

rachipagus (2%)

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prenatal diagnosis common

frequently advised to terminate

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postnatallyis separation

desirable?

possible?

mandatory? if so,

when?

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separationalways

possiblebut

what will each have?

can each survive?

is conjoined life so terrible?

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who should do this?standard surgical

techniquesbut

approach is unusual

anatomy complicated

some structures absent

Twins

thoracopagus

conj. livers 100%conj. hearts 100%conj. gi tracts 50%

Twins

ischio/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

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final decision to proceed

death without

separation

conjoined life

intolerable

two survivors

likely

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when to operate?

given a choice – at about 3 months

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planning meeting surgery anaesthesia

theatre staff picu staff labs ward staff

radiology psychology chaplain press office

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pre-operative planningplan

initial stagesplan major

separations do not plan the order

of events

options for closure planned in detail

Twins

for the proceduretwo anaesthetic teamsone surgical team initiallyother surgical specialties as needed

later two surgical teamstwo operating theatres

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male twinstwin 1 ileostomy, rectumtwin 2 sigmoid colostomy

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male twinspost-operation

twin 1 stable twin 2 unstable (needed low

CVP) prostheses plicated as toleratedtwin 1 closed 12 d.; twin 2 closed

16 d.

Twins

1985 – 2010

33 sets

2 sets left for operation elsewhere

31 sets managed by GOS

Twin

types of unionthoracopagus 13 (41%)

omphalopagus 6 (18%)

parapagus 6 (18%)

pygopagus 3 (9%)

ischiopagus 3 (9%)

craniopagus 1 (3%)

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other problemsabn. duod. bile ducts imperforate anuscardiac abn./ insuff. intestinal atresiaabsent hepatic vs. ruptured livercrossed ureters hypoplastic lungsureters not crossed bladder

extrophy

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no operation 8 sets

conjoined hearts

7 sets died

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operated

emergency separation9 sets

4 survivors (22%)

elective separation12 sets

22 survivors (91%)

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emergency separationof the 14 who died

already dead 2

uncorrectable hearts 5

cot death 1

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elective separationthe 2 who died cardiac insuff. 1 aspiration 1

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we recommendpre-natal consultationdelivery by CSdelivery close to

surgeonsexpect the unexpected