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%)
Twins
prenatal diagnosis common
frequently advised to terminate
Twins
postnatallyis separation
desirable?
possible?
mandatory? if so,
when?
Twins
separationalways
possiblebut
what will each have?
can each survive?
is conjoined life so terrible?
Twins
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
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 chaplain press office
Twins
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
Twins
male twinstwin 1 ileostomy, rectumtwin 2 sigmoid colostomy
Twins
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%)
Twins
other problemsabn. duod. bile ducts imperforate anuscardiac abn./ insuff. intestinal atresiaabsent hepatic vs. ruptured livercrossed ureters hypoplastic lungsureters not crossed bladder
extrophy
Twins
no operation 8 sets
conjoined hearts
7 sets died
Twins
operated
emergency separation9 sets
4 survivors (22%)
elective separation12 sets
22 survivors (91%)
Twins
emergency separationof the 14 who died
already dead 2
uncorrectable hearts 5
cot death 1
Twins
elective separationthe 2 who died cardiac insuff. 1 aspiration 1
Twins
we recommendpre-natal consultationdelivery by CSdelivery close to
surgeonsexpect the unexpected
