~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
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
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
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ischio/para/pygo-pagus complicated urological anatomy
may have single set of genitalia
if genitals not divisible, what then?
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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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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
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for the proceduretwo anaesthetic teamsone surgical team initiallyother surgical specialties as needed
later two surgical teamstwo operating theatres
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.
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
Twins
operated
emergency separation9 sets
4 survivors (22%)
elective separation12 sets
22 survivors (91%)