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 MuseumAnkara
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 widelybecame 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. 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 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 2 sigmoid colostomy
Twins
male twinspost-operation
twin 1 stable
twin 2 unstable (needed low CVP)
prostheses plicated as tolerated
twin 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 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