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