Craniopagus twins
By: Bindu Peter RN,BSN
Staff Nurse
Montefiore Medical Center, Bronx, NY
Conjoined Twins
• Are identical twins joined in
utero
• Skin and internal organs are
fused together
• Occurs in the range of one
in 50,000 to one in 200,000
births.
• Can be joined to each other
in different configurations.
• Approximately 40 to 60
percent are stillborn.
• About 35 percent live only
for one day.
• Overall survival rate is about
5 to 25 percent
Myths and Legends
Roman God
Janus
Centaur
Art copying life
or
Artistic expression of imagination?
Double-Headed Figure [Ecuador; Valdivia] end of 3rd mellenia
B.C.(1980.34.1) | Heilbrunn Timeline of Art History | The Metropolitan
Museum of Art
Terra cotta figure of pre-Columbian Tlatilco/Michoacan
Pottery Conjoined Twin "Pretty Lady"
www.artemisgallerylive.com
First documentation of conjoined twins
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The Biddenden Maids, Mary and Eliza Chulkhurst were
born circa 1100 AD in Biddenden, County Kent, England.
Pygopagus twins joined who were joined at the hips and
shoulder
The earliest case illustrating
craniopagus twins
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The earliest case illustrating craniopagus twins is a report of
a set born in Bavaria, Germany. Described as a case of “Ein
Monstrum” (a monster)
Being born to a woman as a warning from God
These concept of a warning is an ancient concept dating
back at least to biblical time.
Early image of conjoined twins from
Cosmographia universalis by Sebastian
Münster (1488-1552), a Basel edition
printed by Sebastian Heinrich-Petric in
1550 ( leaves DCCLXVIIDCCLXVIII[767-768})
From personal collection of J.T.G.
Chang and Eng Bunker (May 11, 1811 – January 17, 1874) were
Thai-American conjoined twin brothers whose condition and
birthplace became the basis for the term “Siamese twins”
Chang had 11 children and Eng had 10 children.
Conjoint Twining Even in
other Living Things!
Development of Conjoined Twins:
Cause Unknown
Fission theory
• A single fertilized egg
does not completely divide
and results in two fetuses
that are joined together
wherever the division
fails.
Fusion theory
• The fertilized egg
completely separated into
identical twins, but due to
proximity in utero, the
twins re-fuse and
eventually conjoined.
The term conjoined twinning refers to an incomplete
splitting of monozygotic twins after 12 days of
embryogenesis. Image created by Samantha Cloutier and
Aaron Cloutier.
Types of conjoined twin:
subdivided into eight types
1. Omphalopagus-fixed
at the umbilicus- (10%).
2. Thoracopagus-chest (18.5%) are fused from the
upper thorax to the lower
belly
3. Cephalopagus-head-two faces
on opposite sides of a conjoined
head with a fused upper body
4. Ischiopaguship - fused lower half of
two bodies with spines conjoined
end-to-end
5. Craniopagus-helmet - (6%) fused at 6. Rachipagus-spine - 2% most common
site is at the midportion of the posterior
the skull but have separate bodies
vertebral column above the sacrum
7. Pygopagus-rump-( 6–19%)joined at the sacral area
8. Parapagus are fused at the abdomen and pelvis
Craniopagus Conjoined Twins
•
Craniopagus: 2% of all conjoined births
•
1/2.5 million births
•
> 90% have died by age of 10 years.
•
Peri-operative mortality pre-1974 was 67%
•
Mortality post 1974 is 36%
•
Morbidity - enormous!
Classification :
Craniopagus Conjoined Twins
1976- O’ConnellBased on anatomical and
circulatory anomalies,
• Partial and Total, further
subtypes I, II, III
• That describe the tilting and
rotation
• Of the twin’s heads in
relation to each other.
•
1987- Bucholz et al•Grouping the area of
conjoining into
• Frontal
• Parietal
• Temporoparietal
• Occipital.
Stone-Goodrich Brain
2006 Classification
Is based on presence or
absence of shared dural
venous sinuses
added to O’Connell by
angle. Partial angular,
total angular, partial
vertical, total vertical.
Importance of Classification System
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Improves the understanding of the anomaly
Serves as a guide to measure the outcome of the separation
attempt.
Helps surgeons be aware of common problems, but surgeons
must have a plan in place and be ready to tackle a host of
obstacles once the twins are on the operating table.
Indicates the surgical risk factors in order to determine
mortality and the extent of surgery required.
Predicts which cases are possible to separate
Anatomical Issues in Separation
Surgery
• Shared major venous sinuses
(circumferential sinus)
• Large scalp defects
• Large skull defects
• Plane between brains, where to
divide?
• Gravitational distortion of poorly
supported brain
• Large and rapid loss of blood
• Unbalanced intra-twin sharing of
blood (and anesthesia!)
• Contamination/infection
• Diversion of CSF
Thank You
I would like to thank Dr. James Goodrich who has
helped contribute with this presentation. I also
would like to thank Dr. Rick Abbott for his support,
and all of the neurosurgical team from Montefiore,
for guiding and supporting me to complete this
presentation. Thank You.