Childs Nerv Syst (2004) 20:618624

DOI 10.1007/s00381-004-1001-8

James Tait Goodrich

David A. Staffenberg

Received: 15 April 2004

Published online: 10 June 2004

Springer-Verlag 2004
J. T. Goodrich ())
Division of Pediatric Neurological Surgery,
Center for Craniofacial Disorders
at the Childrens Hospital at Montefiore,
Albert Einstein College of Medicine,
Bronx, New York, USA
e-mail: goodrich@aecom.yu.edu
Tel.: +1-718-9204187
Fax: +1-718-5196876
D. A. Staffenberg
Department of Plastic
and Reconstructive Surgery,
Center for Craniofacial Disorders
at the Childrens Hospital at Montefiore,
Albert Einstein College of Medicine,
Bronx, New York, USA

SPECIAL ANNUAL ISSUE

Craniopagus twins:
clinical and surgical management

Abstract Case report: We present

the case of 2-year-old craniopagus
twins who are in the process of undergoing a staged separation of their
craniums. In addition, they will undergo a full calvarial vault remodeling to reconstruct the lacking skull
and scalp tissue in each child. We
elected to do a staged separation rather than a single marathon operation
for various reasons, which we will
detail in this report. Discussion: The
philosophy and surgical management
of each step will be discussed along
with the pros and cons behind the
reasoning that was used. At the time
of this report the children have undergone three of what are projected to

Introduction
The craniopagus twins reported here were born on April
21, 2002 via a cesarean section. A prenatal ultrasound had
been done and viable craniopagus twins were determined
at 30 weeks gestation. Their obstetrical deliveries were
uneventful and shortly after birth they were sent to a major university center where an extensive work-up was
done to determine if any anomalies of the major organs
were present; a multi-system work-up was unremarkable.
A surgical separation was discussed by the local surgeons
but the case was deemed too complex to be done at the
local facilities. The Center for Craniofacial Disorders at
the Childrens Hospital at Montefiore was contacted and
asked if we would be willing to undertake a separation of
these craniopagus twins. After completion of an extensive
medical and radiological work-up in their country, we
decided to accept the children for a possible surgical separation.

be four stages of separation. Each of

the stages has had unique characteristics and surgical judgments involved, and those will be reviewed.
Conclusion: Successful separation of
viable conjoined twins has been historically a great rarity. Successful
separation of twins where both have
come out of surgery without any
neurological deficit remains a rare
occurrence and in our mind the ultimate goal.

Our team initially asked the local physicians for a series of medical and radiological studies to help us determine what would need to be done for these children. The
reports that we were sent suggested that these were two
healthy 14-month-old children, with no medical issues; a
different situation emerged when the children arrived in
the United States. Working with a local neurosurgeon we
arranged a series of radiology examinations. Due to problems with anesthesia and the inability to deliver it successfully, not all of these studies were completed. We
deferred these studies until the childrens arrival at our
center. An early photograph of the twins sent suggested to
our team that these children were underweight and that
both had neurological problems. This proved to be the
case. We will discuss our management of both the preoperative and operative phases of this separation. We
made the decision to do a multiple-stage cranial separation rather than a single-stage separation for various
reasons, which will be discussed (Fig. 1).

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Fig. 1 Twin A on the left and

twin B on the right, at about
1 year of age. Early neurological problems are clearly evident
in twin A with a partial left side
weakness secondary to positional disuse of his left side

Fig. 2 a MR with twin A on top

and twin B below, the interdigitation of the respective brains
can be seen, a discrete plane
between the two brains can be
appreciated. b The plane of fusion at the vertex of each child
is clearly evident in this MR

Fig. 3 a CT angiograms showing the vertex vascular anomalies with twin A on the top and
twin B below. Twin A has a
large sagittal sinus draining the
circumferential venous plexus
(seen clearly in B). Twin B has
minimal venous outflow in the
posterior (occipitalparietal region) portion of the vein. There
is deep venous circulation
clearly present in twin B and the
goal of a staged separation is to
have the deeper circulation take
over. b In this image twin B is
now at the top, the large midline
circumferential venous plexus
can be clearly seen in the center, at the fusion of the two
brains, draining into a large
anomalous sagittal venous
plexus

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Case report
Six months elapsed between our first contact and the arrival of the
children at the Childrens Hospital at Montefiore. During this interval, we attempted to obtain a series of radiological studies to
determine the anatomy of their conjoined brains. Due to aspiration
pneumonia, chronic medical illness, and anesthesia problems,
these studies were never completed. Due to their vertex joining,
these children laid chronically on their backs. This position led to
repeated aspiration pneumonia in both children with oral feeding.
Both children arrived at about 60% of their normal body weight, a
weight loss clearly due to the chronic aspirations and illness. The
children were initially placed at a local pediatric rehabilitation
hospital for the purpose of health care, weight gain, and motor and
intellectual development. In retrospect, this proved to be a most
important decision and one that became quite critical in the childrens care. Our initial reports on these children detailed no significant medical issues; such was not case on their arrival. Twin A
was severely hypertensive with a blood pressure of 210/125 mmHg
and twin B was hypotensive at 60/40 mmHg. Both children had
aspiration pneumonia with fevers and chest X-rays showing diffuse
infiltrates. Neurologically, both children had mild but definite
hemipareses from a chronic positioning on one side that did not
allow them to use one side of their bodies. Both children were
developmentally delayed secondary to social isolation in their
country and exhibited minimal smiles, no babbling and no form of
speech development. Our pediatric team did an extensive work-up
initially to evaluate the blood pressure issues; a full systemic workup, including an extensive renal evaluation was normal. Twin A
initially required four cardiac medications to control the pressure,

Fig. 5 A series of reconstructions done by Medical Modeling (Denver, CO) showing in 3D different views of the venous
system. Twin B is at the top and
twin A below. Twin A clearly
has most of the venous outflow
structures. The anomalous circumferential nature of the sagittal sinuses can be appreciated
here

Fig. 4 A medical model reconstructed from the CT venous angiogram showing the anomalous venous patterns. Twin A is on the
right and twin B is on the left. Image courtesy of Medical Modeling
bearing in mind that some degree of hypertension was essential for
the perfusion of both brains. Interestingly, medication given to one
child did not seem to affect the other. Twin A was routinely urinating but twin B rarely needed a diaper change. As the blood
pressures normalized twin B was no longer oliguric. Close col-

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laboration with the rehabilitation team corrected the feeding issues

with the use of feeding tubes. Repositioning of the children during
feeding led to an almost complete resolution of the aspiration
pneumonia. Over a period of 6 weeks, adequate weight gain was
achieved in both children and a complete medical work-up was
finished.
Our initial radiology work-up was directed towards determining
the potential connection between the twins brains (Figs. 2, 3). We
were particularly interested in any brain fusion, interdigitation, and
possible sharing of vascular supply. An MRI and CT study showed
that the brains abutted vertex to vertex with a 15% rotation, an
OConnell type I malformation. Twin A possessed the primary
venous outflow pattern with the major sagittal sinuses. Twin B
lacked any major venous outflow with the exception of some
smaller tributaries with his venous outflow directed to twin A. After
an extensive discussion with our neuroradiology and pediatric
colleagues, it was determined that a staged separation would be the
appropriate method, as we hoped to develop new deep venous
collaterals in twin B. Without these collaterals, the risk of venous
stasis and stroke would be unacceptably high. We discussed but
decided not to perform any form of venous bypasses due to the
anatomy and the known high failure rates of venous grafts.
Very helpful in our radiology work-up was the use of 3-D reconstructions of the arterial and venous circulations. The venous
circulation was anomalous but typical of what has been seen in
previous vertex-joined craniopagus twins. The sagittal sinus in
these children travels around the skull in a circumferential fashion.
Rudimentary midline sagittal sinuses are seen in the forehead region only in each child. As is typical in vertex joining there is a
major circumferential venous plexus at a fusion point of the two
brains. Key to making a separation plane is deciding which twin is
to get this anomalous venous structure. Attempts to split this
sinus will lead to major and unnecessary complications. We decided that the circumferential venous plexus was primarily possessed by twin A. However, separating it from twin B would require that twin B develop a deeper venous outflow pattern to avoid
venous stasis and congestion. Failure to develop this collateral
pattern would lead to a serious stroke or even death secondary to
severe venous congestion, a point made by several previous surgical teams. With this anomalous anatomy in mind, our team decided to perform a staged separation in the hope of re-routing the
superficial venous outflow present in twin A to a deeper cerebral
circulatory pattern in twin B. The model for this plan came from L.
Marion Walker, M.D., whose team at Primary Childrens Hospital
in Salt Lake City, Utah, USA had performed three staged separations of craniopagus twins with good results (discussed elsewhere
in this issue) (Figs. 4, 5).

of 140180 mmHg systolic and 85100 mmHg diastolic).

These children needed separate anesthesia teams during the
pre-operative work-ups. MRI machines are able to perform
only one exam at a time so a separate and second monitor
had to be ordered to provide the necessary monitoring
during sedation for both twins in the MR machine. Each
twin needed to be clearly documented in the MR or CT
scanner so there would later be no confusion in identifying
each child appropriately. In the operating room there were
separate anesthesia teams for each child. In addition, a
third senior-level anesthesiologist was present to act as a
bridge between the two teams. Slave monitors were made
available so that each team could see the other twins
physiologic responses.

Operating room issues

Due to the nature of these operations and the number of
necessary personnel, the largest operating room was made
available. We positioned the children at the foot of two
operating tables with the heads suspended freely between
the two tables. Each child was draped separately giving
full exposure of each calvarium from the ears up. Preoperatively, the plastic surgery team had marked out the
flaps to be rotated for the final skin coverage. Using

Anesthesia issues
Several anesthesia issues became apparent early on. Despite being conjoined twins, each twin has his own unique
circulation and reaction to medications. Medication given
to one twin does not necessarily affect the other twin.
Giving intravenous fluids to one child can lead to the other
twin urinating. In our case one child was severely hypertensive and the other hypotensive. However, this began to
normalize as the separation went forward. Our pediatric
team recognized early on that normalizing the hypertension
in twin A might not necessarily be advantageous, as the
hypertension was most likely needed to provide adequate
cerebral perfusion for both twins. So a higher than normal
blood pressure was considered acceptable (we used a range

Fig. 6 Intraoperative positioning of the twins, placed on separate

operating room tables with the heads suspended in between. Each
head is draped separately allowing 360 access to each child. At the
time of separation each child goes onto the respective OR table for
the reconstruction

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Fig. 7ad Imaging studies

done just before the third separation phase. a 3-D CT reconstruction showing the first two
staged craniotomy flaps. b Operative positioning using acrylic
models reconstructed from the
CT studies (provided by Medical Modeling. Denver, CO). c, d
Two CT angiograms showing
the collateral venous circulation
that has developed in twin B.
These studies were done a
month after the second staged
separation where approximately
5060% of separation had been
completed

acrylic models provided by Medical Modeling (Denver,

CO, USA), we were able to map out the skin flaps and the
various craniotomies. We used only one OR scrub nurse
who provided the instrumentation for both the plastic surgeon and neurosurgeon. There were two separate circulating nurses, one for neurosurgery and one for plastic
surgery. The children were positioned so that two surgeons could sit on opposite sides. By rotating the tables
each up to 30, full circumferential exposure of the skulls
could be obtained (Fig. 6).
At the initial surgery, tissue expanders were placed,
but these later became infected with bowel flora and had
to be removed. Because of the subcutaneous seromas that
normally developed and the risk of infections we later
made the decision to place the tissue expanders before the

final operation and not associate them with any craniotomy

Staged separation issues

The principle behind the staged separation is to allow
conjoined fused brains, which share arterial and venous
circulation, to develop adequate collateral circulation and
avoid serious neurological sequelae. To accomplish this
goal we decided that at least three, if not possibly five
stages would be needed to accomplish this. The initial
craniotomy was performed to separate a large frontal
midline sagittal sinus in twin B and this was achieved
without sequelae. At this first operation, it became clear
that twin A embryologically owned the majority of the

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circumferential venous sinus and the natural plane was on

the side of twin B. In addition, twin A had a remnant dural
membrane present while twin B had none, although an
arachnoid plane was present. At each stage of separation a
silicone sheet was placed in the dissected plane to prevent
fusion and scarring of the plane. These sheets proved to
be extremely helpful as the staged separation went forward. To date, three separations have been done in a
circumferential fashion and at this point, the twins are
90% separated. At each stage approximately 34 large
venous vessels were gradually clamped and taken. A
dural separation was done just lateral to the venous sinus
of twin A, on twin Bs side. The brains were gradually
separated following the arachnoid/dural plane. Metallic
clips were used to seal the venous sinus allowing us to
separate the children with minimal bleeding. As each of
the large veins was encountered, a temporary clip was
placed and we carefully looked for brain swelling and
venous engorgement. By doing this in a staged fashion we
have not seen any significant venous stasis. As a result of
this staging, twin B has developed a deep cerebral venous
outflow circulation that is clearly evident on MRV studies. As each of the separation stages has been completed
(three to date), the follow-up studies clearly reveal this
necessary re-routing of venous outflow. Neither of the
twins has developed any neurological deficits, neither
temporary nor permanent, nor is there any evidence of
venous or arterial infarction on MR. After the second
separation stage, which involved taking a large posterior
sagittal vein, twin B was noted by the mother to be not
acting right. Twin B seemed a bit slower and less affable, but this personality change resolved over about 2
weeks.
As the separations went forward, several interesting
physiological changes became evident. Twin As severe
hypertension began to normalize. Instead of four cardiac
medications for controlling the hypertension, twin A only
required two and in lesser amounts. In addition, the wide
swings in blood pressures began to normalize after the
second and third operations. Twin B who was barely
urinating, now began to go from only one diaper a day to
three per day. So clearly some physiological dynamics
were evolving (Fig. 7).

Discussion
Vertex joined craniopagus twins clearly share some complex vascular anatomy. Most of the vascular sharing,
however, appears to be on the venous side. Historically,
these twins have been separated in a single-stage operation, forcing both children to undergo rapid and sometimes disastrous changes in blood flow patterns. Following up on the initial studies of L. Marion Walker,
M.D. and his team it seems intuitive that a staged separation is the ideal way to go in these complex cases.

Fig. 8 a Twin A to the left and twin B to the right, postoperative

day 2 after the third separation procedure. (Reprinted with permission by Montefiore Medical Center. Photography by Alice
Addie.) b Twin A to the left and twin B to the right on postoperative day 3 after the third separation. At this point, about 90% of the
separation had been completed. Photograph by Alice Addie

Review of the previous cases in the literature on vertex

craniopagus twins typically reveal these anomalous vertex circumferential venous plexuses. One twin always
seems to have the major venous outflow while the other
twin has little and is shunting to its respective twin.
Successful separation would dictate that the twin without
the collateral venous outflow needs to gradually develop
its own deep venous circulation before final separation and a staged approach seems to us the ideal way to
achieve that goal. An equally important goal is the availability of adequate soft tissue coverage at the completion
of the separation. Here, tissue expansion of the scalps are
key so that adequate scalp coverage can be obtained to
prevent later CSF leakage and infection. Here, the cooperation between the plastic surgeon and neurosurgeon
became essential and key to a good outcome. Persistent
CSF leakage and meningitis can only lead to unacceptable further neurological compromise. The anatomical
separation of vertex-joined twins often leads to a disruption of the CSF absorptive pathways over the vertex,
so it is not uncommon for one or both twins to develop

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hydrocephalus at some point. The neurosurgical team has

to be prepared to divert CSF in a satisfactory fashion at
some point. To date, neither of our twins has developed
hydrocephalus, but we are prepared to deal with that it
should it occur.
For a separation to be successful an enormous amount
of cooperative efforts are needed from the pediatric, anesthesia, neurosurgical, and plastic surgery teams. In our

case, the contribution of the physical and occupational

therapist was key in getting these children to the appropriate body weight and in good health to tolerate these
complex surgical procedures. In our case, resolution of
systemic high blood pressures and continual aspiration
pneumonias had to be resolved before any staged cranial
separation could be accomplished (Fig. 8a, b).