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The Pygopagus
Conjoined Twins
Separation and Perfect
Nerve Systems Rebuilt

www.elsevier.com/locate/jpedsurg

Successful separation of the conjoined thecal sac with an

epidermal cyst in pygopagus twins
Yu-Cheng Chou a , Hai-Chyi Peng b , Chia-Hsiang Chu c , Sheng-Tzung Tsai a ,
Joseph Chang Ming Ling d , Yung-Hsiang Hsu e , Shin-Yuan Chen a , Tomor Harnod a,
a

Department of Neurosurgery, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien 970, Taiwan, ROC
Department of Pediatric Surgery, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien 970, Taiwan, ROC
Department of Pediatrics, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien 970, Taiwan, ROC
Department of Medical Imaging, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien 970, Taiwan, ROC
Department of Pathology, Buddhist Tzu Chi General Hospital and Tzu Chi University, Hualien 970, Taiwan, ROC

b
c

d
e

Received 23 March 2011; revised 23 May 2011; accepted 29 May 2011

Key words:
Conjoined;
Twins;
Pygopagus;
Thecal sac;
Epidermal cyst

Abstract Pygopagus conjoined twins are very rare, and half of all pygopagus twins have other
anomalies. This report describes the successful surgical separation of pygopagus twins who had a
conjoined thecal sac and an epidermal cyst. Meticulous preoperative planning, development of a model
simulator, multispecialty teamwork, and a staged surgery contributed to a successful outcome.
2011 Elsevier Inc. All rights reserved.

Pygopagus twins, those joined at the rump, are classified

as symmetrical conjoined twins [1]. Pygopagus twins are
exceedingly rare, accounting for only 10% to 18% of all
conjoined twins, yielding an incidence of approximately 1 in
1,000,000 live births. Pygopagus conjoined twins commonly
share the gluteal region; terminal spine; and gastrointestinal,
urologic, and reproductive systems to various degrees. Half
of all pygopagus twins have anomalies unrelated to the
classic fused organs, including a high incidence of vertebral
anomalies [2,3]. This report describes the neurosurgical
management of pygopagus twins with spinal abnormalities
and an epidermal cyst.

Corresponding author. Tel.: +886 3 8561825x2151; fax: +886 3

8463164.
E-mail addresses: nsha@tzuchi.com.tw, chouycns@tzuchi.com.tw,
tomorha@yahoo.com.tw (T. Harnod).

1. Case report
Female pygopagus conjoined twins were delivered by
cesarean delivery at 39 weeks of gestation in the
Philippines. They were transferred to the Buddhist Tzu
Chi General Hospital in Taiwan at 5 months of age (Fig. 1).
The twins shared a common urogenital sinus with separate
vaginal and urethral openings and one anus with low fusion
of the rectums. Both twins had good movement of the lower
limbs and were each capable of urinating and defecating.
Magnetic resonance imaging (MRI) revealed a single dural
sac between 2 separate sacral bones (Fig. 2). Based on the
amassed computed tomographic and MRI images, a model
of the twins was developed and used for surgical planning
and simulation.
To ensure adequate skin flaps to cover the underlying
tissues and organs after their separation, the twins underwent
staged operations. First, tissue expanders were implanted in

0022-3468/$ see front matter 2011 Elsevier Inc. All rights reserved.
doi:10.1016/j.jpedsurg.2011.05.023

The thesis on separating the Rose Sister,

received international recognition,
and was published in the Journal of
Pediatric Surgery.

Conjoined twins are

rare cases, but pygopagus

E27

Acknowledgments
The authors are grateful to their mentor, Master Cheng
Yen, the president of Buddhist Compassion Relief Tzu Chi

Foundation. The authors thank Dr Chien-Hsing Wang who

provided the photograph for Fig. 1.

References
[1] Saguil E, Almonte J, Baltazar W, et al. Conjoined twins in the Philippines:
experience of a single institution. Pediatr Surg Int 2009;25:775-80.
[2] Spitz L, Kiely EM. Experience in the management of conjoint twins.
Br J Surg 2002;89:1188-92.
[3] Fieggen AG, Dunn RN, Pitcher RD, et al. Ischiopagus and pygopagus
conjoined twins: neurosurgical considerations. Childs Nerv Syst 2004;20:
640-51.
[4] Hockley AD, Gornall P, Walsh R, et al. Management of pyopagus
conjoined twins. Childs Nerv Syst 2004;20:635-9.
[5] Shikata J, Yamamuro T, Mikawa Y, et al. Intraspinal epidermoid and
dermoid cysts. Surgical results of seven cases. Arch Orthop Trauma
Surg 1988;107:105-9.
[6] van Aalst J, Hoekstra F, Beuls EA, et al. Intraspinal dermoid and
epidermoid tumors: report of 18 cases and reappraisal of the literature.
Pediatr Neurosurg 2009;45:281-90.
[7] Fieggen G, Millar A, Rode H. Spinal cord involvement in pygopagus
conjoined twins: case report and review of the literature. Childs Nerv
Syst 2003;19:183-7.
[8] Matta H, Jacobsz A, Auchincloss J. Successful separation of
pygopagus conjoined twins. J Pediatr Surg 2006;41:586-8.
[9] Gupta JM. Pygopagus conjoined twins. Br Med J 1966;2:868-71.
[10] Votteler TP. Necrotizing enterocolitis in a pygopagus conjoined twin.
J Pediatr Surg 1982;17:555-7.

more rare. They often have

other congenital anomalies
and underdeveloped

the Nerve Architect, commended Hualien Tzu Chi Medical Center in

central nerve, which lead

separating the twin Rose Sisters while attending the second International

to prognostic symptom

thought that the complexity of this surgery should be very difficult, because

Separation of a conjoined thecal sac

and divided the neural elements before the bowel was opened
to abrogate concerns regarding a contaminated surgical field
[3]. The surgical team strived to apportion neural elements as
equally as possible to avoid damaging either twin. The
option of not sectioning roots between the twins did not
exist, so intraoperative physiologic monitoring was presumed to be of little help [3] and was, therefore, not used.
The twins had no neurologic deficits after the separation,
supporting this decision. Next, primary closure of the dura in
these pygopagus conjoined twins was achieved without a
graft or patch, both of which have previously been described
[7,8]. Despite the fact that CSF leakage in pygopagus twins
surviving separation with union of the spinal cords is as high
as 37.5% [7] and given that postoperative CSF leakage can
necessitate a second surgery [9,10], CSF leakage did not
occur in this case. Suction drains were also avoided to
minimize the risk of a CSF fistula [3]. Instead, a careful,
watertight dural closure and meticulous myofascial sutures
were chosen.

conjoined twins are even

Professor Wise Young of Rutger University in the US, who is known as

Conference of Stem Cells and Regenerative Medicine on Sept. 9, 2011. He

similar to cerebral palsy.

The twins nicknamed Rose Sisters from Philippine
has been successfully separated by Hualien Tzu Chi

the twins are conjoined not only the bottoms but also the end of their spines,

medical team. Of which, the neurosurgery team was

especially that they shared urogential and reproduction parts, which made

the key part responsible for the most difficult part of

the surgery even more difficult.

Written by Yu-Cheng Chou and Tomor Harnod, , neurosurgeon, Hualien Tzu Chi Medical Center

82

Journal of Pediatric Surgery (2011) 46, E25E27

Tzu Chi Medical Care Vol.15 January 2013

their body conjoined thecal sac and central nerve.

After we investigated the details of the imitating
operation meeting contents and related nerve surge
details, we shared our experience with professionals

Tzu Chi Medical Care Vol.15 January 2013

83

Fountain of
Innovation

history. In 2010, when the Rose Sisters were about

five months old, Tzu Chis Philippine Chapter arranged
t h e m t o t r a n s f e r t o o u r h o s p i t a l . D i re c t o r H a i - C h i
Peng of the Pediatric Surgery Dept. went to Philippine
to exam the Rose Sisters and brought back related
information for further evaluation. Then, they were
admitted to our hospital and Dr. Chia-Hsiang Chu as
their attending Pediatrician. The anesthesiology division
carefully arranged the imaging examination so that
we completed CI and MRI. Then, the 3D model was
made according to the digital exams. This allowed us
Dr. Tomor Harnod takes over
separating and repairing spinal
nerve after general surgery team
completed the separation.

to repeat simulation, setup and remodel the operation

plan.
To e n s u re t h e re d b e e n o u g h t i s s u e c o v e r i n g

The spine and nerve root of the

Rose Sisters were connected,
with rare epidermal cysts.
After neurosurgery cutting off
the epidermal cysts, the spine
and nerve separation was also
completed. Picture sketched by
Tomor Harnod

at the Annual Conference

organs after the separation, the first step is to have the

o f Ta i w a n N e u r o s u r g i c a l

plastic surgery team placing a tissue expander at the

Society in 2010. They were

Rose Sisters lateral thigh. During this step, we also

touched by our detailed

performed cystoscopy, proctoscopy, and colposcopy

planning and team work.

to check the genitourinary and the gastrointestinal

On September of 2011, the

s y s t e m s . We a l s o p e r f o r m e d t h e p a r a l l e l s i g m o i d

Journal of Pediatric Surgery

colostomy to avoid future fecal contamination.

confirmed our work, and

published our article,
Successful separation of

Delicately Assigned Layers

to Retain the Best Nerve Function

the conjoined thecal sac

with an epidermal cyst in
pygopagus twins.

84

The second stage was performed when the Rose

Sisters were seven months old. We first placed them

Pygopagus twins are about 10 to 18 percent of

facing up. The pediatric surgery and general surgery

all conjoined twins. The chance having pygopagus

teams first separating their reproduction part. The

twins is nearly one out of a million. They usually share

plastic surgery team removed the tissue expander. Then

the hips, the end of the spine, stomach, and urine

we turned them facing down. We, the neurosurgery

and reproduction parts. The Rose Sisters is different

team, took over separating the spine. We first opened

from previous cases. Besides the conjoined thecal sac

the exposed marrow of the conjoined spinal cavity. We

they had an epidermal cyst. This never happened in

cut the associated nerve marrow into Y shape, and

Tzu Chi Medical Care Vol.15 January 2013

Tzu Chi Medical Care Vol.15 January 2013

85

Fountain of
Innovation

and nerve marrow separation. After that, the pediatric

surgery and general surgery team separated the rectal,
and the plastic surgery team sutured the skin to protect
the organ structure.
The third stage was performed when they were nine
months old. We closed the sigmoid colostomy. By now,
the two sisters were living separately for two months.
The overall post-operation condition was good.
There were no leakage of spinal fluid and neurologic
deterioration.
In order to successfully separating conjoined twins,

The separation surgery in June,

2010 was successful. It gave Rose
Sisters of Philippine their own
independent future.

it relays on all teams understanding how to overcome

challenges and difficulties, repeat simulations with the
model made from the image, and with the medical and

After successful separation, Rose

Sisters returned to Hualien Tzu Chi
Medical Center with Tzu-en and
Tzu-ai, conjoined twins separated
successfully in 2003, for the 25th
anniversary of Tzu Chi Medical
Mission. They visit the pediatric
team to say thank you.

nutritional support and performing bold but cautious

phased surgeries. The death rate for emergency
separation of conjoined twins is up to 80%. The foreign
experts suggest that the conjoined twins will have 70%
of survive rate if separated between six to nine months.
Our team learned from the wisdom of related teams
in the world and completed the separation of the Rose
Sisters when they were seven months old. The keys to
successful separation in neurosurgery are: 1)

to cut

the nerve part prior to intestinal part can avoid infection

from the surgery. 2) To cut the nerve system evenly to
ensure the best nerve function for the two individuals in
the future. We are very thankful for the support from the

86

cut off the epidermal cyst which was connecting to the

Master and the foundation, the hospitals organization,

end of the nerve marrow. This made the nerve marrow

all divisions teamwork, and volunteers care. Seeing

changed from Y shape to V shape. By then, there

the Rose Sisters joyfully returned to the home of Tzu

were excess neural myeloid dural formed. We separate

Chi members, all our hard works are worth it. We are

the nerve marrow from V shape to II shape. Then,

truly grateful to have this opportunity to utilizing our

we turned the excess nerve myelinated dural upward

professional skills and accompany the Rose Sisters to

and sealed the edges close to complete the thecal sac

grow.

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