The Turkish Journal of Pediatrics 2013; 55: 99-103

Case Report

Parapagus (dicephalus, tetrabrachius, dipus) conjoined twins

and their rehabilitation
Sibel Başaran1, Rengin Güzel1, Erbuğ Keskin2, Tunay Sarpel1
1Department of Physical Medicine and Rehabilitation, Çukurova University Faculty of Medicine, Adana, and 2Pediatric

Surgeon, İstanbul University Faculty of Medicine, İstanbul, Turkey. E-mail: sbasaran@cu.edu.tr

SUMMARY: Başaran S, Güzel R, Keskin E, Sarpel T. Parapagus (dicephalus,

tetrabrachius, dipus) conjoined twins and their rehabilitation. Turk J Pediatr
2013; 55: 99-103.
A case of female parapagus conjoined twins living as unseparated is presented
in this report. The twins had two heads, four arms and two legs. As a result
of their fusion, operative care had been considered to be unacceptable, and
the family had refused to take care of them. At three years of age, they were
referred to the Rehabilitation Department with the complaint of inability
to walk. While designing the rehabilitation program, somatosensory evoked
potential evaluation was performed and showed that twin 1 controlled the
right leg and twin 2 controlled the left. The program consisted of coordinated
pacing training by verbal commands, upper and lower extremity reeducation,
and balance and coordination exercises. After two months of inpatient
rehabilitation, they were able to walk independently with a specially designed
walker.
Currently, the twins are 11 years old with normal academic achievement, and
they are able to walk, run and climb the stairs independently.
Key words: conjoined twins, dicephalus, magnetic resonance imaging, parapagus,
rehabilitation.

The incidence of conjoined twins is reported to umbilicus, thoracopagus-chest, cephalopagus-

be in the range of one in 50,000-200,000 live
births1. Conjoined twins are always genetically
identical and the same sex. Females are most
commonly affected, with a ratio of female/
male of 4:1. No association of race, age,
parity, maternal age, heredity, or environmental
influences has been reported2.
Conjoined twins are classified according to the
most prominent site of union together with
the suffix pagus, meaning fixed, and divided
into two groups depending upon the aspect
of the embryonic disc involved3. The ventral
subgroups are joined over a single yolk sac with
a shared abdomen and umbilicus and include
those joined rostrally, caudally and laterally. The
dorsal subgroup is conjoined in the neural tube,
each with a separate abdomen and umbilical
cord. The terminology describing the various
types of conjoined twins can be subdivided
into eight types: omphalopagus-fixed at the
This case was reported in the 5th Mediterranean Congress of Physical and Rehabilitation Medicine, Antalya, Turkey,
2004.
head, ischiopagus-hip, craniopagus-helmet,
rachipagus-spine, pygopagus-rump, and
parapagus-side2.
Thoracopagus is the commonest variety,
accounting for 40% of reported cases. It is
followed by omphalopagus (32%), pygopagus
(19%), ischiopagus (6%), and craniopagus
(2%). Parapagus is the term used where there
is extensive side-to-side fusion, and this is
a rare form of conjoined twins4. Parapagus
twins joined anterolaterally result from two
nearly parallel notochords in close proximity.
This anomaly represents less than 0.5% of
all reported cases of conjoined twins5. In the
literature, there are case reports presenting
dicephalic conjoined twins, either tetrabrachius
or dibrachius5-13. However, some of them are
reported to be stillborn while others died
shortly after birth.
The prognosis of conjoined twins is related
100  Başaran S, et al. The Turkish Journal of Pediatrics • January-February 2013

cesarean section in December 2000. When the

family was informed about the impossibility
of surgical separation, they rejected the twins
and disappeared. During those three years after
birth, the twins lived in the pediatric surgery
ward, and in addition to their medical care, all
the basic needs were met by the hospital staff.
They had two heads, four arms, two legs,
and two thoraxes fused below the level of
the nipples. The hemithoraxes near to the
midline were smaller than the lateral ones (Fig.
1). They had two separate vertebral columns
met at the sacrum, two functional hearts, a
Figure 1: The parapagus conjoined twins at 2 months
of age.
shared abdominal aorta and inferior vena cava,
one shared liver, one spleen, two kidneys, a
partially shared gastrointestinal tract with two
to the type and extent of the union, and separate stomachs, and fused small intestine
the management of conjoined twins can that continued with a single colon and rectum.
be divided into four separate time frames: All structures of genitourinary organs (ureter,
prenatal care and counseling, non-operative bladder, urethra, uterus and vagina) were
treatment, emergency separation, and elective single with a shared pelvis (Figs. 2, 3). As a
separation. Non-operative care is indicated result of their fusion, operative care had been
in the presence of complex cardiac fusion or considered to be unacceptable.
where there would be severe unacceptable In addition to the systemic physical examination,
deformity following separation4. According to a detailed neurologic and musculoskeletal
our literature search, we could not find any evaluation was performed. Although they were
research or case report about the results of
non-operative care. Unseparated conjoined
twins should be evaluated for their functional
capacity (e.g. musculoskeletal evaluation,
mobility and ambulation potential) as early
as possible. Rehabilitation goals should be
tailored for each case and the twins should
be encouraged to be independent.
Each case has particular importance in advancing
scientific knowledge of rare diseases14. In this
case report, we present a case of female
parapagus (dicephalus, tetrabrachius, dipus)
conjoined twins because of its rarity and
the results of successful rehabilitation for
ambulation. To our knowledge, they are the
only living conjoined twins in Turkey and one
of the few living parapagus conjoined twins in
the world currently.

Case Report
The twins were three years old when they
were referred to the department of Physical
Medicine and Rehabilitation with the complaint
of inability to walk. The mother did not have
prenatal care so the cases were not diagnosed
until term evaluation. They were delivered by Figure 2: X-ray of the conjoined twins after birth.
Volume 55 • Number 1 Conjoined Twins and Rehabilitation  101

Figure 3: Coronal MR imaging of the conjoined twins

after birth.

crawling awkwardly on their belly without

coordination using the upper extremities Figure 4: The conjoined twins at 3 years of age.
for forward progression, they were unable
to sit or stand (Fig. 4). Their feet were in
equinus position. The hip and knee range
of motion were normal. While designing
the rehabilitation program, somatosensory
evoked potential evaluation was performed,
and the results indicated that the twin on
the right side controlled the right leg and
the twin on the left side controlled the left
leg. The rehabilitation program consisted
of upper and lower extremity reeducation,
balance and coordination, and gait training.
The advantage in the rehabilitation process
was that the cognitive abilities, perception and
the cooperation of the twins were compatible Figure 5: The parapagus conjoined twins are shown
with their normal counterparts and both were pacing in coordination.
enthusiastic to walk. In order to prevent the
progression of the flexible equinus deformity
name of the twin on the right side for the right
of the feet, a pair of custom-made orthopedic
step and vice versa for the left step. Rhythmic
shoes was prescribed. Additionally, a special
hand clapping and verbal commands enabled
wide pediatric walker was designed, which
would be suitable for their large trunk. With coordinated pacing and with time they were
the help of these specially designed shoes and able to walk without commands. The major
the walker, equilibrium in the standing position difficulty of the rehabilitation process was the
was achieved (Fig. 5). During gait training, management of two different individuals with
since each twin was controlling the leg on two upper bodies and four arms sharing two
their respective side, the therapist was clapping legs. It was very difficult to balance the center
hands in a rhythmic pattern and calling the of gravity and to stand with such a huge body.
102  Başaran S, et al. The Turkish Journal of Pediatrics • January-February 2013
Sometimes one of the twins did not want to
participate in the treatment, while the other
was eager to do so. The rehabilitation program
was designed taking into account factors such
as the age of the children, focusing their
attention and fatigue. The program consisted
of two 30 minutes sessions a day, five days
a week. After two months, they were able
to walk independently using their specially
designed walker.
When the parents learned that the twins were
able to walk independently, they accepted their
children and the twins were discharged from
the hospital. The socioeconomic status of the
family was poor and they attended their follow-
up evaluation only once, after which they were
lost to follow-up. Later, we learned from the
media that their father had exploited them,
making them beg in marketplaces. Upon the
complaints of the neighbors and the relatives,
the twins were taken under state protection.
We were pleased to learn through the press
that they are still living and healthy. They are
now 11 years of age with a normal academic
achievement, and they graduated from the
first level (fifth grade) of primary school. They
are not using any special aids and are able to
walk, run and climb the stairs independently.
Discussion
Conjoined twinning is a rare phenomenon,
occurring in 1 in 50,000 to 100,000. However,
since 60% are stillborn or die shortly after,
the true incidence is around 1 in 200,000 live
births15. In a recent epidemiological study,
it was found that the total prevalence was
1.47 per 100,000 births. A significant female
predominance particularly of the thoracopagus
type and a significant male predominance
in parapagus and parasitic types have been
detected. No significant genetic-, environmental-
or demographic-associated factors have been
identified16. Parapagus twins represent less than
0.5% of all reported cases of conjoined twins5.
Whereas the incidence of conjoined twinning in
our country is unknown, there have been a few
previous reports of parapagus conjoined twins
from Turkey5,8,11,12,17,18. The case presented by
Tansel et al5. was male parapagus (dicephalus,
tetrabrachius, dipus) conjoined twins. However,
as this case was prenatally diagnosed, the
pregnancy was terminated and male conjoined
twins were vaginally delivered at 22 weeks of
gestation. Ince et al17. presented dicephalic
parapagus conjoined twins recently. The twins
had two heads, a single thorax and abdomen,
one rudimentary and two independent upper
and lower limbs, and one genitalia and anus.
They were postnatal 42 days of life and still
being followed in the neonatal intensive care
unit when this case was presented17. Taner
et al19. presented an early prenatal diagnosis
of thoracopagus conjoined twins at 7 weeks
and 6 days of gestation with two-dimensional
Doppler ultrasound. Early diagnosis is very
important to escape this anomaly and the
unimaginable stress for the family. For that
reason, early prenatal sonographic examination
should be performed in each suspected case
by experts in this field. Another case from
Turkey involved female parapagus conjoined
twins (dicephalus, dibrachius, dipus). The
condition was not diagnosed prenatally and the
twins were dead at birth11. To our knowledge,
this case represents the only living conjoined
twins in Turkey.
Dicephalic conjoined twins may have a long life.
The main predictor of survival is the degree
of conjunction and abnormality of the hearts.
The majority of stillborns have cardiopulmonary
malformations that are incompatible with
extrauterine life. It is desirable to separate
less extensively conjoined cases. In dicephaly,
and also in certain other types of extensively
conjoined twins, the anatomic structure is such
that it is unlikely that both twins will survive
an attempt at separation1,20. In a review by
Bondeson1, dicephalic conjoined twins in the
past and present were investigated. The Tocci
brothers (1877-1940) were also parapagus
conjoined twins (dicephalic, tetrabrachius,
dipus) like our case. Each boy controlled the
leg on his respective side, but they were never
able to coordinate their movements and could
never walk without assistance throughout their
life. Poor muscular development, caused by
prolonged bed rest and inactivity, is presumed
to be the reason for their immobility. Their
immobility was also advantageous for their
parents because this made their exploitation
much easier1.
The Hensel twins (dicephalic, dibrachius,
dipus) are parapagus conjoined twins currently
living as unseparated. Although each twin
Volume 55 • Number 1 Conjoined Twins and Rehabilitation  103

controlled the arm and leg on her side, they 10. Groner JI, Teske DW, Teich S. Dicephalus dipus
were remarkably agile, coordinated their dibrachius: an unusual case of conjoined twins. J
Pediatr Surg 1996; 31: 1698-1700.
movements perfectly, and could not only walk,
11. Harma M, Harma M, Mil Z, et al. Vaginal delivery
but ran, swam, and rode a bicycle1. They are
of dicephalic parapagus conjoined twins: case report
now 21 years old, alive and well. and literature review. Tohoku J Exp Med 2005; 205:
Based on our literature search, there are few 179-185.
dicephalic (either tetrabrachius or dibrachius) 12. Mete A, Cebesoy FB, Dikensoy E, et al. Dicephalic
parapagus conjoined twins: a rare second trimester
parapagus conjoined twins who remained alive
sonographic diagnosis. J Clin Ultrasound 2010; 38:
as unseparated. Cases of conjoined twins occur 89-90.
so rarely, it is important to learn as much as 13. Onuoha CE, Iyare FE. Dicephalic-dipus: a case report.
possible from each case. To the best of our West Afr J Med 2006; 25: 161-163.
knowledge, our case represents one of the few 14. Carey JC. The importance of case reports in advancing
currently living parapagus conjoined twins in scientific knowledge of rare diseases. In: Posada de la
the world. Furthermore, they can perform their Paz M, Groft SC (eds). Rare Diseases Epidemiology
activities of daily living, can walk independently (Advances in Experimental Medicine and Biology series,
vol 686). New York: Springer; 2010: 77-86.
and are continuing their education.
15. Spitz L, Kiely EM. Experience in the management of
In conclusion, unseparated conjoined twins conjoined twins. Br J Surg 2006; 89: 1188-1192.
should not be left to their fate. Rehabilitative 16. Mutchinick OM, Luna-Munoz L, Amar E, et al. Conjoined
approaches can help them to become functionally twins: a worldwide collaborative epidemiological study
active, and if needed, with the help of assistive of the International Clearinghouse for Birth Defects
devices, they can live independently. Surveillance and Research. Am J Med Genet C Semin
Med Genet 2011; 157: 274-287.
17. Ince DA, Ecevit A, Kurt A, et al. Dicephalic parapagus
Acknowledgement conjoined twins. Indian J Pediatr 2012; 79: 818-819.
We thank Professor Sevim Balci, MD for 18. Ulker K, Akyer SP, Temur I, et al. First trimester
reviewing our case report and physiotherapist diagnosis of parapagus diprosopus dibrachius dipus
Yıldız Sahin for her contribution to the twins with craniorachischisis totalis by three-
dimensional ultrasound. J Obstet Gynaecol Res 2012;
rehabilitation process of the twins. 38: 431-434.
19. Taner MZ, Kurdoglu M, Taskiran C, et al. Early prenatal
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