Main Page | Editorial Staff | Editorial Board | About | Contents | Archive | Search | Instructions | Association | E-Mail
The Turkish Journal of Pediatrics

[Summary]  [PDF]  [Mail to Editor ]  [Back]
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:
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.

Keywords: conjoined twins, dicephalus, magnetic resonance imaging, parapagus, rehabilitation.
The incidence of conjoined twins is reported to be in the range of one in 50,000-200,000 live births[1]. 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 reported[2].

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 involved[3]. 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 umbilicus, thoracopagus-chest, cephalopagushead, ischiopagus-hip, craniopagus-helmet, rachipagus-spine, pygopagus-rump, and parapagus-side[2].

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 twins[4]. 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 twins[5]. In the literature, there are case reports presenting dicephalic conjoined twins, either tetrabrachius or dibrachius[5]-[13]. However, some of them are reported to be stillborn while others died shortly after birth.

The prognosis of conjoined twins is related to the type and extent of the union, and the management of conjoined twins can be divided into four separate time frames: prenatal care and counseling, non-operative treatment, emergency separation, and elective separation. Non-operative care is indicated in the presence of complex cardiac fusion or where there would be severe unacceptable deformity following separation[4]. According to our literature search, we could not find any 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.

Figure 1: The parapagus conjoined twins at 2 months of age.

Each case has particular importance in advancing scientific knowledge of rare diseases[14]. 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 Presentation
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 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 shared abdominal aorta and inferior vena cava, one shared liver, one spleen, two kidneys, a partially shared gastrointestinal tract with two separate stomachs, and fused small intestine that continued with a single colon and rectum. All structures of genitourinary organs (ureter, bladder, urethra, uterus and vagina) were single with a shared pelvis (Figs. 2, 3). As a result of their fusion, operative care had been considered to be unacceptable.

In addition to the systemic physical examination, a detailed neurologic and musculoskeletal evaluation was performed. Although they were crawling awkwardly on their belly without coordination using the upper extremities 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 with their normal counterparts and both were enthusiastic to walk. In order to prevent the progression of the flexible equinus deformity of the feet, a pair of custom-made orthopedic shoes was prescribed. Additionally, a special wide pediatric walker was designed, which would be suitable for their large trunk. With the help of these specially designed shoes and the walker, equilibrium in the standing position was achieved (Fig. 5). During gait training, since each twin was controlling the leg on their respective side, the therapist was clapping hands in a rhythmic pattern and calling the name of the twin on the right side for the right step and vice versa for the left step. Rhythmic hand clapping and verbal commands enabled coordinated pacing and with time they were able to walk without commands. The major difficulty of the rehabilitation process was the management of two different individuals with two upper bodies and four arms sharing two legs. It was very difficult to balance the center of gravity and to stand with such a huge body.

Figure 2: X-ray of the conjoined twins after birth.

Figure 3: Coronal MR imaging of the conjoined twins after birth.

Figure 4: The conjoined twins at 3 years of age.

Figure 5: The parapagus conjoined twins are shown pacing in coordination.

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 followup 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.

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-, environmentalor demographic-associated factors have been identified[16]. Parapagus twins represent less than 0.5% of all reported cases of conjoined twins[5]. Whereas the incidence of conjoined twinning in our country is unknown, there have been a few previous reports of parapagus conjoined twins from Turkey[5],[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 al[17]. 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 presented[17]. Taner et al[19]. 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 birth[11]. 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 separation[1],[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 easier[1].

The Hensel twins (dicephalic, dibrachius, dipus) are parapagus conjoined twins currently living as unseparated. Although each twin controlled the arm and leg on her side, they were remarkably agile, coordinated their movements perfectly, and could not only walk, but ran, swam, and rode a bicycle1. They are now 21 years old, alive and well.

Based on our literature search, there are few dicephalic (either tetrabrachius or dibrachius) parapagus conjoined twins who remained alive as unseparated. Cases of conjoined twins occur so rarely, it is important to learn as much as possible from each case. To the best of our knowledge, our case represents one of the few currently living parapagus conjoined twins in the world. Furthermore, they can perform their activities of daily living, can walk independently and are continuing their education.

In conclusion, unseparated conjoined twins should not be left to their fate. Rehabilitative approaches can help them to become functionally active, and if needed, with the help of assistive devices, they can live independently.


We thank Professor Sevim Balci, MD for reviewing our case report and physiotherapist Yıldız Sahin for her contribution to the rehabilitation process of the twins.


1. Bondeson J. Dicephalus conjoined twins: a historical review with emphasis on viability. J Pediatr Surg 2001; 36: 1435-1444.

2. Walker M, Browd SR. Craniopagus twins: embryology, classification, surgical anatomy, and separation. Childs Nerv Syst 2004; 20: 554-566.

3. Spitz L, Kiely EM. Conjoined twins. JAMA 2003; 289: 1307-1310.

4. Spit L. Conjoined twins. Prenat Diagn 2005; 25: 814-819.

5. Tansel T, Yazıcıoglu F. Cardiac and other malformations in parapagus twins. Arch Gynecol Obstet 2004; 269: 211-213.

6. Anastasakis E, Zhang EG, Bates AW, et al. Parapagus dicephalus dibrachius tripus: an unusual case of conjoined twins. Prenat Diagn 2007; 27: 1165-1166.

7. Aparna C, Renuka IV, Sailabala G, et al. Dicephalus dipus tribrachius: a case report of unusual conjoined twins. Indian J Pathol Microbiol 2010; 53: 814-816.

8. Camuzcuoglu H, Toy H, Vural M, et al. Prenatal diagnosis of dicephalic parapagus conjoined twins. Arch Gynecol Obstet 2010; 281: 565-567.

9. Gessessew A. Dicephalus tetrabrachius. Ethiop Med J 2001; 45: 391-394.

10. Groner JI, Teske DW, Teich S. Dicephalus dipus dibrachius: an unusual case of conjoined twins. J Pediatr Surg 1996; 31: 1698-1700.

11. Harma M, Harma M, Mil Z, et al. Vaginal delivery of dicephalic parapagus conjoined twins: case report and literature review. Tohoku J Exp Med 2005; 205: 179-185.

12. Mete A, Cebesoy FB, Dikensoy E, et al. Dicephalic parapagus conjoined twins: a rare second trimester sonographic diagnosis. J Clin Ultrasound 2010; 38: 89-90.

13. Onuoha CE, Iyare FE. Dicephalic-dipus: a case report. West Afr J Med 2006; 25: 161-163.

14. Carey JC. The importance of case reports in advancing scientific knowledge of rare diseases. In: Posada de la Paz M, Groft SC (eds). Rare Diseases Epidemiology (Advances in Experimental Medicine and Biology series, vol 686). New York: Springer; 2010: 77-86.

15. Spitz L, Kiely EM. Experience in the management of conjoined twins. Br J Surg 2006; 89: 1188-1192.

16. Mutchinick OM, Luna-Munoz L, Amar E, et al. Conjoined twins: a worldwide collaborative epidemiological study of the International Clearinghouse for Birth Defects 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 conjoined twins. Indian J Pediatr 2012; 79: 818-819.

18. Ulker K, Akyer SP, Temur I, et al. First trimester diagnosis of parapagus diprosopus dibrachius dipus twins with craniorachischisis totalis by threedimensional ultrasound. J Obstet Gynaecol Res 2012; 38: 431-434.

19. Taner MZ, Kurdoglu M, Taskiran C, et al. Early prenatal diagnosis of conjoined twins at 7 weeks and 6 days’ gestation with two-dimensional Doppler ultrasound: a case report. Cases J 2009; 22: 8330.

20. Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst 2004; 20: 508-525.
[Summary ]  [PDF]  [Mail to Editor ]  [Back]
Main Page | Editorial Staff | Editorial Board | About | Contents | Archive | Search | Instructions | Association | E-Mail