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Etiological aspects of double monsters
Europ. J. Obstet. Gynec. 0 Elsevier/North-Holland
CASE
reprod. Biol., 1980, Biomedical Press
10/5,
343-349
343
REPORTS
ETIOLOGICAL
ASPECTS OF DOUBLE MONSTERS
O.E. JASCHEVATZKY,
B. GOLDMAN,
Department of Obstetrics Hadera and Cytogenetics University, Tel Hashomer,
and Gynecoiogy, Institute, Sheba Israel
Accepted
29 November
for publication
D. KAMPF,
H. WEXLER
and S. GRUNSTEIN
Hillel Jaffe Memorial Government Medical Center and Medical School,
Hospital, Tel Aviv
1979
JASCHEVATZKY, O.E., GOLDMAN, B., KAMPF, D., WEXLER, H. and GRUNSTEIN, S. (1980): Etiological aspects of double monsters. Europ. J. Obstet. Gynec. reprod. Biol., 1 o/5, 343-349. Four cases of double monsters are reported, including a rare case of craniofacial duplication (diprosopus). Based on the findings observed, etiological factors of these malformations are discussed. We suggest that exogenous (environmental) factors such as habits, way of life or religious practices of certain populations can influence the development of double monsters. etiology
of double
monsters;
development;
chromosomal
study;
environmental
influence
INTRODUCTION
Double monsters have always been a subject of interest for researchers and physicians, on account of their rarity and the lack of information relating to etiological factors and the form of their development. The frequency of double monsters in large series in hospitals is approximately 1 in 40,000 births (Beischer and Fortune, 1968), but there are certain types of double monsters which are extremely rare, like diprosopus, of which only about 13 cases have been reported (Changaris and McGavran, 1976). Over a period of 15 years (1963-1978), there have been 63,229 deliveries in our department, and of the total of women who gave birth 54,944 were Jews (86.9%) and 8285 were Arabs (13.1%). Of these, 4 births were of double monsters (2 pairs of conjoined twins and 2 monsters with duplications); all 4 were of Arab mothers. The frequency of double monsters among the total births was 1 in 15,807 births, whereas among Arab mothers alone the frequency was 1 in 2071 births. We will now report on 4 cases of double monsters and discuss etiological aspects of these malformations.
344 CASE
REPORTS
Case 1 (Fig. 1)
A 20-yr-old female Arab, gravida 1, para 1, was admitted (October 1964) with signs of active labor at 36 wk gestation. During the pregnancy she had received no medication. There was no history of consanguinity or of any malformations in the family. Because of prolonged labor it was decided to deliver by cesarian section. Female live twins were delivered, joined at the thorax (Fig. l), with a total weight of 4100 g. The infants died immediately after delivery with signs of respiratory distress. External examination showed that the twins had a generally normal appearance apart from the fusion at the thorax. There was a common umbilical cord 50 cm in length.
Fig. 1. Twins joined at the thorax
(thoracopagus).
345
Case 2 A 24-yr-old female Arab, gravida 3, para 3, was admitted (May 1966) at 38 wk gestation with complete cervical dilatation. The woman had received no medication during pregnancy. There was no history of consanguinity. A short time after admission the body of an infant was born by breech delivery, but all attempts to extract the head failed. An abdominal examination revealed a large protuberance above the symphisis, and malformation of the head was suspected. A vaginal re-examination revealed two necks on one body. Decapitation was performed followed by manual extraction of the heads. The sex of the infant was male and its weight was 4200 g. External
Fig. 2. Diprosopus monster organs into thoracic cavity.
showing
craniofacial
duplication
and herniation
of abdominal
346
examination revealed one body with two normal necks to which were connected two normal heads. The single umbilical cord was 45 cm in length. Case 3 (Fig. 2) A 26-yr-old female Arab, gravida 4, para 4, was admitted (February 1973) in active phase of labor at 27 wk gestation. The woman had received no medication during pregnancy. An investigation of the family history of the couple revealed no consanguinity. A female infant weighing 1100 g was born by breech delivery after spontaneous labor. A short time after delivery the infant died. Examination revealed an anencephalic infant with facial duplication (diprosopus). The infant had a face with two noses, two mouths with a split lip and two chins (Fig. 2). Autopsy revealed herniation of the left lobe of the liver, the spleen, stomach and loops of the small intestine into the thoracic cavity (Fig. 2). Immediately after death of the infant a blood sam-
Fig. 3. Conjoined twins fused at the pelvis (pygopagus).
347
ple was taken via transcardiac puncture for a chromosomal female karyotypes were found in 23 cells of the culture.
analysis. Normal
Case 4 (Fig. 3) A 23-yr-old female Arab, gravida 1, para 1, was admitted (February 1976) in active phase of labor at 30 wk gestation. The woman became pregnant during clomifene treatment. Family history revealed that both her mother and grandmother had had identical twins. There was no evidence of consanguinity. Female conjoined twins fused at the pelvis were born by assisted breech delivery. Total weight was 1800 g. The infants developed respiratory distress and died a short time later. External examination of the infants revealed characteristic signs of conjoined twins of the pygopagus type (Fig. 3). Immediately on the twins’ death, blood samples were taken via transcardiac puncture and a skin sample was taken from the external common genitalia for a chromosomal study. In 30 cells of the blood culture and 23 cells of the skin culture, normal female karyotypes were found. DISCUSSION
Various factors regarding the development of double monsters were previously studied, including the genetic factor. In 2 of the cases reported where a cytogenetic study was performed, the karyotype was normal. In 4 previous reports, where cytogenetic studies were carried out in cases of conjoined twins (Kreutner et al., 1963; Ingalls and Bazemore, 1969; Kim et al., 1971; Bhettay et al., 1975), the karyotype was also found to be normal. On the basis of these findings it can be deduced that the development of malformations of this type is not related to abnormal karyotypes. However, it is a fact that a high frequency of monozygote twins has been observed in women who have given birth to double monsters (Kallen and Rybo, 1978), a fact which indicates that a genetic tendency exists in the development of monozygote twins, and therefore in double monsters, which are generally accepted as a representation of abnormal development of monozygote twins (Kim et al., 1971). In Case 4 of this series there was evidence of this phenomena. Bhettay et al. (1975) suggest the possibility of an epidemic incidence of conjoined twins. This theory is based on the findings of the abovementioned author, who found an increase in incidence of conjoined twins over a particular period of time in South Africa. This opinion was recently supported by KallCn and Rybo (1978). The theory of Bhettay et al. (1975) was rejected by Hanson (1975), who, in various statistical surveys all over the world, found a varying frequency among different reports, and in a certain series a much higher frequency than that found by Bhettay et al. (1975) in South Africa. In this report the cases were observed over a long period of time, affecting only a certain type of the population in the same region (i.e. Arab population); therefore an epidemic can not be claimed. In the present survey a high frequency of double monsters born of Arab mothers was observed, a finding also reported by El-Minawi et al. (1970).
348
Our obstetric population is composed of two groups, Arabs and Jews. Oriental Jews represent more than 50% of the total Jewish population; their ethnic background, folklore and areas of origin are very similar to those of the Arab population. The fundamental differences among the Arabs are certain habits (i.e. occupational, such as farm work done by the women); religious practices such as prolonged fasting during Ramadan (holy days to the Moslems); way of life (diet and community life). It remains a fact that no incidence of births of double monsters was observed among Oriental Jews, in spite of their similarities to Arabs. Two principal mechanisms were proposed in order to explain the development of double monsters (Aird, 1959). According to one of the mechanisms, double monsters could stem from incomplete fission of one embryonic area. According to Witschi (1934), the incomplete fission of the embryonic area could be due to aging of the maternal ovum, as occurs after delayed ovulation. A second possibility is that double monsters stem from partial fusion of two embryonic shields. Stockard (1921) has proved experimentally, in Fundulus and trout eggs, that influence of exogenous factors such as low temperature or low oxygen concentration can cause partial fusion of the two axes after arrest of the developmental rate. Similar results were obtained most recently by Ingalls et al. (1969) in zebra fish embryos after they had been submitted to moderate hyperthermia. Ferm (1969) experimentally developed conjoined twinning in the golden hamster after having submitted it to the influence of exogenous substances, i.e. vitamin A, dimethylsulfoxide and urethane. On the basis of the facts commented on above, we can suppose that there is an exogenous (environmental) factor which influences the development of double monsters. It can be related to habits, religious practices or way of life of certain populations with genetic tendency to develop such malformations. Periodic influence of an exogenous factor could be the reason why the appearance of double monsters is sometimes regarded as an epidemic phenomenon. REFERENCES Aird, I. (1959): Conjoined twins -further observations. &it. med. J., 1, 1313-1315. Be&her, N.A. and Fortune, D.W. (1968): Double monsters. Obstet. Gynec., 32, 158-170. Bhettay, E., Nelson, M.M. and Beighton, P. (1975): Epidemic of conjoined twins in Southern Africa. Lancet, 2, 741-743. Changaris, D.G. and McGavran, M.H. (1976): Craniofacial duplication (diprosopus) in a twin. Arch. Path. Lab. Med., 100, 392-394. Human El-Minawi, F.M., Shaaban, H., Naguib, Y., El-Sadek, M. and Shaaban, A. (1970): double monsters, management and radiologic observations. Znt. J. Gynaec. Obstet., 8, 648-652. Ferm, V.H. (1969): Conjoined &inning in mammalian teratology. Arch. environ. Hlth, 19, 353-357. Hanson, J.W. (1975): Incidence of conjoined twinning. Lancet, 2, 1257. Ingalls, T.H. and Bazemore, M.K. (1969): Prenatal events antedating the birth of thoracopagus twins. Arch. environ. Hlth, 19, 358-364.
349
Ingalls, T.H., Philbrook, F.R. and Majima, A. (1969): Conjoined twins in zebra fish, Arch. environ. Hlth, 19, 344-352. Kahen, B. and Rybo, G. (1978): Conjoined twinning in Sweden. Acta obstet. gynaec. stand.,
57,
257-259.
Kim, C.K., Barr, R.J. and Benirschke, K. (1971): Cytogenetic studies of conjoined twins, a case report. Obstet. Gynec., 38, 877~-881. Kreutner, A.K., Levine, J. and Thiede, H. (1963): A double truncus arteriosus in thoracopagus twins. New Engl. J. Med., 268, 1388-1390. Stockard, C.R. (1921): Developmental rate and structural expression: an experimental study of twins, ‘double monsters’ and single deformities and the interaction among embryonic organs during their origin and development. Amer. J. Anat., 28, 115~-277. Witschi, E. (1934): Appearance of accessory ‘organizers’ in overripe eggs of the frog. Proc. Sot. exp. Biol. Med., 31, 419-420.
CASE
reprod. Biol., 1980, Biomedical Press
10/5,
343-349
343
REPORTS
ETIOLOGICAL
ASPECTS OF DOUBLE MONSTERS
O.E. JASCHEVATZKY,
B. GOLDMAN,
Department of Obstetrics Hadera and Cytogenetics University, Tel Hashomer,
and Gynecoiogy, Institute, Sheba Israel
Accepted
29 November
for publication
D. KAMPF,
H. WEXLER
and S. GRUNSTEIN
Hillel Jaffe Memorial Government Medical Center and Medical School,
Hospital, Tel Aviv
1979
JASCHEVATZKY, O.E., GOLDMAN, B., KAMPF, D., WEXLER, H. and GRUNSTEIN, S. (1980): Etiological aspects of double monsters. Europ. J. Obstet. Gynec. reprod. Biol., 1 o/5, 343-349. Four cases of double monsters are reported, including a rare case of craniofacial duplication (diprosopus). Based on the findings observed, etiological factors of these malformations are discussed. We suggest that exogenous (environmental) factors such as habits, way of life or religious practices of certain populations can influence the development of double monsters. etiology
of double
monsters;
development;
chromosomal
study;
environmental
influence
INTRODUCTION
Double monsters have always been a subject of interest for researchers and physicians, on account of their rarity and the lack of information relating to etiological factors and the form of their development. The frequency of double monsters in large series in hospitals is approximately 1 in 40,000 births (Beischer and Fortune, 1968), but there are certain types of double monsters which are extremely rare, like diprosopus, of which only about 13 cases have been reported (Changaris and McGavran, 1976). Over a period of 15 years (1963-1978), there have been 63,229 deliveries in our department, and of the total of women who gave birth 54,944 were Jews (86.9%) and 8285 were Arabs (13.1%). Of these, 4 births were of double monsters (2 pairs of conjoined twins and 2 monsters with duplications); all 4 were of Arab mothers. The frequency of double monsters among the total births was 1 in 15,807 births, whereas among Arab mothers alone the frequency was 1 in 2071 births. We will now report on 4 cases of double monsters and discuss etiological aspects of these malformations.
344 CASE
REPORTS
Case 1 (Fig. 1)
A 20-yr-old female Arab, gravida 1, para 1, was admitted (October 1964) with signs of active labor at 36 wk gestation. During the pregnancy she had received no medication. There was no history of consanguinity or of any malformations in the family. Because of prolonged labor it was decided to deliver by cesarian section. Female live twins were delivered, joined at the thorax (Fig. l), with a total weight of 4100 g. The infants died immediately after delivery with signs of respiratory distress. External examination showed that the twins had a generally normal appearance apart from the fusion at the thorax. There was a common umbilical cord 50 cm in length.
Fig. 1. Twins joined at the thorax
(thoracopagus).
345
Case 2 A 24-yr-old female Arab, gravida 3, para 3, was admitted (May 1966) at 38 wk gestation with complete cervical dilatation. The woman had received no medication during pregnancy. There was no history of consanguinity. A short time after admission the body of an infant was born by breech delivery, but all attempts to extract the head failed. An abdominal examination revealed a large protuberance above the symphisis, and malformation of the head was suspected. A vaginal re-examination revealed two necks on one body. Decapitation was performed followed by manual extraction of the heads. The sex of the infant was male and its weight was 4200 g. External
Fig. 2. Diprosopus monster organs into thoracic cavity.
showing
craniofacial
duplication
and herniation
of abdominal
346
examination revealed one body with two normal necks to which were connected two normal heads. The single umbilical cord was 45 cm in length. Case 3 (Fig. 2) A 26-yr-old female Arab, gravida 4, para 4, was admitted (February 1973) in active phase of labor at 27 wk gestation. The woman had received no medication during pregnancy. An investigation of the family history of the couple revealed no consanguinity. A female infant weighing 1100 g was born by breech delivery after spontaneous labor. A short time after delivery the infant died. Examination revealed an anencephalic infant with facial duplication (diprosopus). The infant had a face with two noses, two mouths with a split lip and two chins (Fig. 2). Autopsy revealed herniation of the left lobe of the liver, the spleen, stomach and loops of the small intestine into the thoracic cavity (Fig. 2). Immediately after death of the infant a blood sam-
Fig. 3. Conjoined twins fused at the pelvis (pygopagus).
347
ple was taken via transcardiac puncture for a chromosomal female karyotypes were found in 23 cells of the culture.
analysis. Normal
Case 4 (Fig. 3) A 23-yr-old female Arab, gravida 1, para 1, was admitted (February 1976) in active phase of labor at 30 wk gestation. The woman became pregnant during clomifene treatment. Family history revealed that both her mother and grandmother had had identical twins. There was no evidence of consanguinity. Female conjoined twins fused at the pelvis were born by assisted breech delivery. Total weight was 1800 g. The infants developed respiratory distress and died a short time later. External examination of the infants revealed characteristic signs of conjoined twins of the pygopagus type (Fig. 3). Immediately on the twins’ death, blood samples were taken via transcardiac puncture and a skin sample was taken from the external common genitalia for a chromosomal study. In 30 cells of the blood culture and 23 cells of the skin culture, normal female karyotypes were found. DISCUSSION
Various factors regarding the development of double monsters were previously studied, including the genetic factor. In 2 of the cases reported where a cytogenetic study was performed, the karyotype was normal. In 4 previous reports, where cytogenetic studies were carried out in cases of conjoined twins (Kreutner et al., 1963; Ingalls and Bazemore, 1969; Kim et al., 1971; Bhettay et al., 1975), the karyotype was also found to be normal. On the basis of these findings it can be deduced that the development of malformations of this type is not related to abnormal karyotypes. However, it is a fact that a high frequency of monozygote twins has been observed in women who have given birth to double monsters (Kallen and Rybo, 1978), a fact which indicates that a genetic tendency exists in the development of monozygote twins, and therefore in double monsters, which are generally accepted as a representation of abnormal development of monozygote twins (Kim et al., 1971). In Case 4 of this series there was evidence of this phenomena. Bhettay et al. (1975) suggest the possibility of an epidemic incidence of conjoined twins. This theory is based on the findings of the abovementioned author, who found an increase in incidence of conjoined twins over a particular period of time in South Africa. This opinion was recently supported by KallCn and Rybo (1978). The theory of Bhettay et al. (1975) was rejected by Hanson (1975), who, in various statistical surveys all over the world, found a varying frequency among different reports, and in a certain series a much higher frequency than that found by Bhettay et al. (1975) in South Africa. In this report the cases were observed over a long period of time, affecting only a certain type of the population in the same region (i.e. Arab population); therefore an epidemic can not be claimed. In the present survey a high frequency of double monsters born of Arab mothers was observed, a finding also reported by El-Minawi et al. (1970).
348
Our obstetric population is composed of two groups, Arabs and Jews. Oriental Jews represent more than 50% of the total Jewish population; their ethnic background, folklore and areas of origin are very similar to those of the Arab population. The fundamental differences among the Arabs are certain habits (i.e. occupational, such as farm work done by the women); religious practices such as prolonged fasting during Ramadan (holy days to the Moslems); way of life (diet and community life). It remains a fact that no incidence of births of double monsters was observed among Oriental Jews, in spite of their similarities to Arabs. Two principal mechanisms were proposed in order to explain the development of double monsters (Aird, 1959). According to one of the mechanisms, double monsters could stem from incomplete fission of one embryonic area. According to Witschi (1934), the incomplete fission of the embryonic area could be due to aging of the maternal ovum, as occurs after delayed ovulation. A second possibility is that double monsters stem from partial fusion of two embryonic shields. Stockard (1921) has proved experimentally, in Fundulus and trout eggs, that influence of exogenous factors such as low temperature or low oxygen concentration can cause partial fusion of the two axes after arrest of the developmental rate. Similar results were obtained most recently by Ingalls et al. (1969) in zebra fish embryos after they had been submitted to moderate hyperthermia. Ferm (1969) experimentally developed conjoined twinning in the golden hamster after having submitted it to the influence of exogenous substances, i.e. vitamin A, dimethylsulfoxide and urethane. On the basis of the facts commented on above, we can suppose that there is an exogenous (environmental) factor which influences the development of double monsters. It can be related to habits, religious practices or way of life of certain populations with genetic tendency to develop such malformations. Periodic influence of an exogenous factor could be the reason why the appearance of double monsters is sometimes regarded as an epidemic phenomenon. REFERENCES Aird, I. (1959): Conjoined twins -further observations. &it. med. J., 1, 1313-1315. Be&her, N.A. and Fortune, D.W. (1968): Double monsters. Obstet. Gynec., 32, 158-170. Bhettay, E., Nelson, M.M. and Beighton, P. (1975): Epidemic of conjoined twins in Southern Africa. Lancet, 2, 741-743. Changaris, D.G. and McGavran, M.H. (1976): Craniofacial duplication (diprosopus) in a twin. Arch. Path. Lab. Med., 100, 392-394. Human El-Minawi, F.M., Shaaban, H., Naguib, Y., El-Sadek, M. and Shaaban, A. (1970): double monsters, management and radiologic observations. Znt. J. Gynaec. Obstet., 8, 648-652. Ferm, V.H. (1969): Conjoined &inning in mammalian teratology. Arch. environ. Hlth, 19, 353-357. Hanson, J.W. (1975): Incidence of conjoined twinning. Lancet, 2, 1257. Ingalls, T.H. and Bazemore, M.K. (1969): Prenatal events antedating the birth of thoracopagus twins. Arch. environ. Hlth, 19, 358-364.
349
Ingalls, T.H., Philbrook, F.R. and Majima, A. (1969): Conjoined twins in zebra fish, Arch. environ. Hlth, 19, 344-352. Kahen, B. and Rybo, G. (1978): Conjoined twinning in Sweden. Acta obstet. gynaec. stand.,
57,
257-259.
Kim, C.K., Barr, R.J. and Benirschke, K. (1971): Cytogenetic studies of conjoined twins, a case report. Obstet. Gynec., 38, 877~-881. Kreutner, A.K., Levine, J. and Thiede, H. (1963): A double truncus arteriosus in thoracopagus twins. New Engl. J. Med., 268, 1388-1390. Stockard, C.R. (1921): Developmental rate and structural expression: an experimental study of twins, ‘double monsters’ and single deformities and the interaction among embryonic organs during their origin and development. Amer. J. Anat., 28, 115~-277. Witschi, E. (1934): Appearance of accessory ‘organizers’ in overripe eggs of the frog. Proc. Sot. exp. Biol. Med., 31, 419-420.