Skip to main content
Top
Published in: Journal of Medical Case Reports 1/2020

Open Access 01-12-2020 | Case report

A parapagus dicephalus tripus tribrachius conjoined twin with a unique morphological pattern: a case report

Authors: Jeske F. M. Bovendeert, Rutger A. J. Nievelstein, Ronald L. A. W. Bleys, Cindy G. J. Cleypool

Published in: Journal of Medical Case Reports | Issue 1/2020

Login to get access

Abstract

Background

Conjoined twinning is a rare congenital malformation with an incidence of about 1.5 per 100,000 births. Because no consensus has been reached regarding the dysmorphology, thorough descriptions of conjoined twins as part of teratological collections can be useful to increase knowledge of this congenital malformation. In this case report, we describe a parapagus dicephalus twin from the collection of the Department of Anatomy of the University Medical Center Utrecht in the Netherlands. External anatomical characteristics were assessed through a detailed macroscopic examination and internal characteristics by means of whole-body computed tomography and magnetic resonance imaging (3 Tesla).

Case presentation

Macroscopic examination showed a Caucasian male parapagus dicephalus tripus tribrachius conjoined twin a type of conjoined twinning in which there are two heads side by side, one rump, and three upper and three lower limbs. In addition, anencephaly was observed in the left twin. Radiological imaging showed a normal central nervous system in the right twin and absence of the calvaria, cerebrum, diencephalon, and mesencephalon in the left twin. There was clear duplication of the vertebral column, rib cage, respiratory system, and gastrointestinal system at least up to and including the first part of the duodenum. The heart consisted of a monoatrium with two separate ventricles. There was a fused liver with a single gallbladder, a single spleen, three kidneys, two bladders, and duplication of the penis. The third upper and lower extremities were articulating with a fused glenoid and acetabulum, respectively. The third foot showed both polydactyly and syndactyly of the toes.

Conclusion

This case report describes a unique case of a male dicephalus parapagus tripus tribrachus conjoined twin discordant for anencephaly. Radiological imaging proved to be an adequate noninvasive method to provide insights into the internal (dys)morphology of this specific specimen, improving its scientific and educational value. This approach could be generally applied to other teratological specimens, thereby strengthening arguments regarding pathogenetic hypotheses, which may lead to new or improved insights into both normal and abnormal embryonic development.
Literature
1.
go back to reference Boer LL, Van Asten JJA, Bosboom DGH, et al. Radiological imaging of teratological fetuses: what can we learn? Insights Imaging. 2017;8(3):301–10.CrossRef Boer LL, Van Asten JJA, Bosboom DGH, et al. Radiological imaging of teratological fetuses: what can we learn? Insights Imaging. 2017;8(3):301–10.CrossRef
2.
go back to reference Mutchnick OM, Luna-Muñoz 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. 2015;157C(4):274–87. Mutchnick OM, Luna-Muñoz 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. 2015;157C(4):274–87.
3.
go back to reference Spitz L, Kiely EM. Experience in the management of conjoined twins. Br J Surg. 2002;89(9):1188–92.CrossRef Spitz L, Kiely EM. Experience in the management of conjoined twins. Br J Surg. 2002;89(9):1188–92.CrossRef
4.
go back to reference Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst. 2004;20(8–9):508–25.PubMed Kaufman MH. The embryology of conjoined twins. Childs Nerv Syst. 2004;20(8–9):508–25.PubMed
5.
go back to reference Spencer R. Theoretical and analytical embryology of conjoined twins: part I: embryogenesis. Clin Anat. 2000;13(1):36–53.CrossRef Spencer R. Theoretical and analytical embryology of conjoined twins: part I: embryogenesis. Clin Anat. 2000;13(1):36–53.CrossRef
6.
go back to reference Papageorghiou AT, Ohuma EO, Altman DG, et al. International standards for fetal growth based on serial ultrasound measurements: the Fetal Growth Longitudinal Study of the INTERGROWTH-21st Project. Lancet. 2014;384(9946):869–79.CrossRef Papageorghiou AT, Ohuma EO, Altman DG, et al. International standards for fetal growth based on serial ultrasound measurements: the Fetal Growth Longitudinal Study of the INTERGROWTH-21st Project. Lancet. 2014;384(9946):869–79.CrossRef
7.
go back to reference Guihard-Costa AM, Menez F, Delezoide AL. Standards for dysmorphological diagnosis in human fetuses. Pediatr Dev Pathol. 2003;6(5):427–34.CrossRef Guihard-Costa AM, Menez F, Delezoide AL. Standards for dysmorphological diagnosis in human fetuses. Pediatr Dev Pathol. 2003;6(5):427–34.CrossRef
8.
go back to reference Maroun LL, Graem N. Autopsy standards of body parameters and fresh organ weights in nonmacerated and macerated human fetuses. Pediatr Dev Pathol. 2005;8(2):204–17.CrossRef Maroun LL, Graem N. Autopsy standards of body parameters and fresh organ weights in nonmacerated and macerated human fetuses. Pediatr Dev Pathol. 2005;8(2):204–17.CrossRef
9.
go back to reference Spencer R. Theoretical and analytical embryology of conjoined twins: part II: adjustments to union. Clin Anat. 2000;13(2):97–120.CrossRef Spencer R. Theoretical and analytical embryology of conjoined twins: part II: adjustments to union. Clin Anat. 2000;13(2):97–120.CrossRef
10.
go back to reference Chatkupt S, Chatkupt S, Kohut G, et al. Antepartum diagnosis of discordant anencephaly in dicephalic conjoined twins. J Clin Ultrasound. 1993;21:138–42.CrossRef Chatkupt S, Chatkupt S, Kohut G, et al. Antepartum diagnosis of discordant anencephaly in dicephalic conjoined twins. J Clin Ultrasound. 1993;21:138–42.CrossRef
11.
go back to reference Usang UE, Olasode BJ, Archibong AE, et al. Dicephalus parapagus conjoined twins discordant for anencephaly: a case report. J Med Case Rep. 2010;4:38.CrossRef Usang UE, Olasode BJ, Archibong AE, et al. Dicephalus parapagus conjoined twins discordant for anencephaly: a case report. J Med Case Rep. 2010;4:38.CrossRef
12.
go back to reference Ülker K, Akyer ŞP, Temur Ǐ, et al. First trimester diagnosis of parapagus diprosopus dibrachius dipus twins with cranirachischisis totalis by three-dimensional ultrasound. J Obstet Gynaecol Res. 2012;38(2):431–4.CrossRef Ülker K, Akyer ŞP, Temur Ǐ, et al. First trimester diagnosis of parapagus diprosopus dibrachius dipus twins with cranirachischisis totalis by three-dimensional ultrasound. J Obstet Gynaecol Res. 2012;38(2):431–4.CrossRef
13.
go back to reference Yang PY, Wu CH, Yeh GP, et al. Prenatal diagnosis of parapagus diprosopus dibrachius dipus twins with spina bifida in the first trimester using two- and three-dimensional ultrasound. Taiwan J Obstet Gynecol. 2015;54(6):780–3.CrossRef Yang PY, Wu CH, Yeh GP, et al. Prenatal diagnosis of parapagus diprosopus dibrachius dipus twins with spina bifida in the first trimester using two- and three-dimensional ultrasound. Taiwan J Obstet Gynecol. 2015;54(6):780–3.CrossRef
14.
go back to reference Itoh K, Imai Y, Obayashi C, et al. Pathological findings in dicephalus dipus dibrachius: implications for mechanisms in two pairs of lateral conjoined twins. Kobe J Med Sci. 1993;39(3):95–106.PubMed Itoh K, Imai Y, Obayashi C, et al. Pathological findings in dicephalus dipus dibrachius: implications for mechanisms in two pairs of lateral conjoined twins. Kobe J Med Sci. 1993;39(3):95–106.PubMed
15.
go back to reference Schmidt W, Heberling D, Kubli F. Antepartum ultrasonographic diagnosis of conjoined twins in early pregnancy. Am J Obstet Gynecol. 1981;139(8):961–3.CrossRef Schmidt W, Heberling D, Kubli F. Antepartum ultrasonographic diagnosis of conjoined twins in early pregnancy. Am J Obstet Gynecol. 1981;139(8):961–3.CrossRef
16.
go back to reference Levin M, Roberts DJ, Holmes LB, et al. Laterality defects in conjoined twins. Nature. 1996;384(6607):321.CrossRef Levin M, Roberts DJ, Holmes LB, et al. Laterality defects in conjoined twins. Nature. 1996;384(6607):321.CrossRef
17.
go back to reference Yamaguchi Y, Miyazawa H, Miura M. Neural tube closure and embryonic metabolism 1. Congenit Anom (Kyoto). 2017;57(5):134–7.CrossRef Yamaguchi Y, Miyazawa H, Miura M. Neural tube closure and embryonic metabolism 1. Congenit Anom (Kyoto). 2017;57(5):134–7.CrossRef
18.
go back to reference Journel H, Parent P, Leguern H, Roussey M, Mulliez N, Toudic L, et al. Anencephaly and diprosopy: 2 cases. J Gynecol Obstet Biol Reprod. 1986;15(8):1075–80. Journel H, Parent P, Leguern H, Roussey M, Mulliez N, Toudic L, et al. Anencephaly and diprosopy: 2 cases. J Gynecol Obstet Biol Reprod. 1986;15(8):1075–80.
19.
go back to reference Sieswerda-Hoogendoorn S, Van Rijn RR. Current techniques in postmortem imaging with specific attention to paediatric applications. Pediatr Radiol. 2010;40(2):141–52.CrossRef Sieswerda-Hoogendoorn S, Van Rijn RR. Current techniques in postmortem imaging with specific attention to paediatric applications. Pediatr Radiol. 2010;40(2):141–52.CrossRef
20.
go back to reference Cohen MC, Paley MN, Griffiths PD, et al. Less invasive autopsy: benefits and limitations of the use of magnetic resonance imaging in the perinatal postmortem. Pediatr Dev Pathol. 2008;11(1):1–9.CrossRef Cohen MC, Paley MN, Griffiths PD, et al. Less invasive autopsy: benefits and limitations of the use of magnetic resonance imaging in the perinatal postmortem. Pediatr Dev Pathol. 2008;11(1):1–9.CrossRef
Metadata
Title
A parapagus dicephalus tripus tribrachius conjoined twin with a unique morphological pattern: a case report
Authors
Jeske F. M. Bovendeert
Rutger A. J. Nievelstein
Ronald L. A. W. Bleys
Cindy G. J. Cleypool
Publication date
01-12-2020
Publisher
BioMed Central
Published in
Journal of Medical Case Reports / Issue 1/2020
Electronic ISSN: 1752-1947
DOI
https://doi.org/10.1186/s13256-020-02501-x

Other articles of this Issue 1/2020

Journal of Medical Case Reports 1/2020 Go to the issue