Skip to main content

Acardia

  • Living reference work entry
  • First Online:
  • 634 Accesses

Abstract

Acardia is a bizarre fetal malformation occurring only in twins or triplets. It is also called acardius acephalus, acardiac twinning, or twin reversed arterial perfusion (TRAP) syndrome or sequence. This condition is very rare and occurs in 1 in 34,600 births or 1 in 100 monozygotic twins (Gillim and Hendricks 1953) and 1 in 30 monozygotic triplets (Van Allen et al. 1983; Napolitani and Schreiber 1960; Moore et al. 1990; Sanjaghsaz et al. 1998; Blenc et al. 1999) and even in quintuplets. Almost all cases reported are monozygotic twins. However, there are reports of dichorionic monozygotic twin gestations with TRAP sequence (French et al. 1998; Gewolb et al. 1983).

This is a preview of subscription content, log in via an institution.

References

  • Aggarwal, N., Suri, V., Saxena, S. V., et al. (2002). Acardiac acephalus twins: A case report and review of literature. Acta Obstetricia et Gynecologica Scandinavica, 81, 983–984.

    Article  PubMed  Google Scholar 

  • Alderman, B. (1973). Foetus acardius amorphous. Postgraduate Medical Journal, 49, 102–105.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  • Arias, F., Sunderji, S., Gimpelson, R., et al. (1998). Treatment of acardiac twinning. Obstetrics and Gynecology, 91, 818–821.

    Article  PubMed  CAS  Google Scholar 

  • Bebbington, M. W., Danzer, E., Moldenhauer, J., et al. (2012). Radiofrequency ablation vs bipolar umbilical cord coagulation in the management of complex monochorionic pregnancies. Ultrasound in Obstetrics and Gynecology, 40, 319–324.

    Article  PubMed  CAS  Google Scholar 

  • Benirschke, K., & des Roches Harper, V. (1977). The acardiac anomaly. Teratology, 15, 311–316.

    Article  PubMed  CAS  Google Scholar 

  • Blaicher, W., Repa, C., & Schaller, A. (2000). Acardiac twin pregnancy: Associated with trisomy 2. Human Reproduction, 15, 474–475.

    Article  PubMed  CAS  Google Scholar 

  • Blenc, A. M., Gömez, J. A., Collins, D., et al. (1999). Pathologic quiz case. Pathologic diagnosis: Acardiac fetus, acardius acephalus type. Archives of Pathology and Laboratory Medicine, 123, 974–976.

    PubMed  CAS  Google Scholar 

  • Bonilla-Musoles, F., Machado, L. E., Raga, F., et al. (2001). Fetus acardius. Two- and three-dimensional ultrasonographic diagnoses. Journal of Ultrasound in Medicine, 20, 1117–1127.

    PubMed  CAS  Google Scholar 

  • Bornstein, E., Monteagudo, A., Dong, R., et al. (2008). Detection of twin reversed arterial perfusion sequence at the time of first-trimester screening: The added value of 3-dimensional volume and color Doppler sonography. Journal of Ultrasound in Medicine, 27, 1105–1109.

    PubMed  Google Scholar 

  • Buyukkaya, A., Tekbas, G., & Buykkaya, R. (2015). Twin reversed arterial perfusion (TRAP) sequence: Characteristic gray-scale and Doppler ultrasonography findings. The Iranian Journal of Radiology, 12, e14979.

    Article  PubMed  Google Scholar 

  • Chen, H., Gonzalez, E., Hand, A. M., & Cuestas, R. (1983). The acardius acephalus and monozygotic twinning. Schumpert Medical Quarterly, 1, 195–199.

    Google Scholar 

  • Coulam, C. B. (1996). First trimester diagnosis of acardiac twins. Journal of Obstetrics and Gynaecology, 88, 729.

    Article  CAS  Google Scholar 

  • Donnenfeld, A. E., Van de Woestijne, J., Craparo, F., et al. (1991). The normal fetus of an acardiac twin pregnancy: Perinatal management based on echocardiographic and sonographic evaluation. Prenatal Diagnosis, 11, 235–244.

    Article  PubMed  CAS  Google Scholar 

  • French, C. A., Bieber, F. R., Bing, D. H., et al. (1998). Twins, placentas, and genetics: Acardiac twinning in a dichorionic, diamniotic, monozygotic twin gestation. Human Pathology, 29, 1028–1031.

    Article  PubMed  CAS  Google Scholar 

  • Gewolb, I. H., Freeman, R. M., Kleinman, C. S., et al. (1983). Prenatal diagnosis of a human pseudoacardiac anomaly. Obstetrics and Gynecology, 61, 657–662.

    PubMed  CAS  Google Scholar 

  • Gillim, D. L., & Hendricks, C. H. (1953). Holoacardius: Review of the literature and case report. The Obstetrician and Gynaecologist, 2, 647–652.

    CAS  Google Scholar 

  • Hanafy, A., & Peterson, C. M. (1997). Twin-reversed arterial perfusion (TRAP) sequence: Case reports and review of literature. The Australian and New Zealand Journal of Obstetrics and Gynaecology, 37, 187–191.

    Article  PubMed  CAS  Google Scholar 

  • Healey, M. G. (1994). Acardia: Predictive risk factors for the co-twin’s survival. Teratology, 50, 205–213.

    Article  PubMed  CAS  Google Scholar 

  • Hecher, K., Lew, L., Gratacos, E., et al. (2006). Twin reversed arterial perfusion: Fetoscopic laser coagulation of placental anastomoses or the umbilical cord. Ultrasound in Obstetrics and Gynecology, 28, 688–691.

    Article  PubMed  CAS  Google Scholar 

  • Ibne, A., Afshan, S. N., Mehtab, A., et al. (2013). Diagnostic dilemma in twin-reversed arterial perfusion sequence. Journal of Obstetrics & Gynaecology of India, 63, 282–284.

    Article  Google Scholar 

  • Langlotz, H., Sauerbrei, E., & Murray, S. (1991). Transvaginal Doppler sonographic diagnosis of an acardiac twin at 12 weeks gestation. Journal of Ultrasound in Medicine, 10, 175–179.

    PubMed  CAS  Google Scholar 

  • Martins, C. F., Santos, A. V., Miranda, M., et al. (2014). Twin reversed arterial perfusion (TRAP) sequence: An acardiac fetus. BMJ Case Reports, 11, 1–2.

    Article  Google Scholar 

  • Moore, T., Gale, S., & Benirschke, K. (1990). Perinatal outcome of forty-nine pregnancies complicated by acardiac twinning. American Journal of Obstetrics and Gynecology, 163, 907–912.

    Article  PubMed  CAS  Google Scholar 

  • Nakata, M., Sumie, M., Murata, S., et al. (2008). Fetoscopic laser photocoagulation of placental communicating vessels for twin-reversed arterial perfusion sequence. Journal of Obstetrics and Gynaecology Research, 34, 649–652.

    Article  PubMed  Google Scholar 

  • Napolitani, F. H., & Schreiber, I. (1960). The acardiac monster: A review of the world literature and presentation of two cases. American Journal of Obstetrics and Gynecology, 80, 582–589.

    PubMed  CAS  Google Scholar 

  • Nicolaidis, P., Nasrat, H., & Tannirandorn, Y. (1990). Review: Fetal acardia: Etiology, pathology and management. Journal of Obstetrics and Gynecology, 10, 518–525.

    Article  Google Scholar 

  • Obladen, M. (2010). From monster to twin reversed arterial perfusion: A history of acardiac twins. Journal of Perinatal Medicine, 38, 247–253.

    PubMed  Google Scholar 

  • Okai, T., Ichizuka, K., Hasegawa, J., et al. (2013). First successful case of non-invasive in-utero treatment of twin reversed arterial perfusion sequence by high-intensity focused ultrasound. Ultrasound in Obstetrics and Gynecology, 42, 112–114.

    Article  PubMed  CAS  Google Scholar 

  • Rehder, H., Schoner, K., & Kluge, B. (2012). Klinefelter twins presenting with discordant aneuploidies, acardia, forked umbilical cord and with different gonadal sex despite monozygosity. Prenatal Diagnosis, 32, 173–179.

    Article  PubMed  Google Scholar 

  • Sanjaghsaz, H., Bayram, M. O., & Qureshi, F. (1998). Twin reversed arterial perfusion sequence in conjoined, acardiac, acephalic twins associated with a normal triplet. A case report. The Journal of Reproductive Medicine, 43, 1046–1050.

    PubMed  CAS  Google Scholar 

  • Schwarzler, P., Ville, Y., Moscosco, G., et al. (1999). Diagnosis of twin reversed arterial perfusion sequence in the first trimester by transvaginal color Doppler ultrasound. Ultrasound in Obstetrics and Gynecology, 13, 143–146.

    Article  PubMed  CAS  Google Scholar 

  • Shalev, E., Zalel, Y., Ben-Ami, M., et al. (1992). First-trimester ultrasonic diagnosis of twin reversed arterial perfusion sequence. Prenatal Diagnosis, 12, 219–222.

    Article  PubMed  CAS  Google Scholar 

  • Sherer, D. M., Armstrong, B., Shah, Y. G., et al. (1989). Prenatal sonographic diagnosis. Doppler velocimetric umbilical cord studies and subsequent management of an acardiac twin pregnancy. The Obstetrician and Gynaecologist, 74, 472–475.

    CAS  Google Scholar 

  • Søgaard, K., Skibsted, L., & Brocks, V. (1999). Acardiac twins: Pathophysiology, diagnosis, outcome and treatment. Six cases and review of the literature. Fetal Diagnosis and Therapy, 14, 53–59.

    Article  PubMed  Google Scholar 

  • Steffensen, T. S., Gilbert-Barnes, E., Spellacy, W., et al. (2008). Placental pathology in TRAP sequence: Clinical and pathogenetic implications. Fetal and Pediatric Pathology, 27, 13–29.

    Article  PubMed  CAS  Google Scholar 

  • Stiller, R. J., Romero, R., Pace, S., et al. (1989). Prenatal identification of twin reversed arterial perfusion syndrome in the first trimester. American Journal of Obstetrics and Gynecology, 160, 1194–1196.

    Article  PubMed  CAS  Google Scholar 

  • Thelmo, M. L. C., Fok, R. Y., & Schertukde, S. P. (2007). Acardiac twin fetus with severe hydrops fetalis and bilateral talipes varus deformity. Fetal and Pediatric Pathology, 26, 235–242.

    Article  PubMed  Google Scholar 

  • Tsao, K., Feldstein, V. A., Albanese, C. T., et al. (2002). Selective reduction of acardiac twin by radiofrequency ablation. American Journal of Obstetrics and Gynecology, 187, 635–640.

    Article  PubMed  Google Scholar 

  • Van Allen, M. I., Smith, D. W., & Shepard, T. H. (1983). Twin reversed arterial perfusion (TRAP) sequence: A study of 14 twin pregnancies with acardius. Seminars in Perinatology, 7, 285–293.

    PubMed  Google Scholar 

  • Ville, Y., Hyett, J. A., Vandenbussche, F. P., et al. (1994). Endoscopic laser coagulation of umbilical cord vessels in twin reversed arterial perfusion sequence. Ultrasound in Obstetrics and Gynecology, 4, 396.

    Article  PubMed  CAS  Google Scholar 

  • Zucchini, S., Borghesani, F., Soffriti, G., et al. (1993). Transvaginal ultrasound diagnosis of twin reversed arterial perfusion syndrome at 9 weeks of gestation. Ultrasound in Obstetrics and Gynecology, 3, 209–211.

    Article  PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Harold Chen .

Rights and permissions

Reprints and permissions

Copyright information

© 2015 Springer Science+Business Media New York

About this entry

Cite this entry

Chen, H. (2015). Acardia. In: Atlas of Genetic Diagnosis and Counseling. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-6430-3_1-2

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-6430-3_1-2

  • Received:

  • Accepted:

  • Published:

  • Publisher Name: Springer, New York, NY

  • Online ISBN: 978-1-4614-6430-3

  • eBook Packages: Springer Reference Biomedicine and Life SciencesReference Module Biomedical and Life Sciences

Publish with us

Policies and ethics