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Acardiac Twin with Externalized Intestine Adherent to Placenta: Unusual Manifestation of Omphalocele

  • Case Report
  • Published:
Pediatric and Developmental Pathology

Abstract

TRAP (twin reversed arterial perfusion) syndrome produces an acardiac twin (acardiac monster, acardius, acardiacus, chorioangiopagus parasiticus, etc.). Acardiacs result from monozygotic multiple births in which three anatomic anomalies occur: (1) a fetus’ cardiac development is disturbed; (2) artery–artery anastomosis carries blood from a normal (“pump”) twin to the acardiac; (3) vein–vein anastomosis carries blood from the acardiac back to the normal twin. Whether reversal of blood flow in the acardiac results from or causes cardiac dysmorphogenesis has not been resolved. Acardiac twins demonstrate a complex constellation of malformations usually thought to result from reversed blood flow; omphalocele is particularly common. We report monochorionic monoamnionic male twins in which an acardiac twin demonstrated externalized intestines adherent to the placenta. The twins were delivered from a 30-year-old primigravida mother by cesarean section without maternal complications at 33 w. The mother has no significant past medical history. The macerated acardius had a 4-cm long attenuated umbilical cord with indeterminate number of vessels. Structures rostral to the thorax were absent save for one poorly developed hand and arm. The abdomen contained loose mesenchyme and no organs. The entire intestine (21 cm) along with two testes was located in a sac on the surface of the placenta. No histopathologic anomalies of formed structures were identified aside from spatial relationships and incomplete development. The normal twin required no intensive care and is doing well. To our knowledge, this is the first report of externalized intestine, which may represent an unusual consequence of omphalocele.

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Acknowledgments

We thank Dr. Kenneth Lyons Jones and Dr. Frank Mannino for their enthusiastic clinical correlations and perspectives.

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Authors and Affiliations

  1. Department of Pathology, Autopsy Service, University of California San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103-8321, USA

    Shawn Clark Emery, Eliezer Masliah & Kurt Benirschke

  2. Department of Pediatrics, University of California San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103, USA

    Keith K. Vaux

  3. Department of Radiology, University of California San Diego Medical Center, 200 West Arbor Drive, San Diego, CA 92103, USA

    Dolores Pretorius

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  1. Shawn Clark Emery
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  2. Keith K. Vaux
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  3. Dolores Pretorius
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  4. Eliezer Masliah
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  5. Kurt Benirschke
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Correspondence to Kurt Benirschke.

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Emery, S., Vaux, K., Pretorius, D. et al. Acardiac Twin with Externalized Intestine Adherent to Placenta: Unusual Manifestation of Omphalocele . Pediatr. Dev. Pathol. 7, 81–85 (2004). https://doi.org/10.1007/s10024-003-5052-8

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  • DOI: https://doi.org/10.1007/s10024-003-5052-8

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