Skip to main content Skip to main navigation menu Skip to site footer

The pentalogy of cantrell in limited resources: A case report


Background: Pentalogy of Cantrell (PC) is a rare anomaly, and the incidence varies from 5.5 to 7.9 per million live births. The components are defined as a defect of the lower sternum, a midline supraumbilical thoracoabdominal wall defect, a deficiency of the diaphragmatic pericardium, a deficiency of the anterior diaphragm, and congenital cardiac anomalies. The pathognomonic characteristic of this syndrome is the coexistence of omphalocele and ectopia cordis. Diagnosis is possible by antenatal or physical examination, and other tests should be performed to detect other anomalies, including the heart's anomaly. Surgical treatment is complex, and various operative techniques often require a staged approach. The mortality rate is quite high, with a survival rate of less than 5%.

Case presentation: Two-day-old male infant was complained that his heart had been outside his chest wall since birth without covering by skin or sternum. He was born on aterm through section cesarean with a vigorous baby condition. Section cesarean was chosen because his mother has Human Immunodeficiency Virus (HIV)—no family history with similar symptoms. While pregnant, the mother regularly checks with the midwife and fetal ultrasonography only once during the third trimester but did not find the defect, no history of drug abuse, teratogen exposure, smoking, alcohol, or consanguinity. The midline defect extended from the lower part of the sternum completely. Echocardiography found defects such as classic Tetralogy of Fallot, mild atrial regurgitation, mild mitral regurgitation, mild tricuspid regurgitation, and suspect mass at left atrium (LA). The patients underwent surgery by covering the heart with a silo bag. Three days after the procedure, the patient's condition worsened and death because of the sepsis.

Conclusion: Prenatal diagnosis is important in guiding the surgical team to estimate the prognosis and reduce a postnatal management strategy. Early management can provide the basic care necessary to keep the patients until definitive management can be provided.


  1. Williams AP, Marayati R, Beierle EA. Pentalogy of Cantrell. Semin Pediatr Surg. 2019/04/09. 2019;28(2):106–10. Available from:
  2. Mărginean C, Mărginean CO, Gozar L, Meliţ LE, Suciu H, Gozar H, et al. Cantrell Syndrome-A Rare Complex Congenital Anomaly: A Case Report and Literature Review. Front Pediatr. 2018;6:201. Available from:
  3. Lack V, Oetzmann von Sochaczewski C, Naidoo K, Loveland J. Pentalogy of Cantrell with thoracoabdominal ectopia cordis: Attempted surgical correction and review of recent literature to aid prognostication prior to surgery. J Pediatr Surg Case Rep. 2015;3(11):476–80. Available from:
  4. Chandran S, Ari D. Pentalogy of cantrell: an extremely rare congenital anomaly. J Clin Neonatol. 2013;2(2):95–7. Available from:
  5. Zhang X, Xing Q, Sun J, Hou X, Kuang M, Zhang G. Surgical treatment and outcomes of pentalogy of Cantrell in eight patients. J Pediatr Surg. 2014;49(8):1335–40. Available from:
  6. Gao Z, Duan Q-J, Zhang Z-W, Ying L-Y, Ma L. Pentalogy of Cantrell Associated With Thoracoabdominal Ectopia Cordis. Circulation. 2009;119(15). Available from:
  7. Amato JJ, Zelen J, Talwalkar NG. Single-stage repair of thoracic ectopia cordis. Ann Thorac Surg. 1995;59(2):518–20. Available from:
  8. Hochberg J, Ardenghy MF, Gustafson RA, Murray GF. Repair of Thoracoabdominal Ectopia Cordis with Myocutaneous Flaps and Intraoperative Tissue Expansion. Plast Reconstr Surg. 1995;95(1):148–51. Available from:
  9. Hill DS. First-Trimester Ectopia Cordis in a Twin Gestation, Using 3D Surface Rendering and Reconstruction. Journal of Diagnostic Medical Sonography. 2005;21(5):420–3. Available from:
  10. Prieto LM, González-Tomé MI, Muñoz E, Fernández-Ibieta M, Soto B, Álvarez A, et al. Birth defects in a cohort of infants born to HIV-infected women in Spain, 2000-2009. BMC Infect Dis. 2014;14:700. Available from:
  11. Williams PL, Crain MJ, Yildirim C, Hazra R, Van Dyke RB, Rich K, et al. Congenital anomalies and in utero antiretroviral exposure in human immunodeficiency virus-exposed uninfected infants. JAMA Pediatr. 2015;169(1):48–55. Available from:
  12. Limanto DH, Soebroto H. Thoracic ectopia cordis: A case report. Jurnal Kedokteran dan Kesehatan Indonesia. 2020;11(1):99–104. Available from:

How to Cite

Maharini, K. ., Eka Gunawijaya, Yantie, N. P. V. K. ., Kardana, I. M. ., & Sudarma, W. . (2023). The pentalogy of cantrell in limited resources: A case report. Intisari Sains Medis, 14(2), 511–514.




Search Panel