Baby girl with pentalogy of Cantrell: a case report on an extremely rare condition

  • Widiastuti Soewondo Department of Radiology Dr. Moewardi Public Hospital, Sebelas Maret University, Surakarta, Indonesia
  • Suyono Suyono Department of Radiology Dr. Moewardi Public Hospital, Universitas Sebelas Maret, Surakarta, Central Java
  • Johannes Berchmans Prasodjo Department of Radiology Dr. Moewardi Public Hospital, Universitas Sebelas Maret, Surakarta, Central Java
  • Sri Lilijanti Widjaja Department of Child Health, Universitas Sebelas Maret Medical School/Dr. Moewardi Hospital, Surakarta, Central Java
Keywords: pentalogy of Cantrell; ectopia cordis; omphalocele

Abstract

In 1958, Cantrell et al. described an extremely rare syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart malformation.1 The incidence of pentalogy of Cantrell (POC) is one in 65,000 live births.1,2,3 Only one case was reported in Dr. Moewardi Public Hospital between 1999 and 2016. The exact etiology of this condition is unknown, but developmental failure of mesoderm at 4 weeks of gestational age may contribute to the condition.4 The prognosis depends on the degree of heart failure and the malformations that occur. Heart failure, arrhythmia, cardiac rupture, cardiac tamponade, endocarditis, and peripheral emboli are described as the main complications and causes of death.5,6 The aim of this report was to add to reference data about complete POC and the prognostic outcome.

References

Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet 1958;107:602-14. PMid:13592660

Kurt A, Kurt ANC, Şengül I, Yılmaz E, Doğan Y, Aygün D. Cantrell syndrome: a rare case report. J Neonatal Biol. 2016;5:1. https://doi.org/10.4172/2167-0897.1000212

Kaouthar H, Jihen A, Faten J, Hela M, Fatma O, et al. Cardiac anomalies in Cantrell's pentalogy: from ventricular diverticulum to complete thoracic ectopia cordis. Cardiol Tunis. 2013;9:94-7. PMid:25541632 PMCid:PMC4274934

Figueroa JR, Cruz EFS, Garcia LD, Daza DC. Cardiac malformations in patients with pentalogy of Cantrell and ectopia cordis. Rev Esp Cardiol. 2011;64:615-8. https://doi.org/10.1016/j.recesp.2010.07.010 PMid:21652132

Gao Z, Duan QJ, Zhang ZW, Ying LY, Ma LL. Images in cardiovascular medicine: pentalogy of Cantrell associated with thoracoabdominal ectopia cordis. Circulation. 2009;119:e483-5. https://doi.org/10.1161/CIRCULATIONAHA.108.811000 PMid:19380628

Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: a case report and review of the syndrome. Pediatrics. 1972;50:778-92. PMid:4263752

Çelik Y, Hallıoğlu O, Basut N, Demetgul H, Esin Kibar A. A rare case of cardiac anomaly: prenatally diagnosed ectopia cordis. Turk Pediatri Ars. 2015;50:129-31. https://doi.org/10.5152/tpa.2015.927 PMid:26265899 PMCid:PMC4523987

Khanna PC, Bharati A, Merchant SA. Pentalogy of cantrell or one of its variants. Appl Radiol. 2005;34:40-3.

Chandran S, Ari D. Pentalogy of Cantrell: an extremely rare congenital anomaly. J Clin Neonatol. 2013;2:95-7. https://doi.org/10.4103/2249-4847.116410 PMid:24049753 PMCid:PMC3775145

Hoorn JH, Moonen RM, Huysentruyt CJ, van Heurn WE, Offermans JP, Mulder AL. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr. 2008;167:29-35. https://doi.org/10.1007/s00431-007-0578-9 PMid:17674044 PMCid:PMC2668557

Vazquez-Jimenez JF, Muehler EG, Daebritz S, Keutel J, Nishigaki K, Huegel W, et al. Cantrell's syndrome: a challenge to the surgeon. Ann Thorac Surg.1998;65:1178-85. https://doi.org/10.1016/S0003-4975(98)00089-7

Published
2018-12-10
How to Cite
1.
Soewondo W, Suyono S, Prasodjo J, Widjaja S. Baby girl with pentalogy of Cantrell: a case report on an extremely rare condition. PI [Internet]. 10Dec.2018 [cited 29Mar.2024];59(1):51-. Available from: https://paediatricaindonesiana.org/index.php/paediatrica-indonesiana/article/view/1803
Section
Case Report
Received 2018-03-05
Accepted 2018-11-26
Published 2018-12-10