Abstract
The pentalogy of Cantrell is a rare midline developmental field complex often with a dismal outcome. We present a newborn qualifying for Cantrell’s pentalogy with an abdominal wall defect, a diaphragmatic defect and a heart defect, a ventricular septal defect (VSD) and a left ventricular diverticulum. Early operative correction following accurate diagnostic work-up was prompted at the age of 11 weeks because of progressive heart failure. Beside correction of the VSD and the abdominal and diaphragmatic defects, resection of the distal part of the diverticulum was possible without damage to the LAD and preserving good contractility. Two years postoperative follow-up is uneventful.
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Cantrell JR, Haller JA, Ravitch MM (1958) A syndrome of congenital defect involving the abominal wall, sternum, diaphragm, pericardium and heart. Surg Gynecol Obstet 107:602–614
Carmi R, Boughman JA (1992) Pentalogy of Cantrell and associated midline anomalies; a possible ventral mindline developmental field. Am J Med Genet 42:90–95
Ghidini A, Sirtori M, Romero R, Hobbins JC (1988) Prenatal diagnosis of pentalogy of Cantrell. J Ultrasound Med 7:567–772
Haller JA, Cantrell JR (1966) Diagnosis and surgical correction of combined congenital defects of supra-umbilical abal wall, lower sternum and diaphragm. J Thorac Cardiovasc Surg 51:286–291
Martin RA, Cuniff C, Erickson L, Jones KL (1992) Pentalogy of Cantrell and ectopia cordis, a familial developmental field complex. Am J Med Genet 42:839–841
Morales JM, Patel SG, Duff JA, Villareal RL, Simpson JW (2000) Ectopia cordis and other midline defects. Ann Thorac Surg 70:111–114
Tabbers MM, Veer van der EMA, Lam J, Van der Horst CCAM, Heenekam RCM (2002) Een pasgeborene met pentalogie van Cantrell en frontonasale dysplasie. Tijdschr Kindergeneeskd 70:164–169
Vanamo K, Sairanen H, Louhimo I (1991) The spectrum of Cantrell’s syndrome. Pediatr Surg Int 6:429–433
Vasquez-Jimenez JF, Muehler EG, Daebritz S, Keutel J, Nishigaki K, Huegel W, Messmer BJ (1998) Cantrell’s syndrome; a challenge to the surgeon. Ann Thorac Surg 65:1178–1185
Acknowledgments
We thank F.J.A. Beek, M.D., Ph.D., radiologist and W.A. Avis, M.D., pediatrician, for their substantial support.
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Korver, A.M.H., Haas, F., Freund, M.W. et al. Pentalogy of Cantrell. Pediatr Cardiol 29, 146–149 (2008). https://doi.org/10.1007/s00246-007-9032-z
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DOI: https://doi.org/10.1007/s00246-007-9032-z