Seminars in Pediatric Surgery
Volume 17, Issue 3 , Pages 154-160 , August 2008

Embryology, sternal clefts, ectopia cordis, and Cantrell's pentalogy

  • Scott A. Engum, MD

      Affiliations

    • Corresponding Author InformationAddress reprint requests and correspondence: Scott A. Engum, MD, Division of Pediatric Surgery, James Whitcomb Riley Hospital for Children, 702 Barnhill Drive, Suite 2500, Indianapolis, IN 46202.

References 

  1. Shamberger RC, Welch KJ. Sternal defects. Paediatr Surg Int. 1990;5:156–164
  2. Shao-tsu L. Ectopia cordis congenital. Thoraxchirurgie. 1957;5:197–212
  3. Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. Surg Gynecol Obstet. 1958;107:602–614
  4. Ravitch MM. Congenital Deformities of the Chest Wall and Their Operative Correction. Philadelphia, PA: WB Saunders; 1977;
  5. Shamberer R. Congenital chest wall deformities. Curr Probl Surg. 1996;33:517–529
  6. Shamberger R. The chest wall. In:  Shields T editors. General Thoracic Surgery. (ed 4). Baltimore, MD: Williams & Wilkins; 1994;p. 529–557
  7. Ruge G. Untersuchungen über Entwicklungsvorgänge am Brustbein und der Sternoclavicularverbindung der Menschen. Morphol Jahrb. 1880;6:362–414
  8. Paterson AM. The sternum: its early development and ossification in man and mammals. J Anat Physiol. 1900;35:21–32
  9. Chen JM. Studies on the morphogenesis of the mouse sternum. I. Normal embryonic development (II. Experiments on the origin of the sternum and its capacity for self-differentiation in vitro). J Anat. 1952;86:373–401
  10. Skindalakis JE, Gray SW, Ricketts R, et al. The anterior body wall. In:  Skindalakis JE,  Gray SW editor. Embryology for Surgeons (Chapter 16). Baltimore, MD: Williams and Wilkins; 1994;p. 540–593
  11. Greenberg BM, Becker JM, Pletcher BA. Congenital bifid sternum: repair in early infancy and literature review. Plast Reconstr Surg. 1991;88:886–889
  12. Chappius JP, Takuorian P, Philibert M, et al. A propos de deux cas de fissure sternale congenitale. Pédiatrie. 1984;39:195
  13. Acastello E, Majluf R, Garrido P, et al. Sternal cleft: a surgical opportunity. J Paediatr Surg. 2003;38:178–183
  14. Forzano F, Daubeney PEF, White SM. Midline raphe, sternal cleft and other midline abnormalities: a new dominant syndrome?. Am J Med Genet. 2005;135A:9–12
  15. Kotzot D, Huk W, Pfeiffer RA. Genetic counseling of sternal cleft. Genet Couns. 1994;5:147–150
  16. O'Neal M, Dwornik J, Ganey T, et al. Postnatal development of the human sternum. J Pediatric Orthop. 1998;18:398–405
  17. Hanson FB. The ontogeny of the sternum. Am J Anat. 1919;26:41
  18. Twomey EL, Moore AM, Ein S, et al. Prenatal ultrasounography and neonatal imaging of complete cleft sternum: a case report. Ultrasound Obstet Gynecol. 2005;25:599–601
  19. Herman TE, Siegel M. Superior congenital sternal cleft. J Perinatol. 2001;21:334–335
  20. Freiden IJ, Reese V, Cohen D. PHACE syndrome (The association of posterior fossa brain malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities). Arch Dermatol. 1996;132:307–311
  21. James PA, McGaughran J. Complete overlap of PHACE syndrome and sternal malformation-vascular dysplasia association. Am J Med Genet. 2002;110:78–84
  22. Spitz L, Bloom R, Milner S, et al. Combined anterior abdominal wall, sternal, diaphragmatic, pericardial, and intracardiac defects: a report of five cases and their management. J Pediatr Surg. 1975;10:491–496
  23. Firmin RK, Fragomeni LS, Lennox SC. Complete cleft sternum. Thorax. 1980;35:303–306
  24. Hebra A, Davidoff A, O'Neill J. Neonatal sternal cleft associated with an extrathoracic cystic mass. J Pediatr Surg. 1997;32:627–630
  25. Suri RK, Sharma RK, Jha NK, et al. Complete congenital sternal cleft in an adult: repair by autogenous tissues. Ann Thorac Surg. 1996;62:573–575
  26. Mathai J, Cherian VK, Chacko J, et al. Bridging the cleft over the throbbing heart. Ann Thorac Surg. 2006;82:2310–2311
  27. Abel RM, Robinson M, Gibbons P, et al. Cleft sternum: case report and literature review. Pediatr Pulmonol. 2004;37:375–377
  28. Maier HC, Bortone F. Complete failure of sternal fusion with herniation of perdicardium (Report of a case corrected surgically in infancy). J Thorac Surg. 1949;18:851–859
  29. Salley RK, Stewart S. Superior sternal cleft: repair in newborn. Ann Thorac Surg. 1985;39:582–583
  30. Sabiston DC. The surgical management of congenital bifid sternum with partial ectopia cordis. J Thorac Surg. 1958;35:118–122
  31. Tobin GR. Pectoralis major segmental anatomy and segmentally split pectoralis major flaps. Plast Reconstr Surg. 1985;75:814–824
  32. Donnelly LF, Frush DP, Foss JN, et al. Anterior chest wall: frequency of anatomic variations in children. Radiology. 1999;212:837–840
  33. Hill CA, Argenta LC, Hines M. Superior sternal cleft repair using autologous rib grafts in an infant with complex congenital heart disease. Ann Thorac Surg. 2007;84:673–674
  34. Hazari A, Mercer N, Pawade A, et al. Superior sternal cleft: construction with a titanium plate. Plast Reconstruct Surg. 1998;101:167–170
  35. Hoffman E. Surgical correction of bifid sternum using Marlex mesh. Arch Surg. 1965;88:76–80
  36. Ravitch MM. Disorders of the sternum and thoracic wall. In:  Sabiston DC,  Spencer FC editor. Gibbon's Surgery of the Chest. (ed 4). Philadelphia, PA: W.B. Saunders; 1983;p. 318–360
  37. Hummer HP, Rupprecht H. Atypical thoracic deformities: evaluation and operative sequelae. Z Orthop. 1985;123:913–917
  38. Knox L, Tuggle D, Knott-Craig CJ. Repair of congenital sternal clefts in adolescence and infancy. J Pediatr Surg. 1994;29:1513–1516
  39. Kaplan LC, Matusoka R, Gilbert EF, et al. Ectopia cordis and cleft sternum: evidence of mechanical teratogenesis following rupture of the chorion or the yolk sac. Am J Med Genet. 1985;21:187–202
  40. Harrison MR, Filly RA, Stanger P, et al. Prenatal diagnosis and management of omphalocele and ectopia cordis. J Pediatr Surg. 1982;17:64–66
  41. Leca F, Thibert M, Khoury W, et al. Extrathoracic heart (ectopia cordis): report of two cases and review of the literature. Int J Cardiol. 1989;22:221–228
  42. Diaz JH. Perioperative management of neonatal ectopia cordis: report of three cases. Anesth Analg. 1992;75:833–837
  43. Soper SP, Roe LR, Hoyme HE, et al. Trisomy 18 with ectopia cordis, omphalocele and ventricular septal defect: case report. Pediatr Pathol. 1986;5:481–483
  44. Bick D, Markowitz RI, Horwich A. Trisomy 18 associated with ectopia cordis and occipital meningocele. Am J Med Genet. 1988;30:805
  45. Fox JE, Gloster ES, Mirchandani R. Trisomy 18 with Cantrell pentalogy in a stillborn infant. Am J Med Genet. 1988;31:391–394
  46. Hornberger LK, Colan SD, Lock JE, et al. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996;94:32–37
  47. Haynor DR, Shuman WP, Brewer DK, et al. Imaging of fetal extopia cordis: roles of sonography and computed tomography. J Ultrasound Med. 1984;3:25–27
  48. Van Allen MI, Mytire S. Ectopia cordis thoracalis with craniofacial defects resulting from early amnion rupture. Teratology. 1985;32:19
  49. Mohta A, Bagga D, Anand R. Ectopia cordis: report of two cases. Indian Heart J. 1993;45:133–134
  50. Morrello M, Quaini E, Nenov G, et al. Extrathoracic ectopia cordis case report. J Cardiovasc Surg. 1994;35:511–515
  51. Morales JM, Patel SG, Duff JA, et al. Ectopia cordis and other midline defects. Ann Thorac Surg. 2000;70:111–114
  52. Mittermayer C, Bernaschek G, Lee A. Diagnosis of ectopia cordis with three-dimensional power Doppler ultrasound in first-trimester pregnancy. Ultrasound Obstet Gynecol. 2002;20:203–208
  53. Fortunato SJ. The use of power Doppler and colour power angiography in fetal imaging. Am J Obstet Gynecol. 1996;174:1828–1831
  54. Liang RI, Huang SE, Change FM. Prenatal diagnosis of ectopia cordis at 10 weeks of gestation using two-dimensional and three-dimensional ultrasonography. Ultrasound Obstet Gynecol. 1997;10:137–139
  55. Lilje C, Weiss F, Lacour-Gayet F, et al. Complete ectopia cordis. Circulation. 2006;113:e757–e758
  56. Klingensmith WC, Cioffi-Ragan DT, Harvey DE. Diagnosis of ectopia cordis in the second trimester. J Clin Ultrasound. 1988;16:204–206
  57. Sharma VK, Kiran U, Sharma J, et al. Challenges in the management of ectopia cordis. J Cardiothorac Vasc Anesth. 2001;15:618–623
  58. Abdallah HI, Marks LA, Balsara RK, et al. Staged repair of pentalogy of Cantrell with tetralogy of Fallot. Ann Thorac Surg. 1993;56:979–980
  59. Hornberger LK, Colan SD, Lock JE, et al. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996;94(suppl):II32–II37
  60. Watterson KG, Wilkinson JK, Kliman L, et al. Complete thoracic ectopia cordis with double-outlet right ventricle: neonatal repair. Ann Thorac Surg. 1992;53:146–147
  61. Amato JJ, Zelen JU, Talwalkar NG. Single-stage repair of thoracic ectopia cordis. Ann Thorac Surg. 1995;59:518–520
  62. 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;50:778–786
  63. Bognoni V, Quartuccio A, Quartuccio A. First-trimester sonographic diagnosis of Cantrell's pentalogy with exencephaly. J Clin Ultrasound. 1999;27:276
  64. Martin RA, Cunnif C, Erickson I, et al. Pentalogy of Cantrell and ectopis cordis, a familial developmental field comples. Am J Med Genet. 1992;42:839–841
  65. Peer D, Moroder W, Delluca A. Prenatal diagnosis of the pentalogy of Cantrell combined with exencephaly and amniotic band syndrome. Ultraschall Med. 1993;1494:94–95
  66. Barrow MV, Willis LS. Ectopia cordis (ectocardia) and gastroschisis induced in rates my maternalo administration of lathyrogen, betaaminopropionitrile (BAPN). Am Heart J. 1972;83:518–526
  67. Sosa MO. Pentalogia de Cantrell con malformaciones multiples y asociada a inhalación de cloro. Ultrason Med. 1994;10:27–33
  68. Fogel M, Copoel JA, Cullen MT, et al. Congenital heart disease and fetal thoracoabdominal anomalies: association in utero and the importance of cytogenetic analysis. Am J Perinatol. 1991;8:411–416
  69. Loureiro T, Oliveira C, Aroso J, et al. Prenatal sonographic diagnosis of a rare Cantrell's pentalogy variant with associated open neural tube defect: a case report. Fetal Diagn Ther. 2007;22:172–174
  70. Emanuel PG, Garcia GI, Angtuaco TL. Prenatal detection of anterior abdominal wall defects with US. Radiographics. 1995;15:517–530
  71. Saxena NC. Ectopia cordis child surviving: prosthesis fails. Pediatr News. 1976;10:3
  72. Ley EJU, Roth JJ, Kim KA, et al. Successful repair of ectopia cordis using alloplastic materials: 10-year follow-up. Plast Reconstr Surg. 2004;114:1519–1522

PII: S1055-8586(08)00013-9

doi: 10.1053/j.sempedsurg.2008.03.004

Seminars in Pediatric Surgery
Volume 17, Issue 3 , Pages 154-160 , August 2008