PerCar - Perinatal Cardiology

Pagine ed articoli di informazioni sulle cardiopatie congenite dal feto al giovane adulto. Potete usare questa sezione per potere interagire con i medici

Cantrell's pentalogy

Cantrell’s pentalogy, first described in 1958, consists of
a lower sternal defect, anterior diaphragmatic defect,
parietal pericardial defect, omphalocoele, and herniation
of the heart . Ectopia cordis describes the herniation
or extrusion of the heart, which may occur
through either the thorax or the thoracoabdominal
region. The hypothesis underlying this condition is
abnormal development of the mesoderm at a very
early stage in embryonic life, occurring prior to or
immediately after differentiation of the primitive intraembryonic
mesoderm into its splanchnic and somatic
layers, since derivatives of both these layers are involved.
To date, there have been no etiologic factors
elucidated for this lesion. At approximately the
10th week of embryological life, the midgut, which had
previously been extraabdominal, returns to the
abdominal cavity and undergoes rotation followed by
fixation. The final structure of the abdominal wall is
made up of five distinct body folds that fuse at the base
of the umbilical cord. Failure of growth and fusion of
the cephalic fold results in either isolated ectopia cordis
or the pentalogy of Cantrell or one of its variants.
In a report of 13 cases of ectopia cordis—4 thoracic
and 9 thoracoabdominal—10 infants survived to term.
There were 5 long-term survivors, each of whom
had significant cardiac diagnoses, including doubleoutlet
right ventricle in 2 patients, hypoplastic left
ventricle in association with conoventricular septal
defects in 2 patients, and tetralogy of Fallot with pulmonary
atresia in 1 patient. Interestingly, all survivors
had isolated ectopia cordis with no significant extrathoracic
Prenatal diagnosis of pentalogy of Cantrell is most
common using ultrasound and has been extensively reported.
Fetal MRI has been reported recently.


  • 1. Baker ME, Rosenberg ER, Trofatter KF, et al. (1984) The in
    utero findings in twin pentalogy of Cantrell. J Ultrasound
    Med 3:525–527
  • 2. Cantrell JR, Haller JA, Ravitch MM (1958) A syndrome of
    congenital defects involving the abdominal wall, sternum,
    diaphragm, pericardium and heart. Surg Gynecol Obstet
  • 3. Carmi R, Boughman JA (1992) Pentalogy of Cantrell and
    associated midline anomalies: a possible ventral midline
    developmental field. Am J Med Genet 42:90–95
  • 4. Coakley FV, Glenn OA, Qayyum A, et al. (2004) Fetal MRI:
    a developing technique for the developing patient. Am J
    Roentgenol 182:243–252
  • 5. DiBernardo S, Sekarski N, Meijboom E (2004) Left ventricular
    diverticulum in a neonate with Cantrell syndrome.
    Heart 90:1320
  • 6. Ghidini A, Sirtori M, Romero R, et al. (1988) Prenatal
    diagnosis of pentalogy of Cantrell. J Ultrasound Med 7:567–572
    174 Pediatr Cardiol (2007) 28:172–175
  • 7. Halbertsma FJ, van Oort A, van der Staak F (2002) Cardiac
    diverticulum and omphalocele: Cantrell’s pentalogy or syndrome.
    Cardiol Young 12:71–74
  • 8. Hornberger LK, Colan SD, Lock JE, et al. (1996) Outcome
    of patients with ectopia cordis and significant intra-cardiac
    defects. Circulation 94:II32–II37
  • 9. Jochems L, Jacquemyn Y, Blaumeiser B (2004) Prenatal
    diagnosis of pentalogy of Cantrell: a case report. Clin Exp
    Obstet Gynecol 31:141–142
  • 10. Kawabata I, Takahashi Y, Iwagaki S, et al. (2003) MRI
    during pregnancy. J Perinat Med 31:449–458
    11. Levine D (2004) Fetal magnetic resonance imaging. J Matern
    Fetal Neonatal Med 15:85–94
  • 12. Liang RI, Huang SE, Chang FM (1997) Prenatal diagnosis of
    ectopia cordis at 10 weeks of gestation using two-dimensional
    and three-dimensional ultrasonography. Ultrasound
    Obstet Gynecol 10:137–139
  • 13. Oka T, Shiraishi I, Iwasaki N, et al. (2003) Usefulness of
    helical CT angiography and MRI in the diagnosis and
    treatment of pentalogy of Cantrell. J Pediatr 142:84
  • 14. Robson CD, Barnewolt CE (2004) MR imaging of fetal head
    and neck anomalies. Neuroimaging Clin North Am 14:273–291
  • 15. Song A, McLeary MS (2000) MR imaging of pentalogy of
    Cantrell variant with an intact diaphragm and pericardium.
    Pediatr Radiol 30:638–639
  • 16. Toyama WM (1972) Combined congenital defects of the
    anterior abdominal wall, sternum, diaphragm, pericardium,
    and heart: a case report and review of the syndrome. Pediatrics
    50:778–792 Pediatr Cardiol (2007) 28:172–175 175
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