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2007-02-06-19 Pentalogy of Cantrell © Sanchez www.thefetus.net/


Pentalogy of Cantrell

Marcos Antonio Velasco Sanchez, MD; Candelairo Conda Moreno, MD, Hilario Bibiano Pano, MD.

Unidad Obstetrica Quirurgica, Hospital General de la S. S. A. de Acapulco, Guerrero, Mexico. Instituto Mexicano de Diagnostico por Imagen (I.M.D.I.). Facultad de Mexicana, Division de Posgrado de la Universidad Autonoma de Guerrero, Mexico.

 

Definition

Pentalogy of Cantrell is a disorder characterized by:

  • a midline, supraumbilical abdominal wall defect;
  • a defect ot the lower sternum
  • a deficiency of the anterior diaphragm;
  • a defect in the diaphragmatic pericardium; and
  • Cardiac anomalies.

Synonyms

Thoraco-abdominal ectopia cordis.

History

Pentalogy of Cantrell was described by Cantrell in 1958 [1].

Prevalence

0.5:10.000 to 0.1:10,000 births [2].

Etiology

Unknown.

Pathogenesis

The constellation of defects observed in Pentalogy of Cantrell is thought to result from abnormalities in the differentiation of the intraembryonic mesoderm at approximately 14 to 18 days after conception. The diaphragmatic and pericardial defects result from abnormal development of the septum transversum whereas the sternum and abdominal wall defects are probably related to impaired migration of mesodermal structures [3].

Sonographic findings

The association of ectopia cordis and an abdominal wall defect are the most obvious ultrasonographic findings. The diagnosis of the Cantrell"s pentalogy is certain when all five defects are present; is probable when four of them are present (including intracardiac and ventral abdominal abnormalities); and is incomplete when various combination of defects are present, as long as a sternal abnormality is observed.

Case report

A 26-year-old patient (G1P0) presented at 24th week of pregnancy for routine ultrasound investigation. We found a large ventral wall defect, ectopia cordis, extruded abdominal content through the wall defect, clubfeet, short two-vessel umbilical cord. The findings were consistent with the pentalogy of Cantrell. Here are a few images that we obtained.

Images 1, 2. 2D oblique sagittal plane showing ventral thoraco-abdominal wall defect with herniated abdominal organs through the defect and displacement of the heart to the defect (left); and transverse 2D scan showing displaced heart to the thoraco-abdominal defect and herniated abdominal organs (right).

 

Images 3, 4. Color Doppler (left) and power Doppler (right) transverse images showing heart and course of umbilical vein in hernial sac.

 

Images 5, 6. Power Doppler (left) and color Doppler (right) transverse images showing displaced heart to the ventral wall defect.

 

Images 7, 8. Color Doppler images showing short two-vessel umbilical cord.

 

Images 9, 10. 2D images showing clubfoot (left); and deformed spine (right).

 

References

1.  

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

2.  

Bianchi DW, Crombleholme TM, D’Alton ME. Pentalogy of Cantrell. In: Fetology – diagnosis & management of the fetal patient.  New York: McGraw-Hill, 2000, p 493-98.

3.  

Buyse ML. Birth Defects Encyclopedia. Cambridge: Blackwell Scientific Publications, 1999.

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