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Review
. 2008 Jan;167(1):29-35.
doi: 10.1007/s00431-007-0578-9. Epub 2007 Aug 4.

Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Jeroen H L van Hoorn  1 , Rob M J Moonen, Clément J R Huysentruyt, L W Ernest van Heurn, Jos P M Offermans, A L M Twan Mulder
Affiliations
Review

Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach

Jeroen H L van Hoorn et al. Eur J Pediatr. 2008 Jan.

Abstract

Two patients with incomplete pentalogy of Cantrell are described. The first was a girl with a large omphalocele with evisceration of the heart, liver and intestines with an intact sternum. Echocardiography showed profound intracardiac defects. The girl died 33 h after birth. The second patient was a female fetus with ectopia cordis (EC) without intracardiac anomalies; a large omphalocele with evisceration of the heart, stomach, spleen and liver; a hypoplastic sternum and rib cage; and a scoliosis. The pregnancy was terminated. A review of patients described in the literature is presented with the intention of finding prognostic factors for an optimal approach to patients with the pentalogy of Cantrell. In conclusion the prognosis seems to be poorer in patients with the complete form of pentalogy of Cantrell, EC, and patients with associated anomalies. Intracardial defects do not seem to be a prognostic factor.

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Figures

Fig. 1
Fig. 1
Patient 1 with a large omphalocele with evisceration of the heart (arrow), liver and intestines
Fig. 2
Fig. 2
Post-mortem examination of patient 2 showed a large omphalocele with evisceration of the liver, spleen and a major part of the gastro-intestinal tract. The arrow indicates the heart covered with skin
See this image and copyright information in PMC

Comment in

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