Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach.
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.
Department of Pediatrics, University Hospital Maastricht, P.O. Box 5800, 6202 AZ, Maastricht, The Netherlands.
Jeroen H L
JH
van Hoorn
Rob M J
RM
Moonen
Clément J R
CJ
Huysentruyt
L W Ernest
LW
van Heurn
Jos P M
JP
Offermans
A L M Twan
AL
Mulder
D034861:Abdominal Wall / Q000002:abnormalities*; D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006554:Hernia, Umbilical / Q000150:complications* / Q000000981:diagnostic imaging / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D011296:Prenatal Diagnosis; D011379:Prognosis; D013771:Tetralogy of Fallot / Q000150:complications* / Q000000981:diagnostic imaging; D014463:Ultrasonography