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Ann Thorac Surg 1998;65:1178-1185
© 1998 The Society of Thoracic Surgeons

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Current Review

Cantrell’s Syndrome: A Challenge to the Surgeon

^a Thoracic and Cardiovascular Surgery, RWTH Aachen, Aachen, Germany
^b Pediatric Cardiology, Rheinisch-Westfälische-Technische Hochschule Aachen, Aachen, Germany
^c Pediatric Cardiology, Zentralkrankenhaus "Links der Weser," Bremen, Germany
^d First Department of Surgery, Osaka University Medical School, Osaka, Japan

Address reprint requests to Dr Vazquez-Jimenez, Thoracic and Cardiovascular Surgery, Universitätsklinikum RWTH Aachen, Pauwelsstr 30, 52057 Aachen, Germany

We present a case of partial Cantrell’s syndrome with ventricular septal defect, left ventricular diverticulum, dextrorotation of the heart, an anterior diaphragmatic defect, and a midline supraumbilical abdominal wall defect with omphalocele. At the age of 20 months, the patient underwent a successful cardiac surgical procedure. To detect risk factors and to define therapeutic strategies, we analyzed the spectrum and the frequency of malformations described in 153 patients with Cantrell’s syndrome. Despite modern surgical standards, Cantrell’s syndrome represents a challenge to the surgeon because of the wide spectrum of anomalies, the severity of the abdominal and cardiac malformations, and the high mortality.

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