Current Review
Cantrell’s Syndrome: A Challenge to the Surgeon

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Abstract

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.

Section snippets

Case Report

The patient was born May 17, 1994, after a normal pregnancy, the mother’s first pregnancy. There was no family history of congenital defects. After birth, an omphalocele was immediately apparent, but it did not require surgical treatment. The sternum was short but complete, and beneath the xiphoid process a pulsatile structure extending into the epigastrium was seen. There was a harsh systolic murmur, with its maximum at the left and right third intercostal spaces. The neonate had a flat and

Review of Literature

A year after the report of Cantrell and associates [1], Mulder and co-workers [4] presented the cases of 3 more patients. All were seen with dextroversion, ventricular septal defect, and left ventricular diverticulum, and 1 also had infundibular and valvular pulmonary stenosis.

In 1972, Toyama [5] reported the case of a patient with dextroversion, ventricular septal defect, pulmonary stenosis, common atrium, left superior vena cava, overriding aorta, and total anomalous venous return to the

Analysis of Literature

An analysis of the malformations in 153 patients (the 61 found by Tomaya [5] and 92 others) was carried out.

Comment

Patients with Cantrell’s syndrome are usually products of normal gestation. No familial tendency has been reported 6, 12, 13. The incidence of this malformation is estimated to be 1 in 65,000 live births [13]. According to our review, male patients are affected more often than female patients (1.35:1).

As previously pointed out by Cantrell and associates [1] and others 6, 11, 13, 18, a developmental failure of appropriated segments of the mesoderm is considered the embryologic background of

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