Current ReviewCantrell’s Syndrome: A Challenge to the Surgeon
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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Cited by (119)
Pentalogy of Cantrell With a Left Ventricular Diverticulum and Muscular VSD
2022, Annals of Thoracic SurgeryTwin pregnancy in which both fetuses have Cantrell's pentalogy: A case report and literature review
2021, European Journal of Obstetrics and Gynecology and Reproductive BiologyNorwood Operation of a Neonate With Pentalogy of Cantrell
2020, Annals of Thoracic SurgeryFirst reported case of Pentalogy of Cantrell variant with good outcome in a 32 week gestational age monozygotic twin with twin-twin transfusion syndrome
2019, Journal of Pediatric Surgery Case ReportsCitation Excerpt :Familial occurrence has also been reported. An associated intracardiac anomaly is the rule and in 75% of cases, pericardial defects may be absent and an abdominal wall defect, mostly omphalocele is present (63%) [7–10]. Previous reports on a fetus with pentalogy of Cantrell in a twin pregnancy are limited with approximately only eight such case reports in the English literature [11].
Pentalogy of Cantrell
2019, Seminars in Pediatric SurgeryCitation Excerpt :However, the survival rate has improved to 61% with considerable advances in pediatric surgery and neonatal intensive care. Mean age at which patients underwent surgical operation was shown to be a predictor of survival, with older age associated with better prognosis; all non-survivors had surgery on the first postnatal day, whereas mean age of operation of survivors was 9 months.12,28 Postoperative care of children with Pentalogy of Cantrell is often complicated by respiratory insufficiency and the prolonged need for ventilator support.41