Ectopia Cordis in Man

Kanagasuntheram, R.; Verzin, J. A.

doi:10.1136/thx.17.2.159

Cited by 32 publications

(17 citation statements)

References 15 publications

(7 reference statements)

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“…Surgical treatment attempted on preterm infants meets with variable success; one such patient survived 8 months. 16 To our knowledge, the cardiac defects in our patient (pulmonary artery hypoplasia, aortic and pulmonary valve insufficiency, and malposition of great vessels) have not been reported previously with PC.…”

Section: Surasak Puvabanditsin Valeria DI Stefano Eugene Garrow Ronalmentioning

confidence: 47%

Ectopia cordis

Puvabanditsin¹,

Stefano²,

Garrow³

et al. 2013

Hong Kong Med J

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Section: Surasak Puvabanditsin Valeria DI Stefano Eugene Garrow Ronalmentioning

confidence: 47%

Ectopia cordis

Puvabanditsin¹,

Stefano²,

Garrow³

et al. 2013

Hong Kong Med J

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“…Although frequently referred to as the Pentalogy of Cantrell when anterior diaphragmatic deficiency, cardiac defects, sternal clefts, ventral wall deficiencies, and pericardial defects are present [Cantrell et al, 19581, this spectrum ofdefects is more appropriately categorized as a midline developmental field complex and as such is causally heterogeneous. Evidence for this heterogeneity is suggested by the occasional occurrence of this complex in chromosomal and other multiple malformation syndromes [Baker et al, 1984;Fox et al, 1988;Ghidini et al, 1988;Haynor et al, 1984;Kanagasuntheram and Verzin, 1962;Leca et al, 1989;MedinaEscobedo et al, 1991;Milne et al, 1990;Toyama, 1972;Spitz et al, 1975;Wesselhoeft and DeLuca, 19841. However, despite heterogeneity, the boys reported here represent the first recurrence of this developmental field defect in a family.…”

Section: Discussionmentioning

confidence: 96%

“…A wide spectrum of midline defects has been seen in association with sternal fusion abnormalities [Cantrell et al, 1958;Kanagasuntheram and Verzin, 1962;Toyama, 19721. Although frequently referred to as the Pentalogy of Cantrell when anterior diaphragmatic deficiency, cardiac defects, sternal clefts, ventral wall deficiencies, and pericardial defects are present [Cantrell et al, 19581, this spectrum ofdefects is more appropriately categorized as a midline developmental field complex and as such is causally heterogeneous.…”

Section: Discussionmentioning

confidence: 99%

“…Within this spectrum lies the association of sternal clefts, pericardial defects, cardiac defects, anterior diaphragmatic deficiency and ventral wall defects known as the Pentalogy of Cantrell. Several reviews describing this heterogeneous group of conditions lack any reference to familial recurrence [Cantrell et al, 1958;Crittenden et al, 1959;Kanagasuntheram and Verzin, 1962;Leca et al, 1989;Toyama, 19721. We have evaluated 3 brothers each with extensive diaphragmatic deficiency that includes the anterior diaphragm. The first born child had absence of both hemidiaphragms but was otherwise normal from a structural standpoint.…”

Section: Introductionmentioning

confidence: 99%

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Pentalogy of Cantrell and ectopia cordis, a familial developmental field complex

et al. 1992

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The association of sternal fusion defects with various cardiac, diaphragmatic, and anterior body wall defects represents a developmental field complex that includes the Pentalogy of Cantrell and ectopia cordis. No familial cases have been reported previously. We present 3 consecutively born brothers with extensive diaphragmatic defects, 2 who had the Pentalogy of Cantrell. One of the 2 also had ectopia cordis.

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“…Midline fusion and formation of the thoracic and abdominal cavities is complete by the 9 th embryonic week. [17][18] Complete or incomplete failure, of midline fusion at this stage, results in disorders varying from isolated EC to complete ventral evisceration. Genesis of EC has not been fully explained, although several theories have been offered.…”

Section: Embryologymentioning

confidence: 99%

Ectopia cordis: a case report and review of literature

et al. 2018

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The Ectopia cordis is a rare congenital condition. It is characterized by the abnormal position of the heart outside the thoracic cavity, associated with defects in the parietal pericardium, diaphragm, sternum, and, in most cases, cardiac malformations. The reported prevalence is 5.5 to 7.9 per million live births. The designation Ectopia cordis was first proposed by Abott in 1998, although cases of patients with similar defects had been reported in past. Only 267 cases have been reported as of 2001, most (95%) associated with other cardiac abnormalities. A 10 hour old, normal, vaginally delivered, full-term male neonate, weighing 2020 grams was brought with an externally visible, beating heart over the lower chest wall. The antenatal period was uneventful, though no ultrasonography was done. The beating heart was covered with a membrane with greenish purulent matter over it, most probably, due to pseudomonas/proteus infection. Rest of the anterior abdominal wall was intact. Ectopia cordis is produced by segmental defects in mesodermal development in the third week of intrauterine life, and/or amniotic band syndrome that causes simultaneous cerebral and Thoraco-abdominal malformations. The existence of Ectopia cordis with severe congenital heart disease may be confirmed in the prenatal period by vaginal echocardiography at 10-12 weeks of gestation or by abdominal echocardiography by 20-22 weeks. Surgery is the only hope of survival, for such patients, although the overall success rate is very poor. In recent years, surgery has been attempted in one or two phases with variable results that depend, mainly on the type of associated heart disease.

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Ectopia Cordis in Man

Cited by 32 publications

References 15 publications

Ectopia cordis

Ectopia cordis

Pentalogy of Cantrell and ectopia cordis, a familial developmental field complex

Ectopia cordis: a case report and review of literature

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