Pentalogía de Cantrell: un caso en embarazo gemelar / Cantrell`s Pentalogy: A case in twin pregnancy

La pentalogía de Cantrell es una enfermedad muy poco frecuente sobre todo en embarazos múltiples. Está caracterizado por presentar malformaciones integradas por defectos de cinco anomalías: tercio inferior del esternón, defecto epigástrico de la línea media abdominal, alteración del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardiacas. Se reporta un caso en una paciente primigesta de 22 años de edad y embarazo gemelar que acude para su control ecográfico a las 27,3 semanas de gestación, en donde se encuentran los siguientes hallazgos ecográficos: anencefalia, anormalidad facial y corazón fuera de la cavidad torácica. Se da el nacimiento de los gemelos por vía cesárea a las 35 semanas: el primero de aspecto normal y el segundo con Pentalogía de Cantrell y otras malformaciones asociadas.

Pentalogy of Cantrell is a low frequency disease within multiple pregnancy, characterized with malformations with five anomalies defects: lower sternal defect, midline supraumbilical abdominal wall defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations. We report a case in a patient in her first pregnancy of 22 year old, twin pregnancy. She is 27, 3 weeks pregnant, comes for ultrasound control of the gestation, where the following ultrasound results are found: anencephaly, facial abnormalities and the heart out of the chest cavity. The birth of the twins is given via caesarian section at 35 weeks; the first of normal appearance and the second with Pentalogy of Cantrell and other associated malformations

Pentalogía de Cantrell en el primer gemelo de un embarazo gemelar monocigótico: presentación de un caso y revisión de la literatura / Cantrell's pentalogy in the first twin froma monozygotic twin pregnancy: a case presentation and literature review

Introducción: la Pentalogía de Cantrell (PC) se compone de cinco anomalías: defecto superior de pared abdominal, defecto esternal inferior, defecto diafragmático anterior, defecto pericárdico diafragmático y anormalidades del corazón. Cerca de 250 casos han sido reportados, solo 6 involucran gemelos, y de estos 4 describen discordancia de esta anomalía. Objetivo: realizar la presentación de un caso de PC asociado a embarazo gemelar y la revisión de la literatura de esta asociación con énfasis en el diagnóstico prenatal y la etiología. Materiales y métodos: se presenta un caso de Pentalogía de Cantrell que afecta solo al primer gemelo de un embarazo gemelar monocigótico, se realizó una búsqueda de la información en las bases de datos SciELO y PubMed con la terminología MeSH "Pentalogy of Cantrell", "twin" y en español "Pentalogía de Cantrell" y "gemelar". Conclusión: la PC puede ser sospechada ecográficamente por ectopia cordis y defecto toracoabdominal. La presentación de un caso en un embarazo gemelar es una asociación poco frecuente.

Introduction: Cantrell's pentalogy (CP) consists of five anomalies: upper abdominal wall defects, lower sternal defects, anterior diaphragmatic defects, diaphragmatic pericardial defects and abnormalities of the heart. Around 250 cases have been reported; only 6 have involved twins and 4 of these have described discordance of such anomaly. Objective: presenting a case of twin pregnancy-associated CP and literature review regarding such association, emphasizing prenatal diagnosis and etiology. Materials and methods: a case of Cantrell's pentalogy is presented which only affected the first twin from a monozygotic twin pregnancy. Information was sought in SciELO and PubMed databases, using the following MeSH terminology "Pentalogy of Cantrell", "Twin" and "Pentalogía de Cantrell" and "gemelar" in Spanish. Conclusion: CP may be ecographically suspected by ectopia cordis and thoracoabdominal defects. The presentation of a case in twin pregnancy is an extremely rare association.

Use of prosthetic materials in cardiac surgery with special reference to pentalogy of cantrell.

Use of prosthetic materials has not only helped but revolutionalised surgery especially cardiac surgery. Various types of patches are now available to match the needs of the various types of cardiac surgery performed today. Use of prosthetic patches has not only met the demand of day to day cardiac surgeries like ASD, VSD but also helped in repair and better survival of complicated surgeries like repair of Pentalogy of Cantrell

Successful Vaginal Delivery of a Pregnant Woman with Cantrell's Pentalogy

Cantrell's Pentalogy is a rare condition that consists of defects involving the abdominal wall, lower sternum, anterior diaphragm, pericardium, and heart. In the literature to date, pregnant women with Cantrell's Pentalogy have not been discussed. We performed successful vaginal delivery of a 23-yr-old nulliparous, primigravid woman who had been diagnosed with this condition. Diagnosis was based on cardiac catheterization, angiography, and echocardiogram, and abdominopelvic CT. Vaginal delivery may be an option for women with Cantrell's Pentalogy and may be attempted with caution.

One-Stage Operation for Cantrell's Pentalogy

Cantrell's pentalogy may be defined as a failure of fusion of the midline from the sternum to the umbilicus. Thus, this malady consists of multiple anomalies of the sternum, heart, pericardium, diaphragm and umbilicus or anterior abdominal wall. According to the degrees of each anomaly, various operations can be planned as a one-stage operation or as a multi-stage operation and then palliative or corrective operations. The authors experienced a case of Cantrell's pentalogy that consisted of a bifid sternum, ventricular septal defect, atrial septal defect, ventricular diverticulum, dextrocardia, pericardial defect, anterior diaphragmatic defect and diastasis recti; all of these problems were corrected by a one-stage operation.

A Case of Cantrell's Pentalogy with Anencephaly Diagnosed by Prenatal Ultrasonography / 대한산부인과학회잡지

Pentalogy of Cantrell was very rare congenital anomaly and represent a regional prevalence of 5.5/1 million live births. The syndrome, Cantrell's pentalogy is characterized by defects in the lower sternum with ectopia cordis, anterior diaphragm defects, midline supraumbilical abdominal wall defects, defects in the diaphragmatic pericardium, and various intracardiac defects. Anencephaly involves acrania with a degenerated mass of brain tissue. Combination of both conditions are extremely rare. We experienced a case of Cantrell's pentalogy with anencephaly detected by ultrasonography at 15 weeks 1 day menstural age and confirmed at autopsy. Thus we report a case with brief review of the literature.