Pentalogy of Cantrell

Newberry, Desi; England, Amanda

doi:10.1097/anc.0000000000000209

Cited by 24 publications

(10 citation statements)

References 33 publications

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“…Хорион первого плода располагался по передней стенке матки, а второго плода -по задней. Обнаруженные ультразвуковые признаки соответствуют врожденной аномалии плода -пентаде Кантрелла [8,9]. Кроме того, по передней стенке матки визуализировались два участка отслойки плодного яйца размером 37 × 9 и 39 × 15 мм в стадии организации.…”

Section: описание клинического случаяunclassified

Peculiarities of high-risk pregnancy management after the IVF procedure: a clinical observation report

et al. 2018

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This article presents an annotation and discussion of our own clinical observation of a high-risk multiple pregnancy after in vitro fertilization in a patient with tuboperitoneal infertility. We report a case of early prenatal diagnosis of a rare malformation of a single fetus in dichorionic twin pregnancy (pentalogy of Cantrell). The feasibility of cumulative assessment of the risks of assisted reproductive technology complications to determine the tactics of management and intensive observation during pregnancy, as well as the use of invasive intrauterine interventions for multiple pregnancy to reduce perinatal risks is discussed.

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Section: описание клинического случаяunclassified

Peculiarities of high-risk pregnancy management after the IVF procedure: a clinical observation report

et al. 2018

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“…Pentalogy of Cantrell (POC) is a rare congenital disease that includes sternal fissure, lower sternal defects, anterior semilunar defects of the diaphragm, pericardial wall defects with pericardioperitoneal communication, upper abdominal wall defects with umbilical hernia, and intracardiac malformation. 1 2 A normal person's heart is on the left side of the thorax, while the heart of a patient with POC is below the middle of the thorax. Because of the exterior of the heartbeat, it is also called “people with hearts outside.” The disease was first described by Cantrell in 1958.…”

Section: Introductionmentioning

confidence: 99%

Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center

Wang

et al. 2021

Eur J Pediatr Surg

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Objective To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects. Materials and Methods A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019. Results The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal. Conclusion The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax.

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“…However, up until now this theory has never been evaluated. Similarly, no specific genetic abnormality has been conclusively correlated with PC, even though multiple genetic influences and chromosomal associations with PC have been described 6 . The spectrum of Cantrell has been reported in association with chromosomal anomalies like trisomy 18, trisomy 21, trisomy 13 and Turner syndrome 7 , 8 .…”

Section: Introductionmentioning

confidence: 99%

Abrogation of TGF-beta signalling in TAGLN expressing cells recapitulates Pentalogy of Cantrell in the mouse

Aldeiri

Roostalu

Albertini

et al. 2018

Sci Rep

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Pentalogy of Cantrell (PC) is a rare multi-organ congenital anomaly that impedes ventral body wall closure and results in diaphragmatic hernia, intra- and pericardial defects. The underlying cellular and molecular changes that lead to these severe developmental defects have remained unknown largely due to the lack of representative animal models. Here we provide in depth characterization of a mouse model with conditional ablation of TGFβRII in Transgelin (Tagln) expressing cells. We show that Tagln is transiently expressed in a variety of cells that participate in the embryonic development and patterning of ventral structures. Genetic ablation of TGFβRII in these cells leads to ventral midline closure defect, diaphragmatic hernia, dilated cardiac outflow tract and aberrant cardiac septation, providing a reliable model to study the morphological changes leading to PC. We show that myogenisis in the diaphragm is independent of TGFβ and the diaphragmatic hernia arises from fibroblast-specific migration defect. In the dorsal body wall Tagln expression is initiated after the closure process, revealing a remarkable difference between ventral and dorsal body walls development. Our study demonstrates the use of micro-CT scanning to obtain a 3-dimensional high-resolution overview of embryonic anomalies and provides the first mechanistic insight into the development of PC.

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Pentalogy of Cantrell

Abstract: Future research can focus on further elucidating genetic etiologies of pentalogy of Cantrell.

Cited by 24 publications

References 33 publications

Peculiarities of high-risk pregnancy management after the IVF procedure: a clinical observation report

Peculiarities of high-risk pregnancy management after the IVF procedure: a clinical observation report

Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center

Abrogation of TGF-beta signalling in TAGLN expressing cells recapitulates Pentalogy of Cantrell in the mouse

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