Complete and Incomplete Pentalogy of Cantrell

Kylat, Ranjit I

doi:10.3390/children6100109

Cited by 8 publications

(8 citation statements)

References 22 publications

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“…• An anterior abdominal wall defect with herniation of liver and bowel loops through it • A defect in the lower anterior chest wall with heart lying outside the thoracic cavity • The brain showed deficient skull vault with dilated ventricles suggestive of anencephaly Since the patient had the following findings, it leads to the diagnosis of Pentalogy of cantrell-class 3.  A wide range of intracardiac anomalies have been associated with PC including septal defects, tetralogy/pentalogy of Fallot, total anomalous pulmonary venous drainage, and left ventricular diverticulum [3,4,5,6,7,8] . Holt-Oram syndrome, which was seen in the family of Case 1, is associated with cardiac anomalies but has never been reported with PC.…”

Section: Resultsmentioning

confidence: 99%

“…Holt-Oram syndrome, which was seen in the family of Case 1, is associated with cardiac anomalies but has never been reported with PC. A variety of mutations including deletions or duplications of the PORCN, thoraco-abdominal syndrome (TAS), ALDH1A2, and teneurin-4 (TENM4) genes have been identified [8,9,10,11,12] to be associated with Pentalogy of Cantrell. It can also be associated with rare anomalies, such as craniorachischisis, pulmonary extrophy, and Goltz-Gorlin syndrome [8,13,14,15,16] .…”

Section: Resultsmentioning

confidence: 99%

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Early detection of pentalogy of cantrell by sonography

Mane¹,

Goyal²,

Joshi³

2020

Int. J. Radiol. Diagn. Imaging

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Pentalogy of Cantrell is a rare congenital condition with a thoraco-abdominal disruption showing five characteristics i.e. ectopia cordis and intracardiac anomalies; lower sternal defect; midline supraumbilical thoraco-abdominal wall defect; anterior diaphragmatic defect; defect of pericardium. Ultrasound can be used for its early and confirmatory diagnosis. This is a study of a rare case of Pentalogy of Cantrell based on sonography findings in a 23 year old primigravida presenting for routine antenatal check-up.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Early detection of pentalogy of cantrell by sonography

Mane¹,

Goyal²,

Joshi³

2020

Int. J. Radiol. Diagn. Imaging

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“…Patients can show the existence of all five defects, or they may only have some defects. It can be divided into complete type and incomplete type ( 6 , 7 ). Defects also vary greatly in degree.…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of neonatal Cantrell pentalogy: a case report and literature review

Liao¹,

Huang²,

Li³

2023

AME Case Rep

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Background Pentalogy of Cantrell (PC) is a congenital multiple malformation consisting of midline supraumbilical thoracoabdominal wall defects, anterior and pericardial diaphragm defects, lower sternum defects, ectopia cordis and various intracardiac anomalies, that present a distinctive challenge for care-providers and surgeons. Case Description Cases of PC in twin pregnancies is rare. We report a new born infant, weighing 2,400 g, the younger twin. After birth, he was transferred to the Children’s Hospital of Soochow University due to the prenatal echocardiography indicated that he has ventricular septal defect (VSD), atrial septal defect (ASD) and aortic arch hypoplasia. From the appearance, the lower end of sternum is suspiciously missing, the apical beating point is located at the lower edge of the median xiphoid process of sternum, and the upper abdominal muscle below the beating area is defective. He has the clinical and imaging features of complete type of PC. Three surgical operations were performed in the neonatal period, including end-to-side anastomosis of aortic arch under cardiopulmonary bypass (CPB), patch repair of VSD, repair of ASD, ligation of ductus arteriosus, correction of cardiac vascular malformation and repair of chest and abdominal wall defect, and the postoperative recoveries went smoothly. Conclusions PC is a rare congenital dysplasia, and its condition is complex. Our case shows that surgery is an effective treatment method, and the prognosis is related to the complexity of malformations.

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“…It is interesting that the two other canine case reports about pentalogy of Cantrell, present in the literature, are describing this anomaly in the German shepherd dog as well ( 15 , 16 ), suggesting that it is likely an abnormality occurring more often in this breed. In humans, it is suspected to have a multifactorial inheritance ( 18 ). In the German shepherd dog, pentalogy of Cantrell may also have a hereditary background.…”

Section: Discussionmentioning

confidence: 99%

Case Report: Unusual Peritoneopericardial Diaphragmatic Hernia in an 8-Month-Old German Shepherd Dog, Associated With a Pericardial Pseudocyst and Coexisting Severe Pericardial Effusion Resulting in Right-Sided Heart Failure

et al. 2021

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Objective: This study aims to describe an unusual peritoneopericardial diaphragmatic hernia (PPDH) in an 8-month-old German shepherd dog, associated with a pericardial pseudocyst and coexisting severe pericardial effusion resulting in right-sided heart failure.Case Summary: An 8-month-old, male, intact, German shepherd dog, was referred for ascites. Echocardiography revealed a severe pericardial effusion with a cyst-like structure within the pericardium and consequently decompensated right-sided heart failure. The ascites was secondary to right-sided heart failure (cardiac tamponade). Computed tomography (CT) of the thorax and abdomen was performed and showed PPDH with severe pericardial effusion and presence of a pericardial cyst-like structure; xyphoid cleft and Y-shaped seventh sternebra; and mild thickening along the cranioventral abdominal wall consistent with scar tissue from the previous umbilical hernia surgical repair. During surgery, the PPDH was corrected, and it was revealed that the remnant of the umbilical cord passed through it, into the pericardium. The cyst-like structure was successfully resected and sent for pathology. Histopathology showed signs of a chronic suppurative inflammation, with absence of a mesothelial or endothelial wall layer, thus consistent with a pseudocyst. Based on tomographic and surgical findings, it is suspected that the pseudocyst, together with the pericardial effusion, evolved by an inflammation of the remnant of the umbilical cord during the umbilical hernia surgical repair 1 month prior to presentation. The underlying PPDH most likely favored the development of the pericardial pseudocyst. However, due to prior antibiotic therapy initiated by the private vet, an infectious origin cannot be ruled out completely.New or Unique Information Provided: There are a few case reports describing PPDH and/or pericardial pseudocysts in veterinary patients, but the current case report is unique, since it describes PPDH associated with a pericardial pseudocyst and coexisting severe pericardial effusion resulting in cardiac tamponade. As far as the authors know, such a case has not been described in veterinary medicine before.

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Complete and Incomplete Pentalogy of Cantrell

Cited by 8 publications

References 22 publications

Early detection of pentalogy of cantrell by sonography

Early detection of pentalogy of cantrell by sonography

Surgical treatment of neonatal Cantrell pentalogy: a case report and literature review

Case Report: Unusual Peritoneopericardial Diaphragmatic Hernia in an 8-Month-Old German Shepherd Dog, Associated With a Pericardial Pseudocyst and Coexisting Severe Pericardial Effusion Resulting in Right-Sided Heart Failure

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