Research: The pentalogy of Cantrell: A rare and challenging prenatal diagnosis

Int J Surg Case Rep


. 2023 Oct 10:112:108941.

 doi: 10.1016/j.ijscr.2023.108941. Online ahead of print.

The pentalogy of Cantrell: A rare and challenging prenatal diagnosis

Fathi Mraihi 1Jihene Basly 2Amani Mezni 2Zeineb Ghali 2Montasar Hafsi 2Dalenda Chelli 2

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Introduction: The sternum, pericardium, diaphragm, abdominal wall, and heart are all affected by the unusual congenital condition known as Cantrell pentalogy. It is a rare congenital disorder that requires multidisciplinary care. Early diagnosis and appropriate management are crucial for improving outcomes in affected individuals. To illustrate the difficulties and complexity of Cantrell pentalogy, we provide two cases.

Presentation of case: In case 1, a routine antenatal scan at 12 weeks’ gestation revealed thoracoabdominal ectopia cordis in a 29-year-old woman. The pregnancy was terminated medically due to the severity of the anomalies and the poor prognosis. A 32-year-old patient in case 2 had a diaphragmatic hernia, thoracoabdominal ectopia cordis and midline abdominal wall abnormalities. After counselling, a medical termination was chosen. The ultra-sonographic features were confirmed by autopsy results in 2 cases.

Discussion: Early diagnosis is feasible in the first trimester if ectopia cordis and omphalocele exist. Additionally, development in ultrasound technology provides us with better visualization and early diagnosis. With patients who have fully developed Cantrell syndrome and those who also have accompanying anomalies, the prognosis is often poor, with short survival and quality of life.

Conclusions: Cantrell pentalogy is a rather uncommon congenital condition. Early detection is possible in the first trimester. The severity of the illness varies greatly, and treatment is determined by the precise abnormalities present. Early diagnosis necessitates adequate initial training as well as ongoing in-service training for sonographers. Early detection and treatment are critical for improving outcomes in affected persons.

Keywords: Pentalogy of Cantrell-; Pregnancy; Prenatal diagnosis; Ultrasonography.

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