Images of Extremely Rare Cantrell Phenomenon
Artur Fabijan1, Sara Korabiewska-Pluta2, Tomasz Puzio3
1Department of Neurosurgery, Polish-Mother's Memorial Hospital Research Institute, 93-338 Lodz, Poland.
Insights
Cantrell syndrome, a rare congenital disorder, requires early multimodality imaging for management. This case highlights complex cardiac defects and the need for a multidisciplinary approach in treating ectopia cordis.
Area of Science:
- Medical Imaging
- Pediatric Cardiology
- Congenital Disorders
Background:
- Cantrell syndrome is an extremely rare congenital disorder.
- It is characterized by a thoracoabdominal wall defect, often including ectopia cordis (heart outside the chest).
- Prenatal diagnosis is crucial for planning management.
Purpose of the Study:
- To present a case of Cantrell syndrome with ectopia cordis.
- To demonstrate the utility of three-dimensional computed tomography angiography in visualizing complex anatomy.
- To emphasize the need for multimodality imaging in managing this condition.
Main Methods:
- Prenatal diagnosis of Cantrell syndrome and ectopia cordis.
- Three-dimensional computed tomography angiography for detailed anatomical assessment.
- Postnatal evaluation of intracardiac defects.
Main Results:
- CT angiography confirmed complex intracardiac defects.
- Diagnosed defects included Tetralogy of Fallot, total anomalous pulmonary venous return, and persistent left superior vena cava.
- The thoracoabdominal wall defect with complete ectopia cordis was visualized.
Conclusions:
- Cantrell syndrome requires a multidisciplinary approach from prenatal diagnosis through surgical intervention.
- Multimodality imaging is essential for effective management planning.
- This rare disorder, including ectopia cordis, often has a fatal outcome.
Abstract:
We present a case of a neonate born with prenatal diagnosis of Cantrell syndrome and ectopia cordis. This extremely rare congenital disorder underscores the significant need for multimodality imaging to plan further management. The aim of the study was to present the thoracoabdominal syndrome using a three-dimensional computed tomography angiography. The CT scans confirmed complex intracardiac defects consisting of tetralogy of Fallot, total anomalous pulmonary venous return and persistent left superior vena cava. In conclusion, Cantrell syndrome necessitates a multidisciplinary approach, from the onset of the prenatal diagnosis followed by prompt medical imaging and surgical interventions after birth. The thoracoabdominal wall defect including complete ectopia cordis is an extremely rare disorder with a fatal outcome.
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