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Radiology
Pentalogy of Cantrell
Built from Obstetric Imaging
What’s inside
8 sections · 55 slides
Overview
- Topics covered
What the condition is
The five defects, the terms behind them, and how partial cases are classified
- Overview of pentalogy of Cantrell
- Definitions of the main terms
- The five defects of the pentad
- Components of the pentad
- Defects of the anterior diaphragm and lower sternum
- Sternum, costal cartilages and the rectus abdominis attachment
- Midline supraumbilical abdominal wall defect
- Axial ultrasound view of a fetal omphalocele
- Defect in the diaphragmatic pericardium
- Ectopia cordis
- Omphalocele with ectopia cordis on prenatal ultrasound
- Newborn with a large uncovered ventral midline defect
- Toyama classification of pentalogy of Cantrell
- Toyama classes at a glance
- Proposed hexalogy and heptalogy of Cantrell
Frequency, causes and embryology
How often it occurs, and the early developmental failure thought to produce it
- Prevalence and epidemiology
- Embryologic origin of the defects
- Germ layers of the early embryo and what each forms
- Proposed mesodermal mechanism
- Inheritance and familial reports
- Reported chromosomal and gene associations
- The ventral midline as a developmental field
Findings in the fetus and newborn
The defects themselves, the heart lesions, and the other anomalies that travel with them
- Common findings in pentalogy of Cantrell
- Cardiac malformations reported with the syndrome
- Ventricular septal defect
- Normal heart compared with tetralogy of Fallot
- Associated brain and craniofacial defects
- Associated limb and abdominal organ defects
- Skeletal, skin and umbilical cord findings
Imaging technique and findings
Ultrasound first, then three-dimensional imaging, fetal MRI and fetal echocardiography
- Ultrasound diagnosis and timing
- Sonographic clues when ectopia cordis is not obvious
- First-trimester markers
- Three-dimensional ultrasound
- Three-dimensional ultrasound of a fetus with an omphalocele
- Fetal magnetic resonance imaging
- Fetal echocardiography
- Imaging pathway after a suspicious finding
Conditions that look similar
What else can produce these sonographic findings, and why chromosomal testing is advised
- Differential diagnosis from imaging findings
- Further conditions in the differential diagnosis
- Role of chromosomal studies
Management and outcome
What can and cannot be done before birth, the surgical options afterwards, and survival
- Prenatal management
- Fetal pericardiocentesis
- Prognosis and survival
- Surgical repair after birth
- Timing of surgical repair
Summary
What the referring clinician needs to carry away
- What the referring physician needs to know
- Key points
- Key points on diagnosis and outcome
- References
- References (continued)
- References (continued)
- References (continued)
- References (continued)
- Obstetric Imaging: Fetal Diagnosis and Care, 2nd Edition