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[Ivemark syndrome: 2 case reports]

P Roth1, G Agnani, F Arbez-Gindre

  • 1Clinique Universitaire de Gynécologie, d'Obstétrique et de la Reproduction, CHU Saint-Jacques, Besançon.

Journal De Gynecologie, Obstetrique Et Biologie De La Reproduction
|January 1, 1993
PubMed
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Ivemark Syndrome is a rare congenital condition affecting multiple organs, including the spleen, heart, and abdominal viscera. Early ultrasound detection and understanding cardiac malformation are key for managing this splenic abnormality syndrome.

Area of Science:

  • Medical Genetics
  • Developmental Biology
  • Pediatric Cardiology

Background:

  • Ivemark Syndrome is a rare congenital disorder characterized by splenic abnormalities, complex cardiac defects, and visceral malformations.
  • The estimated incidence is 1 in 6000 deliveries, with teratogenic effects potentially occurring between 30-40 days of intrauterine development.
  • The exact etiology of Ivemark Syndrome remains unknown.

Observation:

  • The primary clinical warning sign for Ivemark Syndrome is fetal bradycardia.
  • Prenatal diagnosis is feasible through obstetric ultrasound.
  • Prognosis is significantly influenced by the severity of cardiac malformations.

Findings:

  • Ivemark Syndrome presents as a complex multiple organ abnormality.

Related Experiment Videos

  • Splenic anomalies are a consistent feature.
  • Associated conditions include complex congenital heart disease and abdominal organ malformations.

    • Implications:

    • Early prenatal diagnosis via ultrasound allows for timely intervention planning.
    • Understanding the spectrum of cardiac pathology is crucial for patient management and prognosis.
    • Further research into the teratogenic causes of Ivemark Syndrome is warranted.