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Renohepaticopancreatic dysplasia: diagnostic dilemma.

Alicia Fernández-Sein1, María S Correa-Rivas, Keila Pratts

  • 1Department of Pediatrics, Medical Sciences Campus, University of Puerto Rico, San Juan, Puerto Rico. afernandez@rcm.upr.edu

Puerto Rico Health Sciences Journal
|May 18, 2005
PubMed
Summary

A rare Ivemark II syndrome case in an infant presented with abdominal mass, jaundice, and renal issues. Despite intensive care, the infant succumbed to multisystem organ failure, highlighting the condition's severity.

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Area of Science:

  • Pediatric Surgery
  • Neonatal Medicine
  • Medical Genetics

Background:

  • Ivemark II syndrome, or renohepaticopancreatic dysplasia, is a rare congenital disorder.
  • This condition involves anomalies of the kidneys, liver, and pancreas.
  • Early diagnosis and management are crucial in affected neonates.

Observation:

  • An 18-day-old infant presented with abdominal mass, renal insufficiency, and jaundice.
  • Diagnostic workup included radiographic, radionuclear, and surgical procedures.
  • The clinical presentation indicated a severe congenital anomaly.

Findings:

  • The infant was diagnosed with renohepaticopancreatic dysplasia (Ivemark II syndrome).
  • Despite aggressive intensive care, the patient experienced progressive multisystemic organ failure.

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  • Autopsy confirmed the clinical diagnosis and revealed widespread organ damage.

    • Implications:

    • This case underscores the critical need for prompt recognition of Ivemark II syndrome in neonates.
    • Aggressive supportive care may be insufficient for severe presentations.
    • Further research into the genetic and developmental mechanisms of this syndrome is warranted.