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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
Insights
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.
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.
- 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.
Abstract:
We present the case of an eighteen day old baby boy hospitalized with an abdominal mass, renal insufficiency and jaundice. Multiple radiographic, radionuclear and surgical interventions were required to diagnose renohepaticopancreatic dysplasia, also known as Ivemark II syndrome. In spite of aggressive intensive care support, the patient developed multisystemic organ failure and died. Clinical presentation and autopsy findings are presented.
