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Congenital pancreatic cyst with Ivemark II syndrome: a rare case

Jamila Chahed1, Mongi Mekki, Sameh Aloui

  • 1Pediatric Surgery Department, University Hospital Center of Monastir, 5000 Monastir, Tunisia. j.chahed@voila.fr

Journal of Pediatric Surgery
|March 20, 2012
PubMed

Insights

This report details a rare case of congenital pancreatic cyst in an infant with Ivemark II syndrome, featuring situs inversus and asplenia. While the cyst was managed, the infant

Area of Science:

  • Pediatric Surgery
  • Medical Genetics
  • Developmental Biology

Background:

  • Ivemark II syndrome, characterized by asplenia and visceral heterotaxy, presents complex congenital anomalies.
  • Congenital pancreatic cysts are rare, and their association with Ivemark II syndrome is exceptionally uncommon.
  • Early diagnosis and management of associated malformations are critical for infant outcomes.

Observation:

  • A neonate presented with Ivemark II syndrome, including situs inversus, asplenia, and complex congenital heart disease.
  • The infant also exhibited a congenital pancreatic cyst with a connection to the biliary tract.
  • The pancreatic cyst was successfully treated with cystoduodenostomy.

Findings:

  • The successful surgical management of the pancreatic cyst highlights a potential therapeutic approach.
  • Despite cyst management, the infant's prognosis was dictated by severe, life-threatening cardiac malformations.
  • This case underscores the significant impact of complex congenital heart disease in Ivemark II syndrome.

Implications:

  • This case emphasizes the importance of recognizing rare syndrome associations in neonates.
  • Effective management of pancreatic cysts in infants with complex congenital conditions is feasible.
  • The study highlights that survival in Ivemark II syndrome is primarily determined by the severity of associated cardiac anomalies.

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