Journal of Pediatric Surgery Case Reports (Aug 2023)

Giant liver cyst in a newborn with pseudoprune-belly syndrome - A case report

  • João Baptista Ormonde,
  • Michelle Patrocínio,
  • Francisco Nicanor Macedo

Journal volume & issue
Vol. 95
p. 102667

Abstract

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Introduction: Pseudoprune-belly syndrome is a rare anomaly, an incomplete form of Prune-belly syndrome. The etiology is not clear, and though this syndrome may present associated with other malformations, the association with a liver cyst is extremely rare. Case presentation: We report the case of a male newborn who was referred to our hospital with an antenatal ultrasound that suggested a gastric duplication. Clinical examination revealed lax abdominal wall, unilateral undescended testis and a palpable mass on the right upper quadrant with fuzzy boundaries. On laparotomy we found a giant hepatic cyst displacing the bowel to the left. The baby underwent a partial ressection and marsupialization of the cyst as total resection was infeasible. The patient's recovery was uneventful. Conclusion: Although rarely, the Pseudoprune-belly syndrome can present with a liver cyst. Large liver cysts should be partially or totally ressected as soon as possible.

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