First reported case of Pentalogy of Cantrell variant with good outcome in a 32 week gestational age monozygotic twin with twin-twin transfusion syndrome

Preethi Srinivasakumar, Ganapathy Rama, Nathan Jesse, Rogers G. Howell, Joseph Lawrence Brady, Tania Tejera Morales, Hima Maramreddy, Jenna Hogan, Graham H. Cosper, Thomas Schmelzer, Daniel A. Bambini, Andrew Schulman, Jay Kothadia

Research output: Contribution to journalArticle

Abstract

We report a case of a 32 week gestational age preterm monozygotic twin neonate with a rare variant of Pentalogy of Cantrell who presented with severe respiratory distress syndrome, possible pulmonary hypoplasia, large congenital diaphragmatic hernia, large omphalocele, and atrial septal defect. Pentalogy of Cantrell poses a unique challenge for both neonatologists and surgeons, as it has a high mortality and morbidity rate. Prognosis of newborns with Pentalogy of Cantrell depends upon severity of both cardiac and extracardiac defects, and other associated anomalies. This report describes an extremely rare variant of the Pentalogy of Cantrell spectrum in a patient with a co-existent twin – twin transfusion syndrome and transient hydrops fetalis in utero, who survived with favorable outcome.

Original languageEnglish (US)
Article number101321
JournalJournal of Pediatric Surgery Case Reports
Volume51
DOIs
StatePublished - Dec 2019

Keywords

  • Giant omphalocele
  • Monochornionic twin
  • Pentalogy of cantrell
  • Prematurity

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Surgery

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    Srinivasakumar, P., Rama, G., Jesse, N., Howell, R. G., Brady, J. L., Morales, T. T., Maramreddy, H., Hogan, J., Cosper, G. H., Schmelzer, T., Bambini, D. A., Schulman, A., & Kothadia, J. (2019). First reported case of Pentalogy of Cantrell variant with good outcome in a 32 week gestational age monozygotic twin with twin-twin transfusion syndrome. Journal of Pediatric Surgery Case Reports, 51, [101321]. https://doi.org/10.1016/j.epsc.2019.101321