Thoracoschisis: case report and review of the literature

  • Dustin L. Eck
  • Brooke N. Maryak
  • Nicholas D. Poulos
  • Joseph J. Tepas III
  • Gohalem G. Felema
  • Daniel K. Robie
Keywords: gastroschisis, limb–body wall complex, thoracoabdominal schisis, thoracoschisis

Abstract

Introduction: Thoracoschisis is a rare congenital malformation characterized by herniation of intraabdominal contents through a thoracic wall defect. There have been six previously reported cases. We describe our novel approach incorporating closure of the chest wall defect with temporary abdominal wall expansion utilizing a silastic pouch.

Case report: A male child born at 29 weeks’ gestation was transferred to our institution for the management of a right anterior chest wall defect with herniation of intraabdominal contents through this defect. The patient was taken to the operating room for reduction of the herniated viscera from the right chest wall defect into the abdomen utilizing a spring-loaded silastic pouch to cover the abdominal viscera.

Discussion: The cause of thoracoschisis is unclear. Multiple mechanisms have been proposed for the development of thoracoschisis, including amnionic rupture, vascular injury, and embryologic maldevelopment. In previously reported cases, a majority of patients had associated limb abnormalities. It has been proposed that this association between extremity agenesis/deformity  and chest wall defects is related to the limb–body wall complex. In addition, most of the cases reported also had an accompanying diaphragmatic defect, allowing the abdominal viscera to enter the chest and then herniate through the thoracic defect.

Conclusion: Overall, thoracoschisis is a very rare congenital abnormality characterized by a chest wall defect with herniation of intra-abdominal organs through this defect. Previously, only six cases have been reported, most of which had an associated limb anomaly or diaphragmatic hernia.

Keywords: gastroschisis, limb–body wall complex, thoracoabdominal schisis, thoracoschisis

Published
2018-03-27
Section
Articles

Journal Identifiers


eISSN: 1687-4137
print ISSN: 1687-4137