Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity

  • Stéphanie Cupers Department of Pediatrics, University Hospital Liège, Liège, Belgium.
  • Christine Van Linthout Department of Gynecology and Obstetrics, University Hospital Liège, Liège, Belgium.
  • Brigitte Desprechins Medical Imaging, University Hospital Liège, Liège, Belgium.
  • Léon Rausin Department of Medical Imaging, Regional Hospital Center La Citadelle, Liège, Belgium.
  • Martine Demarche Department of Surgery, Regional Hospital Center La Citadelle, Liège, Belgium.
  • Marie-Christine Seghaye | mcseghaye@chu.ulg.ac.be Department of Pediatrics, University Hospital Liège, Liège, Belgium.

Abstract

Heterotaxy syndrome is a situs anomaly that comprises a large spectrum of cardiac and extracardiac malformations. Its association with intestinal malrotation is frequent. This later might be asymptomatic or manifest by signs of abdominal discomfort or of intestinal obstruction. We report on the case of a full term, small for gestational age neonate in whom heterotaxy syndrome with partial situs inversus abdominalis, intestinal malrotation, polysplenia and vena azygos continuity was diagnosed at prenatal echography. Due to the high risk of volvulus carried by the malrotation, Ladd’s procedure was performed at the age of two months. Follow-up examination at the age of four years shows excellent post-operative result and normal development of the child.

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Author Biographies

Stéphanie Cupers, Department of Pediatrics, University Hospital Liège, Liège

Department of Pediatrics

Christine Van Linthout, Department of Gynecology and Obstetrics, University Hospital Liège, Liège
Department of Gynecology and Obstetrics
Brigitte Desprechins, Medical Imaging, University Hospital Liège, Liège
Department of Medical Imaging
Léon Rausin, Department of Medical Imaging, Regional Hospital Center La Citadelle, Liège
Department of Medical Imaging
Martine Demarche, Department of Surgery, Regional Hospital Center La Citadelle, Liège
Department of Surgery
Marie-Christine Seghaye, Department of Pediatrics, University Hospital Liège, Liège

Department of Pediatrics 

Published
2018-01-08
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Section
Case Reports
Keywords:
Heterotaxy, intestinal malrotation, azygos continuity.
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How to Cite
Cupers, S., Van Linthout, C., Desprechins, B., Rausin, L., Demarche, M., & Seghaye, M.-C. (2018). Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clinics and Practice, 8(1). https://doi.org/10.4081/cp.2018.1004

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