Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity

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Stéphanie Cupers
Christine Van Linthout
Brigitte Desprechins
Léon Rausin
Martine Demarche
Marie-Christine Seghaye *
(*) Corresponding Author:
Marie-Christine Seghaye | mcseghaye@chu.ulg.ac.be

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 

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