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PEDIATRICS Vol. 113 No. 3 March 2004, pp. e256-e258

ELECTRONIC ARTICLE

Congenital Diaphragmatic Hernia and Situs Inversus Totalis

Coranne D. Tesselaar, MD*, Roelf R. Postema, MD*, Marieke F. van Dooren, MD*, Karel Allegaert, MD{ddagger} and Dick Tibboel, MD, PhD*

* Department of Pediatric Surgery, Sophia Children’s Hospital, Rotterdam, The Netherlands
{ddagger} Department of Neonatology, University Hospital, Leuven, Belgium

Abbreviations: ECMO, extracorporeal membrane oxygenation • VA-ECMO, venoarterial ECMO

The first 20% of the full text of this article appears below.

Congenital diaphragmatic hernia is a relatively rare disorder (1:3000 newborns) that frequently presents with respiratory distress in the immediate neonatal period due to severe pulmonary hypertension and lung hypoplasia. Extracorporeal membrane oxygenation (ECMO) can be used as a last resort when artificial ventilation and/or modulation of the pulmonary vascular tone fail to improve the clinical condition.

Situs inversus totalis is a rare condition in which orientation of all asymmetrical organs in the body is a mirror image of the normal morphology.1 Diaphragmatic hernia may be caused by predisposing genes that are involved in left-right axis determination.

A few cases of an eventration of the diaphragm combined with situs inversus totalis have been described in the literature. We report for the first time a patient with a right-sided posterolateral diaphragmatic hernia, type Bochdalek, and a situs inversus totalis for which contralateral cannulation for the institution of venoarterial ECMO (VA-ECMO) was warranted.


   CASE REPORT
 
A girl with a birth weight of 3810 g, born at the postmenstrual age of 39 + 6 weeks, developed acute respiratory distress, cyanosis, and lethargy within 30 minutes after birth. Physical examination revealed a scaphoid abdomen and decreased breathing sounds at the right side of the thorax. A chest radiograph revealed that the heart and mediastinum were displaced to the left and that the right pleural cavity was filled with intestines (Fig 1). Ultrasound confirmed that the right hemithorax was almost completely filled with loops of intestine, mesenterial fat, . . . [Full Text of this Article]

Address correspondence to Dick Tibboel, MD, PhD, Head Pediatric Surgical Intensive Care Unite, Erasmus MC-Sophia, Dr Molewaterplein 60, 3015 GJ Rotterdam, The Netherlands. E-mail: illsley@chis.azr.nl