Abstract
Situs inversus totalis (SIT) is a rare condition of complete inversion and mirroring of normal human anatomy. The incidence is approximately 1 in 8,000 to 1 in 25,000 live births. SIT is inherited in an autosomal recessive pattern and is associated with multiple gene mutations. It is also commonly seen in a condition known as primary ciliary dyskinesia.A 39-year-old pregnant woman presented to the Labor and Delivery unit to rule out pre-eclampsia due to high blood pressure recordings in the office setting. The infant was delivered preterm at 36 weeks gestation via spontaneous vaginal delivery. The infant presented with symptoms of respiratory distress. The newborn was transferred to the neonatal intensive care unit (NICU) for further work-up and to rule in/rule out an etiology known as Wet Lung. Upon retrieving a chest X-ray for the newborn, the results demonstrated situs inversus totalis. The newborn was transferred to a level III NICU for further management and work-up for other potential etiologies. Situs inversus totalis was not seen on prenatal work-up.In summary, situs inversus totalis is a rare condition which can be associated with other detrimental conditions. In the future, if situs inversus totalis is detected in utero, patients should be instructed to deliver in a setting in which any possible etiology can be accommodated. Pediatricians should follow these infants closely and with caution as common presentations may be obscured due to complete inversion of normal human anatomy. It is also important to screen these infants for other etiologies which may present in later developmental stages such as bronchiectasis and respiratory infections.
Highlights
Situs inversus totalis (SIT) is a rare condition that is defined by the complete positional inversion of cardiac and abdominal viscera
Situs inversus is closely associated with another condition termed primary ciliary dyskinesia (PCD)
Situs inversus with dextrocardia is commonly accompanied by the following congenital cardiac anomalies: atrial situs solitus, discordant atrioventricular (AV) connection, discordant ventriculoatrial (VA) connection, and to a lesser extent congenitally corrected transposition of great arteries (TGA) [6]
Summary
Situs inversus totalis (SIT) is a rare condition that is defined by the complete positional inversion of cardiac and abdominal viscera. In situs inversus totalis, the apex points towards the right due to the positional mirroring of the normal anatomy. This is termed situs inversus with dextrocardia. These findings are suggestive of dextrocardia with situs inversus. The findings demonstrated an opacity in the left superior mediastinum which could be secondary to the rotation of the patient and the thymic shadow Preceding these findings, the infant was transferred to a level III NICU for further observation and work-up regarding this rare etiology
Sign-in/Register to view highlights & summary Talk to us
Join us for a 30 min session where you can share your feedback and ask us any queries you have
Schedule a callDisclaimer: All third-party content on this website/platform is and will remain the property of their respective owners and is provided on "as is" basis without any warranties, express or implied. Use of third-party content does not indicate any affiliation, sponsorship with or endorsement by them. Any references to third-party content is to identify the corresponding services and shall be considered fair use under The CopyrightLaw.
