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Situs inversus totalis with pleural empyema: a clue to ciliary dysfunction
Suraj Pai1, Anusha Induraj2, Aparna Mohan K1
1Cardiovascular and Thoracic Surgery, Kasturba Medical College Mangalore, Manipal Academy of Higher Education, Mangaluru, Karnataka, India.
Situs inversus totalis (SIT) can indicate primary ciliary dyskinesia (PCD), a condition causing recurrent lung infections. Early recognition of SIT aids in diagnosing PCD and preventing severe pulmonary complications like empyema.
Area of Science:
- Medical Genetics
- Pulmonology
- Congenital Anomalies
Background:
- Situs inversus totalis (SIT) is a rare congenital condition with complete organ reversal.
- SIT is frequently associated with primary ciliary dyskinesia (PCD), a disorder of mucociliary clearance.
- Delayed diagnosis of PCD can lead to severe, progressive pulmonary damage.
Purpose of the Study:
- To highlight the association between SIT and PCD.
- To emphasize the importance of recognizing SIT as an early diagnostic clue for PCD.
- To underscore the need for timely intervention in patients with suspected ciliary dysfunction.
Main Methods:
- Case report of an adolescent male with SIT presenting with left-sided pleural empyema.
- Clinical evaluation including imaging and pleural fluid analysis.
- Surgical intervention: thoracotomy with decortication for trapped lung and empyema.
Main Results:
- The patient presented with symptoms suggestive of underlying ciliary dysfunction.
- Conservative management was unsuccessful, necessitating surgical intervention.
- Thoracotomy with decortication successfully re-expanded the lung and treated the empyema.
Conclusions:
- Situs inversus totalis serves as a crucial phenotypic marker for primary ciliary dyskinesia.
- Early identification of SIT can facilitate prompt diagnosis and management of PCD.
- Proactive intervention is essential to mitigate long-term pulmonary morbidity in patients with PCD.
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