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Pulmonary sequestration and related congenital disorders. A clinico-radiological review of 41 cases
P R John1, S W Beasley, V Mayne
1Department of Radiology, Royal Children's Hospital, Melbourne, Australia.
Insights
Pulmonary sequestration is a complex lung disorder with varied presentations. Early radiologist suspicion is key for diagnosing this condition in children, especially outside infancy.
Area of Science:
- Pediatric Radiology
- Thoracic Surgery
- Pulmonology
Background:
- Pulmonary sequestration is a spectrum of congenital lung malformations.
- It involves abnormal bronchopulmonary, arterial, and venous structures.
- Understanding its varied presentations is crucial for diagnosis.
Purpose of the Study:
- To review cases of pulmonary sequestration in children.
- To highlight the diverse clinical and imaging features.
- To emphasize the importance of early diagnosis and appropriate imaging.
Main Methods:
- Retrospective review of 41 children diagnosed with pulmonary sequestration.
- Analysis of clinical presentations and imaging findings.
- Evaluation of the role of angiography and alternative imaging techniques.
Main Results:
- Pulmonary sequestration presented ubiquitously, with "classical" features in 49% of cases.
- Diagnosis outside infancy requires a high index of suspicion.
- Angiography effectively demonstrated anomalous systemic arterial supply.
Conclusions:
- Pulmonary sequestration has diverse presentations, not always fitting the classical description.
- Radiologists must maintain a high suspicion for early diagnosis in pediatric patients.
- Angiography remains valuable for identifying anomalous arterial supply in suspected cases.
Abstract:
Forty-one children with pulmonary sequestration complex were reviewed. This disease is a spectrum of disorders involving the bronchopulmonary airway, the arterial supply to the lungs, the lung parenchyma and its venous drainage. The review showed pulmonary sequestration to be ubiquitous in its presentation: "classical" features of sequestration occurred in 20 (49%) children studied. The early diagnosis of children with sequestration outside infancy demands a high index of suspicion by the radiologist. The role of angiography in demonstrating an anomalous systemic arterial supply to the lung in suspected bronchial and pulmonary arterial sequestration is described in relation to alternative imaging techniques available.