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Congenital Thoracic Malformations: A Single-Center Retrospective Study From Hong Kong
Chung Yan Michelle Lam1, Shu Yan David Lam1, Kin-Hoi Thung2
1Department of Paediatrics and Adolescent Medicine, Tuen Mun Hospital, Hong Kong, HKG.
Insights
Congenital thoracic malformations (CTMs) often have an indolent course. A conservative management approach with delayed surgery may be suitable for asymptomatic CTMs, including congenital pulmonary airway malformation (CPAM).
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Developmental Biology
Background:
- Congenital thoracic malformations (CTMs) are developmental lung anomalies, with congenital pulmonary airway malformation (CPAM) being the most common subtype.
- Management strategies for asymptomatic CTMs remain a subject of debate.
- This study reviews CTMs in a Chinese population to inform clinical decision-making.
Purpose of the Study:
- To analyze the clinical course, management options, and outcomes of congenital thoracic malformations (CTMs).
- To provide evidence-based guidance for the future management of CTMs, particularly in asymptomatic cases.
Main Methods:
- Retrospective review of electronic medical records from Tuen Mun Hospital, Hong Kong (2002-2024).
- Inclusion of all cases with computed tomography (CT)-confirmed CTM diagnosis.
- Categorization into antenatally diagnosed (AN) and postnatally diagnosed (PN) groups for comparative analysis.
Main Results:
- A cohort of 45 CTM cases was analyzed (18 AN, 27 PN).
- Most AN cases (61.1%) remained asymptomatic; 48.2% of PN cases were incidental findings.
- Pneumonia was the most frequent complication (33.3% AN, 59.3% PN), with a low incidence of malignancy (one case of lymphoepithelioma-like carcinoma in bronchopulmonary sequestration).
Conclusions:
- CTMs exhibit a relatively indolent and benign clinical course.
- A conservative management approach, including delayed surgical intervention, is a viable alternative for asymptomatic CTMs.
- Findings support individualized management strategies based on clinical presentation and diagnosis.
Abstract:
Background Congenital thoracic malformations (CTMs) are a group of developmental lung anomalies, with congenital pulmonary airway malformation (CPAM) being the most prevalent subgroup of all. There had been controversies over the management approaches in asymptomatic CTMs. The study aims to review the clinical course, management options, and outcomes of CTMs in a Chinese population to provide guidance in the future management of the disease. Methods This retrospective study reviewed electronic medical records of Tuen Mun Hospital, Hong Kong, from 2002 to 2024 to include all cases with the diagnosis of CTM confirmed through computed tomography (CT). Cases were categorised into the antenatally diagnosed (AN) group and postnatally diagnosed (PN) group for further analysis. Results The cohort analyzed 45 cases, of which 18 belonged to the AN group and 27 belonged to the PN group. The majority of patients in the AN group remained asymptomatic throughout follow-up (61.1%); 48.2% of the PN group presented as incidental findings. Pneumonia was the most common complication, affecting 33.3% of the AN group and up to 59.3% of the PN group. Most of the patients had a single uncomplicated pneumonia (AN group: 83.3%; PN group: 75%). Surgeries were performed in 44.4% of the AN group and 66.7% of the PN group, with notable postoperative complications observed (AN: 25%, PN: 22.2%). Importantly, only one case of lymphoepithelioma-like carcinoma in a patient with bronchopulmonary sequestration (BPS) was identified, and no malignancies were found in other CTM entities. Conclusion In view of the relatively indolent and benign course found in this study, a conservative approach with surgery at a later age could be an alternative to early surgery in asymptomatic CTMs.
