Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate

Rana, Nisha; Dalal, Poonam; Lohchab, Shamsher Singh; Singh, Sandeep; Kumar, Basant; Singh, Sunita

doi:10.1007/s12055-023-01510-x

Indian J Thorac Cardiovasc Surg

2023

DOI: 10.1007/s12055-023-01510-x

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Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate

Nisha Rana

Poonam Dalal

Shamsher Singh Lohchab

et al.

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2024

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Congenital Pulmonary Airway Malformation in a 1-Month-Old Infant: A Call for Strengthening Antenatal Diagnosis

Ngalya,

Jackson,

Khamis

et al. 2024

Preprint

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Background Congenital pulmonary airway malformation (CPAM) is a rare condition that poses diagnostic and management challenges because of a low index of suspicion. Most reported cases of CPAM are diagnosed postnatally via chest X-ray and/or chest CT scan or MRI, despite the high diagnostic efficacy of obstetric ultrasound. The management of symptomatic CPAM is surgical excision. Case details This is a case report of a delayed diagnosis of a 1-month-old male infant who presented with signs of respiratory distress since week one of life associated with one episode of fever and a history of being treated for pneumonia with no improvement. On admission to our tertiary unit, the infants’ preliminary differential diagnoses were severe pneumonia, suspected left-sided congenital diaphragmatic hernia or acyanotic congenital heart defect. A chest X-ray revealed a large left-sided cystic lesion with a pressure effect on the left lung and a mediastinal shift to the right side of the chest, which was confirmed by a CT scan of the chest. Left lower lobectomy was performed, resulting in significant improvement with complete resolution of the signs of respiratory distress by postoperative day 13. Conclusion A low index of suspicion among healthcare practitioners can lead to significant morbidity in infants with a delayed diagnosis of CPAM. Postnatal chest X-ray and CT scans play crucial roles in confirming the diagnosis, assessing the extent of the lesion, and guiding the surgical approach in our case, resulting in a favorable outcome. Prenatal diagnosis is still the cornerstone for early detection and early intervention.

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Congenital Pulmonary Airway Malformation in a 1-Month-Old Infant: A Call for Strengthening Antenatal Diagnosis

Ngalya,

Jackson,

Khamis

et al. 2024

Preprint

View full text Add to dashboard Cite

show abstract

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Congenital pulmonary airway malformation manifesting with severe respiratory distress and pneumothorax in a neonate

Cited by 1 publication

References 8 publications

Congenital Pulmonary Airway Malformation in a 1-Month-Old Infant: A Call for Strengthening Antenatal Diagnosis

Congenital Pulmonary Airway Malformation in a 1-Month-Old Infant: A Call for Strengthening Antenatal Diagnosis

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