Late Onset Congenital Cystic Adenomatoid Pulmonary Malformation

Congenital cystic adenomatoid pulmonary malformations are defined as the presence of a cystic mass of undifferentiated lung tissue; a third of the cases affect one of the lower lobes, and multilobar or bilateral affection is rare. The entity is a rare congenital malformation in the lungs, with an incidence of one in 11,000 to one in 35,000 live births. It is mainly discovered during the fetal period, although a low percentage of patients will be diagnosed postnatally. The disease has a wide spectrum of clinical manifestations, from asymptomatic to life-threatening respiratory symptoms. The diagnosis is made by imaging studies, and the treatment depends on the patient's clinical status. We reported the case of an infant with a rare congenital pulmonary airway malformation and a late diagnosis, which makes the case even rarer, who required open lobectomy as treatment. Keywords: Congenital abnormalities; cystic adenomatoid malformation of lung; lung diseases.

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Publication Details

Journal
Journal of Nepal Health Research Council
Published
2025-10-17
DOI
https://doi.org/10.33314/jnhrc.v23i02.4809
Primary Topic
Congenital Diaphragmatic Hernia Studies
Type
article
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article

Late Onset Congenital Cystic Adenomatoid Pulmonary Malformation

Lorena García Agudelo, Alejandro Rojas Urrea, Duván Felipe Velandia Siabato, Natalia Sánchez Coronel et al.
Journal of Nepal Health Research Council
Congenital Diaphragmatic Hernia Studies
article

Late Onset Congenital Cystic Adenomatoid Pulmonary Malformation

Lorena García Agudelo, Alejandro Rojas Urrea, Duván Felipe Velandia Siabato, Natalia Sánchez Coronel, Daniela Marino, Linette Marliece Olivares Calderon
article en

Abstract

Congenital cystic adenomatoid pulmonary malformations are defined as the presence of a cystic mass of undifferentiated lung tissue; a third of the cases affect one of the lower lobes, and multilobar or bilateral affection is rare. The entity is a rare congenital malformation in the lungs, with an incidence of one in 11,000 to one in 35,000 live births. It is mainly discovered during the fetal period, although a low percentage of patients will be diagnosed postnatally. The disease has a wide spectrum of clinical manifestations, from asymptomatic to life-threatening respiratory symptoms. The diagnosis is made by imaging studies, and the treatment depends on the patient's clinical status. We reported the case of an infant with a rare congenital pulmonary airway malformation and a late diagnosis, which makes the case even rarer, who required open lobectomy as treatment. Keywords: Congenital abnormalities; cystic adenomatoid malformation of lung; lung diseases.

Journal of Nepal Health Research CouncilVol. 23(02)
Regional Health (US)
Openalex Percentile: Top 50%
Congenital Diaphragmatic Hernia Studies
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