Isolated Unilateral Pulmonary Agenesis

Gabarre, Juan Arbués; Izquierdo, Alberto Galindo; Ponferrada, M. Rasero; Gallardo, C. Orbea; Agueda, José Manuel Puente; Pérez, Pedro de la Fuente

doi:10.7863/jum.2005.24.6.865

Cited by 29 publications

(3 citation statements)

References 15 publications

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“…Gabarre et al found a 50% mortality rate by the age of 5 years in the patient cohort [7]. 50% of patients with right lung agenesis also have anomaly affecting at least one other organ system.…”

Section: Discussionmentioning

confidence: 99%

Right Lung Agenesis with Tracheal Stenosis due to Complete Tracheal Rings and Postpneumonectomy Like Syndrome Treated with Tissue Expander Placement

Agrawal

Patri

Kalavakunta

2016

Case Reports in Pulmonology

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Congenital lung agenesis is an extremely rare condition with an estimated prevalence of 34 in 1,000,000 live births. It is often associated with other congenital malformations of the skeletal, cardiovascular, urogenital, and gastrointestinal systems. We discuss the case of a 5-month-old who presented with increasing stridor over 1 month. Imaging revealed right lung agenesis, complete dextromalposition of heart, and compression of distal trachea. An intrathoracic saline tissue expander was placed which marked improved distal tracheal stenosis. In patients who are symptomatic it becomes imperative to perform surgeries to increase survival as was the case in this patient.

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Section: Discussionmentioning

confidence: 99%

Right Lung Agenesis with Tracheal Stenosis due to Complete Tracheal Rings and Postpneumonectomy Like Syndrome Treated with Tissue Expander Placement

Agrawal

Patri

Kalavakunta

2016

Case Reports in Pulmonology

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“…Color Doppler scans are of great value in detecting the pulmonary vasculature prenatally; even in the first trimester (6). The prenatal differentiation between BPA and other thoracic abnormalities, including congenital diaphragmatic hernia (CDH), congenital cystic adenomatoid malformation (CCAM), and pulmonary sequestration, is clinically important because the management and postnatal outcome are different; however, it is very difficult to do so because of sonographically-similar features, especially when the diaphragm cannot be visualized (2).…”

Section: Discussionmentioning

confidence: 99%

Prenatal Diagnosis of Bilateral Pulmonary Agenesis: a Case Report

et al. 2010

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We report a case of bilateral pulmonary agenesis (BPA), which was suspected during a prenatal US examination and diagnosed by fetal magnetic resonance imaging (MRI). BPA is an extremely rare congenital anomaly and, although many fetal structural defects can be detected with a high degree of confidence after introducing high-resolution US, the prenatal diagnosis of BPA remains problematic. Other thoracic abnormalities, such as a congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, and pulmonary sequestration, should be excluded from the list of possible diagnoses before coming to the conclusion of BPA, because BPA is absolutely incompatible with extrauterine life, and an accurate internal diagnosis can prevent a futile intervention from being performed.

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“…Pulmonary agenesis is a rare congenital malformation in which there is total absence of the bronchial tree, alveolar parenchyma, pulmonary and bronchial vasculature. [1]…”

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confidence: 99%