A Note on the Prognosis of Pulmonary Agenesis and Hypoplasia According to the Side Affected

Schaffer, Alexander J.; Rider, Rowland V.

doi:10.1016/s0096-5588(20)30530-4

Cited by 40 publications

(4 citation statements)

References 4 publications

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“…The multivariable analysis revealed that right‐sided agenesis was an independent risk factor for mortality, which is consistent with the results of previous reports 24–27 . Furthermore, the present review identified that right‐sided agenesis was associated with tracheal stenosis.…”

Section: Discussionsupporting

confidence: 92%

Clinical outcomes of pulmonary agenesis: A systematic review of the literature

Fukuoka

Yamamura

Nagata

et al. 2022

Pediatric Pulmonology

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Introduction Pulmonary agenesis is a complete absence of the pulmonary parenchyma, airways, and vasculature unilaterally or bilaterally. Although bilateral cases are lethal, the outcome of unilateral cases remains not well described. We performed a comprehensive literature review to assess the clinical features of pulmonary agenesis. Methods Four database sources were searched on October 10, 2021 and two cases were included from our institution. Studies related to the clinical impact of comorbidity and intervention on the survival outcome in pulmonary agenesis were included for full‐text review. Results We identified 259 patients—with right‐sided (59%), left‐sided (34%), and bilateral agenesis (7%)—among 195 articles and our two cases. Additional anomalies included cardiovascular (40%), skeletal (30%), gastrointestinal (20%), tracheal (20%: all stenoses), and genitourinary (14%) anomalies. Fifty‐seven (24%) individuals in unilateral pulmonary agenesis had isolated disease. Outcomes related to survival were not uniformly reported, but the 2‐year overall survival rate of unilateral agenesis was 62% and no subsequent death was reported until 13 years of age. The right‐sided agenesis was more frequently associated with tracheal stenosis (27% vs. 11%, p = 0.003) than the left‐sided disease. A multivariable analysis indicated that tracheal stenosis (hazard ratio [HR]: 2.2, 95% confidence interval [CI]: 1.3–4.1, p = 0.003) and gastrointestinal anomalies (HR: 2.0, 95% CI: 1.1–3.3, p = 0.010) were prognostic factors for mortality. Conclusions The poor prognostic factors were tracheal stenosis, right agenesis, and gastrointestinal anomalies. Treatment for these comorbidities is a key point for improving the survival of unilateral pulmonary agenesis.

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Section: Discussionsupporting

confidence: 92%

Clinical outcomes of pulmonary agenesis: A systematic review of the literature

Fukuoka

Yamamura

Nagata

et al. 2022

Pediatric Pulmonology

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“…Symptoms result when agenesis is complicated by vascular compression, bronchomalacia, or tracheal stenosis from fused tracheal rings (4). It has been suggested that agenesis of the right lung has a poorer prognosis compared to agenesis of the left lung due to greater mediastinal shift and consequently more kinking of vascular structures (7). Associated malformations of the cardiovascular, skeletal, genitourinary, and gastrointestinal systems have been described in 60% of cases of unilateral lung agenesis (5).…”

Section: Discussionmentioning

confidence: 99%

Adult presentation of right lung agenesis and left pulmonary artery sling

Espinosa

Agarwal

2008

Acta Radiol

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The combination of right lung agenesis and left pulmonary artery (LPA) sling is a rare entity that has been described only in the pediatric population. Cross-sectional imaging modalities such as magnetic resonance imaging (MRI) and computed tomography (CT) have an advantage over echocardiography and pulmonary angiography in demonstrating the anomalous left pulmonary artery, particularly in the presence of coexisting lung agenesis, as exemplified in this case. We report the first case of this rare entity in an adult. It is important to be aware that this abnormality, though rare, can present even in adulthood, and therefore close attention should be paid to the course of the pulmonary artery to ensure detection of a sling in association with lung agenesis.

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“…Unilateral lung agenesis can affect the right or the left side, but right lung agenesis has been found to have a worse prognosis than the left. 2 The presentation can vary from prenatal diagnosis with ultrasound (US) or magnetic resonance imaging (MRI) to a symptomatic infant with severe respiratory failure postnatally. Rarely, unilateral lung agenesis is diagnosed in later life on routine chest imaging.…”

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confidence: 99%

Unilateral Lung Agenesis: A Case Series and Review of Literature

Weber

Sati

Karody

2023

AJP Rep

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Unilateral lung agenesis is a rare congenital abnormality that typically presents with respiratory distress after birth. Prognostic factors include the side of the lung affected along with the presence or absence of other congenital abnormalities. Prenatal imaging can make the diagnosis which can assist the healthcare team in preparing to care for the neonate, as well as set expectations for the family. In this case series, we describe three cases of unilateral lung agenesis, two infants with right lung agenesis and one with the left. We describe their presentation, provide a brief clinical course, and discuss outcomes.

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A Note on the Prognosis of Pulmonary Agenesis and Hypoplasia According to the Side Affected

Cited by 40 publications

References 4 publications

Clinical outcomes of pulmonary agenesis: A systematic review of the literature

Clinical outcomes of pulmonary agenesis: A systematic review of the literature

Adult presentation of right lung agenesis and left pulmonary artery sling

Unilateral Lung Agenesis: A Case Series and Review of Literature

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