Bilateral Pulmonary Agenesis: A Rare and Unexpected Finding in a Newborn

Nguyen, Lananh N.; Parks, W. Tony

doi:10.1055/s-0036-1584530

Cited by 4 publications

(5 citation statements)

References 10 publications

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“…Suspicion on BPA is usually done by ultrasound examination and diagnosis made by MRI in the second and the third trimester of pregnancy [11]. Unfortunately, it is not rare that BPA can be missed on routine prenatal screening [2]. As BPA is incompatible with extrauterine life, there is a need to make a reliable diagnosis in pregnancy at the earliest.…”

Section: Discussionmentioning

confidence: 99%

“…Congenital lung malformations can be identified in the immediate birth period or in early childhood; however, they can be diagnosed incidentally on routine imaging or autopsy [1]. Bilateral pulmonary agenesis (BPA) is a very rare congenital lung malformation incompatible with extra-uterine life [2]. BPA is usually suspected during a prenatal US scan and diagnosed by fetal MRI, in the second and third trimesters [3][4][5].…”

Section: Introductionmentioning

confidence: 99%

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Bilateral pulmonary agenesis diagnosed in the 13 th week of gestation - a case report and literature review

Mihailovic¹,

Kuanova²,

Terzić³

et al. 2019

Clin. Exp. Obstet. Gynecol.

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Bilateral pulmonary agenesis (BPA) is a rare congenital lung malformation incompatible with extra-uterine life, diagnosed by various prenatal imaging modalities in the second and third trimesters. Essentials for diagnosis are complete absence of lungs and bronchi and no pulmonary vascular supply. In the present case, both convex and vaginal ultrasound scan performed at 13 th GW revealed an anechoic content in the entire fetal thorax with no evidence of pericardial effusion, fetal heart diseases, or any other accompanying congenital malformations. On three-vessel view (3VV), bifurcation of pulmonary artery could not be identified, which confirmed the diagnosis of BPA. In conclusion, clinical importance of combining the up-to-date technological tools and clinical expertise in establishing the diagnosis as early as in the first trimester allows the clinicians to terminate the pregnancy with the lowest incidence rate of eventual complications related to the procedure. According to the literature data, this is the first and therefore unique case of BPA diagnosed in the 13 th week of gestation.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Bilateral pulmonary agenesis diagnosed in the 13 th week of gestation - a case report and literature review

Mihailovic¹,

Kuanova²,

Terzić³

et al. 2019

Clin. Exp. Obstet. Gynecol.

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“…Esta condición consiste en la ausencia de bronquios, parénquima y vasculatura pulmonar 2,7,10,13,14 , pudiendo ser unilateral o bilateral 7,15 , siendo la primera mas frecuente, la agenesia derecha se asocia a mayores complicaciones 7 , defectos cardiovasculares (14%), musculoesqueléticos (12%), gastrointestinales, (14%) genitourinarios (9%), vasculares (9%). Puede aparecer de forma aislada o hacer parte de una entidad clínica como por ejemplo, sín-drome de Goldenhar, asociación VACTER, síndrome por microdeleción 22q11, síndrome de Holt-Oram y síndrome de Opitz G 1,4,6,[9][10][11][12]16 .…”

Section: Discussionunclassified

“…Uno de los casos reportados de agenesia pulmonar bilateral fue una paciente prematura de 29 semanas, con ecografía prenatal dentro de limites normales, que nace sin esfuerzo respiratorio y en el que no se logra intubación endotraqueal por vía aérea difícil. La paciente falleció a los 26 min y en la necropsia se documentó agenesia pulmonar bilateral con tráquea de 1 cm blindada al final, una condición incompatible con la vida 15 .…”

Section: Discussionunclassified

Asociación LACHT con hipoplasia de la vía aérea superior. Caso clínico

Gómez

Rodríguez²,

Carrero

2017

Rev. chil. pediatr.

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“…85,87 This is relevant for the study of human CDH, as there have been a few reported cases of pulmonary agenesis in humans that have intact diaphragms. 86,[88][89][90] Although genetics can account for only a small percentage of CDH cases in humans, seminal studies analyzing genes like COUP-TFII (NR2F2) have suggested that screening and genetic status should be considered when performing a differential diagnosis of CDH. 91 Integrating genetic data and the lessons learned from animal models of CDH has provided many insights into the pathways that are disrupted in CDH and is a crucial step in translating novel therapies from bench to bedside.…”

Section: Genetic Modelsmentioning

confidence: 99%

Congenital Diaphragmatic Hernia: State of the Art in Translating Experimental Research to the Bedside

et al. 2019

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Congenital diaphragmatic hernia (CDH) is a devastating disease that still carries a high mortality and morbidity rate. Poor outcomes for fetuses and infants with CDH are mainly related to pulmonary hypoplasia (PH) and pulmonary vascular remodeling that leads to pulmonary hypertension (PHTN). Over the last five decades, research efforts have focused on modeling CDH not only to study the pathophysiology of the diaphragmatic defect, pulmonary hypoplasia, and pulmonary hypertension, but also to identify therapies that would promote lung growth and maturation, and correct vascular remodeling. As CDH is a multifactorial condition whose etiology remains unknown, there is not a single model of CDH, rather several ones that replicate different aspects of this disease. While small animals like the mouse and the rat have mainly been used to uncover biological pathways underlying the diaphragmatic defect and poor lung growth, larger animals like the lamb and the rabbit models have been instrumental for pursuing medical and surgical interventions. Overall, the use of animal models has indeed advanced our knowledge on CDH and helped us test innovative therapeutic options. For example, the lamb model of CDH has been the paradigm for testing fetal surgical procedures, including tracheal occlusion, which has been translated to clinical use. In this review, we outline the induction protocols of CDH in animals with the use of chemicals, dietary changes, genetic alterations, and surgical maneuvers, and we describe the studies that have translated experimental results to the bedside.

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Bilateral Pulmonary Agenesis: A Rare and Unexpected Finding in a Newborn

Cited by 4 publications

References 10 publications

Bilateral pulmonary agenesis diagnosed in the 13 th week of gestation - a case report and literature review

Bilateral pulmonary agenesis diagnosed in the 13 th week of gestation - a case report and literature review

Asociación LACHT con hipoplasia de la vía aérea superior. Caso clínico

Congenital Diaphragmatic Hernia: State of the Art in Translating Experimental Research to the Bedside

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