Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome

Muensterer, Oliver J.; Abellar, Rosanna; Otterburn, David M.; Mathew, Rajamma

doi:10.1055/s-0034-1395986

Cited by 14 publications

(12 citation statements)

References 23 publications

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“…Our patient successfully underwent tissue expander placement which significantly relieved the tracheal stenosis. There are only four other cases where this procedure has been successfully performed [5, 9]. The fact that our patient is currently having reasonable exercise tolerance with a single lung about 4 years after the procedure shows that it may be a good option for these patients.…”

Section: Discussionmentioning

confidence: 88%

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: 88%

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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“…1 Pulmonary agenesis was first discovered by De Pozze in 1673 during an autopsy of an adult woman. 7 It was first described as a clinical entity by Klebs in 1874, when he described it as a 'missing lung'. 8 The exact aetiology of pulmonary agenesis is unknown.…”

Section: Discussionmentioning

confidence: 99%

Unilateral opaque chest radiograph in paediatrics: A case series

et al. 2021

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Pulmonary underdevelopment is a rare congenital disease which manifests as persistent hemithorax opacification at chest radiography. We present three patients with different types of pulmonary underdevelopment, their imaging features and associated anomalies. Case 1 is a premature neonate with persistent respiratory distress. Further imaging confirmed right pulmonary hypoplasia, associated with a patent foramen ovale, patent ductus arteriosus and vertebral anomalies. Case 2 is a 6-year-old child with corrected anorectal malformation, and recurrent pneumonia. Further imaging confirmed left pulmonary aplasia, associated with an aberrant right subclavian artery and vertebral anomaly. Case 3 is a full term neonate who developed excessive drooling of saliva and respiratory distress. Further imaging confirmed right pulmonary agenesis, associated with an atrial septal defect, patent ductus arteriosus and tracheo-oesophageal fistula. Pulmonary underdevelopment is classified into three types: hypoplasia, aplasia and agenesis. The majority of them have associated anomalies. This condition should be considered a differential diagnosis in paediatric patients with an opaque hemithorax on chest radiography.

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“…Pulmonary hypertension, noted in this case, could be secondary to the anatomical shift and the resultant effect on the pulmonary vessels and bronchus. 11 The compression of the lung by abdominal viscera can result in hypoplasia of a developing lung or lung collapse. In pulmonary hypoplasia, there is impaired homeostasis of the immature respiratory epithelium which leads to vascular remodeling (endothelial dysfunction, increased media thickness and increased adventitia thickness), and vasoconstriction, while in mediastinal diseases there is compression of pulmonary vessels caused by the abdominal content pressure against the atelectatic lung in this index case.…”

Section: Discussionmentioning

confidence: 99%

Thoracoabdominal compartment syndrome complicating right-sided diaphragmatic eventration with co-existent unilateral renal agenesis

2020

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Diaphragmatic eventration (DE) associated with intestinal malrotation and renal agenesis is a rare entity. The authors report a case of a 69-year-old man who had symptoms of heart failure. He had a previous imaging diagnosis of right diaphragmatic eventration and dilated cardiomyopathy. He died on the second day after the hospital admission and had a post mortem examination that confirmed complete right diaphragmatic eventration, intestinal malrotation, left renal agenesis, dilated cardiomyopathy, and anteriorly rotated right kidney and had findings suggestive of a thoracoabdominal compartment syndrome. Thoracoabdominal compartment syndrome is described as transmission of abdominal pressure through a defective diaphragm causing compression of the hemithorax viscera and mediastinal shift with a hemodynamic alteration. The association of these anomalies is rare, and the possibility of this finding in a patient with eventration should always be considered.

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Pulmonary Agenesis and Associated Pulmonary Hypertension: A Case Report and Review on Variability, Therapy, and Outcome

Cited by 14 publications

References 23 publications

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

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

Unilateral opaque chest radiograph in paediatrics: A case series

Thoracoabdominal compartment syndrome complicating right-sided diaphragmatic eventration with co-existent unilateral renal agenesis

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