Congenital Avalvular Pulmonary Artery and Infantile Lobar Emphysema

Borg, Seth A.; Young, Lionel W.; Roghair, Garry D.

doi:10.2214/ajr.125.2.412

Cited by 13 publications

(1 citation statement)

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“…The cause of the aneurysmal dilatation of the pulmonary artery is uncertain; it may result from increased right ventricular stroke volume caused by pulmonary regurgitation and a left to right shunt through the ventricular septal defect. The dilatation of the pulmonary artery and proximal branches contrasts with the peripheral pulmonary vasculature, that is either normal or slightly increased.12 [38][39][40][41][42][43] Pulmonary vascularity depends upon the degree of stenosis in the pulmonary outflow tract, the presence of a ventricular septal defect, and the anatomical characteristics of the pulmonary artery. Differences in the vascularity of the two lungs were observed in patients with absence of one pulmonary artery.…”

Section: Congenital Absence Ofpulmonary Valve Leafetsmentioning

confidence: 99%

Congenital absence of pulmonary valve leaflets.

Buendı́a¹,

Attié²,

Ovseyevitz³

et al. 1983

Heart

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Section: Congenital Absence Ofpulmonary Valve Leafetsmentioning

confidence: 99%

Congenital absence of pulmonary valve leaflets.

Buendı́a¹,

Attié²,

Ovseyevitz³

et al. 1983

Heart

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Congenital lobar emphysema: Evaluation and long‐term follow‐up of thirty cases at a single center

Özçeli̇k

Göçmen

Ki̇per

et al. 2003

Pediatric Pulmonology

102

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Congenital lobar emphysema (CLE) is characterized by overdistension and air-trapping in the affected lobe, and is one of the causes of infantile respiratory distress. In this report, we review our 27 years of experience with 30 CLE patients. Patients' medical records were evaluated with regard to age, clinical presentation, diagnostic methods, associated diseases, treatment, histopathologic findings, and final clinical and laboratory findings at the end of a long-term period. The mean age of 30 patients (18 male) at diagnosis was 4.9 +/- 6.7 months (range, 2 days-2.5 years). Tachypnea, dyspnea, cough, cyanosis, wheezing, hoarseness, and decreased breath sounds on the affected side were the main symptoms and clinical findings. On chest X-rays, emphysema was seen in all patients; shift/herniation to the opposite lung, atelectasis, and pneumothorax were observed in 16, 5, and 2 cases, respectively. Computerized tomography of the thorax was performed in 16 cases and revealed emphysema at affected lobe/lobes in all, a shift/herniation to the opposite side in 12 cases, and atelectasis of neighbor lobe/lobes in 7 cases. All 8 patients who had perfusion scintigraphy showed reduced perfusion in the affected lobe. Narrowed and flaccid bronchi were detected in one patient by using flexible bronchoscopy. Blood gas analysis was performed in 11 patients, and hypoxia and hypercarbia were revealed in 9 and 7 of these patients, respectively. The most common affected lobe was the left upper lobe (57%), followed by the right upper lobe (30%) and right middle lobe (27%). Two lobes were involved in 4 patients. Associated abnormalities were observed in 5 patients. Twenty-one patients underwent lobectomy; 9 were followed conservatively. Ages at diagnosis were significantly younger in surgically treated patients. Emphysema was detected in all pathological specimens, with an additional bronchial cartilage deficiency in 2 patients. In the surgically treated group, 2 patients died and 2 patients were lost to follow-up. In the conservatively treated group, one patient was lost to follow-up. Mean follow-up duration of all patients was 63.2 +/- 56.2 months (range, 1-209 months). At follow-up visits, all patients were doing well. In surgically treated patients, chest X-rays were normal (9 cases), or showed hyperlucency on the operated side (6 cases) or chronic changes in the operation area (2 cases). Hyperexpansion in the affected lobe was found to be reduced in all cases in the conservatively treated group.

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The clinical and imaging spectrum of findings in patients with congenital lobar emphysema

Stigers

Woodring

Kanga

1992

Pediatric Pulmonology

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Congenital lobar emphysema (CLE) is an important cause of infantile respiratory distress that may require surgical intervention. We retrospectively reviewed the clinical and imaging findings in eight infants with CLE. In our series, CLE was more common in females, predominantly involved the right lung, often presented with an opaque lobe from retained fetal lung fluid, and frequently involved the lower lobes, multiple segments or lobes, and both lungs. Most patients with CLE were diagnosed and managed on the basis of clinical and plain radiographic findings alone. Computed tomography, and occasionally ventilation/perfusion scintigraphy, were helpful in confirming the diagnosis and in guiding management decisions in several cases; bronchoscopy showed that stenosis of the right mainstem bronchus was the cause of CLE in one case. Three patients experienced progressive worsening of respiratory distress and required surgical resection of the affected lobe for cure; the remaining five patients were managed medically with eventual remission of symptoms.

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Congenital Avalvular Pulmonary Artery and Infantile Lobar Emphysema

Cited by 13 publications

References 0 publications

Congenital absence of pulmonary valve leaflets.

Congenital absence of pulmonary valve leaflets.

Congenital lobar emphysema: Evaluation and long‐term follow‐up of thirty cases at a single center

The clinical and imaging spectrum of findings in patients with congenital lobar emphysema

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