Histopathologic features of alveolar capillary dysplasia with misalignment of pulmonary veins with atypical clinical presentation

•Atypical alveolar capillary dysplasia with misalignment of pulmonary veins.•Clinical presentation does not always correlate with the histopathologic changes.•Abnormal pulmonary veins and septal vessels may be diagnostic clues in atypical form. Alveolar capillary dysplasia with misalignment of pulmo...

Full description

Saved in:
Bibliographic Details
Published in:Cardiovascular pathology 2021-01, Vol.50, p.107289-107289, Article 107289
Main Authors: Alturkustani, Murad, Li, Duo, Byers, Joshua T., Szymanski, Linda, Parham, David M., Shi, Wei, Wang, Larry L.
Format: Article
Language:English
Subjects:
Aberrant arteriovenous vessels
Alveolar capillary dysplasia
Atypical features
Autopsy
Female
Gestational Age
Humans
Male
Misalignment of pulmonary veins
Persistent Fetal Circulation Syndrome - mortality
Persistent Fetal Circulation Syndrome - pathology
Prognosis
Pulmonary Alveoli - abnormalities
Pulmonary Alveoli - pathology
Pulmonary Veins - abnormalities
Retrospective Studies
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:•Atypical alveolar capillary dysplasia with misalignment of pulmonary veins.•Clinical presentation does not always correlate with the histopathologic changes.•Abnormal pulmonary veins and septal vessels may be diagnostic clues in atypical form. Alveolar capillary dysplasia with misalignment of pulmonary veins (ACD/MPV) is a rare neonatal lung disease with fatal outcome. Typically, respiratory symptoms present in the first 24 hours of life and patients die within the neonatal period. Atypical, delayed clinical presentations and/or longer survival have also been reported. Here, we studied the clinicopathologic relationship of ACD/MPV by examining 16 cases of ACD/MPV, focusing on atypical features. Based on the presence of diffuse vs. focal/patchy ACD/MPV histopathologic changes, we divided the cases into classic and nonclassic pathology groups. MPV was found in all ACD/MPV. Ten of 16 cases exhibited classic diffuse abnormalities, while 6 of 16 had a nonclassic focal/patchy distribution. However, among 7 patients with atypical clinical features, only 2 had nonclassic pathology, while 4 out of 9 clinically typical cases had nonclassic ACD/MPV pathology. Marked intrapulmonary aberrant arteriovenous vessels were present in all atypical cases. In conclusion, clinical presentation is not always correlated with histopathology in ACD/MPV. Atypical ACD/MPV should be suspected in any infants with fulminant pulmonary hypertension. Abnormal pulmonary veins and aberrant intraseptal vessels are the most important clues for diagnosis. Additional studies are needed for further elucidation of diagnostic histological criteria of atypical ACD/MPV and to explore its pathogenesis.
ISSN:1054-8807
1879-1336
DOI:10.1016/j.carpath.2020.107289