Hypertensive Vascular Changes in Sequestration

Plexogenic angiopathy has been described in resected sequestrations from children as young as 3 years old [1], and in patients with systemic arteries to normal lung in infants as young as 8 months [2]. These changes implicate systemic pressure as a cause.

artery

Figure 1. Hypertensive Vascular Changes

This bronchiolovascular bundle in a sequestration from a 28-year-old shows medial thickening of the pulmonary artery.

Find the pulmonary artery with thickened media.

vessplex

Figure 2. Plexiform Lesion

Other vessels in this sequestration show mural destruction and extravascular proliferation of endothelial cells (plexiform lesion) in a setting of chronic inflammation and fibrosis.

Outline the artery and plexiform lesion.

plexplex

Figure 3. Elastic Tissue Stain of Plexiform Lesion

An elastic tissue stain (black) of figure 2 shows focal disruption of the elastic layers of 2 cuts of a vessel. The lower portion of the vessel is narrowed by intimal proliferation. An ill-defined nodule (plexiform lesion) lies adjacent to, and above, the parent artery.

Outline the plexiform lesion.

Figure 4. Fibrinoid Necrosis of Artery

Another artery shows homogeneous pink necrosis (fibrinoid necrosis) of the wall of an artery and a focal acute inflammatory infiltrate.

These changes are similar to those seen in other forms of plexogenic angiopathy and reflect the response to prolonged systemic pressure in the lung.

References: To return to reference section after viewing abstract, click here before clicking on "abstract".

1. Tandon M, Warnock M. Plexogenic angiopathy in pulmonary intralobar sequestrations: pathogenetic mechanisms. Hum Pathol 1993; 24:263-273. Abstract

2. Holder P, Langston C. Intralobar pulmonary sequestration (a nonentity?). Pediatr Pulmonol 1986; 2:147-153. Abstract

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