An Autopsy Case of Idiopathic Pleuroparenchymal Fibroelastosis with Left Vocal Cord Paralysis and a Rapid Deterioration without an Acute Exacerbation

Abstract

Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a generally slow-progressing rare disorder of unknown etiology. The direct cause of death in cases of IPPFE is rarely investigated. We experienced an autopsy case of a Japanese man with IPPFE and found aspiration pneumonia to be the major trigger of death. The individual had left vocal cord paralysis at admission, which may have contributed to aspiration pneumonia, and which probably was affected by the fibrous adhesion of the left apex of the chest wall resulting from IPPFE. The prevention of aspiration pneumonia is important for maintaining the respiratory function, especially in IPPFE patients with repeated pneumothorax.

Left Vocal Cord Paralysis in Idiopathic Pleuroparenchymal Fibroelastosis: A Case Report

“…Generally, unilateral vocal cord dysfunction secondary to mediastinal abnormalities occurs more occasionally on the left due to the course and length of the left recurrent laryngeal nerve (8) , (9) . There have been three case reports, except for our case, that describe vocal cord paralysis or paresis in PPFE (5) , (6) , (7) . Three out of the four cases, including our case, were left-sided.…”

“…Vocal cord paralysis or paresis has been rarely reported in PPFE (5) , (6) , (7) , and its underlying mechanism remains unknown. Several mechanisms of vocal cord paralysis have been previously discussed in lung parenchymal diseases (8) .…”

“…Several mechanisms of vocal cord paralysis have been previously discussed in lung parenchymal diseases (8) . A possible mechanism is proposed, that is, fibrous adhesion of the recurrent laryngeal nerve to the chest wall due to PPFE can cause stretching of the nerve (5) . Generally, unilateral vocal cord dysfunction secondary to mediastinal abnormalities occurs more occasionally on the left due to the course and length of the left recurrent laryngeal nerve (8) , (9) .…”

“…Three out of the four cases, including our case, were left-sided. Another possible mechanism is that the traction or compression of the recurrent laryngeal nerve due to the distortion of the tracheobronchial tree can cause paralysis of the vocal cord (5) , (8) , (10) , (11) . In this case, CT revealed that the aortic arch and left pulmonary artery were closely contacted due to the distortion of the tracheobronchial tree toward the right side ( Figure 2B, C, D ), possibly suggesting the compression of the left recurrent laryngeal nerve, as in cases with pulmonary artery enlargement or aortic arch aneurysm (8) , (11) .…”

“…Idiopathic PPFE (IPPFE) was included as a group of rare idiopathic interstitial cases of pneumonia in the official American Thoracic Society/European Respiratory Society 2013 classification (4) . Although pneumothorax and pneumomediastinum are frequent, vocal cord paralysis or paresis is a rare complication of PPFE (5) , (6) , (7) . In this report, we present an unusual case of IPPFE with left vocal cord paralysis that developed repeated pneumonia.…”