Bubbly and cystic appearance in chronic lung disease: Is this diagnosed as Wilson–Mikity syndrome?

Abstract

Wilson-Mikity syndrome (WMS) was first reported in 1960 by Wilson and Mikity. They described preterm infants who developed areas of cystic emphysema in the first month of life with subsequent progression to chronic lung disease (CLD) of infancy, although these infants did not exhibit early respiratory distress, such as respiratory distress syndrome (RDS). This condition was widely accepted over the next 20 years, but WMS is now rarely mentioned and is commonly considered an anachronism. In Japan, CLD is classified into six types according to the presence of RDS and/or intrauterine inflammation and appearance on chest X-ray. One type of CLD (type III, which accounts for 13.5% of all CLD) is defined as history of intrauterine inflammation and the typical bubbly and cystic appearance on chest X-ray described in the original report of WMS. There is insufficient evidence to determine whether WMS exists or whether WMS is relatively common only in Japan and not in other countries. It is important, however, to distinguish this type of CLD from other types because the strategy for the prevention or treatment of CLD should be different according to its origin, cause, and risk factors.

Impact of chest radiography‐based definition of bronchopulmonary dysplasia

“…Diagnosis based on chest radiography was made after 28 postnatal days by the neonatologists at each institution. A typical radiographic bubbly/cystic pattern is shown in Figure a; the other Japanese NICU reports also have shown the same bubbly/cystic appearance patterns . The postnatal radiographic bubbly/cystic appearance is recognized widely in all Japanese NICU.…”

“…Almost 90% of the subjects, however, were admitted to level 3 NICU, which might uphold the diagnostic value of the chest radiograph classification. Additionally, the bubbly/cystic chest radiograph pattern has been recognized widely as signifying the presence of severe BPD in Japanese NICU, and it might be relatively easy to recognize this chest radiograph pattern . Second, the chest radiograph classification did not have a scoring system.…”

“…The etiology‐based BPD classification, including chest radiography appearance, which is used in Japan, might be helpful in analyzing the pathophysiology of BPD. In the Japanese BPD classification, BPD28 is classified according to the evidence of respiratory distress syndrome (RDS), intrauterine infection/inflammation, and chest radiograph appearance postnatally (Table ) . Of these factors, chest radiography is the most important for BPD classification: it is classified into two types: the severe type showing a bubbly/cystic appearance; and the mild type showing a hazy shadow without a bubbly/cystic appearance.…”

“…Of these factors, chest radiography is the most important for BPD classification: it is classified into two types: the severe type showing a bubbly/cystic appearance; and the mild type showing a hazy shadow without a bubbly/cystic appearance. In addition, bubbly/cystic appearance on chest radiograph is an important factor in patients with the severe type of BPD in Japan . The bubbly/cystic pattern on chest radiograph is recognized widely and diagnosed easily as severe BPD in all Japanese neonatal intensive care units (NICU; Fig.…”

Bubbly and cystic appearance on chest radiograph of extremely preterm infants with bronchopulmonary dysplasia is associated with wheezing disorder

“…The CXR appearance is classified into two findings: severe types showing a bubbly/cystic appearance (bubbly/cystic+) and mild types showing hazy shadows without a bubbly/cystic appearance (bubbly/cystic−). It was reported that the bubbly/cystic appearance on CXR is an important factor in patients with severe BPD in Japan . We showed an association between extremely preterm infants with bubbly/cystic+ BPD28 and a higher rate of home oxygen therapy in a Japanese population‐based cohort study.…”