Respiratory characteristics of infants with pulmonary hypoplasia syndrome following preterm rupture of membranes: a preliminary study for establishing clinical diagnostic criteria.
BACKGROUND
At present, the diagnosis of pulmonary hypoplasia is based on postmortem findings, and there are no clear clinical diagnostic criteria to facilitate its identification and management.


OBJECTIVE
To characterise the respiratory status of pulmonary hypoplasia syndrome (PHS) following preterm rupture of membranes so as to establish its clinical diagnostic criteria.


METHODS
Retrospective comparison of respiratory characteristics of six typical PHS infants with six wet lung syndrome (WLS) infants who served as controls.


METHODS
The PHS and WLS infants were selected from 1094 patients admitted to a tertiary care neonatal unit over a 6-year period, with criteria based on perinatal history, respiratory signs, X-ray and laboratory findings, and ventilator settings.


METHODS
The compared variables were lung volume index (LVI) calculated from lung dimensions on chest X-ray, ventilatory index (VI), ventilatory efficiency index (VEI), response to artificial surfactant treatment, and ventilation days.


RESULTS
In PHS compared to WLS infants, LVI was lower (4.5 +/- 0.5 vs. 9.5 +/- 1.5; p < 0.01), VI was higher (0.108 +/- 0.030 vs. 0.022 +/- 0.005; p < 0.05), and VEI was lower (0.083 +/- 0.012 vs. 0.258 +/- 0.052; p < 0.01) (mean +/- S.E.). Artificial surfactant was given to four PHS infants, but none of them showed respiratory improvement. Ventilation days were 11-79 in three surviving PHS infants and 2-14 in WLS infants.


CONCLUSIONS
In this preliminary study, low LVI (< 6.5) and VEI (< 0.15) were the most useful indicators of PHS.
Division of Neonatology, Perinatal Center, St Marianna University School of Medicine, Yokohama City Seibu Hospital, Yokohama, Japan. dks@med.monash.edu.au
Keiji
K
Suzuki
D005260:Female; D005322:Premature Rupture of Fetal Membranes / Q000150:complications*; D005865:Gestational Age; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000473:pathology / Q000503:physiopathology*; D008171:Lung Diseases / Q000000981:diagnostic imaging / Q000503:physiopathology*; D011247:Pregnancy; D011663:Pulmonary Surfactants / Q000008:administration & dosage; D011859:Radiography; D012119:Respiration; D012121:Respiration, Artificial; D012189:Retrospective Studies; D013577:Syndrome; D013997:Time Factors
D011663:Pulmonary Surfactants