Abstract
To provide useful information for diagnosing and predicting fetal intraabdominal extralobar pulmonary sequestration (IEPS), a retrospective review of diagnostic approaches was conducted. Ultrasonography was performed serially in 21 fetuses with IEPS from 2005 to 2017. Prenatal sonographic features, treatment, and outcomes of each case were evaluated and collected. These cases of IEPS were also compared to 43 cases previously reported by other researchers from 1986 to 2017. Of the 21 sonographic features, 14 (67%) were hyperechoic, 21 (100%) were well circumscribed, and 17 (81%) depicted a mass that shifted with fetal breaths/hiccups non-synchronized with adjacent organs (sliding sign). Feeding arteries were detected prenatally in 18 patients (86%). The lesion volume was 10.17 ± 4.66 cm3, the congenital cystic adenomatoid malformation volume ratio and cardiothoracic ratio were in normal range. The gestational age at diagnosis, location and echotexture of the lesion, and rate of surgical treatment were similar to previous studies, but with a significantly higher rate of detected feeding arteries (P < 0.01), and associated anomalies (P < 0.01). All infants who underwent surgery after birth had satisfactory outcomes. The sliding sign and feeding artery are essential features of IEPS in prenatal diagnosis.
Highlights
Intraabdominal extralobar pulmonary sequestration (IEPS) is a rare type of pulmonary sequestration (PS)
Abortion was performed in 43% (9/21) of the patients with intraabdominal extralobar pulmonary sequestration (IEPS), according to the preferences of the parents who did not want to suffer potential adverse outcomes
Postmortem autopsy confirmed all findings on prenatal ultrasound
Summary
Intraabdominal extralobar pulmonary sequestration (IEPS) is a rare type of pulmonary sequestration (PS). There are 2 types of PS that have been previously determined: intralobar sequestrations (ILS; 75%), and extralobar sequestrations (ELS; 25%). In ILS, the sequestration shares the common pleura with the normal lung; in ELS, the sequestration has its own pleural envelope[4,5]. Appropriate prenatal diagnosis of IEPS is important to optimize the management strategy for affected neonates. Most previous studies did not describe the details of sonographic features of IEPS, congenital cystic adenomatoid malformation volume ratio, or cardiothoracic ratio. Due to the lack of studies with a larger sample size, the prenatal sonographic features, natural course, and perinatal outcomes have not been discussed definitively
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