Prenatal diagnosis of fetal intraabdominal extralobar pulmonary sequestration: a 12-year 3-center experience in China

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.

Figure 1

IEPS in autopsy and histopathology. (a) Autopsy showing a soft, solid mass at upper abdomen with no adherence to its surrounding structures and no communication to the gastrointestinal tract. (b) The cut surface of the mass showing spongy structure. (c) Autopsy showing a mass with a feeding vessel arising from the celiac trunk (CT), which is the first visceral branch of the descending aorta (DAO). SMA, superior mesenteric artery. (d) The mass containing mixed dysplastic and hyperplastic lung tissue, with bronchiole-like structures (asterisk) and cartilage (arrows) on histopathology. (H&E stain, ×40).

Figure 2

IEPS in postnatal imagings and histopathology. (a) Postnatal color Doppler ultrasound showing a mass at the left adrenal area with the feeding artery (arrows) arising from the descending aorta (DAO). (b) CT scans showing an ellipsoidal low-density mass at the left adrenal area. This mass was delineated by a clear boundary, and CT value was approximately 35 HU. The left side of the diaphragm was pushed upwards. Sp, spleen; K, kidney. (c,d) Postoperative histopathology showing alveolar tissue (asterisk), surface covered with mesothelial cells (arrows) (H&E stain, ×40).

Figure 3

Feeding vessel of IEPS in color Doppler of prenatal US. Color Doppler ultrasound showing masses with the feeding artery (arrows) arising from the abdominal aorta (AO). ST, stomach.

Figure 4

IEPS in gray-scale imaging of prenatal US. Ultrasound imaging of the upper fetal abdomen showing well-defined solid masses. (a) A homogenous and hyperechoic mass on the left side. (b,c) Heterogeneously hyperechoic solid masses with a small cystic component (asterisk) on the left side. (d) A homogenous and hyperechoic mass on the right side. ST, stomach; GB, gall bladder; UV, umbilical vein; L, left; R, right.