J Med Ultrasonics (2013) 40:279–281 DOI 10.1007/s10396-012-0425-0
CASE REPORT
Antenatal three-dimensional sonographic features of fetal biliary atresia Masato Mashima • Hirokazu Tanaka • Atsuo Numoto • Hiroyuki Kubo • Ryuichi Shimono Takashi Kusaka • Susumu Itoh • Toshiyuki Hata
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Received: 1 October 2012 / Accepted: 26 November 2012 / Published online: 20 December 2012 Ó The Japan Society of Ultrasonics in Medicine 2012
Abstract We present antenatal three-dimensional (3D) sonographic features of fetal biliary atresia. A 38-year-old pregnant Japanese woman was referred to our ultrasound clinic because of a fetal intra-abdominal cyst at 19 weeks of gestation. Conventional two-dimensional (2D) sonography and 3D sonography with an inversion mode showed a round cyst with a tiny dip in the subhepatic area. At 34 weeks, conventional 2D sonography and 3D sonography with inversion and transparent X-ray modes clearly revealed a round to oval cyst connected with the gallbladder via the cystic duct. A diagnosis of choledochal cyst (type I) was suggested antenatally. However, the final diagnosis made by employing operative cholangiography during surgery was biliary atresia (type I cyst) at 25 days of life. To the best of our knowledge, this is the first report of biliary atresia employing antenatal 3D sonography with inversion and transparent X-ray modes.
M. Mashima H. Tanaka T. Hata (&) Department of Perinatology and Gynecology, Kagawa University School of Medicine, 1750-1 Ikenobe, Miki, Kagawa 761-0793, Japan e-mail:
[email protected] A. Numoto Department of Obstetrics and Gynecology, Numoto Ladies Clinic, 4-8 Higashitamachi, Takamatsu, Kagawa 760-0058, Japan H. Kubo R. Shimono Department of Pediatric Surgery, Kagawa University School of Medicine, 1750-1 Ikenobe, Miki, Kagawa 761-0793, Japan T. Kusaka S. Itoh Department of Pediatrics, Kagawa University School of Medicine, 1750-1 Ikenobe, Miki, Kagawa 761-0793, Japan
Keywords 3D ultrasound Inversion mode Transparent X-ray mode Fetus Biliary atresia
Introduction The incidence of biliary atresia is one in 10,000–12,000 live births [1]. The main symptoms of biliary atresia are jaundice, clay-colored stools, and hepatomegaly after birth. Its successful treatment depends on early diagnosis and surgery, prevention of cholangitis, and appropriate postoperative management [1]. There have been only three reports on antenatal two-dimensional (2D) sonographic diagnosis of biliary atresia during pregnancy [2–4]. To the best of our knowledge, however, there has been no report on antenatal three-dimensional (3D) sonographic diagnosis of biliary atresia during pregnancy. This prompted us to present our experience with antenatal diagnosis of biliary atresia employing 3D sonography with inversion and transparent X-ray rendering modes.
Case report A 38-year-old pregnant Japanese woman, gravida 5, para 2, was referred to our ultrasound clinic because of a fetal intra-abdominal cyst at 19 weeks of gestation. 2D sonographic biometric measurements were consistent with 19 weeks of gestation. 2D sonography (Voluson E8; GE Healthcare, Milwaukee, WI, USA) showed a round cyst with a tiny dip in the subhepatic area (Fig. 1). Color Doppler sonography revealed no flow within the cyst. 3D sonography (Voluson E8; GE Healthcare, Milwaukee, WI, USA) with an inversion mode revealed a spatial relationship among the round cyst, gallbladder, stomach, and
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J Med Ultrasonics (2013) 40:279–281
Fig. 1 Three-dimensional reconstruction of the fetal intraabdominal cavity using the inversion mode in a fetus with biliary atresia at 19 weeks of gestation. A round cyst (asterisks) with a tiny dip (arrow) in the subhepatic area is noted. Ao aorta, BL bladder, GB gallbladder, L liver, St stomach. a Two-dimensional transverse image of the fetal abdomen; b two-dimensional coronal image of the fetal abdomen; c three-dimensional image with inversion mode
Fig. 2 Three-dimensional reconstruction of the fetal intraabdominal cavity using the inversion mode in a fetus with biliary atresia at 34 weeks of gestation. A round to oval cyst (asterisks) connected with the gallbladder (GB) via the cystic duct (arrow) can be clearly identified. Ao aorta, BL bladder, GB gallbladder, IVC inferior vena cava, St stomach, UV umbilical vein. a Twodimensional transverse image of the fetal abdomen; b threedimensional image with transparent X-ray mode; c threedimensional image with inversion mode
bladder (Fig. 1). However, the connection between the cyst and gallbladder could not be identified employing both 2D and 3D techniques. At 34 weeks’ gestation, 2D sonography and 3D sonography with inversion and transparent X-ray modes clearly revealed a round to oval cyst connected with the gallbladder via the cystic duct (Fig. 2). A diagnosis of choledochal cyst (type I) was suggested before birth. The size of the cyst remained unchanged on follow-up sonographic examinations. A healthy female infant weighing 3,230 g was delivered vaginally at 39 weeks and 4 days of gestation with an Apgar
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score of 8/9 at 1 and 5 min, respectively. Laparotomy was performed at 25 days of life because of elevated serum direct bilirubin and increasing liver enzymes. Biliary atresia (type I cyst) was diagnosed by operative cholangiography. A standard Kasai operation was performed, and the infant was discharged at 37 days of life. She is now doing well.
Discussion There have been three reports on antenatal 2D sonographic diagnosis of biliary atresia during pregnancy [2–4].
J Med Ultrasonics (2013) 40:279–281
The antenatal 2D sonographic finding of biliary atresia was an intra-abdominal cyst in the fetal hepatic hilum. However, the presence of a cyst in the fetal hepatic hilum suggests a choledochal cyst or biliary atresia [4]. A landmark feature for antenatal diagnosis of biliary atresia is a cystically dilated extrahepatic bile duct [3]. A similar 2D sonographic finding is also a clue for the antenatal diagnosis of choledochal cyst [5–7]. In the present case, conventional 2D sonography and 3D sonography with inversion and transparent X-ray modes clearly revealed a round to oval cyst connected with the gallbladder via the cystic duct. A diagnosis of choledochal cyst (type I) was suggested antenatally. However, the final diagnosis made by employing operative cholangiography during surgery after delivery was biliary atresia (type I cyst). So, there seem to be no characteristic and unique findings for the antenatal diagnosis of biliary atresia. However, the 3D inversion mode facilitated visualization of the relationship among the cyst and surrounding fluid-filled structures, thus helping the examiner to more effectively understand the spatial relationships among the cyst, connecting cystic duct, gallbladder, umbilical vein, bladder, and stomach. Based on our search of the literature, we identified five investigations that described the use of 3D inversion rendering mode imaging for visualization of abnormal fluidfilled structures in the fetal intra-abdominal cavity [8–12]. However, there has been no report on antenatal 3D inversion mode diagnosis of biliary atresia during pregnancy. In the present report, we demonstrated additional possibilities for 3D sonography with an inversion mode with respect to the spatial relationship among the cyst, connecting cystic duct, gallbladder, umbilical vein, bladder, and stomach in the case of biliary atresia. Further studies are needed to confirm the potential use of the 3D inversion mode to diagnose fetal anomalies.
281 Conflict of interest
The authors have no conflicts of interest.
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Acknowledgments The work reported in this paper has been supported by a Grant-in-Aid for Scientific Research on Innovative Areas ‘‘Constructive Developmental Science’’ (no. 24119004) from The Ministry of Education, Culture, Sports, Science, and Technology, Japan.
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