SD.a Bnoieolp Ja. tMhoy lVluijraayaraghavan Matthew P. Lungren Michael R.B. Evans Editors Clinical Medicine Covertemplate Atlas of Fetal Imaging ASubbdtoitmlee fnor Clinical Medicine Covers T3_HB Second Edition 112323 Atlas of Fetal Imaging S. Boopathy Vijayaraghavan Atlas of Fetal Imaging Abdomen S. Boopathy Vijayaraghavan SONOSCAN-Ultrasonic Scan Centre Coimbatore Tamil Nadu India ISBN 978-981-13-0931-1 ISBN 978-981-13-0932-8 (eBook) https://doi.org/10.1007/978-981-13-0932-8 Library of Congress Control Number: 2018951068 © Springer Nature Singapore Pte Ltd. 2019 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors, and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made. The publisher remains neutral with regard to jurisdictional claims in published maps and institutional affiliations. This Springer imprint is published by the registered company Springer Nature Singapore Pte Ltd. The registered company address is: 152 Beach Road, #21-01/04 Gateway East, Singapore 189721, Singapore Preface The field of fetal medicine has evolved as an independent specialty over this past decade. It is, to a major extent, due to the advancement in obstetric ultrasound. Because of improvement in the technology and resolution of machines, we are able to see more and earlier in gestation. Study of fetal abdomen has come to limelight, only recently. Most of the anomalies of fetal abdomen are rare, and their description has been mostly in the form of case reports or case series because of the lack of numbers. This atlas is designed to lead the reader from an abnor- mal ultrasound image of fetal abdomen to a precise diagnosis. This is made possible by the help of high-frequency probe in most of the cases and dynamic study in many cases. The dynamic study is illustrated in the form of cine loops which are made available online for the readers. This book is an atlas of fetal abdomen giving description of various anomalies of fetal abdomen using abundant illustrative scan images with essential descriptive text containing a brief mention of other aspects of the anomalies. The readers are encouraged to refer to more literature for detailed description of these aspects. This atlas will be a practical guide to the imaging specialists and those who practice fetal medicine. Coimbatore, Tamil Nadu, India S. Boopathy Vijayaraghavan v Acknowledgment I acknowledge with thanks the support extended by my family and the professional help by my colleagues, fellows, and staff of SONOSCAN which made this book possible. vii Contents 1 Protocol of Performance of  Sonography of Fetal Abdomen and Normal Appearance . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.2 Technique and Protocols . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 1 1.3 Amniotic Fluid Dynamics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4 2 Sonography of Fetal Gastrointestinal Tract (GIT) . . . . . . . . . . . . . . . . . . . . . . . . . 5 2.1 Oesophagus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 2.2 Visualisation of Stomach by Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7 2.3 Esophageal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8 2.3.1 Sonographic Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9 2.4 Gastric Pseudomass . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 2.5 Pyloric Atresia (PA) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12 2.6 Duodenal Obstruction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 2.6.1 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16 2.7 Duodenal and Esophageal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 2.8 Gastrointestinal Duplication Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 2.8.1 Gastric Duplication Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17 2.8.2 Small Bowel Duplication Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 19 2.9 Small Bowel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 21 2.9.1 Small Bowel Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 22 2.10 Congenital Chloride Diarrhea (CCD) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 25 2.11 Echogenic Bowel (EB). . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 26 2.12 Fetal Large Bowel . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 27 2.13 Anorectal Malformation (ARM) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 2.13.1 Sonographic Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 2.13.2 Technique of Seeing the Fetal Anus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 2.13.3 Nonvisualized Fetal Anus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 28 2.14 Urorectal Septal Malformation (URSM) Sequence or Cloacal Malformation . . . . 31 Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 37 3 Fetal Genitourinary Tract Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 3.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39 3.2 Ultrasound Scanning Technique of Fetal Urinary Tract (Extended Fetal Urosonography) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 40 3.3 Amniotic Fluid and Urinary Tract Anomaly . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46 3.4 Urinary Tract Dilatation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 49 3.5 Cystic Renal Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 54 3.5.1 Decision Tree Approach to Renal Cystic Disease . . . . . . . . . . . . . . . . . . 55 3.5.2 Obstructive Dysplasia of Kidneys . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 56 3.5.3 Multicystic Dysplasia (MCD) of Kidney . . . . . . . . . . . . . . . . . . . . . . . . . 56 3.5.4 Simple Renal Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 59 ix x Contents 3.5.5 Inherited Renal Cystic Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 60 3.5.6 Renal Anomalies with Typical Ultrasound Pattern as Part of Polymalformative Syndromes . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 66 3.6 Renal Anomalies with Atypical Ultrasound Pattern . . . . . . . . . . . . . . . . . . . . . 71 3.7 Nonvisualized Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72 3.7.1 Unilateral Nonvisualized Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 72 3.7.2 Bilateral Nonvisualized Kidneys . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 75 3.8 Unilateral Smaller Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76 3.9 Hydronephrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 78 3.10 Hydroureteronephrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80 3.10.1 Unilateral Hydroureteronephrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 80 3.10.2 Ectopic Ureteric Opening . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 84 3.10.3 Duplex Collecting System of Kidneys . . . . . . . . . . . . . . . . . . . . . . . . . . 85 3.10.4 Bilateral Hydroureteronephrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 87 3.11 Megacystis or Dilated Urinary Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 88 3.11.1 Urethral Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 89 3.11.2 Posterior Urethral Valves . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 90 3.11.3 Anterior Urethral Valves . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 94 3.11.4 Anterior Urethral Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 95 3.11.5 Megacystis Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 97 3.11.6 Megacystis Megaureter Complex . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 98 3.11.7 Prune Belly Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 3.12 Nonvisualized Urinary Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 99 3.12.1 Renal Tubular Dysgenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 100 3.13 Duplication of Urinary Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 101 3.14 Megalourethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 104 3.15 Sirenomelia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 106 Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 112 4 Masses and Fluid Collection in Fetal Abdomen . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 4.1 Confirmation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 113 4.1.1 Organ or Plane of Origin Is Determined by the Following Features . . . 114 4.1.2 Characterization . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 114 4.2 Fetal Abdominal Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 4.2.1 Ultrasound Imaging Approach . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 121 4.2.2 Ovarian Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 124 4.2.3 Mesenteric Lymphangioma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 126 4.2.4 Gastrointestinal Duplication Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 4.2.5 Choledochal Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 4.2.6 Liver or Splenic Cysts . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 4.2.7 Retroperitoneal Lymphangioma. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 127 4.2.8 Cystic Lesions of Urinary Tract . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 128 4.2.9 Hydrocolpos . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129 4.2.10 Meconium Cyst . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129 4.2.11 Urachal Cyst. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 131 4.2.12 Vesical Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132 4.2.13 Fetus in Fetu . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133 4.2.14 Umbilical Vein Varix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 133 Contents xi 4.3 Solid Intraabdominal Masses in Fetus . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 4.3.1 Liver . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 135 4.3.2 Adrenal Glands . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 137 4.3.3 Infradiaphragmatic Bronchopulmonary Sequestration . . . . . . . . . . . . . 140 4.3.4 Bronchopulmonary Foregut Malformation . . . . . . . . . . . . . . . . . . . . . . 142 4.4 Echoes in Gall Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 4.5 Fetal Ascites . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 4.5.1 Hydrops Fetalis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 143 4.5.2 Isolated Fetal Ascites . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 145 Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150 5 Fetal Abdominal Wall Sonography . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 5.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 5.2 Embryology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 5.2.1 Development of Cloaca . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151 5.3 Physiological Herniation of Bowel in the Fetus . . . . . . . . . . . . . . . . . . . . . . . . 152 5.4 Omphalocele . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152 5.4.1 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 5.4.2 Associated Genetic and Structural Abnormalities . . . . . . . . . . . . . . . . . 153 5.4.3 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 5.4.4 Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153 5.5 Gastroschisis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155 5.5.1 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155 5.6 Umbilical Cord Hernia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157 5.6.1 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157 5.6.2 Outcome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 157 5.7 Bladder and Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158 5.7.1 Aetiopathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158 5.7.2 Prenatal Ultrasound Findings . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 159 5.7.3 Outcome of Bladder and Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . 160 5.8 Ectopia Cordis and Pentalogy of Cantrell . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 5.8.1 Etiopathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 5.8.2 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 5.8.3 Associated Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 162 5.9 Limb Body Wall Complex/Body Stalk Anomaly . . . . . . . . . . . . . . . . . . . . . . . . 164 5.9.1 Aetiopathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 5.9.2 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 5.9.3 Associated Anomalies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 5.9.4 Prognosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164 5.10 Amniotic Band Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 5.10.1 Aetiopathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 5.10.2 Ultrasound Features . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168 5.11 Prognosis in ABS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 5.12 Parasitic Twin . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 169 Suggested Reading . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 170 About the Author S. Boopathy Vijayaraghavan graduated from Coimbatore Medical College and Madras Medical College, Chennai. He is a consultant diagnostic radiologist in Sonoscan-Ultrasonic Scan Centre and Director of the Sonoscan Institute of Ultrasound in Medicine. He was President of the Coimbatore Ultrasound Society and Chairperson of the Coimbatore subchap- ter of the Indian Radiological and Imaging Association. He is currently the Organizing Chairman of FOCUSSE, a periodical CME in ultrasonography, and he was on the editorial committees of the Indian Journal of Radiology and Imaging and the Indian Journal of Medical Ultrasound. He has published work in several books and journals and has delivered numerous lectures at various national and international CME events and conferences. He has received a number of awards, including the Sir JC Bose Award—the highest honor conferred by the Indian Radiology and Imaging Association—and Gnanadhara’s distinguished teacher award. xiii