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MONOAMNIOTIC TWINS

Monoamniotic twinning occurs in 5% of monochorionic twins (1% of all twins), and the most severe form of splitting disorders in monozygotic twins is conjoined twins. At the 10–14-week scan, the criteria for monoamnionicity are the absence of the intertwin amniotic membrane and the presence of only one yolk sac7 and it should be suspected if the placental cord insertions are close to each other and an unusual intrauterine position of both fetuses, in close proximity to each other, is seen.25 Monoamniotic twins show a significantly increased risk for structural anomalies and for poor perinatal outcome, even in the absence of an intertwin discordance. In both structurally normal and abnormal twins, cord entanglement, which has been reported to be present as early as the first trimester,2 has been responsible for the demise of one or both fetuses in the majority of cases.
The aim of fetal surveillance should be to reach at least 32 weeks of gesta­tion and delivery by elective caesarean section should be performed to avoid acute cord complications during delivery. Cord entanglement may be detected by colour Doppler ultrasound. However, its consequences remain controversial,
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because the incidence of loose entanglement seems to be high, but fetal jeop­ardy occurs only if this leads to compression of the cords, which may be an acute event.
The primary ultrasound feature of conjoined twins is the fact that they are always close to each other with common movement patterns and without sepa­ration from each other if observed over a certain period of time. The chance for survival depends on the site of conjoining and the organs involved, and overall about 50% are stillborn. One third of the live-born twins have defects, which are impossible to correct surgically, and in those cases where surgery is attempted, a survival rate of about 60% of babies is achieved.
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HIGHER-ORDER MULTIPLE PREGNANCIES

Recently the incidence for multiples of a higher order has increased due to the use of different techniques in assisted reproduction. The use of transvaginal and transabdominal ultrasound to assess the number of fetuses and their chorioni­city is fundamental in the early diagnosis and management of these pregnancies. With the measurement of the nuchal translucency, the individual fetal risk for chromosomal abnormalities and other maldevelopments can be calculated and a selective reduction can be performed under ultrasound guidance to reduce the perinatal risk associated with multiples of higher order than twins or trip­lets. Multifetal reduction has been reported to reduce triplets to twins5 and the risk for fetal loss after the procedure has been continuously diminishing during recent years, as more experience in this technique has been gained. In general, the higher the starting number of fetuses, the poorer is the outcome after reduc­tion, with fetal loss rates reported to range from 15.4% to 4.5% according to starting numbers ranging from six to three fetuses, respectively.
13,14

References

1. Appleman Z, Vinkler C, Caspi B. Chorionic villous sampling in multiple pregnancies. Eur J Obstet Gynecol Reprod Biol 1999;85:979
2. Arabin B, Laurini RN, van Eyck J. Early prenatal diagnosis of cord entanglement in monoamniotic multiple pregnancies. Ultrasound Obstet Gynecol 1999;13: 181–186
3. Bajoria R, Wee LY, Anwar S, Ward S. Outcome of twin pregnancies complicated by single intrauterine death in relation to vascular anatomy of the monochorionic placenta. Hum Reprod 1999;14:2124–2130
4. Baldwin VJ. The pathology of monochorionic monozygocity. In: Baldwin VJ (ed) Pathology of multiple pregnancy. Springer Verlag, New York, 1994: 199–214
5. Boulot P, Vignal J, Vergnes C, Dechaud H, Faure JM, Hedon B. Multifetal reduction of triplets to twins: a prospective comparison of pregnancy outcome. Hum Reprod 2000;15:1619–1623
6. Brodtkorb E, Myhr G, Gimse R. Is monochorionic twinning a risk factor for focal cortical dysgenesis? Acta Neurol Scand 2000;102:53–59
7. Bromley B, Benacerraf B. Using the number of yolk sacs to determine amnionicity in early first trimester monochorionic twins. J Ultrasound Med 1995;14:415–419
8. Chitrit Y, Filidori M, Pons JC, Duyme M, Papiernik E. Perinatal mortality in twin pregnancies: a 3-year analysis in Seine Saint-Denis (France). Eur J Obstet Gynecol Reprod Biol 1999;86:23–28
9. De Catte L, Liebaers I, Foulon W. Outcome of twin gestations after first trimester chorionic villous sampling. Obstet Gynecol 2000;96:714–720
10. Deprest JA, Audibert F, Van Schoubroeck D, Hecher K, Mahieu-Caputo D. Bipolar coagulation of the umbilical cord in complicated monochorionic twin pregnancy. Am J Obstet Gynecol 2000;182:340–345
11. Devoe LD, Ware DJ. Antenatal assessment of twin gestation. Semin Perinatol 1995;19:413–423
12. Diehl W, Hecher K, Zikulnig L, Vetter M, Hackelöer BJ. Placental vascular anastomoses visualised during fetoscopic laser surgery in severe mid-trimester twin–twin transfusion syndrome. Placenta 2001;22:876–881
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13. Evans MI, Goldberg JD, Horenstein J et al. Selective termination for structural, chromosomal, and mendelian anomalies: international experience. Am J Obstet Gynecol 1999;181:893–897
14. Evans MI, Berkowitz RL, Wapner RJ et al. Improvement in outcomes of multifetal pregnancy reduction with increased experience. Am J Obstet Gynecol 2001;184:97–103
15. Farina A, Vesce F, Garutti P, Jorizzo G, Bianciotto A. Evaluation of intrauterine growth pattern of twins by linear discriminant analysis of the values of biparietal diameter, femur length and abdominal circumference. Gynecol Obstet Invest 1999;48:14–17
16. Gaziano EP, De Lia JE, Kuhlmann RS. Diamniotic monochorionic twin gestations: an overview. J Matern Fetal Med 2000;9:89–96
17. Hatkar PA, Bhide AG. Perinatal outcome of twins in relation to chorionicity. J Postgrad Med 1999;45:33–37
18. Hecher K, Diehl W, Zikulnig L, Vetter M, Hackelöer BJ. Endoscopic laser coagulation of placental vascular anastomoses in 200 pregnancies with severe mid-trimester twin­to-twin transfusion syndrome. Eur J Obstet Gynecol Reprod Biol 2000;92:135–139
19. Isada NB, Sorokin Y, Drugan A, Johnson MP, Zador I, Evans MI. First trimester interfetal size variation in well-dated multifetal pregnancies. Fetal Diagn Ther 1992;7:82–86
20. Jenkins TM, Wapner RJ. First trimester prenatal diagnosis: chorionic villous sampling. Semin Perinatol 1999;23:403–413
21. Landy HJ, Weiner S, Corson SL, Batzer FR, Bolognese RJ. The ‘vanishing twin’: ultrasonographic assessment of fetal disappearance in the first trimester. Am J Obstet Gynecol 1986;155:14–19
22. Loos R, Demron C, Vlietinick R, Demron R. The East Flanders prospective twin survey (Belgium): a population-based register. Twin Res 1998;1:167–178
23. Manzur A, Goldsman MP, Stone SC, Frederick JL, Balmaceda JP, Asch RH. Outcome of triplet pregnancies after assisted reproductive techniques: how frequent are the vanishing embryos? Fertil Steril 1995;63:252–257
24. Pandya P. Ultrasound and multiple pregnancies. Front Fetal Health 2001;3: 89–91
Multiple pregnancies
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25. Sebire NJ, Souka A, Skentou H, Geerts L, Nicolaides KH. First trimester diagnosis of monoamniotic twin pregnancies. Ultrasound Obstet Gynecol 2000;16:223–225
26. Sebire NJ, Souka A, Skentou H, Geerts L, Nicolaides KH. Early prediction of severe twin-to-twin transfusion syndrome. Hum Reprod 2000;15:2008–2010
27. Sepulveda W, Sebire NJ, Hughes K, Odibo A, Nicolaides KH. The lambda sign at 10-14 weeks of gestation as a predictor of chorionicity in twin pregnancies. Ultrasound Obstet Gynecol 1996;7:421–423
28. Sherer DM. Is less intensive fetal surveillance of dichorionic twin gestations justified? Editorial. Ultrasound Obstet Gynecol 2000;15:167–173
29. Snijders RJM, Noble P, Sebire NJ, Souka AP, Nicolaides KH. UK multicentre project on assessment of risk for trisomy 21 by maternal age and fetal nuchal
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translucency at 10–14 weeks of gestation. Fetal Medicine Foundation First Trimester Screening Group. Lancet 1998;352: 343–346
30. Spitz L. Conjoined twins. Br J Surg 1996;83:1028–1030
31. van den Berg C, Braat AP, Van Opstal D et al. Amniocentesis or chorionic villous sampling in multiple gestations? Experience with 500 cases. Prenat Diagn 1999;19: 234–244
32. Victoria A, Mora G, Arias F. Perinatal outcome, placental pathology, and severity of discordance in monochorionic and dichorionic twins. Obstet Gynecol 2001;97:310–355
33. Zikulnig L, Hecher K, Bregenzer T, Bäz E, Hackelöer BJ. Prognostic factors in severe twin–twin transfusion syndrome treated by endoscopic laser surgery. Ultrasound Obstet Gynecol 1999;14:380–387
34. Kalish RB, Gupta M, Perni SC, Berman S, Chasen ST. Clinical significance of first trimester crown–rump length disparity in dichorionic twin gestation. Am J Obstet Gynecol 2004;191:1437–1440
35. Machin GA. Why is it important to diagnose chorionicity and how do we do it? Best Pract Res Clin Obstet Gynecol 2004;18:515–530
36. Menon DK. A retrospective study of the accuracy of sonographic chorionicity determination in twin pregnancies. Twin Res Hum Genet 2005;8:259–261
37. Geipel A, Berg C, Katalinic A et al. Prenatal diagnosis and obstetric outcomes in triplet pregnancies in relation to chorionicity. Br J Obstet Gynaecol 2005;112:554–558
38. Van der Cruys, Faiola S, Auer M, Sebire N, Nicolaides KH. Screening for trisomy 21 in monochorionic twins by measurement of fetal nuchal translucency thickness. Ultrasound Obstet Gynecol 2005;25: 551–553
39. Wald NJ, Rish S, Hackshaw AK. Combining nuchal translucency and serum markers in prenatal sceening for Down syndrome in twin pregnancies. Prenat Diagn 2003;23:588–592
40. Garne E, Andersen HJ. The impact of multiple pregnancies and malformations on perinatal mortality. J Perinat Med 2004;32:215–219
41. Huber A, Hecher K. How can we diagnose and manage twin-twin transfusion syndrome? Best Pract Res Clin Obstet Gynaecol 2004;18:543–556
42. Fisk NM, Tan TY, Taylor MJ. Stage­saved treatment of twin-twin transfusion syndrome. Am J Obstet Gynecol 2004;190:1491–1492
43. Robyz R, Quarello E, Ville Y. Management of fetofetal transfusion syndrome. Prenat Diagn 2005;25:786–795
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Three-dimensional and four-dimensional ultrasound application in prenatal diagnosis

Rabih Chaoui Bernard Benoit
ABSTRACT
Three-dimensional ultrasound has been the most rapidly evolving technique in fetal imaging in recent years and is mainly known for the demonstration of the face or other fetal surface structures. The potential of this technique in prenatal diagnosis is, however, greater, based on the possibility of acquiring a volume data set of a region of interest which can then be displayed in different ways. This chapter will emphasize these display modes as the demonstrations of reconstructed two-dimensional images either as orthogonal or parallel cross­section planes (tomographic mode) or the rendering of the three-dimensional/ four-dimensional information. Rendering includes the surface mode, the maximum, minimum and inversion modes, as well as glass body mode when combined with colour or power Doppler acquisition. Spatial and temporal image correlation technology enables the acquisition of fetal heart data and the display of one single cardiac cycle in different modes. The chapter supports the idea that we are now moving from the era of ‘sonography in two-dimensional planes’ to ‘volume ultrasound’.
KEYWORDS
Inversion mode, maximum mode, minimum mode, prenatal diagnosis, spatial and temporal image correlation, three-dimensional ultrasound, tomographic imaging, volume ultrasound.

INTRODUCTION

Three-dimensional (3D) ultrasound has become the most rapidly evolving technique in fetal imaging, but some examiners are still using 3D and four­dimensional (4D) techniques only to demonstrate the fetal face to the parents,
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which has made this new technique very popular with them as well. However, the concept of ‘volume ultrasound’ introduced a few years ago enabled a more comprehensive medical and clinical application of 3D technology to prena­tal diagnosis.1 A volume data set of the region of interest is acquired digitally, and the information stored can be displayed in different ways to highlight the spatial arrangement of a specific structure in the region of interest. Many col­leagues may still be unfamiliar with all of these features, which are now well established in targeted prenatal diagnosis for ruling out or clearly demonstrat­ing fetal malformations. In this chapter we will review the potential of volume ultrasound and the application of some display modes in clinical work.

VOLUME ACQUISITION

A volume data set can be acquired in different ways. The acquisition can be achieved as a:

static 3D

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•
real-time 3D or 4D
•
spatial and temporal image correlation for heart and vessels.
•
STATIC 3D
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This is the 3D used in most fetal studies (face, hands, etc.) and consists of a single volume data set. The volume quality is defined by the choice of the acquisition time. Static 3D can also be combined with colour Doppler, power Doppler, high-definition (HD) flow, and B-flow, depending on the question of interest.

REAL-TIME 3D OR 4D ULTRASOUND

Real-time 3D or 4D is achieved today mainly by a mechanical 3D transducer with a rapid acquisition from 1.5 to 40 volumes/sec. A few matrix transducers provide electronic 3D information but their use in obstetric ultrasound is still limited. The advantage of a 4D examination is its ease of use. The direct result on the screen enables online manipulation to acquire the best image by chang­ing the gain and the contrast depending on the mode used. Furthermore, it allows the transducer to be moved depending on the insonation angle. The technique is ideal for studying fetal movements and behaviour throughout pregnancy (smil­ing, yawning, grimacing, etc.). It can also be used for fetal echocardiography but this requires a great deal of experience.

SPATIAL AND TEMPORAL IMAGE CORRELATION

Spatial and temporal image correlation (STIC) is a software application pro­viding an acquisition of a fetal heart volume data set over a period of few seconds (i.e. 7.5–15 sec). It allows the acquisition of numerous planes including
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additional information from an entire cardiac cycle. The software calculates the mean heart rate acquired and the images in the volume are rearranged accord­ing to their temporal event within the heart cycle. The displayed volume then includes a single ‘hypothetical’ heart cycle, which is reconstructed from single selected images of the A-plane (acquisition plane) in the different phases of the heart cycle, whereas the B- and C-planes are reconstructed digitally.2 STIC can be used with grey-scale fetal echocardiography but can also be combined with colour Doppler, power Doppler, HD flow, B-flow, etc.3 Once the acquisition of a volume is achieved, the information can be visualized as either single or mul­tiple 2D images regenerated from the volume and selected by the examiner or as a volume spatial information called 3D rendering, allowing the application of different modes. Some of the actual display modes will be emphasized and illus­trated in this chapter.

VOLUME DATA DISPLAY

SINGLE PLANE OF CHOICE, MULTIPLANAR ORTHOGONAL PLANES OR MULTIPLE TOMOGRAPHIC PARALLEL SLICES

From a digitally stored 3D/4D data set, cross-sectional views can be obtained at any desired orientation (a so-called ‘anyplane’), direction and depth. It must be borne in mind that the acquisition (A-) plane provides the best information, whereas the reconstructed planes (B-, C- or others) are of lesser quality. This should be considered during the volume acquisition. The 2D image analysis from a volume can be achieved from a 3D, 4D or STIC data set. The display format is either a single-plane view or a multiplanar view showing three planes which are perpendicular to each other (Fig. 14.1). In the lateral view the intersection of the three planes is a dot and by moving the position of this dot, the exam­iner can navigate through the volume (see Fig. 14.1). The recent introduction of multislice analysis known as tomographic ultrasound imaging (TUI) is similar to the tomographic assessment known from computed tomography (CT) and mag­netic resonance (MR) workstations (Figs 14.2, 14.3). The examiner can define the slice thickness and the number of planes demonstrated. The multiplanar mode can be used to acquire a plane not directly seen on cross-section 2D during live examination, mainly in cases with non-optimal fetal position, so as to dem­onstrate the corpus callosum, a fetal profile, a limb or the aortic arch. It can be used to visualize exact midline planes after making adjustments in the two other orthogonal planes (for nasal bone assessment).
One of the major advantages of a 3D data set is the potential for offline exami­nation of a few volumes at a remote station. this mode could be the transfer of data via the Internet to a remote site to get a second opinion or a complete offline evaluation without examining the patient.5 However, since the quality of reconstructed images depends mainly on the origi­nal acquisition, the examiner should consider this aspect when acquiring volumes for future studies.
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One of the future potential uses of
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Fig. 14.1 Volume data set of a fetal face shown with the ‘multiplanar mode’ with three orthogonal planes. The dot is the intersection of all three planes and can be used to achieve the best position for assessing the profile. In the C-plane (lower panel) the dot is on the nasal bone. In the lower panel in another fetus the volume information of the face was used to achieve a plane of the soft palate after offline processing.
Fig. 14.2 Tomographic ultrasound imaging (TUI) of the brain demonstrating all important brain structures including the lateral ventricles, the cerebellum, the cavum septum pellucidum and insula.
Three-dimensional and four-dimensional ultrasound application in prenatal diagnosis
Fig. 14.3 In this fetal thorax, the cross-section volume acquisition in anterior–posterior tomographic mode is used to demonstrate the slices of the heart, lungs, stomach, diaphragm, etc. In the lower middle panel the bifurcation of the trachea is well seen.
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Fig. 14.4 TUI can be used for the heart combined with STIC. Within one image mainly moving structures can be seen.
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Once a volume data set of a fetal region is stored, the examiner can scroll through the volume to get the plane of interest independent from the insonation angle. In a STIC volume the reconstructed cardiac volume can be displayed in the multiplanar or tomographic modes (Fig. 14.4), and played in slow motion or stopped at any time for detailed analysis of specific phases of the cardiac cycle. When combined with colour Doppler, events within the cardiac cycle during systole and diastole can be very well demonstrated.

SURFACE MODE RENDERING

The image rendering of the fetal surface is the best known and most commonly used display modality in 3D and 4D. From the volume acquired, the skin is pri­marily demonstrated (surface) and not the organs inside the body. It is used to visualize the surface of a structure which is best achieved in the interlay between fluid and surface, such as the face of a fetus in amniotic fluid or the valves within the heart. The main advantage of the technique is its ease of use and its impact on patients and doctors due to the lifelike image (Fig. 14.5), and comparison with the postnatal appearance. Clinical applications include demonstration of the whole fetus in the first trimester up to 12 weeks' gestation (see Fig. 14.5), and demonstration of the face (Fig. 14.6), limbs, etc. in order to rule out or confirm anomalies involving the skin as well as facial anomalies, spina bifida, limb anoma­lies and others. It is best demonstrated using 3D as well as 4D, whereas the latter
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Fig. 14.5 Surface mode demonstrating two fetuses at 10 weeks (left) and at 13 weeks.
Three-dimensional and four-dimensional ultrasound application in prenatal diagnosis
Fig. 14.6 With surface mode, maturation of the face and changes occurring during pregnancy are well recognized. Three fetuses at 14 weeks (left), 26 weeks (middle) and 31 weeks (right).
can be used to analyse behaviour such as fetal movements, grimacing, yawning or eye opening. Surface rendering can be applied to the fetal heart to visualize car­diac cavities and valves (Fig. 14.7). It can be used in the brain to demonstrate cavi- ties such as the lateral ventricles, especially in the presence of brain anomalies.

MAXIMUM MODE RENDERING

This mode is used to highlight the maximal echo information of a volume data set and is an ideal tool for the 3D reconstruction of bony structures (Fig. 14.8).6 In general, cranial bones, the ribs and other curvilinear bones cannot be clearly seen in a single 2D plane and are therefore better assessed in a maximum mode pro­jection. This technique has been applied in the demonstration of spine and limb abnormalities but was recently used in the assessment of the nasal bones, the cra­nial bones and corresponding sutures in normal and abnormal conditions. technique delivers a picture similar to an x-ray of the bony skeleton in the fetus.
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This
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