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ab

5.4 Frontal Space Distance

33
A
A
**
M
M
MM
c
A
Fig. 5.3 Normal and abnormal appearance of retronasal triangle. (TAS) (a) Normal retronasal triangle arrows point to intact base of triangle, apex (A), mandible (M). (b) Midline defect (* denotes defect seen on either side of
M
M
vomer) in the base of the triangle in a case of bilateral
CLP. (c) Lateralized defect (arrows) in the base of the tri-
angle in a case of unilateral cleft palate
from the midsagittal section in a case of isolated cleft palate at 14weeks.
5.4 Frontal Space Distance
The Frontal Space (FS) distance is the distance between the forehead and an extended line inter­secting the most anterior portions of the mandi­ble and the maxilla (MM line) (Fig. 5.5). In
facial clefts, due to protrusion of the maxilla, there is an alteration in the normal position of the maxilla in relation to the mandible, thus
modifying the FS distance. Hence FS distance
can be used as a screening marker to evaluate
facial clefts [10].
Cases with bilateral CLP (Fig.5.6) and retrog­nathia (Fig.5.7) have a highly abnormal FS dis­tance, and this suggests that it may be useful in identifying a broad spectrum of anomalies. It is important to remember that different types of abnormalities have different effects on the FS distance. In cases of bilateral CLP and in retrog­nathia, measurements are increased, whereas in cases of median cleft, the FS distance is decreased.
34
ab
5 Signs andMarkers in theDetection ofFirst-Trimester Palatine Clefts
0.65 cm
Fig. 5.4 Appearance of the normal and shortened maxil­lary line. (TAS) (a) Midsagittal section illustrating normal maxillary line. (b) 3D sagittal section slightly off the mid­line demonstrating shortened maxillary line in isolated
NB
FS
CP. (Note that the maxillary line posterior to the premax­illa is absent (arrows point to the defect), premaxilla (dashed arrow). (Video 5.2)
with a palatine cleft, and sonographic visualiza­tion of this junction aids in their detection.
In normal fetuses, the posterior aspect of the
ML
M
maxillary line appears bid in midsagittal sec­tion. The shorter cranial line is the vomer bone
which is seen superior to and superimposed on a longer, more caudal line which is the hard second­ary palate. Both the vomer and secondary palate comprise the maxillary line in the posterior aspect.
The superimposed line can be imaged by gen­tly tilting the probe from one side to the other of the fetal nose focusing on the maxillary line using the same technique as to image the nasal bone. This line is better visualized in real-time imaging
Fig. 5.5 Normal frontal space distance. Midsagittal sec­tion of the fetal face showing measurement of FS distance. MM line (dotted line) connects the anterior protuberance of mandible and maxilla, extended superiorly. FS distance (solid line) is measured perpendicularly between the fetal forehead to the MM line. Nasal bone (NB), maxillary line (ML), mandible (M)
with the fetal neck in the neutral position or slight exion, as illustrated in Video 5.3.
The sign is based on the simple anatomical fact that the maxillary line appears single in an isolated cleft palate as the secondary palate is absent and only the vomer contributes to the maxillary line. The application of this sign also

5.5 Superimposed Line Sign

enables interpretation of cleft extension into the secondary palate, as shown in line diagram
The “superimposed line sign” (SLS) is a novel sonographic sign based on 2D imaging of the vomero-maxillary junction in the midsagittal view [11]. We proposed this sign for early diag­nosis of the cleft of the secondary palate. The vomero-maxillary junction is abnormal in fetuses
(Fig.5.8) [11]. Note that the posterior aspect of the maxillary line appears single in clefts involv­ing secondary palate (Fig.5.8 d, f, g and h).
Figure 5.9 is an illustration of the normal (Fig. 5.9a) superimposed line sign and the absence of superimposed line sign in bilateral
0.35 cm
5.5 Superimposed Line Sign
35
ba
NB
ML
Fig. 5.6 FS distance in bilateral CLP. (a) Midsagittal sec- tion illustrating premaxillary protrusion (arrowhead), maxillary gap (arrow), nasal bone (NB), maxillary line (ML), mandible (M). (b) The premaxillary protrusion in
NB
ML
M
M
bilateral CLP causes the MM line (dotted line) to be dis­placed anteriorly, thus increasing the FS distance (solid line)
ba
Fig. 5.7 FS distance in retrognathia. (a) Midsagittal sec- tion illustrating receding chin, nasal bone (NB), maxillary line (ML), mandible (M). (b) The receding chin causes the
(Fig.5.9b), median (Fig.5.9c), and isolated cleft palate (Fig.5.9d). Figure5.9a depicts the normal bid appearance of the posterior aspect of the maxillary line. Figure5.9b is a case of complete bilateral CLP with absent SLS. Note that the maxillary line in Fig.5.9b, d appears single in the posterior aspect. Here the maxillary line is formed only by the premaxilla and vomer.
In the rst trimester, the maxillary gap used
along with PMD serves as an efcient marker to
MM line (dotted line) to be anteriorly displaced, thus increasing the FS distance (solid line)
detect isolated CP [6, 10]. The retronasal triangle does not serve to identify isolated cleft of secondary palate and clefts conned to the premaxilla alone.
The strength of the superimposed line sign is that it gives the initial clue to suspect cleft of the secondary palate in cases where the routine, conventional signs are not present. This sign can
easily be incorporated into routine practice as we are familiar with the anatomical landmarks in midsagittal prole view.
36
a
b
cd
ef
5 Signs andMarkers in theDetection ofFirst-Trimester Palatine Clefts
NB
VB
M
P
PM
VB
PMP
VB
P
g
VB VB
PMP
h
PMP
VB
Fig. 5.8 Line diagram illustrating SLS in various types of palatine clefts. Illustration of superimposed line sign in various types of palatine clefts. NB—Nasal Bone, VB— Vomer Bone, PMP—Premaxillary protrusion, PM— Premaxilla, M—Mandible, P—Palate. (a) Normal Palate (b) Unilateral CLP involving premaxilla (c) Unilateral
CLP involving secondary palate (d) Unilateral CLP with midline extension into secondary palate (e) Bilateral CLP with intact secondary palate (f) Bilateral CLP involving secondary palate (g) Median CLP (h) Isolated cleft of the secondary palate
PMP
PM
5.6 Overview ofMarkers
The measurement of the frontomaxillary facial (FMF) angle and maxilla nasion mandible (MNM) angle are the other proposed markers for the detection of cleft palate [12, 13]. However, the clinical utility of these signs is yet to be estab-
lished in routine practice. The intact alveolar arch seen in the axial view is another important marker in rst-trimester evaluation of palate (see Chap.
4, Fig. 4.7) [14].
Figure 5.10 depicts the various signs and mark­ers described for rst-trimester evaluation of the palate in the sagittal, axial, and coronal planes.
ab
cd
5.6 Overview ofMarkers
37
ML
V
P
PM
V
V
V
PM
Fig. 5.9 Appearance of superimposed line sign in normal and abnormal cases. Midsagittal section - (a) Superim­posed line sign, the vomer bone (V) is seen superimposed on the posterior two-thirds of maxillary line (ML), (Video
5.3) (b) Absent superimposed line sign in a case of bilat-
* Maxillary gap sign * Frontal space distance * Palatino-maxillary diameter * Super imposed line sign * MNM angle * FMF angle
eral CLP premaxillary protrusion (arrow), (c) Median CLP with absent superimposed line sign, note the absence of premaxillary portion (arrow), (d) Absent superim­posed line sign in isolated CP. Palate (P), premaxilla (PM), vomer (V)
Signs and markers
Axial
*Intact alveolar arch
Coronal Sagittal
* Retronasal triangle
Fig. 5.10 Flow chart depicting the various signs and markers in the sagittal, axial and coronal planes
38
5 Signs andMarkers in theDetection ofFirst-Trimester Palatine Clefts

References

1. Syngelaki A, Chelemen T, Dagklis T, Allan L, Nicolaides KH. Challenges in the diagnosis of fetal non-chromosomal abnormalities at 11–13 weeks. Prenat Diagn. 2011 Jan;31(1):90–102.
2. Becker R, Wegner RD. Detailed screening for fetal anomalies and cardiac defects at the 11–13-week scan. Ultrasound Obstet Gynecol. 2006 Jun;27(6):613–8.
3. Salomon LJ, Alrevic Z, Raine-Fenning NJ, Timor­Tritsch I, Seshadri S. ISUOG practice guidelines: performance of rst-trimester fetal ultrasound scan. Ultrasound Obstet Gynecol. 2013;41(1):102–13.
4. Syngelaki A, Hammami A, Bower S, Zidere V, Akolekar R, Nicolaides KH.Diagnosis of fetal non­chromosomal abnormalities on routine ultrasound examination at 11–13 weeks’ gestation. Ultrasound Obstet Gynecol. 2019 Oct;54(4):468–76.
5. Chaoui R, Orosz G, Heling KS, Sarut-Lopez A, Nicolaides KH.Maxillary gap at 11–13 weeks’ gesta­tion: marker of cleft lip and palate. Ultrasound Obstet Gynecol. 2015 Dec;46(6):665–9.
6. Sepulveda W, Wong AE, Martinez-Ten P, Perez­Pedregosa J. Retronasal triangle: a sonographic landmark for the screening of cleft palate in the rst trimester. Ultrasound Obstet Gynecol. 2010 Jan;35(1):7–13.
7. Sepulveda W, Wong AE, Vinals F, Andreeva E, Adzehova N, Martinez-Ten P.Absent mandibular gap in the retronasal triangle view: a clue to the diagno-
sis of micrognathia in the rst trimester. Ultrasound Obstet Gynecol. 2012 Feb;39(2):152–6.
8. Suresh S, Vijayalakshmi R, Indrani S, Devaki G, Bhavani K. The premaxillary triangle: clue to the diagnosis of cleft lip and palate. J Ultrasound Med. 2006 Feb;25(2):237–42.
9. Lachmann R, Schilling U, Brückmann D, Weichert A, Brückmann A.Isolated cleft lip and palate: maxillary gap sign and palatino-maxillary diameter at 11–13 weeks. Fetal Diagn Ther. 2018;44(4):241–6.
10. Hoopmann M, Sonek J, Esser T, Bilardo CM, Wagner P, Abele H, Kagan KO. Frontal space dis­tance in facial clefts and retrognathia at 11–13 weeks’ gestation. Ultrasound Obstet Gynecol. 2016 Aug;48(2):171–6.
11. Lakshmy SR, Rose N, Masilamani P, Umapathy S, Ziyaulla T. Absent ‘superimposed-line’sign: novel marker in early diagnosis of cleft of fetal sec­ondary palate. Ultrasound Obstet Gynecol. 2020 Dec;56(6):906–15.
12. Pan M, Chen M, Sahota DS, Lao TT, Lau TK, Leung TY.Can we use the frontomaxillary facial angle in the rst trimester to predict facial cleft? Prenat Diagn. 2012 May;32(5):491–3.
13. de Jong-Pleij EA, Ribbert LS, Manten GT, Tromp E, Bilardo CM.Maxilla–nasion–mandible angle: a new method to assess prole anomalies in pregnancy. Ultrasound Obstet Gynecol. 2011 May;37(5):562–9.
14. Lakshmy SR, Deepa S, Rose N, Mookan S, Agnees J. First-trimester sonographic evaluation of palatine clefts: a novel diagnostic approach. J Ultrasound Med. 2017 Jul;36(7):1397–414.
3D Techniques toEvaluate Palate at 11–14Weeks
6
It has been observed that it is very easy to visual­ize the entire primary and secondary palate in the axial plane at 11–14weeks using multiplanar imaging. One of the probable explanations for this would be that the secondary palate in the rst trimester is at and not arched as it appears in later gestation. The other is the lack of ossi­cation of all facial structures surrounding the palate at this period of gestation. Therefore there is minimal shadowing from surrounding facial bones, which favors the study in multiplanar mode display.
Acoustic shadowing is always a signicant problem in the second and third trimesters, which hinders visualization of the palate [1, 2]. Indeed,
Campbell considers that in the rst trimester, the absence of acoustic shadowing from the maxilla is an advantage for an early examination of the sec­ondary palate [3]. Further, the secondary palate is larger in the lateral than in the anteroposterior dimension, favoring its examination in the axial plane [1] This chapter deals with the ofine analy­sis of 3D datasets in evaluating the palate. Most
cases of orofacial clefts can be accurately detected and characterized at this gestational age by analyzing the 3D datasets [4, 5].
Supplementary Information The online version of this chapter (https://doi.org/10.1007/978- 981- 16- 4613- 3_6) contains supplementary material, which is available to authorized users.
© The Author(s), under exclusive license to Springer Nature Singapore Pte Ltd. 2021 L. R. Selvaraj, T. Ziyaullah, First and Mid Trimester Ultrasound Diagnosis of Orofacial Clefts,
https://doi.org/10.1007/978-981-16-4613-3_6

6.1 Multiplanar Imaging

For evaluating the palate a volume acquisition is done in the midsagittal view of the fetal face after adequate magnication. 3D data sets are obtained using a 40-degree sweep from one side of the face to the other using optimized sonographic settings. Ofine analysis of the stored 3D data sets is used to assess the sectional anatomy of the palate.
The essential markers to evaluate the palate are an intact maxillary line in the sagittal view, continuity of the alveolar arch of the maxilla in the axial view and intact base of the retronasal triangle in the coronal view. All the abovemen-
tioned 2D markers in the three orthogonal planes can be visualized simultaneously by multiplanar imaging (Fig. 6.1). Video 6.1 illustrates the simultaneous visualization of the maxillary line in the sagittal view and the palate in the axial view in multiplanar imaging.
Multiplanar imaging helps to identify the anatomic structure in the three orthogonal planes simultaneously with the use of a refer­ence dot. The anatomy of the retronasal triangle
is illustrated in Fig.6.2. The reference dot, when placed at the apex of the triangle as in Fig.6.2a shows the nasal bone. The reference dot, when placed at the base of the triangle as in Fig.6.2b shows the palate. Video 6.2 illustrates the anat­omy of the retronasal triangle in multiplanar imaging using the reference dot.
Figure 6.3 is an illustration of the anatomy of vomero-maxillary junction with reference dot
39
40
6 3D Techniques toEvaluate Palate at 11–14Weeks
a
P
Fig. 6.1 Multiplanar imaging of palate in orthogonal planes. (a) 14weeks (TVS) representation of base of the retronasal triangle in orthogonal planes (note that the ref­erence dot is placed at the base of the RNT in plane A, the maxillary line (ML), and palate (P) is visualized in B and
A
ML
C
B
a
A
NB
b
ML
C planes respectively. (b) 14weeks (TAS) representation of the palate in orthogonal planes (note that the reference dot is placed on the maxillary line in plane A, the palate is visualized in axial and coronal planes in B and C planes, respectively.(Video 6.1)
A
P
C
B
b
B
P
Fig. 6.2 Multiplanar imaging illustrating anatomical landmarks of the retronasal triangle (14 weeks TVS). (a) The reference dot placed at the apex (A) of the tri-
placed on the caudal and cranial lines. The refer-
ence dot, when placed on the cranial line highlights, the vomer (Fig. 6.3a) and when placed on the caudal line depicts the secondary palate (Fig. 6.3b).
Figure 6.4 is an illustration of the vomer bone in multiplanar imaging. Note that the vomer fuses with the posterior two-thirds of the palate as shown in plane C (see line diagram Chap. 2, Fig. 2.5).
angle shows the nasal bone (NB). (b) The reference dot placed at the base (B) of the triangle shows the palate (P) (Video 6.2)

6.2 Volume Contrast Imaging

Volume contrast imaging (VCI) is a technique that projects a 1–10mm slice of a volume data­set onto a two-dimensional (2D) screen. As a
result of adding the tissue information from adja­cent layers, there is an enhancement of tissue demarcation leading to better visualization of structures. Figure6.5 illustrates the utility of VCI in imaging the fetal face.

6.3 Omni View Technique

41
a
V
V
P
Fig. 6.3 Multiplanar imaging of the vomer and palate. (a) Reference dot placed on cranial line corresponds to the vomer (V) in axial view. (b) Reference dot placed on caudal line corresponds to the palate (P) in axial view
A
b
V
P
P
C
O
V
NB
V
O
Fig. 6.4 Multiplanar imaging of the vomer (14weeks TVS) Plane A axial section through the nasal cavity, the refer­ence dot placed on the vomer (V) illustrates its appearance in the sagittal section in plane C.Orbits (O), nasal bone (NB)
6.3 Omni View Technique
Omni view technique is a exible technique that allows the operator to draw lines in any direction or angle to visualize the area of interest in the volume dataset. This technique is an excellent
tool in evaluating the palate, especially in mid trimester to assess the curved anatomy of the palate. The entire secondary palate can be visual-
ized as an echogenic plate of bone caudal to the alveolar arch as demonstrated by omni view in Fig.6.6 [6]. The utility of the omni view line in
42
6 3D Techniques toEvaluate Palate at 11–14Weeks
a
a1
b
b1
Fig. 6.5 Utility of VCI in evaluating fetal face. Midsagittal section of the face without VCI, (a, b) and their correspond- ing images with VCI (a1, b1)
evaluating the retronasal triangle is shown in Chap. 4, Fig. 4.6.
6.4 Rendered View
oftheIntraoral Surface ofthePalate
Rendering of the palate can be done by using the ipped face technique described by Platt et al. [7]. After the usual volume acquisition in the sag­ittal plane (Fig.6.7a), the acquired volume was rotated to obtain a facial image, as shown in
Fig.6.7b. The render box is placed on the maxil­lary line with the green dotted line facing the intraoral surface of the palate as shown in Fig.6.7c. By this technique, the axial view of the palate is seen in the rendered view.
Various rendering modes can be used to depict the palate, like surface and maximum mode ren­dering Fig.6.8a and HD live mode Fig.6.8b. The anatomic landmarks of the fetal palate can be well appreciated in the rendered image, as shown in Fig.6.8e [8].
Examination of the palate can be done very quickly by using only the surface rendering mode