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7.4 Atypical Cleft
73
a
NB
M
V
b
c
Fig. 7.26 Median cleft lip and palate at 12weeks (TVS). (a) Midsagittal view Absence of premaxillary portion (arrow), (b) Axial view midline defect in the alveolar arch
(arrows) (c) TUI—Coronal view showing the decient base of the retronasal triangle in all sections (pointer). Nasal bone (NB), mandible (M), vomer (V)
74
f
abdc
NB
7 Protocol forDiagnosing Type andExtent ofaCleft inFirst Trimester
V
M
e
Fig. 7.27 2D vs. 3D imaging in median CLP at 13weeks (a) (TVS) midsagittal section small echogenic line in the posterior aspect formed by vomer (V). (b) (TVS) axial section midline defect seen in the alveolar arch (arrow), (c) (TAS) coronal plane absence of the base of RNT
f
(arrows), (d) (TVS) coronal section midline defect (arrowhead) in upper lip in 3D with VCI, (e) (TVS) Rendered image of the face. (f) (TVS) multiplanar imag­ing reference dot showing defect in three orthogonal planes. Nasal bone (NB), mandible (M) (Video 7.7)
P
M
V
Fig. 7.28 TUI in median CLP at 13weeks. (TVS) Midsagittal section (M) shows the vomer (V) in midline and para­sagittal (P) sections depicting absence of the maxillary line (arrows)
P
7.4 Atypical Cleft
75
a
Fig. 7.29 Flipped face technique in median CLP at 13weeks. (TVS) (a) Flipped face technique. (b) Rendered view of the intraoral surface of palate. Note the midline
a
NB
V
M
b
*
discontinuity (arrow) in the alveolar arch. As the cleft extends into the secondary palate, the vomer (*) is imaged in midline
b
Fig. 7.30 2D vs. 3D imaging in mediolateral CLP at 12 weeks. (a) (TVS) midsagittal section maxillary line shows a large gap (arrowhead) with a single line (V) in the posterior aspect. (b) (TVS) rendered view of the face. (c) 2D coronal section and (d) 3D coronal section showing
defect in the base of RNT (arrows), (e) 2D axial section and (f) 3D axial section showing mediolateral defect in the alveolar arch (arrow). Nasal bone (NB), mandible (M), vomer (V)
76
7 Protocol forDiagnosing Type andExtent ofaCleft inFirst Trimester
c
e
d
f
Fig. 7.30 (continued)
7.4 Atypical Cleft
77
a
M
d
b
e
Fig. 7.31 Median CLP in holoprosencephaly at 14weeks. (a) (TAS) Midsagittal section absence of maxil­lary line. (b) (TVS) axial view midline defect in the alveo­lar arch (arrow). (c) (TAS) coronal view defect in the base
c
of RNT (arrows). (d) (TVS) rendered view of the face, (e) (TAS) Rendered view of fused choroid plexus in holo­prosencephaly (*).Mandible (M) (Video 7.8)
a
NB
M
V
Fig. 7.32 Sagittal section demonstrating the varying appearance of maxillary line in median CLP. (a) 12weeks (TVS) arrow points to the absent premaxillary portion of
b
V
the maxillary line. (b) 13 ened and interrupted maxillary line. Nasal Bone (NB), Mandible (M), Vomer (V)
+3
weeks (TVS) showing short-
78
SAGITTAL
7 Protocol forDiagnosing Type andExtent ofaCleft inFirst Trimester
a
VB
c
MIDSAGITTAL
AXIAL
b
PARASAGITTAL
d
CORONAL
MEDIAN
b
Fig. 7.33 Line diagram illustrating the 2D markers in median CLP. (a) Appearance of maxillary line in midsagittal and parasagittal section in Median CLP. (b) Sagittal plane. (c) Axial plane. (d) Coronal plane
a
Fig. 7.34 Atypical cleft caused by amniotic bands. 14weeks (TAS) (a) Comparison of the alveolar arch of the mandible and maxilla. The mandible is intact, and the maxillary alveolar arch shows an open cleft (arrow). (b) Sagittal view of the face arrow points to the amniotic
band. (c) Coronal view showing the retronasal triangle. Both frontal processes of the maxilla are seen with the absence of the base and the amniotic band (arrow) seen in between (d) Rendered view of the face. Reproduced with permissions from Wiley

References

79
c
Fig. 7.34 (continued)
References
1. 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.
2. 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.
3. 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.
4. Martinez-Ten P, Adiego B, Illescas T, Bermejo C, Wong AE, Sepulveda W.First-trimester diagnosis of cleft lip and palate using three-dimensional ultrasound. Ultrasound Obstet Gynecol. 2012 Jul;40(1):40–6.
5. Yamanishi T, Kobayashi C, Tsujimoto I, Koizumi H, Miya S, Yokota Y, Okamoto R, Iida S, Aikawa T, Kohara H, Nishio J.An uncommon cleft subtype of unilateral cleft lip and palate. J Dent Res. 2008 Feb;87(2):164–8.
6. Platt LD, DeVore GR, Pretorius DH.Improving cleft palate/cleft lip antenatal diagnosis by 3-dimensional
d
sonography: the “ipped face” view. J Ultrasound Med. 2006 Nov;25(11):1423–30.
7. Lakshmy SR, Rose N, Masilamani P, Umapathy S, Ziyaulla T.Role of TUI in rst trimester evaluation of palate. J Fetal Med. 2019 Sep;6(3):113–21.
8. 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.
9. Lakshmy SR, Rose N, Masilamani P, Umapathy S, Ziyaulla T.First trimester ultrasound evaluation of the cleft palate: midsagittal, axial or coronal view-which view is best? J Fetal Med. 2020 Sep;7(3):183–91.
10. Nyberg DA, Hegge FN, Kramer D, Mahony BS, Kropp RJ. Premaxillary protrusion: a sonographic clue to bilateral cleft lip and palate. J Ultrasound Med. 1993 Jun;12(6):331–5.
11. 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.
12. 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.
Sonoanatomy ofPalate at20Weeks
8
There is no sonographic study presently that pro­vides a technique to evaluate the complete fetal secondary palate [1]. Comprehension of the real
anatomy of the fetal palate with what is visual­ized in ultrasound is still now an extremely dif­cult task. This can be attributed to the curved
nature of the palate in mid trimester and more­over, shadowing from the surrounding facial bones hinders visualization of palate.
It is a common perception that with the help of 3D ultrasound, the secondary palate can be imaged better. A number of three-dimensional (3D) techniques have been proposed in imaging the secondary palate.
However, its visualization in 3D sonography requires adequate volume acquisition, with the fetal neck in slight extension and the presence of uid between the fetal tongue and palate. It also
needs expertise with the usage of 3D ultrasound techniques. So the need of the hour is a simple 2D ultrasound technique to assess the fetal palate.
8.1 Technique ofVisualizing
Palate
Analysis of the hard palate is difcult and consid­ered unreliable and is not included in guidelines for the routine anatomic survey. Though the
Supplementary Information The online version of this chapter (https://doi.org/10.1007/978- 981- 16- 4613- 3_8) 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_8
entire secondary palate cannot be imaged, visual­ization of uvula on ultrasound was proposed as a surrogate marker for the intactness of the second­ary palate. As cleft of the secondary palate
always starts at the uvula and proceeds along the midline during embryonic development, visualization of the uvula rules out a secondary cleft palate.
The echo pattern of the normal uvula on ultra­sound can be imaged as the “equals sign” in both sagittal and axial views at the level of the oro­pharynx (Fig.8.1). The soft palate hangs from the posterior margin of the hard palate and extends backward and downward, separating the orophar­ynx from the nasopharynx. The soft palate can be imaged in the sagittal section with the fetal neck in extension and uid in the oral cavity, as shown in Fig.8.1a.
The uvula is a good landmark for directing the examination plane to the soft palate [2]. The
uvula can be visualized in the corresponding transverse section at the base of the skull at the level of the oropharynx (Fig. 8.1b). Video 8.1 illustrates the visualization of uvula as the equal sign in axial view.
The ideal time for visualizing the soft palate is after 20weeks of gestation. In this gestational
period, the uvula can be identied consistently as the soft palate, and the uvula lengthens with advancing gestation age [2, 3]. Figure 8.2 illus­trates the visualization of the soft palate at 16 weeks of gestation. (Compare Fig.8.2 with Fig.8.1a).
81
82
8 Sonoanatomy ofPalate at20Weeks
a
HP
T
SP
Fig. 8.1 Visualization of the uvula at 22weeks (a) sagit- tal section bony hard palate (HP), soft palate (SP), uid in the oral cavity (*), tongue (T). Note that the soft palate (arrows) ends at the uvula (arrowhead). Arrows point to
SP
HP
b
the soft palate, and arrowhead point to the uvula. (b) Axial view demonstrating the equal sign pointers show the uvula
be in slight extension as shown in (Fig. 8.3b) which enhances visualization of the bony sec­ondary palate.

8.1.2 Swallowing Fluid Dynamics

Fig. 8.2 Visualization of the palate at 16 weeks (a)
Sagittal plane shows the bony hard palate (HP) and soft palate (SP) with fetus in slight extension (arrow) (Video
8.1)

8.1.1 Fetal Position

The best way to image the palate is when the fetus faces the transducer and when the head is slightly extended. The premaxilla can be evalu-
ated in the midsagittal section of the fetus, how­ever, the neutral position or slight exion of the fetus is not ideal for visualization of the second­ary palate (Fig.8.3a). Note that the fetus has to
The presence of uid in the oral cavity is a facili­tating factor to visualize the secondary palate in mid trimester [4]. Figure8.4a shows the midsag­ittal prole of the fetus in the neutral position.
Note that though the maxillary line is visible, the entire secondary palate cannot be visualized in this position.
In Fig.8.4b, the hard palate and the muscular soft palate can be imaged in their entire length with the fetus in extension position and uid in the oral cavity. Moreover, at the time of fetal swallowing, movements of the soft palate can be appreciated during real-time ultrasound evaluation. Video 8.2 illustrates uid in the oral cavity during swallowing movements of the fetus, which enhances visualization of the sec­ondary palate. This is a boon to evaluate, espe-
cially the soft palate; the muscular palate moves with fetal swallowing and hence can be clearly assessed on simple 2D ultrasound.
Figure 8.4c is the sagittal prole of a fetus with cleft of the secondary palate. Note that the
8.2 Planes ofEvaluating Palate
83
a
PM
*
Fig. 8.3 Fetal position in the visualization of maxillary line (a) Sagittal section inadequate visualization of the maxillary line due to shadowing (*) from premaxilla
maxillary line is indistinct. However, during
swallowing movement of the fetus, the entire maxillary line is absent clearly depicting the communication between the oral cavity and nasal cavity (Fig.8.4d). Video 8.3 demonstrates
an absent maxillary line in the cleft of the sec­ondary palate.
8.1.3 Angle ofInsonation
The main limitation of visualizing the entire pal­ate is most often due to the unfavorable position of the fetus. Figure8.5a is a fetus in the neutral position in which the posterior aspect of the hard palate is inadequately imaged.
To avoid acoustic shadowing from the alveo­lar arch, the secondary palate needs to be insonated at a 45° angle in the sagittal plane
[5]. The beam has to be focussed on the under surface of the palate with the fetus in a slight extension position, as shown in Fig.8.5b.
The best results can be obtained by a combi­nation of all three factors, namely fetal position, angle of insonation and the presence of uid in the oral cavity (Fig.8.5c). Video 8.4 shows visu-
alization of the secondary palate using the right angle of insonation with the fetal face facing upwards.
b
ML
(PM). (b) Sagittal section adequate visualization of the maxillary line (ML) with fetal neck in slight extension in the same case
8.2 Planes ofEvaluating Palate

8.2.1 Sagittal Plane

The midsagittal facial prole can be used to assess the maxillary line and the primary palate. The intact maxillary line in the sagittal prole represents the palate. The facial prole view is
not sufcient to visualize both the hard and soft palate (Fig.8.4a). The secondary palate needs to
be evaluated with the head in extension and uid in the oral cavity, as shown in Fig.8.4b.
The same midsagittal prole can also be used to evaluate the superimposed line sign, however, this sign is well seen in the late rst trimester and in the early mid trimester [6]. Note that the pos-
terior aspect of the maxillary line has a bid appearance due to the presence of the vomer and the secondary palate (Fig.8.6). This sign is
better visualized when the fetus is in neutral or in a slight exion position. Video 8.5 depicts the superimposed line sign at 20weeks of gestation.
The anatomical landmarks of importance in the midsagittal prole are the nasal bone, max­illary line, and mandible. These three landmarks
in a normal fetus appear to fall on a straight line, as shown in Fig8.7a.
The premaxillary protrusion seen in bilateral CLP is best visualized in this view [7] (Fig.8.7b).