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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5805_Библиотеки_им_академика_М_И_Перельмана.pdf
126
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
c
*
*
b
d
*
*
Fig. 10.24 Anterior axial view in bilateral CLP illustrating phenotypic variations. (a) Prominent paramedian
labial defect (arrowheads) (b) Subtle paramedian defect
(pointers). (c) Asymmetry of palatal shelf separation on
either side of the premaxilla (*) arrow points to the subtle
defect. (d) Marked degree of palatal shelf separation on
both sides (*) (Arrow points to bilateral paramedian
clefts)
10.3 Median
127
a
d
Fig. 10.25 Rendered view of the face in bilateral CLP
with varying degrees of premaxillary protrusion. (a) No
premaxillary protrusion (b) subtle premaxillary protru-
b
e
c
f
sion. (c and d) median process well seen with symmetrical
clefts on either side. (e and f) marked premaxillary protrusion with cleft asymmetry on either side
a
N
UL
*
UL
C
Fig. 10.26 Median cleft lip at 20weeks (a) Nose chin view shows median cleft lip with the absence of midline phil-
trum and median process (*), (b) Corresponding rendered view of the face. Nose (N), Upper lip (UL), chin (C)
b
128
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
UL
N
*
UL
Fig. 10.27 Bilateral vs. Median cleft lip (a) Nose chin
view in bilateral CLP presence of median process(*) with
bilateral paramedian defect (arrows). Note that the upper
a
NB
V
M
b
UL
N
*
UL
lip is partitioned into three segments. (b) Nose chin view
in median cleft lip absence of median process (*) and midline philtrum. Upper lip (UL), nose (N)
b
c d
*
Fig. 10.28 Median CLP at 20weeks (a) Midsagittal sec-
tion absence of proximal portion of maxillary line. Note
that the line visible in the posterior aspect is only the
vomer (V). (b) Parasagittal section complete absence of
maxillary line (arrow). (c) Axial section interrupted alveo-
lar arch (arrows) and central cleft extending into secondary palate (*) denotes the decient posterior bony edge of
the palate. (d) Rendered view of the face shows median
cleft lip. Nasal bone (NB), Mandible (M). (Video 10.5)
10.3 Median
129
a
NB
M
d
Fig. 10.29 Median CLP associated with alobar holoprosencephaly at 23 weeks. (a) Midsagittal section at
facial prole with complete absence of maxillary line
(arrow). (b) Nose chin view where there is a midline
defect (*) in the upper lip (UL). (c) The alae nasi can
b
UL
UL
e
f
c
UL
N
N
UL
f
T
mimic paramedian cleft, and the nostril mimics the
median process (arrows). (d) Axial view illustrates hypotelorism. (e) Depicts the fused thalami (T) with cupshaped monoventricle. (f) Postnatal correlation. Nasal
bone (NB), Mandible (M) and Nose (N)
a
ML
Fig. 10.30 Pitfalls in imaging the maxillary line in midline cleft palate (a) Midsagittal prole illustrating the
absence of premaxilla and shortening of the maxillary line
(ML) the visible portion of the maxillary line is formed by
b
V
T
vomer (V). (b) Same case, the echogenic upper border of
the tongue (T) should not be mistaken for the intact maxillary line
130
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
References
1. Salomon LJ, Alrevic Z, Berghella V, Bilardo C,
Hernandez-Andrade E, Johnsen SL, Kalache K, Leung
KY, Malinger G, Munoz H, Prefumo F.Practice guidelines for performance of the routine mid-trimester
fetal ultrasound scan. Ultrasound Obstet Gynecol.
2011 Jan;37(1):116–26.
2. Rotten D, Levaillant JM.Two-and three-dimensional
sonographic assessment of the fetal face. 1. A systematic analysis of the normal face. Ultrasound Obstet
Gynecol. 2004;23(3):224–31.
3. 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.
4. 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.
5. Rotten D, Levaillant JM.Two-and three-dimensional
sonographic assessment of the fetal face. 2. Analysis
of cleft lip, alveolus and palate. Ultrasound Obstet
Gynecol. 2004;24(4):402–11.
6. Wilhelm L, Borgers H. The ‘equals sign’: a novel
marker in the diagnosis of fetal isolated cleft palate.
Ultrasound Obstet Gynecol. 2010 Oct;36(4):439–44.
7. 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.
8. 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.
9. Maarse W, Pistorius LR, Van Eeten WK, Breugem CC,
Kon M, Van den Boogaard MJ, Mink van Der Molen
AB.Prenatal ultrasound screening for orofacial clefts.
Ultrasound Obstet Gynecol. 2011 Oct;38(4):434–9.
Isolated Cleft ofSecondary Palate
11
Over the years, the detection rate of isolated cleft
palate in ultrasound has been very minimal, and
many studies quote that none of the isolated cleft
palate have been detected prenatally. The poor
detection rate of the isolated cleft palate at 0–
1.4% illustrates that there is no robust technique to evaluate the secondary palate.
Evaluation of palate is not included in the protocol of mid trimester anomaly scan. As the trend
is now to identify anomalies in the rst trimester,
there has been a paradigm shift in our approach to
the diagnosis of facial anomalies at 11–14weeks
scan. This chapter deals with sonographic markers to detect cleft of the secondary palate in both
rst trimester and in mid trimester.
11.1 Description ofIsolated Cleft
ofSecondary Palate
In the isolated cleft of the secondary palate, neither the lip nor the alveolar process is involved.
As the fusion of the hard and soft palates proceeds from front to back, cleft of the posterior
palate always begins with uvula.
From the uvula, the cleft may extend anteriorly to varying degrees involving either the soft
palate alone or both soft and hard palates. In the
Supplementary Information The online version of this
chapter (https://doi.org/10.1007/978- 981- 16- 4613- 3_11)
contains supplementary material, which is available to
authorized users.
extreme form, the cleft palate may extend anteriorly as far as the incisive foramen.
If the cleft extends up to the incisive foramen,
then the nasal chambers communicate with the oral
cavity. Most often, the nasal septum has no attachment to the palatal shelves throughout the extent of
the cleft. In cleft of the secondary palate, the nasal
septum is visible in the midline in axial view,
which serves as a diagnostic clue to identify CP.
11.2 Diagnostic Approach
withSonographic Signs
inFirst Trimester
The diagnostic approach to identify isolated cleft
of the secondary palate at 11–14weeks is to evaluate the bony secondary palate. As embryologically isolated cleft of secondary palate starts at
the uvula and extends anteriorly, visualization of
the uvula as the equal sign in mid trimester indicates that the secondary palate is intact [1].
But, however, this concept is not useful in
diagnosing cleft of the secondary palate in the
rst trimester. Recently we have proposed a new
marker which is the absent superimposed line
sign to detect cleft of the secondary palate in
late rst trimester [2].
In the rst trimester the hard palate is evaluated by a combination of all three planes, namely
sagittal, axial, and coronal planes (see Chap. 4,
Fig. 4.7). However, in the diagnosis of isolated
© 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_11
131
132
c
b
a
NB
11 Isolated Cleft ofSecondary Palate
CP the axial plane and the retronasal triangle are
not useful.
The described markers in the rst trimester,
which are of importance in the diagnosis of isolated cleft palate in the sagittal plane are the
maxillary gap sign, the palatino-maxillary
diameter and the superimposed line sign (see
Chap. 5).
Figure 11.1 is a line diagram that illustrates
the salient ndings in the sagittal plane to diagnose varying degrees of the cleft of secondary
palate. If the cleft is conned only to the soft palate, then the maxillary line appears normal in
midsagittal and parasagittal sections (Fig.11.1a).
The presence of the maxillary gap and shortening of the maxillary line in midsagittal section depends on the size and the anterior extent
of the midline cleft [3, 4]. If the midline cleft
extends up to the incisive foramen, as shown in
Fig.11.1b, there is obvious evidence of maxillary
gap and shortening of the maxillary line in midsagittal view.
If the cleft is conned only to the posterior
aspect, as shown in Fig.11.1c then both the signs
are absent. However, in both types of cleft,
(Fig. 11.1b, c), the superimposed line sign is
absent in the midsagittal section, and there is a
shortening of the maxillary line in the parasagittal section [2].
In isolated CP, the premaxillary portion is
present, and the secondary palate is absent in the
midline. The initial and the only clue to the
diagnosis of isolated CP is the sagittal view.
(Fig.11.2a and b) [3, 4]. Note that the echogenic
border of the tongue shown in Fig.11.2a should
not be mistaken for the intact maxillary line.
The maxillary gap is seen between the premaxilla and the vomer. But in some cases, the
vomer along with the premaxilla gives a deceptive appearance of an intact maxillary line in the
midline. In sagittal sections slightly off the midline, only the premaxilla is seen as a small proximal segment. Figure 11.3 shows three cases of
isolated cleft palate with the absent superimposed
line sign. Also, note that there is no maxillary gap
in the midsagittal view.
However, their corresponding parasagittal
sections off the midline show obvious shortening
of the maxillary line. The normal bid appear-
ance of the posterior aspect of the maxillary line
is not seen in cases of cleft secondary palate.
As cleft of the secondary palate always starts
posteriorly this absent superimposed line sign is
seen in all degrees of clefts which extends to the
Fig. 11.1 Line diagram
illustrating the salient
ndings in the sagittal
plane in isolated CP. (a)
Cleft conned to soft
palate. (b) Cleft
involving the entire
secondary palate. (c)
Cleft conned to
posterior part of
secondary palate
VB
M
P
MIDSAGITTAL PA RASAGITTAL
PM
VB
11.2 Diagnostic Approach withSonographic Signs inFirst Trimester
133
a
NB
T
Fig. 11.2 Midsagittal prole in isolated CP in two cases
(TVS) (a) Case 1: 12
ened and interrupted, arrowhead points to the maxillary gap.
+6
weeks maxillary line appears short-
M
bony secondary palate. The only type of cleft in
which there is a normal superimposed line sign
is a cleft limited to the soft palate (Fig. 11.1a)
and in bid uvula.
The advantage of the absent superimposed
line sign is that it is seen in the routine midsagittal section and does not require an additional
plane. The posterior aspect of the maxillary line
appears single in all cases of the cleft of bony
secondary palate and is seen even when the other
signs are not evident.
Figure 11.4 is an illustration of 2D markers in
the three orthogonal planes and their correlation in
multiplanar imaging. Figure11.4a is a midsagittal
prole showing the absent superimposed line sign,
and Fig.11.4 b shows shortening of the maxillary
line in sagittal sections off the midline.
In isolated CP, the alveolar arch in the axial
view appears normal (Fig.11.4c), and the base of
the retronasal triangle is intact (Fig. 11.4d).
Figure11.4 e illustrates these ndings in multiplanar imaging. As the secondary palate is de-
cient, the nasal septum (vomer) is seen as an
echogenic structure.
Figure 11.5 illustrates the usage of 3D ultrasound in the diagnosis of isolated cleft palate.
The 3D midsagittal section (Fig.11.5a) shows an
intact maxillary line, whereas the parasagittal
section (Fig.11.5b) shows shortening of the max-
b
NB
M
(b) Case 2: 12weeks presence of maxillary gap (arrow) and
a single line on the posterior aspect of the maxillary line
(ML). Nasal bone (NB), mandible (M), tongue (T)
ML
illary line. Figure 11.5c is the rendered face
which shows the associated retrognathia.
The base of the RNT is intact in the anterior
coronal section (Fig. 11.5d), whereas the base is
decient in sections posterior to the retronasal
triangle (Fig. 11.5e). The rendered view of the
palate in the axial section depicts the intact alveolar arch with the vomer seen in the midline.
Tomographic ultrasound imaging in isolated
CP (Fig.11.6a) depicts the normal appearance of
maxillary line in midsagittal section and shortening of maxillary line in the parasagittal section.
(Compare with TUI of a normal palate in
Fig.11.6b).
Tomographic ultrasound imaging is an excellent tool in assessing isolated CP, as the secondary palate is decient in sections posterior to the
retronasal triangle, as shown in Figure 11.7a
(compare with the coronal sections in a normal
case illustrated in Fig.11.7b) [5]. The retronasal
triangle in the rst section appears intact due to
the presence of premaxilla in the isolated cleft
palate.
Figure 11.8a is multiplanar imaging in isolated CP.The reference dot, when placed on the
premaxilla, shows the intact alveolar arch in axial
view and intact soft tissue of the upper lip in coronal view. In Fig. 11.8b, the reference dot is
placed on the defect in axial view, the
134
bb
cc
11 Isolated Cleft ofSecondary Palate
a
NB
ML
a1
PM
1
PM
ML
1
PM
ML
Fig. 11.3 Appearance of maxillary line in isolated CP (a
and a1) Case 1: (a) 14weeks (TAS) midsagittal section no
maxillary gap and a single line in the posterior aspect of
the maxillary line (ML), (a1) (TVS) shortening of the
maxillary line in sagittal section slightly off the midline,
arrows points to the absence of the maxillary line caudal
to premaxilla (PM), nasal bone (NB). (b and b1) Case 2:
+6
12
weeks (TVS), (c and c1) Case 3: 14weeks (TAS)
ab
11.2 Diagnostic Approach withSonographic Signs inFirst Trimester
135
NB
ML
PM
c
d
e
V
Fig. 11.4 Sagittal, axial, and coronal planes in isolated
CP at 14weeks (TAS) (a) midsagittal section illustrating
the absence of superimposed line sign in the posterior
aspect of the maxillary line (ML). (b) sagittal section off
the midline illustrating the absence of the maxillary line
(arrow) caudal to premaxilla (PM). (c) axial section show-
V
ing intact alveolar arch (arrow). (d) coronal section
depicts intact base of the retronasal triangle (arrowhead).
(e) multiplanar imaging allows simultaneous visualization
of all three planes. (note that the vomer (*) is seen in axial
view suggestive of the cleft of the secondary palate),
Nasal bone (NB), Vomer (V)