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Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5805_Библиотеки_им_академика_М_И_Перельмана.pdf
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106
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
UL
N
UL
c
b
d
Fig. 10.4 Unilateral CLP with a slight deformity in the alveolar arch at 21weeks. (a) Nose chin view lateralized defect (arrow) in the upper lip (UL), nose (N). (b) Axial view depicts deformed alveolar arch (arrowhead) on the
depicts the deformed alveolar arch on the cleft side but note that there is no complete interrup­tion. The normal-appearing midsagittal prole and the intact base of the premaxillary triangle is shown in multiplanar imaging in Fig.10.4d.
Figure 10.5 shows the complete interruption in the alveolar arch. Note that there is also a lat­eralized defect in the base of the triangle in Fig.10.5c.
Figure 10.6 is an illustration of labioalveolar palatal cleft or complete unilateral CLP.The nose chin view (Fig.10.6a) depicts a lateralized defect in the upper lip, anterior axial view (Fig.10.6b) depicts a cleft in the alveolar arch extending into the secondary palate. The coronal plane (Fig. 10.6c) depicts a lateralized defect in the
cleft side. (c) Rendered view of the face. (d) Multiplanar imaging depicting the deformed alveolar arch (arrow­head), note that the base of the triangle is intact in the coronal section (arrow)
base of the triangle, which indicates cleft exten­sion into the palate.
Though the midsagittal prole (Fig. 10.6e) appears normal, the parasagittal section shows the absence of the maxillary line on the cleft side (Fig. 10.6f). The key pointers for the involve-
ment of the palate would be lateralized defect in the base of the triangle in the coronal plane, absence of maxillary line in parasagittal section and defect in the bony posterior edge of the pal­ate in the axial plane.
Among individuals with unilateral cleft lip and palate (UCLP), there exists a variation in the ana­tomical relationship between the vomer and the secondary hard palate. Most often, the vomer is
attached to the secondary palate on the non- cleft
10.1 Unilateral
107
a
b
c
Fig. 10.5 2D and 3D correlation in unilateral CLP 22+3 weeks. (a) Axial view showing complete interruption in alveolar arch (arrowhead). (b) Rendered view of face,
side, but sometimes the vomer is detached from the secondary hard palate. In the latter case, there
is a bilateral extension into the secondary hard pal­ate (b-UCLP) (see Chap. 7, Fig. 7.1) [7].
Figure 10.7 is an illustration of unilateral CLP with bilateral extension into the secondary palate. Figure10.7a shows the lateralized defect in the nose chin view and in the alveolar arch (Fig.10.7b). Figure 10.7c shows the lateralized defect in the premaxillary triangle, and Fig.10.7d shows the lateralized defect in the upper lip.
Figure 10.7e shows the detached vomer and bilateral extension of the cleft into the second­ary palate in multiplanar imaging. The vomer bone of the nasal cavity is seen as a midline
arrow points to defect. (c) Coronal view showing defect (arrow) in the base of retronasal triangle
structure with a bilateral cleft on either side of the vomer. Note that there is an absence of the superimposed line sign at 13weeks in this case (Fig.10.7f).
In complete unilateral CLP, cleft extension into the secondary palate can be assessed by the following planes. If there is a complete extension of a cleft into the secondary palate in unilateral CLP, then there would be a defect in the bony posterior edge of the palate (Fig.10.8c), and the uvula cannot be imaged in sagittal and axial view as illustrated in Fig.10.8 d, e.
Third-trimester detection of CLP is most often difcult due to unfavorable position of the fetus. Moreover, the margins of the tongue
108
cd
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
b
N
UL
*
*
UL
ef
NB
ML
M
*
Fig. 10.6 Complete unilateral CLP at 22weeks. (a) Nose chin view lateralized defect (*) in the upper lip (UL) with nasal asymmetry (arrow). (b) Anterior axial view depicts the cleft (arrow) extending into the alveolus, note that the defect (*) in the bony posterior edge of the palate. (c) Coronal view lateralized defect (arrow) in the base of the
triangle. (d) Rendered view of face. (e) Normal appear­ance of midsagittal prole. (f) Parasagittal section shows the absence of the secondary palate (*) on the cleft side. Nose (N), Maxillary line (ML), Mandible (M), Nasal bone (NB)
10.1 Unilateral
109
obscure the palatal defect, and it is very cum­bersome to assess cleft extension into the sec­ondary palate.
The lateralized defect in the lip (Fig. 10.9a) and cleft in the alveolar arch (Fig.10.9b) can be assessed with conventional views, however, the
ab
UL
cleft extension into the palate cannot be assessed due to shadowing.
Figure 10.10 is a demonstration of unilateral
CLP at 34weeks. Though the labial defect and cleft in the alveolar arch can be diagnosed, note
that the acoustic shadows from the tooth buds
N
UL
cd
Fig. 10.7 Unilateral CLP with bilateral extension into secondary palate 22weeks. (a) Nose chin view lateralized defect in upper lip (arrow). (b) Axial view lateralized defect in alveolar arch (arrow). (c) Coronal view lateral­ized defect (arrow) in the base of the triangle. (d) Rendered view of face unilateral cleft lip. (e) Multiplanar imaging
shows bilateral extension of cleft (*) into the secondary palate, vomer (V) of the nasal cavity is seen as a midline structure with bilateral cleft on either side of the vomer. (f) Note that at 13 weeks of gestation there is absence of superimposed line sign. Upper lip (UL), Nose (N). Reproduced with permission from Wiley
110
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
e
V
V
f
*
*
Fig. 10.7 (continued)
hinder further visualization of the palate (Fig.10.10c).
In such cases, the coronal plane helps in detect­ing cleft extension into the palate as shown in Fig.10.11. The sagittal view and axial view shown in Fig.10.11b are not informative about the cleft extension into the palate as the tongue shrouds the defect. The posterior part of the secondary palate
is inadequately visualized due to dense shadow­ing from facial bones and the tongue.
Figure 10.11c coronal section posterior to nose chin view clearly depicts the defect in the
hard palate, and there is a communication between the oral cavity and the nasal cavity. The posterior coronal section clearly illustrates that there is no intervening hard palate above the tongue (Fig. 10.11d) which indicates that the cleft extends into the secondary palate. Note that the vomer is deected to the non-cleft side and is not attached to the palatal shelves below on either sides.
Varied presentations in unilateral CLP and the different types of cleft extension into the palate have been illustrated. The phenotypic variations
de
10.1 Unilateral
111
a
UL
N
UL
NB
M
V
b
Fig. 10.8 Complete unilateral CLP at 20weeks. (a) Nose chin view lateralized defect (arrow) in the upper lip (UL), (b) rendered view of face illustrating unilateral cleft with nasal asymmetry, (*) denotes the excess soft tissue in the upper lip. (c) Axial view cleft extension into the palate
c
(arrow) with defect in bony posterior edge of the palate (*). (d) Midsagittal prole depicts vomer (V) in midline with non-visualization of secondary palate. (e) Axial view absence of uvula (dotted white circle). Nose (N), Nasal bone (NB), mandible (M)
a
UL
N
Fig. 10.9 Third-trimester detection of unilateral CLP (29weeks). (a) Nose chin view lateralized defect (arrow)
UL
b
T
the alveolar arch (arrow), note that the tongue (T) obscures the defect in late gestation. Nose (N)
in the upper lip (UL) (b) anterior axial view shows cleft in
112
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
N
N
UL
UL
UL
UL
b
c
Fig. 10.10 Unilateral CLP at 34 weeks. (a) Nose chin view lateralized defect in the upper lip (UL), arrow points to the nasal deformity. (b) HD live rendering of the face. (c) Axial view depicting cleft extension (arrow) into the
in unilateral CLP and the different degrees of palatal shelf separation are shown in Fig.10.12.
The anterior axial view clearly depicts the
extent of palatal shelf separation and the severity of the defect. Though the nose chin view is the
diagnostic view in unilateral CLP, a combina­tion of the axial, sagittal, and coronal planes help in evaluating the degree of cleft extension.
A systematic stepwise approach to evaluate cleft lip and palate is illustrated in the owchart (Fig.10.13).
alveolar arch. Dense shadowing from the tooth buds does not allow the assessment of cleft extension into the sec­ondary palate. Nose (N)
In mediolateral cleft, there is a large lateral­ized defect of the upper lip involving the midline philtrum. This type of cleft almost invariably has a central extension into the secondary palate. Video 10.2 illustrates a midline interruption in the alveolar arch in the case of median CLP.
Figure 10.14a illustrates a large lateralized defect in the nose chin view. Note that there is also asymmetry of the nostrils. Figure 10.14b illustrates the complete absence of maxillary line in sagittal view showing the communication

10.2 Bilateral

ab
113
c
Fig. 10.11 3D illustration of unilateral CLP at 31weeks (a) Rendered view of the face depicting the defect and nasal asymmetry. (b) Multiplanar image correlation of defect (arrow) in sagittal and axial view (c) coronal plane
T
T
d
V
T
*
*
illustrating the defect (arrow). (d) (*) depicts the commu­nication between the oral and nasal cavity on both sides, vomer (V). Tongue (T)
between the nasal cavity and the oral cavity. Figure10.14c is the rendered image of the face showing a large lateralized defect of the upper lip.
Figure 10.15 is another case of mediolateral CLP with extension into the secondary palate. The defect in the upper lip is demonstrated in the nose chin view (Fig.10.15b). Figure10.15c illus- trates the large lateralized defect in the alveolar arch extending centrally into the secondary palate.
Note that there is absence of uvula in Fig.10.15d. Figure10.15e depicts the defect in the hard palate in the coronal section. Figure10.15f depicts rendered image of the fetal face.
10.2 Bilateral
Bilateral cleft lip is characterized by the upper lip partitioned into three segments in the nose chin view with the median process under the nostrils. There are two clefts on either side of the
median process in the bilateral cleft. The surface of the skin on the median process has excess mucosa, and there tends to be a protrusion of the median process in most cases [7].
Figure 10.16a illustrates the nose chin view in the coronal plane, which shows the median pro­cess and the upper lip divided into three seg­ments. Figure10.16b shows the median process protruding in the midline with bilateral paramedian clefts in the axial view. Figure10.16c
114
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
ab
de
Fig. 10.12 Phenotypic variation in unilateral CLP with varying degrees of palatal shelf separation. (a, b, and c) shows minimal to moderate degree of palatal shelf separa-
shows the premaxillary protrusion in the sagit­tal prole, which is the key nding in bilateral CLP [8].
In bilateral cleft lip, the defect is conned only to the soft tissue of the upper lip. This is best seen in the nose chin view (Fig.10.17a) and is charac­terized by an intact alveolar arch in axial view (Fig.10.17c). The midsagittal prole almost has a normal appearance (Fig. 10.17d) and most often, the premaxillary protrusion is very subtle when the cleft is conned to the labium.
The cleft extension into the palate can be assessed by evaluating the alveolar arch and the horizontal plate of the palatine bone in axial view. Figure 10.18 is an illustration of bilateral CLP involving the secondary palate. The axial view shows interruption of the alveolar arch and cen­tral extension of the cleft into the secondary pal­ate (Fig.10.18b).
The vomer of the nasal cavity is visualized as a midline echo with bilateral clefts extending on either side of the vomer (Fig.10.18c). Complete absence of the secondary palate caudal to the pre­maxilla is depicted in sagittal sections (Fig.10.18d and e). In Fig.10.18e note that the uid in the oral cavity is continuous with the
tion. (d, e, and f) shows a wide degree of palatal shelf separation (arrows)
nasal cavity with no intervening secondary pal­ate. Video 10.3 illustrates complete bilateral CLP with uid in the oral cavity.
Figures 10.19 and 10.20 are two cases of bilat­eral CLP with varying degrees of premaxillary protrusion. The rst case shows the typical pre­maxillary protrusion in axial and sagittal views (Fig. 10.19a, d, e). Video 10.4 illustrates the abrupt ending vomer in the midline with non­visualization of the soft palate and the uvula in a case of complete bilateral CLP.
Alveolopalatal extension of the cleft can be seen in the axial view (Fig.10.19b) depicting the maxillary arch. Figure10.19c shows the vomer in the midline and the vomeropremaxillary junc­tion. Note the symmetric separation of the palatal shelves on either side.
In the second case, the premaxillary protru­sion is very subtle as illustrated in the nose chin view (Fig. 10.20a). Note there is asymmetric separation of the labium on either side of the median process. The sagittal views (Fig.10.20b
and c) does not show an obvious premaxillary
protrusion but clearly illustrates the absence of the secondary palate caudal to the premaxilla. The midline echo imaged in the midsagittal pro-
c
f
10.2 Bilateral
115
Nose chin view
Unilateral
Alveolar arch (Axial view)
Normal Slightly deformed Complete cleft
Labial cleft
Bilateral Median
Labioalveolar cleft
Premaxillary triangle (Coronal view)
Normal
Cleft confined to
premaxilla
Extended views to assess
Abnormal
Cleft extending into
secondary palate
secondary palate
Bony posterior edge of the palate in axial view
Fig. 10.13 Algorithm to detect cleft extension into the secondary palate
le is the vomer which is situated at a slightly superior plane (Fig. 10.20b). The transverse axial view at the base of the skull illustrates the absence of the uvula (Fig.10.20f).
In this type of presentation, the ultrasound
markers in the rst trimester are very subtle and can be easily overlooked. Note that the maxil-
lary line in the midsagittal view at 12 weeks
looks almost normal, and there is a subtle maxillary gap (Fig. 10.20g). The vomer at the
base of the retronasal triangle, along with the artifacts, masks the defect in the base of the RNT (Fig.10.20h). As the PMP is not evident
in the midsagittal prole, the nose chin view in this case gives the lead clue to identify this defect.
Uvula in
sagittal view