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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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116
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
N
UL
UL
b
NB
c
Fig. 10.14 Mediolateral cleft 24+5 weeks (a) Nose chin view large lateralized defect (arrow). (b) Sagittal view complete absence of maxillary line (*) with communica-
Figures 10.21 and 10.22 illustrate the role of the midsagittal prole in the diagnosis of bilat­eral CLP. The rst case shows the typical pre­maxillary protrusion in the midsagittal prole (Fig. 10.21a) and the complete absence of the maxillary line in the parasagittal section (Fig.10.21b). Video 10.5 Illustration of the pre­maxillary protrusion and the vomer in a case of bilateral CLP at 22+6 weeks. The nose chin view conrms the labial defect (Fig. 10.21c) and the
tion between the nasal and oral cavities. (c) Rendered image of the face large lateralized defect of the upper lip (UL), Nose (N), Nasal bone (NB)
cleft extension into the palate can be seen in the axial view (Fig. 10.21d). Figure 10.21e is the coronal view in multiplanar imaging, and the ren­dered face is shown in Fig.10.21f.
In the second case, the coronal view and the axial view clearly demonstrates the bilateral paramedian defect (Fig.10.22a, b). The midsag­ittal view does not give a clue (Fig.10.22c) and the key plane in the diagnosis of the defect is in the nose chin view. Though, the midsagittal pro-
ef
10.2 Bilateral
117
a
NB
ML
M
c
b
UL
N
UL
d
Fig. 10.15 Mediolateral CLP involving secondary palate 21weeks. (a) Normal mid sagittal prole. (b) Nose chin view shows defect (*) in the upper lip. (c) Anterior axial view shows a large lateralized defect (arrow) extending centrally into the secondary palate; note that the horizon-
tal plate of the palatine bone is decient (*). (d) Absence of uvula (dotted circle). (e) Coronal section shows a defect (arrow) in the hard palate. (f) Rendered image of the fetal face. Nasal bone (NB), Maxillary line (ML), Mandible (M), Nose (N), Upper lip (UL)
118
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
UL
b
*
*
UL
c
M
NB
ML
Fig. 10.16 Median process in bilateral cleft lip (a) nose chin view median process (*) and the upper lip (UL) is divided into three segments (arrows points to bilateral paramedian defect). (b) Axial view median process (*)
le view may not give the clue, yet the parasagit­tal sections (Fig.10.22d) clearly demonstrate the cleft extension into the secondary palate.
Further conrmation can be done in multipla­nar imaging, which clearly demonstrates the complete absence of the base in the premaxillary triangle (Fig. 10.22e). Invariably regardless of
the presence or absence of PMP in midsagittal view, in all cases, the diagnostic plane is the coronal and axial sections.
There can be a deviation of the premaxillary protrusion to one side, giving the appearance of a beaked nose. Figure10.23 is an illustration of a
bilateral paramedian clefts (arrows). (c) Midsagittal sec­tion premaxillary protrusion (arrow), Mandible (M), Maxillary Line (ML), Nasal Bone (NB)
case with deviated premaxillary protrusion. Note in Fig.10.23b the premaxilla is deviated to one side in the axial view. Figure 10.23d is a 3D depiction of the beaked nose in bilateral CLP.
Figure 10.24 is a collection of axial views in different cases of bilateral CLP, which shows varying degrees of labial and palatal shelf sep­aration on either side of the premaxilla. Most often, the defect is very subtle on one side, and that can be mistaken as unilateral CLP (Fig.10.24c).
The rendered image of the face illustrates the different degrees of PMP in cases of bilateral

10.3 Median

119
a
b
N
UL
UL
c
d
Fig. 10.17 Bilateral cleft lip at 21weeks. (a) Nose chin view illustrates bilateral paramedian defect (arrows). (b) Axial view arrows point to the defect (c) Anterior axial
CLP (Fig. 10.25). Note the appearance of the median process in the gures shown below.
10.3 Median
Median cleft lip is characterized by the absence of the midline philtrum and the median process (Fig.10.26). There is a midline defect in the soft tissue of the upper lip.
view intact alveolar arch (arrow), (d) Normal midsagittal prole. Upper lip (UL), Nose (N)
The absence of the median process is diag­nostic of median CLP, and this also helps to dif­ferentiate from bilateral cleft lip. Figure10.27a
is an example of bilateral CLP with the presence of median process, and Fig. 10.27b shows a median cleft lip with the absence of median process.
In median CLP, there is absence of premaxilla and hence shortening of the maxillary line in midsagittal view. The midsagittal prole is often
120
10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
ab
UL
N
UL
d
NB
M
Fig. 10.18 Bilateral CLP involving the secondary palate at 24 weeks. (a) Nose chin view bilateral paramedian defect (arrows), Upper lip (UL), Nose (N). (b) Axial view shows interruption of alveolar arch (arrows) and central extension of the cleft into the secondary palate. (c) Vomer is visualized as a midline echo with bilateral clefts on
e
either side (*). (d) Sagittal view shows complete absence of secondary palate caudal to premaxilla (arrow). (e) Sagittal view shows uid in the oral cavity is continuous with the nasal cavity with no intervening secondary palate (*). Nasal Bone (NB), Nasal septum (NS), Tongue (T), Mandible (M), Video 10.3
c
NB
NS
*
*
*
T
M
the diagnostic plane as the proximal portion of the maxillary line is absent. Figure10.28a illus-
trates the midsagittal prole with a small maxil­lary line.
Note that the posterior echogenic line, which is seen in the midline is vomer. Figure 10.28b illustrates the complete absence of the maxillary line slightly off the midline. Figure10.28c depicts the midline interruption in the alveolar arch and the central cleft extension into the secondary pal­ate. Note that the horizontal plate of palatine bone is absent in the posterior aspect. Figure10.28d is the rendered image showing the median cleft lip.
Invariably in most cases of median CLP, there are other associated malformations, and median
CLP is also a component of many genetic syn­dromes [9].
The premaxilla agenesis-holoprosencephaly syndrome has a frontonasal deformity associated with hypotelorism, holoprosencephaly, and facial deformity ranging from cyclopia to midline facial cleft with premaxillary agenesis.
The median cleft face syndrome is often asso­ciated with a nasal deformity and hypertelorism, usually either with no or minimal brain deformity.
Figure 10.29 is an example of median CLP associated with holoprosencephaly. Figure10.29a shows a at facial prole with complete absence of maxillary line. Figure10.29b shows the nose chin view where there is a midline defect.
bc
10.3 Median
a
M
de f
PMP
121
Fig. 10.19 Complete bilateral CLP at 22 weeks. (a) Axial view median process (M) protruding in the midline with bilateral paramedian clefts (pointers). (b) Axial view alveolopalatal extension of the cleft. Arrows depict the defect in the bony posterior edge of the palate. (c) Axial view Vomer is visualized as a midline echo with bilateral
Figure 10.29c illustrates that the alae nasi can mimic the paramedian cleft, and the nostril mim­ics the median process. This can be misdiagnosed as bilateral CLP.Figure10.29d illustrates hypo­telorism. Figure10.29e depicts the fused thalami with cup-shaped monoventricle, suggestive of alobar holoprosencephaly. Figure10.29f shows the postnatal correlation.
Moreover, shadowing from the premaxilla hinders the visualization of the maxillary line leading to a false-positive diagnosis of cleft pal­ate (see Chap. 8, Fig. 8.8a). On the contrary, true cleft of the palate can be missed by mistaking the echogenic upper border of the tongue for the maxillary line. (Fig.10.30).
To conclude, the nose chin view is the diag­nostic view in detecting unilateral, bilateral, or
clefts on either side, arrows point to vomeropremaxillary junction, (*) palatal shelf separation. (d) Midsagittal view premaxillary protrusion (PMP). (e) Sagittal view uid in the oral cavity is continuous with the nasal cavity with no intervening secondary palate (*), arrow points to PMP (f) Rendered view of the face. (Video 10.4)
median cleft lip. A combination of the axial,
sagittal, and coronal planes helps in evaluating the degree of cleft extension.
Premaxillary protrusion seen in midsagittal
prole is a key feature in bilateral CLP. Cleft
extension into the palate can be assessed with the help of axial and coronal views, and sometimes the midsagittal view is misleading.
The nasal septum and vomer can mimic the maxillary line in bilateral CLP in midsagittal view. The parasagittal section is extremely useful as it shows the complete absence of the maxillary line in complete bilateral CLP. This emphasizes
the need to correlate axial and coronal views with ndings in the midsagittal view for the diagnosis of cleft palate.
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10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
a
N
d
g
b
NB
M
V
e
h
c
f
Fig. 10.20 Bilateral CLP with no premaxillary protrusion at 21weeks. (a) Nose chin view showing premaxillary pro­trusion is very subtle with asymmetric separation of the labium on either side of the median process, arrows points to defect, Nose (N). (b) and (c) shows the absence of second­ary palate caudal to premaxilla(*). (d) Rendered view of the face. (e) Axial view vomer is visualized as a midline echo
with bilateral clefts on either sides (*), arrow points to defect. (f) Transverse axial view at the skull base illustrates the absence of uvula (*). (g) Note that the maxillary line in the midsagittal view at 12weeks looks almost normal, and there is a subtle maxillary gap (arrow). (h) Vomer (arrow) in midline and the artifacts mask the defect in the base of the RNT.Vomer (V), Nasal bone (NB), Mandible (M)
10.3 Median
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a
NB
ML
c
UL
N
b
M
d
PM
UL
e
Fig. 10.21 Complete bilateral CLP at 22+6 weeks (a) Midsagittal prole shows premaxillary protrusion (arrow). (b) Parasagittal view shows the absence of maxillary line (arrow). (c) Nose chin view conrms the labial defect (arrows). (d) Axial views show cleft extension into the
f
palate (*). (e) Coronal view in volume imaging arrows points to defect. (f) Rendered view of the face. Nasal bone (NB), Maxillary line (ML), Mandible (M), Upper lip (UL), Nose (N) and premaxilla (PM). Video 10.5
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10 Protocol forDiagnosing Type andExtent ofaCleft inMid Trimester
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UL
N
C
UL
ML
b
UL
M
UL
e
Fig. 10.22 Bilateral CLP with subtle premaxillary pro­trusion at 22weeks (a) Nose chin view bilateral cleft lip (arrows). (b) Axial view median process (M). (c) Midsagittal view no demonstrable premaxillary protru­sion. (d) Parasagittal view absence of the maxillary line
(arrow). (e) Multiplanar imaging axial view bilateral para­median clefts in the alveolar arch (arrows), coronal view defect in the premaxillary triangle (arrow). Nose (N), Chin (C), Upper lip (UL), Maxillary line (ML)
ab
cd
10.3 Median
N
PM
125
*
*
Fig. 10.23 Bilateral CLP with deviated premaxillary protrusion at 31 CLP (*) points to defect. (b) Axial view shows premaxil-
+5
weeks (a) Nose chin view of bilateral
lary deviation to one side. (c) Midsagittal prole illus­trates the beaked nose with premaxillary protrusion (PM). (d) 3D depiction of the beaked nose. Nose (N)