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3.2 Classication ofFacial Cleft
13
by Kernahan and Stark, which was adopted by
the International Society for Plastic and
Reconstructive Surgery [7]. The American Cleft
Palate-Craniofacial Association (ACPA) classication has four categories, namely clefts of the
pre-palate, cleft of the secondary palate, cleft of
the prepalate and secondary palate (cheiloalveolopalatoschisis) and facial clefts other than these.
Cleft of prepalate is further subdivided as cleft
lip (cheiloschisis), cleft alveolus (alveoloschisis)
and cleft lip, alveolus and primary palate (cheiloalveoloschisis). Clefts of the secondary palate
are subdivided into uranoschisis (cleft of the hard
palate), staphyloschisis, or veloschisis, which is
Fig. 3.2 Tessier’s types
of facial cleft
14
cleft of the soft palate and uranostaphyloschisis
(cleft of the hard and soft palate).
3.2 Classication ofFacial Cleft
Facial cleft required their own classication system [18–20]. Paul Tessier in 1976 published a
classication on facial clefts based on the anatomical position at the lines of embryological
fusion [19]. These clefts are numbered from 0 to
14, 30 (Fig.3.2).
Tessier described cleft at the soft tissue level
and bony structure due to slightly different loca-
12
13
0
1
30
11
3
2
4
10
9
8
6
5
7
7
14
3 Classication ofOrofacial Clefts
tions of the cleft on the soft tissue and bone.
These clefts can be further grouped into the
midline, paramedian, orbital, and lateral clefts.
The midline clefts are median craniofacial
dysplasia-0, frontonasal dysplasia-14 and lower
midline facial cleft-30. The paramedian clefts are
clefts 1,2 running through the nose and maxilla
and clefts 12,13 running through the nose and
frontal bone. The orbital clefts 3,4,5 runs through
the orbital oor and maxilla from medial to lateral, respectively.
Clefts 9,10,11 are between the upper side of
orbit and temple of the head numbered lateral to
medial, respectively. The lateral clefts are placed
horizontally on the face, cleft 6 through the
cheek, cleft 7 runs from the lateral corner of the
mouth to the eye and cleft 8 from the lateral corner of the eye. Clefts 6,7,8 are associated with
Treacher Collins syndrome, and cleft 8 is associated with Goldenhar syndrome.
3.3 Recent Nomenclature
Till now, controversies exist on a universally
accepted classication system. Millard in his
review on classication systems, concluded that
“none has been universally accepted because of
language differences, inaccuracies, omissions
and lack of simplicity” [16]. Two of the recent
classication systems which have gained importance are discussed below.
LAHSHAL system is a diagrammatic classication of CLP, and according to this classication, the mouth is divided into six parts, namely
Right lip, Right alveolus, Right Hard palate, Soft
palate, Left Hard palate, Left alveolus, and Left
lip. The rst character is indicative of the
patient’s right lip, and the last character denotes
the patient’s left lip. The capital letter in the
LAHSHAL code indicates complete cleft and
the small letter indicates an incomplete cleft, and
no cleft is represented with a dot [21]. This was
further modied by the Royal College of surgeons as LASHAL denoting H for the hard
palate.
The recent CLAP system is a more complete
description of cleft phenotype. The letter C serves
as the reference point, and the letters L, A, and P
denote the involvement of the lip, alveolus, and
palate, respectively. The nomenclature for cleft
lip alone would feature the letters CL; a cleft lip
and alveolus with an intact palate as CLA and a
cleft lip involving the alveolus and palate as
CLAP.These uppercase letters specify anatomic
involvement, and the lower case prex denotes
laterality and severity [1]. Further subdivisions of
the lower case are illustrated in Fig. 3.3.
Figure3.4(a) is an illustration of bilateral complete CLP, and Fig. 3.4(b) is an illustration of
complete unilateral CLP depicted in the
Kernahan, LAHSAL, and CLAP systems.
All clinicians and academicians would agree
to a uniform system of classication that would
facilitate the description of cleft phenotype and
for easy and accurate communication among
practitioners [1]. Moreover, it is desirable that
the method also be adopted universally, such that
data could be shared among groups and instantly
understood [1].
Most classications based on numbering
alone leads to misinterpretation in clinical practice. The nal consensus of what is to be used as
a universal classication system is yet to be
known but certainly, the Kernahan and Stark
classication persists in widespread use [1].
Left ucCLAPv3
RL
3.3 Recent Nomenclature
15
Side
• Right
• Left
Ccleft
Fig. 3.3 CLAP system
Pre-foraminal Description
Laterality
• u-Unilateral
• b-Bilateral
• med-Median
L Lip
Severity
• c-Complete
• i-Incomplete
• m-Minor/ Micro/
Mini-micro form
• a-Asymmetric
A Alveolus
Post-foraminal Description
P Palate
• bu-Bifid uvula
• sm-Submucous
• v1-Veau I
• v2-Veau II
• v3-Veau III
• v4-Veau IV
Fig. 3.4 Illustration of
CLP by the three
classication systems
namely Kernahan,
LAHSAL, and CLAP.
(a) Bilateral complete
CLP (b) Unilateral
complete CLP
a
b
1
2
R
1
2
3
3 6
7
8
9
4
5
6
7
8
9
L
4
5
LAHSHAL
BcCLAPv4
**HSHAL
16
3 Classication ofOrofacial Clefts
References
1. Allori AC, Mulliken JB, Meara JG, Shusterman S,
Marcus JR.Classication of cleft lip/palate: then and
now. Cleft Palate Craniofac J. 2017 Mar;54(2):175–88.
2. Kernahan DA, Stark RB.A new classication for cleft
lip and cleft palate. Plast Reconstr Surg. 1958 Nov
1;22(5):435–41.
3. Spina V.A proposed modication for the classication on cleft lip and cleft palate. Cleft Palate J. 1973
Jul 1;10(3):251–2.
4. Davis JS, Ritchie HP. Classication of congenital
clefts of the lip and palate: with a suggestion for
recording these cases. J Am Med Assoc. 1922 Oct
14;79(16):1323–7.
5. Veau V, Borel S.Division palatine: anatomie, chirurgie, phonetique; avec la collaboration de S. Borel.
Paris: Masson; 1931.
6. Dorrance GM, Shirazy E.The operative story of cleft
palate. London: WP Saunders Company; 1933.
7. Harkins CS, Berlin A, Harding RL, Longacre JJ,
Snodgrasse RM.A classication of cleft lip and cleft
palate. Plast Reconstr Surg. 1962 Jan 1;29(1):31–9.
8. Tolarová MM, Cervenka J. Classication and birth
prevalence of orofacial clefts. Am J Med Genet. 1998
Jan 13;75(2):126–37.
9. Murray JC.Gene/environment causes of cleft lip and/
or palate. Clin Genet. 2002 Apr;61(4):248–56.
10. Bernheim N, Georges M, Malevez C, De Mey A,
Mansbach A.Embryology and epidemiology of cleft
lip and palate. B ENT. 2006 Jan;1:11.
11. Brophy TW.Cleft palate and harelip procedures. Int J
Orthod Oral Surg. 1919;7(6):319–30.
12. Fogh-Andersen P.Inheritance of harelip and cleft palate: contribution to the elucidation of the etiology of
the congenital clefts of the face. Copenhagen: Nyt
nordisk forlag, A.Busck; 1942.
13. Fogh-Andersen P. Epidemiology and etiology
of clefts. Birth Defects Orig Artic Ser. 1971 Jun
1;7(7):50–3.
14. Kernahan DA.The striped Y—a symbolic classication for cleft lip and palate. Plast Reconstr Surg. 1971
May 1;47(5):469–70.
15. Elsahy NI.The modied striped Y-A systematic classication for cleft lip and palate. Cleft Palate J. 1973
Jul 1;10(3):247–50.
16. Millard DR. The naming and classifying of clefts.
Cleft Craft. 1976;1:41–55.
17. Nyberg DA, Sickler GK, Hegge FN, Kramer DJ,
Kropp RJ.Fetal cleft lip with and without cleft palate: US classication and correlation with outcome.
Radiology. 1995 Jun;195(3):677–84.
18. Van der Meulen JC, Mazzola R, Vermey-Keers C,
Stricker M, Raphael B.A morphogenetic classication of craniofacial malformations. Plast Reconstr
Surg. 1983 Apr 1;71(4):560–72.
19. Tessier P. Anatomical classication of facial, craniofacial and latero-facial clefts. J Maxillofac Surg. 1976
Jan 1;4:69–92.
20. Whitaker LA, Pashayan H, Reichman J. A proposed
new classication of craniofacial anomalies. Cleft
Palate J. 1981 Jul;18(3):161–76.
21. Kriens O. LAHSHAL: a concise documentation
system for cleft lip, alveolus, and palate diagnoses.
In: Kriens O, editor. What is a cleft lip and palate.
Stuttgart: Thieme; 1989. p.32–3.
Sonoanatomy ofPalate
at11–14Weeks
4
The evaluation of the palate by ultrasonography
is impaired due to the dome-shaped structure of
the palate and due to acoustic shadowing from
the surrounding facial bones [1]. Defects of the
lip and alveolar arch can be easily diagnosed by
ultrasonography in mid trimester, but the diagnosis of abnormalities of the palate remains a
challenge [2]. A slight extension of the fetal neck
and uid in the oral cavity are the necessary prerequisites to evaluate the palate in mid trimester.
First trimester evaluation of the palate is very
much different from the mid trimester and certainly has a lot of advantages. This chapter
describes the sonoanatomy of the palate in the
rst trimester and describes the basic planes
required for evaluation of the palate at
11–14weeks.
4.1 Normal Appearance
ofPalate
The hard palate consists of the primary palate and
the bony part of the secondary palate (Fig.4.1).
The hard palate presents a bony framework
formed by the palatine processes of maxillae in
front and horizontal plates of palatine bones
behind. The bony hard palate can be visualized in
the rst trimester 11–14week scan, while the soft
Supplementary Information The online version of this
chapter (https://doi.org/10.1007/978- 981- 16- 4613- 3_4)
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_4
palate and uvula can be visualized in mid trimester scan. The anatomical landmarks of the pal-
ate are best visualized after 13 weeks of
gestation.
The palate is rather at in early gestation,
and there is less shadowing from the facial
bones, which favors complete visualization of
the bony secondary palate. Moreover, as the
tooth buds do not develop in this period of gestation, there is minimal shadowing from the alveolar arch, which enhances the visualization of the
secondary palate in the axial view.
The other advantage is that uid in the oral
cavity is not necessary for its visualization.
Figure4.2 depicts that the maxillary line which
represents the palate can be visualized either with
or without uid in the oral cavity.
4.2 Sections ofBasic
Examination
4.2.1 Sagittal View
Adequate magnication of the face prole in
midsagittal view is essential for the evaluation of
the palate. In the sagittal section, the anatomical
landmark of importance in evaluating the palate is the maxillary line (Fig. 4.2). The same
midsagittal section taken for measuring the
nuchal translucency is ideal for evaluating the
palate (Fig.4.2b).
17
18
Hard Palate
Secondary PalateSecondary Palate
Upper lip
4 Sonoanatomy ofPalate at11–14Weeks
Premaxilla
Incisive foramen
Soft PalateSoft Palate
Fig. 4.1 Line diagram illustrating the hard palate and soft palate
a
*
T
Fig. 4.2 Appearance of the maxillary line with and
without uid in the oral cavity. (a) 12 weeks
(Transvaginal—TVS) Midsagittal section-maxillary line
(arrows) uid in the oral cavity (*) tongue (T).
b
(b) 12weeks (Transabdominal—TAS) Midsagittal section-maxillary line (arrows) note that the entire maxillary line can be clearly visualized without uid in the
oral cavity
UvulaUvula
The continuous maxillary line is seen when
the palate is intact and is formed by fusion of the
primary palate (premaxilla) and the secondary
palate. In the midline, the secondary palate fuses
with the vomer in its posterior part and the vomer
can be visualized at the posterior two-thirds of
the maxillary line.
The three components of the maxillary line
seen in midsagittal section, namely the premaxilla, secondary palate and the vomer, are illustrated in Fig. 4.3. The midsagittal view of the
secondary palate in a normal fetus always displays a double echogenic line in the posterior
aspect of the maxillary line, representing the
4.2 Sections ofBasic Examination
19
a
NB
ML
M
V
P
c
b
d
Fig. 4.3 Components of maxillary line in the midsagittal
section at 14 weeks (TAS). (a) Midsagittal section of
face, (b) Anterior one-third of maxillary line is premaxilla (purple line). (c) Posterior two-thirds of maxillary
line are secondary palate (blue line). (d) The vomer
hard secondary palate caudally and the vomer
cranially [3].
Video 4.1 illustrates the three components of
the maxillary line in real-time imaging. Note that
the superior line is the vomer which is short and
it lies superimposed on the inferior line, which is
the secondary palate.
The entire maxillary line can be visualized
in the sagittal sections with no gap or interrup-
(green line) is seen superimposed on the posterior twothirds of the maxillary line in a slightly superior plane.
Nasal bone (NB), Maxillary line (ML), Mandible (M),
vomer (V). (Video 4.1)
tion when the palate is intact. In palatine
clefts, only a portion of the maxillary line is
imaged, and this line is either shortened or
interrupted or absent depending on the type
of cleft (Fig.4.4) [4]. The premaxillary portion
of the palate and the vomer is seen as two separate segments leading to interruption of the
maxillary line in clefts involving the secondary
palate [5].
20
4 Sonoanatomy ofPalate at11–14Weeks
a
NB
ML
Fig. 4.4 Abnormal appearance of the maxillary line at
13weeks (TVS). (a) Midsagittal section. Note that there
is also premaxillary protrusion (arrowhead) along with
maxillary gap (arrow) in bilateral CLP, (b) Sagittal section
M
4.2.2 Axial View
The alveolar arch of the maxilla and the echogenic
secondary palate can be visualized in the transverse view of the base of the skull at the level of
the pharynx. From a transverse section through the
head at the level of the thalamus, the transducer is
moved parallel to this plane in the caudal direction
until the uid in the oropharynx is visualized [6].
When the transducer is gently moved further
to and fro, the maxillary arch is identied, and
the secondary palate is seen as an echogenic
structure within the curved alveolar arch
(Fig.4.5). Video 4.2 illustrates the technique of
visualizing the transverse axial view of the palate
from the BPD plane.
This is the same plane proposed for imaging
the uvula in mid trimester by Wilhelm and
Borgers to look for “the equals sign” [7]. In the
rst trimester, as the palate is at, the entire palate itself can be visualized in this plane, whereas
in mid trimester, the “equals sign” which is the
uvula, can be demonstrated.
The intact alveolar margin in axial view
rules out clefts involving the premaxilla
(Fig. 4.5a). The secondary palate is seen as an
b
PM
showing absence of maxillary line (arrows) caudal to premaxilla (PM). Nasal bone (NB), mandible (M) and maxillary line (ML)
echogenic structure within the alveolar arch. As
the palate intervenes and separates the nasal cavity from the oral cavity, the vomer bone is not
visible in this plane when the secondary palate is
intact (Fig.4.5b).
Figure 4.5c is an axial section taken at
13 weeks of gestation showing the hard palate.
Note that the palate is rather at at 13 weeks,
whereas it is curved at 20 weeks of gestation
(Fig.4.5d). As gestation age advances, the curvature of the palate increases and the palatal shelves
are inadequately imaged.
4.2.3 Coronal View
Because of the scarce amount of soft tissue present in the upper lip in early fetal development, the
nose chin view routinely done in mid trimester is
not useful at this gestation age. The anatomical
landmark obtained just posterior to the nose
chin view is the retronasal triangle, and this
serves as an important landmark to identify the
palatine cleft. The base of the retronasal triangle
in the coronal view is formed by the palate as
described by Sepulveda etal. [8].
4.3 Integrity ofthePalate
21
a
F
c
P
b
P
F
d
P
Fig. 4.5 Axial view of the normal palate. (a) 12+5 weeks
(TVS) Intact alveolar margin (arrows), uid (F) in the oropharynx (Video 4.2). (b) 13weeks (TAS) Echogenic plate
of bone which is the secondary palate (P) seen within the
The coronal plane displays three easily recognizable echogenic lines, which resembles an outlined triangle. The two frontal processes of the
maxilla constitute the sides of the triangle, and
the primary palate forms the base of the triangle
(Fig.4.6a). Video 4.3 illustrates the translational
movement from the nose chin view to the retronasal triangle view.
Figure 4.6b is an illustration of the anatomical
landmarks of the retronasal triangle in the sagittal
and coronal planes. Note that the omni view line
passes through the nasal bone at the apex and
through the primary palate at the base.
alveolar arch (arrows). (c) 13weeks (TAS) showing the
at hard palate (P). (d) 20weeks (TAS) arched hard palate
(P) bony posterior edge of the palate (arrow)
The retronasal triangle has higher echogenicity than the surrounding tissue and hence an
easily recognizable landmark that is useful in
detecting cleft palate [8]. Figure4.6c illustrates
the coronal sections in TUI note that the base of
the triangle is intact in all the sections.
4.3 Integrity ofthePalate
The integrity of the palate can be assessed by a
combination of the sagittal, axial, and coronal
planes. The essential markers are an intact
22
4 Sonoanatomy ofPalate at11–14Weeks
a
M
A
FM
M
b
M
NB
ML
NB
c
Fig. 4.6 Retronasal triangle in a normal rst-trimester
fetus. (a) 14weeks (TAS) coronal view shows apex (A)
formed by nasal bones, sides by the frontal process of the
maxilla (FM), arrow points to the intact base of the triangle and the two echogenic structures below represent the
mandible (M) (b) 14weeks (TVS) illustration of the anat-
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.
Though the soft tissue of the upper lip can
also be evaluated in all three planes, the bony
landmarks serve as better clues to detect cleft
omy of RNT using omni view line. The omni view line
(dotted yellow line) passes through nasal bone (NB), maxillary line (ML) and the mandible (M), which is simultaneously displayed in the sagittal and coronal plane. (c)
+6
13
weeks (TAS) TUI illustrates intact base (pointer) of
the retronasal triangle (Video 4.3)
palate at this gestational age. Clefts involving
the premaxilla alone can be missed if only the
retronasal triangle is taken into consideration.
The midsagittal view alone is misleading, as
the vomer in the midline may be mistaken for an
intact palate in a fetus with a cleft of the secondary palate. The pitfalls of using only one plane