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6 Classication ofOrofacial Clefts
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Millard (1976)
by Vilar-Sancho and Koch. Subtotal malforma-
tions were indicated with lower case letters for
A few years later, in 1976, D.Ralph Millard fur-
ther expanded Elsahy’s Y-graphic in volume I of
his trilogy titled “Cleft Craft: The Evolution of
Its Surgery” [28]:
Then it became apparent that there is no reason not
to top Elsahy’s triangular tips to Kernahan’s prongs
with inverted tips to be marked with horizontal
lines indicating the amount of nasal deformity
adinnitum.
The Y was capped with triangular peaks for
the nasal oor, and these were topped with similar triangular peaks turned upside down to represent the nasal arch [28]. Millard added these
the lip (l), alveolus (a), hard palate (h), and soft
palate (s). Malformations of microform (minor)
severity were documented with an asterisk (*).
Kriens documented submucosal malformations
in the posterior third of the hard palate with a
lower case “s” (Table6.4).
tem (Fig.6.2) was the inexibility in describing
complex cleft malformations. It was not possible
to differentiate between submucosal and microform malformations. Furthermore, partly submucosal and partially open malformations in the
same cleft region could not be documented [24].
triangles without numbering. All Y-graphics were
intended to be used in a mirror-inverted manner.
Total malformations were indicated by completely blacking the elds and subtotal malfor-
Noordho (1990) andFriedmann
(1991)
mations by hatched elds. Microform or
submucous malformations were not
documented.
In 1990, Noordhoff and his group revised the
Y-graphic again and included their “dual number system.” In this system, each region and side
was represented by one square [22]. This was
Kriens (1985)
Table 6.4 LAHS code proposed by Kriens
In 1985, Otto Kriens from Bremen, Germany,
adopted Josef Koch’s approach proposed in 1968
and published his LAHS code [21, 29]. Kriens
projected the single region codes of both sides
parallel to each other in one line, similar to that
used in an X-ray [21]:
• LAHS: Right-sided deformity of the (L)ip,
(A)lveolus, and (H)ard and (S)oft palates
• SAHL: Left-sided deformity of the (L)ip, (A)
lveolus, and (H)ard and (S)oft palates
• LAHSHAL: In bilateral cases, Kriens relin-
quished the indication of bilateral involve-
ment of the soft palate (LAHSHAL)
In this code, unaffected regions were indicated
by (−) or “0.” Using the LAHS code consequently, the indications for the affected side
(bilateral/B), left (L), and right (R) were not
necessary.
In 1976, Kriens adopted the documentation of
total deformities with capital letters as proposed
The main disadvantage of the LAHSHAL sys-
Right-sided subtotal
malformation of the lip and
alveolus and total
malformation of the hard and
soft palates
Right-sided total
malformation of the lip,
alveolus, and hard and soft
palates
Left-sided total malformation
of the lip, alveolus, and hard
and soft palates
Bilateral total malformation
of the lip, alveolus, and hard
and soft palates
Subtotal hard and soft palate
malformations
Total soft palate
malformation
Subtotal soft palate
malformation
Microform (minor) soft
palate malformation
Submucosal and subtotal
hard palate malformation and
total soft palate malformation
l a H S – – –
L A H S – – –
– – – S H A L
L A H S H A L
– – h s h – –
– – * S * – – –
– – * s * – – –
– – – * – – – –
s S s
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LAHSHAL
Shorthand Notation
[ LAHS···]
[ laHS···]
[ ···SHal ]
[ LAHSHAL ]
[ I*HSH*L ]
[ ··HSH·· ]
[ ···S··· ]
Fig. 6.2 LASHAL classication of cleft lip and palate. The corresponding Veau classication is indicated. (Author:
Felsir at English Wikipedia. Source: Wikipedia, Transferred from en.wikipedia to Commons)
right unilateral complete cleft lip, completee cleft alveolus, and complete ''unilateral'' (Veau-III) cleft palate
right unilateral incomplete cleft lip and alveolus, with complete ''unilateral'' (Veau-III) cleft palate
left unilateral incomplete cleft lip and alveolus, with complete ''unilateral'' (Veau-III) cleft palate
bilateral symmetric complete cleft lip, complete cleft alveolus, and complete ''bilateral'' (Veau-IV) cleft palate
bilateral symmetric incomplete cleft lip, notched cleft alveolus, and complete ''bilateral'' (Veau-IV) cleft palate
complete ''midline'' (Veau-I) cleft of hand and soft palate
complete ''midline'' (Veau-I) cleft of soft palate
correct for the lip, alveolus, hard palate, and
nose, but not for the soft palate, which is a mid-
Longhand Phenotypic Description
Using theLAHS Code
• Sagittal dimensions:
line structure [24].
With an additional square (no. 10), Noordhoff
tried to describe whether the malformation was
submucosal or not. However, this system did not
represent whether each anatomical region of a
Anatomical region Code
Upper lip L
Alveolar process (alveolus) A
Hard palate H
Soft palate S
cleft was submucosal or subcutaneous.
In 1991, Friedmann again revised the
Y-graphic by including Millard’s triangles to
document nasal malformations [23]. However,
the hard palate was only represented by one
square (no. 11). Malformations of the left and
Malformations of the nose and vomer can also
be included.
Anatomical region Code
Outer nose N
Vomer V
right premaxilla were indicated by two semicircles (nos. 14 and 15).
• Transverse dimensions:
Using the LAHS code, the localization can
be clearly indicated as left, right, or bilateral
Koch (1995) andFurther
Development oftheLAHS Code
for a malformation. Documenting “median” is
anatomically correct for malformations of the
soft palate, but controversial for hard palate
Decades after Vilar-Sancho claimed a complete,
simple, exible, and precise tool for the diagnosis
of lip, alveolar, and palatine malformations at the
second Symposium on the Treatment of Patients
with Clefts of Lip, Alveolus and Palate in
Hamburg 1964, the latest update of the
LAHSHAL classication complied with those
malformations. Depending on the extent of
fusion of the horizontal plate with the vomer,
they can be either left, right, or bilateral para-
median [2].
(R)ight side L A H S
S H A L (L)eft
L A H S H A L (B)ilateral
recommendations [2].
Using the LAHSHAL code, any individual
malformation in each anatomical location could
be documented without the need to indicate the
affected side. Furthermore, malformations of the
nose (N) and vomer (V) could be captured using
the LAHS(NV) code.
• Vertical dimensions:
Malformations above the maxilla can also
be documented using the LAHSNV code [2]:
N V V N
L A H S H A L
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• Grading:
A subdivision in only two grades, e.g., sub-
total and total, would not be sufcient because
minor occurrences such as notches in the lip
or alveolus, missing developing teeth, or uvula
bida would be incorrectly assigned to subtotal malformations. However, microform malformations play a key role as important criteria
in genetic inquiries. Furthermore, the grading
should be analogue for all anatomical regions
included [2].
Extent Shape/progression
Grade 1= Microform Submucosal = 1
Grade 2= Subtotal Partly open/partly
submucosal
Grade 3= Total Open = 3
= 2
Allori (2017)
Based on the most enduring elements of the historical schemata, Alexander C.Allori suggested
the:
… next step in the natural progression toward a
comprehensive and clinically useful classication
system [1].
lip, alveolus (and primary palate), and/or secondary palate. Particularly, the integrity of the
alveolar process has to be documented since it
has implications for the choice of therapy
(orthodontics or bone grafting).
• Specication of the laterality and severity
only to the degree that is necessary to describe
morphologic features relevant to treatment
planning or outcome assessment:
– Labial description: Description of the
cleft lip is preferred over categorizing it
into taxonomical groups (left/right/median,
complete/incomplete).
– Palatal description: Veau’s numbering for
morphologic description (I–IV) + bid
uvula and submucous cleft palate (with/
without bid uvula).
• Exclusion of high-level morphological
details, such as the severity of alveolar cleft,
protrusion of the premaxilla, and collapse of
the alveolar segments for simplicity.
In summary, Allori’s classication includes a
complete description of a CL/P phenotype, laterality and severity of the labial defect, acknowledgement of an alveolar defect, and morphological
characterization of the palatal defect.
Allori’s group proposed a universal structured
form to describe different phenotypes. The system was based on the following precepts:
• Acceptance of the incisive foramen as the
embryologically correct border to separate
preforaminal structures (lip, alveolus, and pri-
mary hard palate) from postforaminal struc-
tures (secondary hard palate and soft palate).
• Comprehensive phenotypical description and
specication if the malformation involves the
Clinical Examples
Figure 6.3 demonstrates the typical clinical
aspects of newborns with different cleft types: (a)
incomplete left-sided cleft lip, (b) incomplete
bilateral cleft lip, (c) left-sided cleft lip alveolus,
(d) bilateral cleft lip alveolus, (e) left-sided complete cleft lip and palate, (f) bilateral complete
cleft lip and palate, (g) hard and soft palate cleft,
and (h) soft palate cleft.
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R. M. Zimmerer et al.
Fig. 6.3 (a) Incomplete left-sided cleft lip, (b) incom-
plete bilateral cleft lip, (c) left-sided cleft lip alveolus, (d)
bilateral cleft lip alveolus, (e) left-sided complete cleft lip
and palate, (f) bilateral complete cleft lip and palate, (g)
hard and soft palate cleft, and (h) soft palate cleft. (Source:
Pictures of Lethaus and Zimmerer, University Leipzig)
6 Classication ofOrofacial Clefts
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Fig. 6.3 (continued)
77
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;54(2):175–88.
2. Koch J, Koch H, Grzonka M, Gundlach KK. Die
LKGS(N)-Spalten und ihre Kodierung mit der LAHSNomenklatur [Facial clefts and their coding with
LAHS nomenclature]. Mund Kiefer Gesichtschir.
2003;7(6):339–44.
3. Hillig U. Lippen-Kiefer-Gaumen-Spalten:
Klassikation und Epidemiologie. Fortschritte der
Kieferorthopädie. 1991;52:230–6.
4. von Ammon FA. Die angeborenen chirurgischen
Krankheiten des Menschen in Abbildungen dargestellt und durch erläuternden Text erklärt; mit fünfhundert vierundsiebzig Figuren auf vierunddreissig
Kupfertafeln in Folio. Herbig; 1842.
5. Förster A. Die Missbildungen des Menschen v.
Friedrich Mauke; 1861. p.1–2.
6. Davis JS, Ritchie HP. Classication of congenital
clefts of the lip and palate: with a suggestion for
recording these cases. JAMA. 1922;79(16):1323–7.
7. Brophy TW. Cleft lip and palate. Philadelphia:
P.Blakiston’s Son & Co.; 1923.
8. Veau V, Borel S, avec la collaboration de S.Division
palatine: anatomie, chirurgie, phonetique. Borel:
Masson; 1931.
9. Fogh-Andersen P.Inheritance harelip and cleft palate.
Opara exdome biologiae hareditariae universities hafniensis. 1942;4:1.
10. Pruzansky S. Description, classication, and analysis of unoperated clefts of the lip and palate. Am J
Orthod. 1953;39(8):590–611.
11. Kernahan DA, Stark RB. A new classication
for cleft lip and cleft palate. Plast Reconstr Surg.
1958;22(5):435–41.
12. Harkins CS, Berlin A, Harding RL, Longacre JJ,
Snodgrasse RM.A classication of cleft lip and cleft
palate. Plast Reconstr Surg. 1962;29(1):31.
13. Vilar-Sancho B. A proposed new international classication of congenital cleft lip and cleft palate. Plast
Reconstr Surg Transplant Bull. 1962;30:263–6.
14. Pfeifer G. Documentation, discussion by invitation.
Treatment of patients with clefts lip, alveolus and palate. Stuttgart: Thieme; 1966. p.226.
15. Koch J.Zur Nomenklatur der Lippen-Kiefer-GaumenSegel-Spalten. Acta Chir Plast. 1966;8(1):45–52.
16. Vilar-Sancho B.Documentation, discussion by invitation. Treatment of patients with clefts of lip, alveolus
and palate. Stuttgart: Thieme; 1966. p.223.
17. Santiago A.Classication of cleft lip and palate for
machine record coding. Cleft Palate J. 1969;6:434–9.
18. Kernahan DA. The striped Y–A symbolic classication for cleft lip and palate. Plast Reconstr Surg.
1971;47(5):469–70.
19. Elsahy NI. The modied striped Y—a systematic
classication for cleft lip and palate. Cleft Palate J.
1973;10(3):247–50.
20. Jensen BL, Kreiborg S, Dahl E, Fogh-Andersen
P.Cleft lip and palate in Denmark, 1976–1981: epidemiology, variability, and early somatic development.
Cleft Palate J. 1988;25(3):258–69.
21. Kriens O. LAHSHAL: a concise documentation
system for cleft lip, alveolus, and palate diagnoses.
What is a cleft lip and palate. Stuttgart: Thieme; 1989.
p.32–3.
22. Noordhoff M, Huang C, Wu J.Multidisciplinary management of cleft lip and palate in Taiwan. In: Bardach
J, Morris H, editors. Multidisciplinary management
of cleft lip and palate. Philadelphia: WB Saunders
Company; 1990. p.18–26.
23. Friedman HI, Sayetta RB, Coston GN, Hussey
JR. Symbolic representation of cleft lip and palate.
Cleft Palate Craniofac J. 1991;28(3):252–60.
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24. Koch H, Grzonka M, Koch J.Cleft malformation of lip,
alveolus, hard and soft palate, and nose (LAHSN)—a
critical view of the terminology, the diagnosis and
gradation as a basis for documentation and therapy.
Br J Oral Maxillofac Surg. 1995;33(1):51–8.
25. Rohen JW, Yokochi C. Photographischer Atlas der
systematischen und topographischen Anatomie für
Zahnmediziner. Schattauer. 1988;468:43.
26. Veau V. Hasenscharten menschlicher Keimlinge auf
der Stufe 21–23mmS.St. L.Zeitschrift für Anatomie
und Entwicklungsgeschichte. 1938;108(3):459–93.
27. Koch J.Diagnose, Unterteilung und Dokumentation
der Lippen-, Kiefer-, Gaumen- und Segelspalten.
Deutsch Zahn-, Mund- und Kieferheilkunde.
1969;52:187–96.
28. Millard DR Jr. Cleft craft: the evolution of its surgery—volume II: bilateral and rare deformities.
Boston: Little Brown and Company; 1977. p.51–2.
29. Koch J.On the diagnosis of clefts of the lip, alveolus and palate. In: Clefts of lip, alveolus and palate.
Yugoslav symposium with international participation,
Maribor; 1968. p.99–106.
Treatment Principles inOrofacial
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Clefts
PhilippKaumann andHenningSchliephake
7
Introduction
The term cleft or clefting refers to a lack of fusion
of adjacent embryonic facial processes. Tessier
reported a classication of orofacial clefts in
1978 that is still a good guide for clinicians today
[1, 2]. The classication is based on a numerical
system that combines the results of the clinical
examination with the underlying deformity of the
facial bone at the time of reconstructive repair,
thereby greatly simplifying communication
among practitioners. Along with other classications from 1983 and 2014, Tessier’s classication
still holds its value today, facilitating communication among clinicians and providing guidance
for treatment of the individual deformity [1–5].
This chapter mainly focuses on the basic
therapeutic principles and techniques of primary surgery for cleft lip and palate, as this
deformity is not only among the most common
malformations in the oral and maxillofacial
region, but also among the most common congenital malformations in general [6]. An
increasing incidence has been observed in the
last decades [7]. For the European region, an
P. Kauffmann (*) · H. Schliephake
Department of Oral and Maxillofacial Surgery,
University Medical Center Göttingen,
Göttingen, Germany
e-mail: philipp.kauffmann@med.uni-goettingen.de;
schliephake.henning@med.uni-goettingen.de
incidence of 1:500 is given in the literature,
making it the second most common malformation in Europe [8]. The clinical manifestations
of cleft lip and palate are diverse, and their
causes are multifactorial in addition to the
association with syndromes [9]. Complete cleft
lip and palates are the most frequent forms
(50%), followed by isolated cleft palate and
velum (30%) and cleft lip and alveolus (20%)
[10]. Cleft formation causes severe morphological and functional limitations in the newborns, which lead to concomitant psychological
damage in further development [11].
Understanding of embryology helps to explain
cleft manifestation. Embryologically, disorders of the primary palate development are distinguished from disorders of secondary palate
development. Within the framework of a disturbance in primary palate development, which
leads to the formation of the upper lip and
alveolus, cleft formation of the lip and alveolus
may occur in the fourth to fth intrauterine
weeks [12]. Clefts of the primary palate end in
the region of the foramen incisive [13]. During
formation of the secondary palate, the two lateral palatal plates develop from the maxillary
ridges towards the midline. Simultaneously
with the anteriorly directed growth of the mandible, the tongue shifts caudoventrally, allowing the palatal processes to erect, as the tongue
previously lled the oronasal space between
the lateral palatal plates [14]. With resolution
© Springer Nature Switzerland AG 2023
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-031-28069-6_7
79
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P. Kaumann and H. Schliephake
Table 7.1 Rome classication of 1967
Clefts of the primary
palate
Clefts of the primary and
secondary palate
Clefts of the secondary
palate
Rare clefts Facial clefts
Clefts of the lip and clefts of
the lip and the alveolus
Clefts of the lip and palate
Clefts of the palate (hard
and soft palate)
of the epithelial walls, the lateral palatal plates
normally fuse with the primary palate and the
nasal septum, which grows caudally at this
time. Fusion begins in the seventh intrauterine
week at the foramen incisive and continues
dorsally from there. By the end of the ninth
intrauterine week, the entire hard palate is then
occluded and fusion progresses in the soft palate to the uvula. By the 12th intrauterine week,
the fusion process is then usually complete and
desmal ossication of the hard palate occurs
[9, 12]. If a disruption occurs during this process, clefts develop in the hard and soft palate
behind the foramen incisive. Embryology and
its disturbances in the fusion of the facial
ridges also explain the occurrence of rare cleft
forms such as facial clefts, lower lips, and
nasal clefts. Table7.1 gives the Rome classication of 1967 of the clefts into four groups.
Nowadays, for the most accurate documentation of cleft manifestations, the LAHSHAL
scheme (L=lip, A=alveolus, H=hard palate,
S = soft palate) can be used to evaluate cleft
forms, which also takes microforms into
account [15].
Treatment Principles
Interdisciplinarity
Nowadays, all therapeutic approaches are
based on an interdisciplinary approach in order
to cope with the complex rehabilitation process, aiming at a complete aesthetic and functional recovery of cleft lip and palate patients.
An interdisciplinary treatment concept takes
care of the patient from birth to termination of
growth. The specialist disciplines of oral and
maxillofacial surgery; ear, nose, and throat
medicine; orthodontics; pediatrics; phoniatrics
and pediatric audiology; human genetics; and
dentistry form the ideal composition of a treatment center.
Treatment Strategies
It is important to remember in all surgical treatment procedures that any intervention will take
place on a growing individual and consequently
can potentially provoke growth disturbances
[16]. As the assessment of the quality or the strategy of surgical interventions can often only be
conclusively performed after growth has ended,
the comparison of different treatment concepts is
difcult and no commonly accepted treatment
schedule exists. This is probably the reason for
those 194 different treatment concepts practiced
in the 201 cleft centers in Europe [17].
Delair’s principles form the basis of the surgical therapy. These principles are based on the
idea that growth in the midface receives impulses
and stimulation from the soft tissue covering in
the sense of a functional matrix [18, 19]. Five
muscle systems make up this functional matrix:
the perinasal, perioral, oro-buccopharyngeal, and
oro-zygomaticomaxillary muscle loops. The
Mm. nasalis, levator labii, superioris alaeque
nasi, levator labii superioris, and zygomaticus
minor unite with the M. orbicularis oris and
attach to the periosteum of the spina nasalis anterior and the septal cartilage. In cleft patients, this
functional complex is interrupted in the region of
the piriform apertura, and the muscle insertion is
located in the margin of the cleft, resulting in a
parallel course of the muscle bers in relation to
the cleft (Fig.7.1) [18–20].
Reconstruction of this functional unit by
exact restoration of all muscle systems involved
in the matrix and associated correction of the
nasal entrance is a substantial part of lip closure. The principles of reconstruction on the
muscle systems and tissues involved in the
respective area are also taken into account for
the closure of the cleft alveolus and cleft palate
(Figs.7.2 and 7.5).
is
inferioris
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Fig. 7.1 Illustration of
facial mimic muscles in
cleft lip
81
M. procerus
M. nasalis
M. levator labii
superioris
M. zygomaticus minor
M. zygomaticus major
M. orbicularis oris
M. depressor anguli or
M. depressor labii
Fig. 7.2 (a, b) Patient with unilateral complete lip-alveolus cleft on the left side; (c) illustration of the misinserted
perioral muscles; (d) illustration of the soft tissue needed to be surgically undermined
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Timing ofSurgery
Timing of surgical procedures distinguishes
between primary procedures and secondary procedures, the latter of which are often corrective. All
primary surgeries should be completed at the age
of the 12months [16]. To achieve this goal, all procedures must be carefully planned and agreed
upon by the team. For the lips and simultaneous
nasal oor reconstruction, closure between 4 and
6months of age is generally considered favorable
[21]. As mentioned at the outset, there are differ-
ences between centers regarding methods of closure and timing of surgery. Especially with regard
to palate closure, these discrepancies become
apparent. Some centers close the soft palate
together with the lip in the rst year of life and the
hard palate much later. Other centers prefer to
close the hard and soft palate in one session or
sequentially 3months apart. Nasal corrections or
velopharyngeal surgery can be performed prior to
school enrollment. Skeletal corrections of the jaws
or corrections of the nose should be postponed
until skeletal growth is complete and the child
reaches adulthood. If both rhinoplasty and skeletal
corrections are required, rhinoplasty should be
performed after skeletal corrections [22]. There is
also controversy regarding the timing of bone
grafting of the cleft alveolus. Depending on the
timing of the procedure, it is referred to as primary
osteoplasty if it is performed in the rst dentition,
secondary osteoplasty if it is performed in the
mixed dentition shortly before the eruption of the
permanent canine, and tertiary osteoplasty if it is
performed in the completed second dentition [23].
However, the patient should be closely monitored
by an orthodontic in order to support growth with
orthodontic appliances. Otorhinolaryngology
monitoring is necessary from the age of 3months
to avoid middle ear involvement.
Preoperative Orthodontics
Nowadays, early orthodontics plays a crucial role
in the treatment of patients with cleft lip, jaw, and
palate. Newborn patients with unilateral and
bilateral cleft lip, alveolus, and palate patients are
provided with an orthodontic appliance in the
rst days of their lives, which separates the nasal
cavity from the oral cavity and relocates the
tongue from the cleft area into the oral cavity to
improve oral function during feeding.
Furthermore, such appliances have an additional
effect on jaw development and result in harmonization of the position of the deviated bony segments [24, 25]. This treatment may help to
improve conditions for a tissue-sparing surgical
closure. Different approaches have been
described in the literature. Latham pursued the
strategy of using xed appliances and higher
forces to shape the segments and to achieve a tensionless surgical gap closure by bringing the segments closer together [25–27]. The application of
removable orthodontic devices is done with signicantly lower forces, but also has an effect on
facial development, normalizes the tongue position, and facilitates surgical closure. The removable palatal plates can additionally be combined
with a naso-alveolar molding (Fig.7.3) to further
approximate the nasal wings and improve symmetry [28–30].
Preoperative Preparation
As a preparation for surgery, it is recommended
to perform a preoperative photo documentation,
which can be done using classical 2D photos (en
face and in prole) or by means of 3D stereophotometry (see Fig. 7.3) [25]. Impressions of the
jaws for the fabrication of a palatal plate can be
made conventionally producing classical plaster
casts. Nowadays, digital scan impressions may
be preferred to reduce the hazard of aspiration of
impression material.
All surgical steps are performed under general anesthesia using special spiral tubes placed
in the oral midline. For lip closure, the infant’s
head is only slightly hyperextended and placed
in a gel ring, which prevents the head from slipping. For the sterile covering of the surgical
eld, it is important that the eyes remain visible
in order to have an overall picture of the face
during lip closure.
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