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7 Treatment Principles inOrofacial Clefts
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a
Fig. 7.3 Preoperative orthodontic treatment using naso-alveolar molding (NAM) (a) in a patient with unilateral cleft
lip, alveolus, and palate (b)
Surgical Techniques
b
other hand, described the so-called rotation
advancement technique for lengthening the
Cleft Lip Closure
medial lip stump, using an arcuate incision from
the vermillion to the columella attachment
In all cleft lips, including microforms, there is
dehiscence of the orbicularis muscle and nasal
deformity [31]. The goal for lip closure is to generate a symmetrical upper lip with a well-dened
philtrum and clear lip red-white border, as well as
symmetrically shaped nasal entrances.
Preservation of the existing tissue is paramount.
The anatomical structures belonging to each
(Fig.7.4c, d) [35]. The wavy line repair according to Pfeifer pursues the strategy of achieving lip
lengthening through a wave incision and stretching of the incision edges (Fig.7.4e, d) [36]. For
the procedure of double-sided cleft lips, mainly
straight incisions are used and performed in a
wide variety of variations [16, 25, 37–41]
(Fig.7.5).
other must be connected; for this purpose, the
misinsertion of the facial muscles must be
detached, reorientated in a physiological
Palate Cleft Surgery
horizontal course, and united. Various approaches
for the closure of unilateral cleft lips have been
described in the literature, the complete detailed
treatment of which would go beyond the scope of
this chapter. It should be mentioned, however,
that with the exception if microforms, where the
straight incisions according to Veau are still used,
today various more complex surgical procedures
are described for the more severe cleft lip manifestations. Von Hagedorn as well as further developments by Le Mesurier, Tennison, and Randall
have described the principle of angular incision
guidance for lengthening the medially shortened
lip stump with caudal rotation of the lip red-white
border (Fig. 7.4a, b) [32–34]. Millard, on the
If the palate is affected, in addition to the anatomical cleft formation, there is a malinsertion of
the muscles of the soft palate, which results in
functional decits. The longitudinal parts of the
palatopharyngeus muscle and the levator veli
palatini muscle do not meet in the midline, as in
healthy individuals, but misinsert. The palatopharyngeus muscle attaches to the hard palate, and
the levator veli palatini muscle ends in the cleft
margin. The muscular sling built by these muscles is interrupted. The goal of cleft palate surgery is thus, on the one hand, tight closure
without residual perforations and, on the other
hand, functional reconstruction of the muscle
84
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a
bdf
P. Kaumann and H. Schliephake
Fig. 7.4 (a, b) Schematic illustration of lip closure according to Tennison and Randall; (c, d) according to Millard; (e,
f) according to Pfeifer
Fig. 7.5 Postoperative result after lip closure according to Tennison and Randall (a, b)
sling, by detaching and uniting the abovementioned muscles. The aim is to achieve a good
velopharyngeal closure for unobstructed phonation. Different techniques are described for palatal closure. Using the vomer ap technique
according to Pichler, a mucosal ap from the sep-
tal area is released from the vomer and sutured to
the nasal layer of the lateral palatal process. The
wound surface facing the oral cavity is left to secondary granulation (Fig. 7.6e, f) [42]. This
Pichler plasty is only a single-layer closure.
There is concern about this technique claiming
7 Treatment Principles inOrofacial Clefts
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ac e
bd f
85
Fig. 7.6 Pedicle ap plasty before incision (a) and after closure (b); bridge ap plasty before incision (c) and after
closure (d). Pichler plasty before incision (e) and after closure (f)
that the union of non-corresponding tissue parts
may prevent a proper adjustment of the lateral
segments behind the premaxilla and possibly
lead to a collapse of the lateral segments. Other
authors consider these concerns to be unfounded
[43]. Other approaches have used a mucoperiosteal palatal axial pattern ap, pedicled dorsally
on the palatine artery and simultaneous mobilization of the nasal mucoperiosteum from the hard
palate and a three-layered closure of the separated structures in the midline (Fig.7.6a, b) [37,
44, 45]. In bridge ap plasty, unlike pedicle ap
plasty, the mucoperiosteal ap is not separated
anteriorly and thus remains pedicled anteriorly
and posteriorly (Fig.7.6c, d) [46].
For closure of the velum, the intravelar velo-
plasty according to Kriens and the soft palate clo-
sure according to Widmaier and according to
Furlow should also be mentioned [47, 48]. In
Kriens’ technique, the misinserted muscles are
sharply detached at the posterior margin of the
hard palate, and a nasal mucosal sheet is prepared
and sutured to the opposite side. This is followed
by suturing of the muscles followed by the
mucosa [47] (Fig.7.7a, b).
The Widmaier procedure is similar to the
preparation of pedicle aps. However, the aps
are not prepared subperiosteally but epiperiosteally, and the vascular nerve bundle is spared and
not included in the ap preparation; ap repositioning in terms of a VY plasty allows closure.
Flaps mobilized laterally from the cheek can be
used to close the resulting relief incisions
(Fig.7.7c, d) [48]. Furlow described an inverted
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ace
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P. Kaumann and H. Schliephake
Fig. 7.7 Velum closure according to Kriens (a, b), Widmaier (c, d), and illustration of the double-reversing Z-plasty
(Furlow palatoplasty) (e, f)
double Z-plasty for soft palate closure in 1978
Secondary Operations
(Fig. 7.7e, f) and the double-reversing Z-plasty
(Furlow palatoplasty) [49].
Secondary surgeries are interventions on previously operated cleft regions. Here, a distinction
must be made between those planned as part of
Bone Grafting oftheAlveolar Cleft
the overall surgical concept, e.g., nasal columella
lengthening in previously operated bilateral cleft
Performing surgical reconstruction of the alveolar cleft segment is intended to avoid collapse of
the alveolar crest stumps, augment the deformed
cleft nasal base as well as the hypoplastic hard
palate, x the premaxilla in double-sided clefts,
and resolve the crowding of the dental nuclei
[21, 23].
lips and palates and secondary bone grafting, and
corrective operations to improve the esthetic and
functional result in the case of unsatisfactory primary surgery.
Secondary surgeries include bone grafting,
velopharyngoplasty for speech improvement, rhinoplasty, and closure of residual perforations.
The scheduling of secondary surgeries depends,
on the one hand, on whether the surgery is part of
7 Treatment Principles inOrofacial Clefts
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87
the overall concept or whether it is a corrective
surgery, as explained above. Planned secondary
surgeries usually include nasal columella lengthening, possibly also nasal entry corrections
before enrollment, and secondary osteoplasty
shortly before eruption of the permanent canine.
Velopharyngoplasties are performed before
school enrollment, if necessary. Orthognathic
osteotomies and rhinoplasties are performed after
growth completion [22].
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36. Pfeifer G.Lip corrections following earlier cleft surgery by way of wave-line incisions. Dtsch Zahnarztl
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Part IV
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Diseases: Branchio-oculo Facial Syndromes
Diagnosis andClassication
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ofBranchial Arch Diseases
UlrichMeyer andValentinKerkfeld
8
Introduction
Branchial arch diseases are a difcult-to-classify
craniofacial malformation entity. This is based on
several aspects:
1. The phenotypic expression can vary signicantly (from mild to severe).
2. Involvement of anatomical structures (skull,
face, ears, others) differs within one disease.
3. The underlying course of disease development is often unsolved.
4. Diseases may be syndromal or
non-syndromal.
5. Milder forms are difcult to distinguish from
normal variants.
6. A comprehensive classication does not exist.
Due to the signicant variability and spectrum
of the disease, there are numerous and confusing
classications. Classication schema in craniofacial malformations is mainly based on phenotypic expression. In contrast to such phenotypic
classication schema, genetically based classi-
U. Meyer (*)
Center for Jaw-, Face- and Skull Surgery,
Münster, Germany
e-mail: praxis@mkg-muenster.de,
meyer@kieferklinik-muenster.de
V. Kerkfeld
Clinic for Maxillofacial and Plastic Faial Surgery,
University of Düsseldorf, Düsseldorf, Germany
cation is a modern approach. Knowledge of the
genetic basis of human disease and its effect on
embryologic development has expanded greatly
in recent years [1]. Disorders of the rst and second branchial arch (BA) are generally thought to
result from a combination of inadequate migration and inadequate formation of facial mesenchyma. Because many structures of the head and
neck migrate during fetal development, an understanding of embryologic development helps
determine the origin and nature of congenital
lesions.
The human face is highly multipartite and
results from the complex coordination of genetic,
cellular, and environmental factors [2–4].
Through prior genetic analysis by genome-wide
association studies (GWASs), over 100 loci have
been implicated in normal-range facial morphology [5–21]. However, as with all complex morphological traits, the ability to identify and
describe the genetic architecture of the face is
limited by our ability to accurately characterize
its phenotypic variation, identify variants of both
large and small effects [13], and identify interactions between variants. White [22] described previously a data-driven approach to facial
phenotyping, which facilitated the identication
and replication of 15 loci involved in global-tolocal variation in facial morphology [14]. Many
of these loci harbor genes involved in craniofacial syndromes but had not yet been observed in
GWAS for normal-range facial morphology, but
© Springer Nature Switzerland AG 2023
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-031-28069-6_8
91
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U. Meyer and V. Kerkfeld
53 genome-wide signicant (26 also study-wide
signicant) peaks are in regions with no previously known role in facial development or disease, potentially pointing to previously unknown
genes and pathways involved in facial development. The ndings by White and colleagues [22]
give an insight into the limited knowledge on
gene–phenotype relations that is fundamental to
the recent understanding of classication of
hypoplastic or hyperplastic phenotype developments of the face. The fact that most of the branchial arch diseases are unilateral gives rise to an
even more confusing understanding of how to
classify diseases of the branchial arch complex.
Nearly all tissues in the head and neck region
can be affected by the misdevelopment of branchial arches. Some syndromes have a unilateral
involvement, whereas others present with bilateral involvement. Clinical manifestations are
present in different regions of the skull and face.
Additionally, phenotypic variability is common
in these disease entities. Whereas some individuals have subtle facial involvement (e.g., slight
facial asymmetry), others have severe involvement of multiple tissues and organs. The clinical
extent of these malformations includes the skull
base, the midfacial region, the mandible, and the
neck.
We distinguish here diseases of branchial arch
complex from a clinically (Table8.1) and genetically (Table8.2) driven view in:
• Hypoplastic diseases:
– Unilateral:
Non-syndromic
Syndromic
– Bilateral:
Non-syndromic
Syndromic
• Hyperplastic diseases:
– Unilateral:
Non-syndromic
Syndromic
– Bilateral:
Non-syndromic
Syndromic
– Partial
Diagnostic Approach
Due to the wide variable presentation of branchial arch diseases, several classication systems
have been proposed to better differentiate the
phenotypical presentation to help improve diagnosis, treatment, and prognostic data.
Classication was done predominantly for syndromal diseases but failed to include the whole
range of diseases of the branchial arch complex.
Classication should be based on a thorough
medical history, followed by a clinical and radiographic investigation (Fig.8.1). Genetic evaluation is dependent on the individual patient
situation (Table8.3).
Table 8.1 Classication of diseases concerning their phenotypical features
Phenotypical classication of branchial arch complex diseases
Hypoplasias Hyperplasias
Unilateral Bilateral Unilateral Bilateral
cMicrognathia
• Goldenhar syndrome
• Auriculocondylar syndrome
• Oculo-auriculo-vertebral
dysplasia
• Acrofacial dysostosis subtypes
• Nager syndrome
• Miller syndrome
All photographs by Meyer
• Treacher Collins
syndrome
• Stickler syndrome
• Di George syndrome
• Pierre-Robin syndrome
• Acrofacial dysostosis
• Cincinatty type
• Hemifacial hyperplasia
• Hemimandibular hyperplasia
• Condylar hyperplasia
• Beckwith-Wiedemann
syndrome
• Sotos syndrome
• Weaver syndrome
8 Diagnosis andClassication ofBranchial Arch Diseases
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Table 8.2 Classication of diseases concerning their genetic-pathophysiological courses
Genetic classication of branchial arch complex diseases
Hypoplasias Hyperplasias
Various pathologies Genetic course Various pathologies Genetic course
• Micrognathia
• Goldenhar syndrome
All photographs by Meyer
• Treacher Collins syndrome
• Oculo-auriculo-vertebral
dysplasia
• Auriculocondylar syndrome
• Stickler syndrome
• Di George syndrome
• Pierre-Robin syndrome
• Acrofacial dysostosis with
subtypes
• Cincinatty type
• Nager syndrome
• Miller syndrome
• Hemifacial hyperplasia
• Hemimandibular
hyperplasia
• Condylar hyperplasia
• Beckwith- Wiedemann
syndrome
• Sotos syndrome
• Weaver syndrome
93
Fig. 8.1 Diagnostic approach. (All photographs by Meyer)
– Clinical Investigation
The patient’s history should be evaluated carefully. Special attention should be placed on
the information concerning similar phenotypes
or diseases in the family. The clinical inspection should include the whole head and neck
area. Limb alterations should be recorded
when present. Different medical specialties
should be involved in the clinical evaluation of
syndromal diseased patients: maxillofacial
surgeons, plastic surgeons, ENT specialists,
ophthalmologists, and dentists.
– Radiographic Evaluation
In the beginning, plain radiographs (OPT or
lateral ceph) were used to determine the bony
structure of patients. With the implementation
of 3D imaging techniques, CT, CBCT, or MRI
has become the imaging technique of choice
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