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Treatment Principles ofBranchial
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Arch Diseases
ValentinKerkfeld andUlrichMeyer
9
Introduction
The treatment of branchial arch diseases, like
their classication, is clinically very complex and
requires a good multidisciplinary collaboration
of oral surgeons, plastic surgeons, pediatricians,
orthodontists, and other practitioners depending
on the severity of the disease. In addition to the
purely somatic consideration, the psychological
component must not be disregarded. Changes in
the face have far-reaching effects on psychosocial development. Therefore, psychological support of the patient and his relatives before and
after surgical therapy should not be omitted. This
can also manage any expectations patients and
their relatives may have about surgical outcome.
Surgical Treatments
Branchial arches are metameric in structure, i.e.,
each branchial arch is structurally constituted in
the same way. Thus, each branchial arch has a
nucleus of mesoderm, which later gives rise to
V. Kerkfeld (*)
Clinic for Maxillofacial and Plastic Facial Surgery,
Westdeutsche Kieferklinik; University of Düsseldorf,
Düsseldorf, Germany
U. Meyer
Center for Jaw-, Face- and Skull Surgery,
Münster, Germany
e-mail: praxis@mkg-muenster.de
cartilage, bone and muscle, a branchial arch
nerve from the neural crest, and a branchial arch
artery. Accordingly, branchial arch diseases can
affect the hard tissue, soft tissue, as well as nervous structures [1]. Due to the heterogeneity of
symptoms and manifestations of branchial arch
diseases, there is no one xed treatment concept.
It is much more the task of the practitioner to
determine the correct therapy steps based on the
individual symptoms. Therapeutic options are
described in this chapter depending on the
affected organ.
Hard Tissue
Jaw surgery has a central role in the therapeutic
concept of branchial arch diseases, as it is almost
always accompanied by a change in the jaws
(Fig.9.1).
Orthognathic Surgery
Both mandibular and maxillary displacement as
well as combined maxillary and mandibular displacement in terms of orthognathic surgery may
be necessary. If the indication is purely aesthetic,
treatment should be delayed until growth is complete in order to avoid recurrences. However,
functional jaw relocation surgery may already be
indicated in childhood to prevent growth retardation and functional impairment during development [2].
© Springer Nature Switzerland AG 2023
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-031-28069-6_9
117

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Fig. 9.1 Radiological features of a patient suffering from Goldenhar syndrome
V. Kerkfeld and U. Meyer
Distraction Osteogenesis
Distraction osteogenesis is a widely used therapeutic option to autologously augment local bone
[3]. This technique is able to achieve new bone
formation even after growth has been completed.
The surrounding tissue, soft tissues, and vascular
supply are adapted to the distraction. Mandibular
distraction osteogenesis allows mandibular
lengthening in early childhood, e.g., in patients
with Pierre Robin sequence or Goldenhar syndrome [4].
In PRS, distraction osteogenesis can prevent
further invasive medical measures that otherwise
might become necessary. Having lengthened the
mandible, the tongue is pulled further ventrally
so that the airway is less restricted. The typical
severe restrictions, such as respiratory distress
and eating disorders, can thus be reduced, so that
tracheotomies and tube feeding may not be necessary [5].
Distraction osteogenesis is able to generate
long-term size increases of the mandible, contributing to a symmetrical appearance [6, 7].
Augmentation
However, orthognathic surgery can be of little
help in cases of aplasia of the jaw. In these cases,
complex reconstruction of the unattached jaw is
necessary. This can be achieved by autologous
bone transplantation or articial bone replacement. Both procedures have their individual
advantages and disadvantages and must be carefully considered with the patient and the patient’s
guardians.
For transplantation, autologous tissue is taken
from a healthy donor site (e.g., grafted ribs [8, 9],
bula [10]) and implanted at the missing site.
However, transplantation suffers from the risk of
donor-site morbidity. The bone defect is decisive
in the choice of transplantation procedure.
In recent years, the technique of augmentation
with articial materials such as polyether ether
ketone (PEEK) has therefore become increasingly widespread. In this way, CAD/CAMsupported patient-specic implants can be
manufactured, which can be implanted quickly
and safely [11].
In this way, even larger defects can be treated
adequately.
In patients with more complex defects, the use
of temporomandibular joint implants may also be
necessary [12].
Soft Tissue
In addition to the bony structures, the soft tissue
structures, including fatty tissue and muscles,
play a decisive role in facial appearance and
facial symmetry. Various therapeutic approaches
have been established to counteract this primarily
aesthetic decit.

9 Treatment Principles ofBranchial Arch Diseases
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119
Adipose Graft
Autologous adipose grafting allows relatively
uncomplicated augmentation of soft tissue. The
advantage of autologous adipose tissue grafting
is complete biocompatibility and subsequent
modication by harvesting or further addition of
adipose tissue. In addition, the human body has
an almost inexhaustible source of this adipose tissue, which can also be removed effortlessly and
without great donor-site morbidity. Nevertheless,
the possible complications, such as over- or
undercorrection, lumps, bumps, infection, damage to adjacent structures, and ischemic necrosis,
must be considered [13].
Pedicled Flaps
Pedicled aps can ll larger soft tissue defects.
This involves autologous transplantation of soft
tissue consisting of skin, fatty tissue, and often
attached muscle. This procedure, established in
reconstructive surgery, is often used after trauma
or tumor, but has been little studied in the therapy
of congenital defects [14].
Free Flaps
For large soft tissue defects, the free ap technique is also an option. Here, the autologous graft
is cut off from the blood supply at its origin and
implanted freely at the defect site. Reanastomosis
with the local blood vessels is performed here.
However, this also represents the critical step in
healing, as free ap grafting carries the risk of
nonhealing or insufcient blood supply.
Nevertheless, this procedure has also been
described in congenital defect situations [14].
Implants
In addition to autologous grafts, articial implants
are also available to ll soft tissue defects. The
materials used are mainly silicone and polyethylene (PEEK). Especially mandibular defects, such
as aplastic jaw angles, have recently achieved
excellent results. However, since the materials
are static and do not grow with the patient, this
procedure is only indicated for fully grown
patients [15].
Nervous Structure
In the context of branchial arch diseases, there
may also be underdevelopment of the nerves,
with the failure of the facial nerve (N.VII as a
nerve component of the second branchial arch)
being particularly clinically prominent. The
facial paralysis leads to considerable functional,
aesthetic, and consequently also psychosocial
problems.
Cause Oriented: Cross-Facial Nerve
Grafting
Cross-facial nerve grafting is possible as a causal
therapy. In this procedure, a nerve interposition
device is moved from the healthy side to the diseased side. The nerve interposition is usually the
sural nerve, which is easy to reach and whose
sensitive innervation area is also very small, so
that no major neuronal decits are to be expected
after removal. A branch of the facial nerve on the
healthy side is split and connected to the interposition graft. Subsequently, a symmetrical motor
function of the mimic musculature is achieved
[16, 17].
Symptom Oriented
Alternatively, attempts can be made to conservatively compensate for the negative effects caused
by facial paralysis.
Lagophthalmos
Restricted eyelid closure results in drying of the
cornea. Moisture chambers as well as creams
protect against drying out for the time being [18].
However, a denitive therapy should be aimed at
for the sake of patient comfort. Therefore, lid
loading is an easy, cost-effective, and reversible
attempt to manage lagophthalmos [19]. An ophthalmologic presentation is mandatory.
Mouth Angle
Drooping corners of the mouth on the affected
side have a strong impact on the psychosocial
appearance in addition to functional limitation. If
cross-facial nerve grafting is not possible, an

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V. Kerkfeld and U. Meyer
attempt is made to achieve symmetry of both
sides by reducing the muscle tone of the depressor anguli oris muscle on the healthy side by
botulinum toxin administration. As ultima ratio,
surgical removal of the depressor anguli oris
muscle is also possible [20].
Microtia
Microtia is one of the most common and often
most obvious symptoms of patients with branchial arch diseases (Fig.9.2). This can be accompanied by sometimes signicant functional
disturbances up to anacusis. An otolaryngologic
evaluation is necessary in all cases, and some
otolaryngologists have additional qualications
in the eld of ear reconstruction. In principle,
there are three therapeutic approaches for ear
reconstruction, if desired [21, 22].
Autologous Reconstruction
The gold standard in ear reconstruction is autologous transplantation of rib cartilage. The procedure offers very good long-term results and is
therefore well suited as a long-term solution.
This therapy option is conceivable from the age
of 8years [21, 23].
Silicone Prosthesis
Silicone prostheses mimic the appearance of the
outer ear. They can be either bonded or implant
supported. In childhood, the desire for a prosthesis often arises from the parents’ motivation to
protect the child from bullying. In this case, the
noninvasive bonded procedure should be used so
as not to generate further damage to the defect
situation in view of the child’s growth. After
growth is complete, an implant is possible, which
sometimes generates more patient comfort [21,
24].
Porous Polyethylene Implant
Porous polyether implants are a well-established
option for obtaining the desired outcome of
autologous reconstructive procedures without
incurring the risks and disadvantages of that
same therapy. Here, patient-specic ear implants
are individually manufactured and subsequently
implanted. Due to the fact that at the age of 6, the
Fig. 9.2 Typical clinical appearance of a patient with Goldenhar syndrome. The right side of the face is shortened, and
both ear and mandible are underdeveloped

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121
Fig. 9.3 Dental status in a patient with Goldenhar syndrome. The very pronounced interlocking of the teeth
with consecutive malocclusion as well as difcult-to-
ear is already almost at the size and width of the
later adult human being, reconstruction can usually already be carried out at school age. However,
as with any invasive reconstructive procedure,
there is a risk of infection. In addition, it can fracture under mechanical stress, e.g., during contact
sports [21, 25, 26].
Dental Problems
Patients with branchial arch diseases often suffer
from secondary dental problems. These include
dental deformities, but also sometimes serious
dental malocclusions (Fig.9.3). Due to tooth and
dental arch anomalies, occlusion problems and a
reduced mouth opening may occur. The consequences are poor oral health (caries, periodontitis) in adulthood as well as nutritional and
respiratory problems, which occur mainly in
infancy and childhood. For this reason, early and
effective orthodontic co-management is
essential.
Conclusion
Treatment of the effects of branchial arch diseases is very complex and must be tailored to the
individual patient. Therefore, a multidisciplinary
team of practitioners versed in the eld is essential for comprehensive and holistic treatment of
accomplish oral hygiene can be seen. Furthermore, the
orthopantomogram shows the severely displaced tooth 43
and the missing ramus of the right mandible
these patients. The outstanding advancement of
many reconstructive therapy approaches makes it
possible to signicantly reduce or even eliminate
the congenital decits. However, the choice of
the specic therapy not only is given by the maximum possible, but also signicantly depends on
intrinsic and extrinsic factors of the patient.
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Part V
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Diseases: Dysgnathias

History ofOrthognathic Surgery
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T.Fillies andT.Seier
10
Mandibular Surgery
The history of orthognathic surgery started in the
mid of the nineteenth century. The American surgeon Simon P.Hullihen is said to be the father of
orthognathic surgery [1, 2]. In 1849, he described
the rst mandibular osteotomy for the correction
of a skeletal anterior open bite. He operated a
woman who suffered from mandibular deformity
due to a burn scar contracture. To improve the
occlusion, Hullihen performed a wedge osteotomy in the premolar region, which enabled him
to set back the anterior mandibular segment and
close the open bite. In these times, modern plate
and screw osteosynthesis was far from development and the segments were stabilized with a
cemented occlusal splint that was constructed on
a plaster cast to allow bony healing [1–3].
In 1887, Vilray P. Blair, who closely worked
together with the pioneering orthodontist Edward
E.Angle, performed a modication of Hullihen’s
operation for the correction of mandibular prognathism. He performed a bilateral wedge resection to set the mandible back [1, 2].
The Austrian surgeon Anton Freiherr von
Eiselsberg published a modied mandibular setback technique in 1906 [1, 4] using a step oste-
T. Fillies (*) · T. Seier
Clinic of Maxillofacial and Plastic Facial Surgery,
Marienhospital Stuttgart, Stuttgart, Germany
e-mail: thomas.llies@vinzenz.de; Thomas.seier@
vinzenz.de
otomy design to increase the surface area of bony
contact to improve osseous consolidation [1], a
concept that should become very important in the
future.
The rst horizontal osteotomy above the
occlusal plane to set back the mandible in prognathism was described by Sir William Arbuthnot
Lane in 1905 and was soon adopted by Blair and
Babcock [2]. In 1906, Blair described a procedure to advance the mandible by using a Gigli
saw in a blind fashion via small extraoral skin
incisions for the horizontal ramus osteotomy [1,
5] (Fig.10.1).
Varaztad Kazanjian, an American surgeon
from Boston, also used a Gigli saw for mandibular body osteotomies and an orthodontic splint
that was cemented after surgery to stabilize the
occlusion after surgical correction of mandibular
prognathism [1]. In 1928, the Czech surgeon
Frantisek Kostecka described a similar technique,
which used a Gigli saw for the horizontal ramus
osteotomy via an extraoral approach that was
widely used in Europe [1].
In the late 1920s, the famous oral and maxillofacial surgeons Martin Wassmund from Berlin
and Hans Pichler from Vienna independently
described the so-called inverted-L osteotomy to
advance the mandible [1]. They used an extraoral
approach and put bone grafts between the osteotomy sites to improve bony consolidation [1].
Hans Pichler was a very inuential surgeon and is
considered to be the father of the Vienna school
© Springer Nature Switzerland AG 2023
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https://doi.org/10.1007/978-3-031-28069-6_10
125
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