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C. Linz and T. Schweitzer
(especially in very young patients), the duration
of the examination and the failure to exactly
depict bones. Recent advances—such as black
bone MRI—will help to compensate for (at least)
this aspect in the near future.
As already mentioned, the workup with ultrasound, radiographs and MRI provides sufcient
information on the intracranial situation, even in
complex craniosynostosis. In many centres, several CT scans are still part of the usual treatment
algorithm. As a CT scan is associated with a risk
of cancer induction, we reserve this examination
for emergency situations or for when exact bone
depiction is needed, for example, for printing 3D
skull models for surgical planning.
Dilated Ventricles inSyndromic
Craniosynostosis
Although some reports on hydrocephalus in monosutural craniosynostosis exist (especially in craniosynostosis of the sagittal suture), dilated
ventricles—or hydrocephalus—are mainly an
issue in syndromal—or complex—craniosynostosis. Different conditions cause hydrocephalus,
including
• Enlarged ventricles without elevated ICP
• Impaired CSF circulation
• Tonsillar herniation (Chiari malformation)
• Elevated intravenous pressure level
nisms that might lead to a premature fusion of
cranial sutures. In genetics, several distinct mutations that are associated with a specic syndrome
have been identied. Additional posttranslational
mechanisms—such as copy number variations
(CNVs)—play a further role in the multifactorial
origin of craniosynostosis. Knowing the distinct
mutation and its associated syndrome has signicant advantages for further treatment strategies,
including in Crouzon syndrome (which is associated with normal intellectual behaviour and has a
very high risk of raised ICP), Apert syndrome
(which has a risk of elevated ICP in ca. 5% of
individuals but determines intellectual restrictions in up to one-third of children) and Muenke
syndrome (which is associated with 30–40% of
intellectual limitations but almost never with elevated ICP) (Fig. 4.3). However, Muenke syndrome is associated with inner-ear hearing
difculties that should be treated adequately and
in time in order to prevent additional developmental limitations.
Surgical Therapy inSyndromal
Craniosynostosis
Generally, surgical therapy is oriented around the
affected sutures. In case of a single affected
suture, the principles demonstrated above remain
valid. If multiple sutures have fused too early, a
different, individualised approach is required.
Any further treatment should be planned
according to these underlying pathologies. It is
important to employ higher pressure levels in
implanted shunt devices in order to restrain the
expansive impulse on the sutures. In coincidence
with elevated ICP in craniostenosis, the treatment
of enlarged ventricles might be even postponed in
order to use resulting expansive forces for optimised remodelling after craniectomy.
Genetic Counselling inSyndromic
Craniosynostosis
We have borne witness to tremendous advances
in understanding the pathophysiological mecha-
Early Decompression
Premature fusion of several cranial sutures may
lead to the severe abnormal skull shape of a socalled cloverleaf skull. Of course, elevated ICP is
patientnICP ↑ %papilledema %cognitive
Crouzon53 92 53 4
Apert 44 64 14 70
S-Chotzen 71 35 40 4
Muenke42 5 - 33
Fig. 4.3 Shortlisting of distinctive craniofacial syndrome
and associated ICP, papilledema and cognitive decit at
our centre. These differences underline the importance of
genetic diagnostics as the knowledge of an underlying
syndrome guides through counselling and follow-ups.
(Own data)
deficit
%
4 Treatment Principles inCraniosynostosis
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53
a likely condition and might need early treatment. It is very important to bear in mind that the
fast growth dynamics in the rst months of life
cause early re-ossication and re-occurrence of
the original suture status. On the other hand, this
re-ossication enables the surgeon to perform a
vast craniectomy in order to create more space
for the growing brain.
In the presence of dilated ventricles, the experienced craniofacial team might even postpone
their treatment in order to gain more space via a
primary decompressive craniectomy. However,
the presence of sharp bony spiculae that dive
deep into the cortical sulci is an enormous challenge intraoperatively.
Generally possible early surgical approaches
include an early open craniectomy as well as
early endoscopically assisted procedures in the
rst 3–4months.
Cranial Remodelling
The indication for surgery might be based not
only on the considerations of restricted intracranial space, but also on the osteoplasticity of the
dural layer, functionality and aesthetic elements.
As mentioned above, the capacity of large skull
areas to re-ossify declines after 8months. Even if
minor defects still ossify, larger skull defects
should be avoided. This means that surgical strategy should aim at reorganising bone segments
with only minor resulting bone defects. These
small defects might be lled with bone crump or
scraps that were created while sawing or that
were even separately harvested.
Split bone techniques that are used to cover
larger areas are not successful before 3–4years
of life.
Active Distraction/Springs
If an active rearrangement of bone segments
seems insufcient or difcult to achieve, an
active distraction with distractors may be
planned. This is a frequently chosen approach for
midface distraction but might also achieve considerable gains in intracranial space. Some centres employ springs to prevent fast reclosure of
the sutures.
The third part of this monography concentrates on the technical aspects of the different surgical strategies.
References
1. Mathijssen IMJ, C.Working group guideline, updated
Guideline on treatment and management of craniosynostosis. J Craniofac Surg. 2021;32(1):371–450.
2. Rice DP. Craniofacial sutures, development, disease
and treatment. Basel: Karger; 2008.
3. Richtsmeier JT, Flaherty K.Hand in glove: brain and
skull in development and dysmorphogenesis. Acta
Neuropathol. 2013;125(4):469–89.
4. Zöller JE, Lober AC, Mühling WD. Kraniofaziale
Chirurgie: Diagnostik und Therapie kraniofazialer
Fehlbildungen. Stuttgart: Thieme; 2003.
5. Hall KM, etal. Effectiveness of screening for craniosynostosis with ultrasound: a retrospective review.
Pediatr Radiol. 2017;47(5):606–12.
6. Regelsberger J, etal. Ultrasound in the diagnosis of
craniosynostosis. J Craniofac Surg. 2006;17(4):623–5.
7. Whittall I, etal. Postnatal diagnosis of single-suture
craniosynostosis with cranial ultrasound: a systematic
review. Childs Nerv Syst. 2021;37(12):3705–14.
8. Schweitzer T, et al. Diagnostic features of prematurely fused cranial sutures on plain skull X-rays.
Childs Nerv Syst. 2015;31(11):2071–80.
9. Schweitzer T, et al. Avoiding CT scans in children
with single-suture craniosynostosis. Childs Nerv Syst.
2012;28(7):1077–82.
10. Cerovac S, etal. Are routine preoperative CT scans
necessary in the management of single suture craniosynostosis? Br J Neurosurg. 2002;16(4):348–54.
11. Rachwalski MWB, Kress W.Klinik und Genetik syndromaler und nichtsyndromaler Kraniosynostosen.
Berlin: Springer; 2013.
12. Behr B, Longaker MT, Quarto N.Differential activation of canonical Wnt signalling determines cranial
sutures fate: a novel mechanism for sagittal suture
craniosynostosis. Dev Biol. 2010;344(2):922–40.
13. Korpilahti P, Saarinen P, Hukki J.Decient language
acquisition in children with single suture craniosynostosis and deformational posterior plagiocephaly.
Childs Nerv Syst. 2012;28(3):419–25.
14. Starr JR, et al. Multicenter study of neurodevelopment in 3-year-old children with and without singlesuture craniosynostosis. Arch Pediatr Adolesc Med.
2012;166(6):536–42.
15. Kapp-Simon KA, et al. Neurodevelopment of children with single suture craniosynostosis: a review.
Childs Nerv Syst. 2007;23(3):269–81.
16. Naumann HL, etal. Duration of exposure to cranial
vault surgery: associations with neurodevelopment
among children with single-suture craniosynostosis.
Paediatr Anaesth. 2012;22(11):1053–61.
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C. Linz and T. Schweitzer
17. Chieffo D, et al. Long-term neuropsychological
development in single-suture craniosynostosis treated
early. J Neurosurg Pediatr. 2010;5(3):232–7.
18. Speltz ML, etal. Intellectual and academic functioning of school-age children with single-suture craniosynostosis. Pediatrics. 2015;135(3):e615–23.
19. Wu RT, et al. SMAD6 genotype predicts neurodevelopment in nonsyndromic craniosynostosis. Plast
Reconstr Surg. 2020;145(1):117e–25e.
20. Maltese G, etal. Spring-assisted surgery in the treatment of sagittal synostosis: a systematic review. J
Plast Surg Hand Surg. 2015;49(3):177–82.
21. Delye HH, et al. Endoscopically assisted craniosynostosis surgery (EACS): the craniofacial team
Nijmegen experience. J Craniomaxillofac Surg.
2016;44(8):1029–36.
22. Gwanmesia I, et al. Frontofacial advancement by
distraction osteogenesis: a long-term review. Plast
Reconstr Surg. 2015;135(2):553–60.
23. Patel N, Fearon JA.Treatment of the syndromic midface: a long-term assessment at skeletal maturity.
Plast Reconstr Surg. 2015;135(4):731e–42e.
24. Agochukwu NB, Solomon BD, Muenke M. Impact
of genetics on the diagnosis and clinical management
of syndromic craniosynostoses. Childs Nerv Syst.
2012;28(9):1447–63.
25. Collmann H, Sorensen N, Krauss J. Hydrocephalus
in craniosynostosis: a review. Childs Nerv Syst.
2005;21(10):902–12.
26. Brooks ED, etal. The etiology of neuronal development in craniosynostosis: a working hypothesis. J
Craniofac Surg. 2018;29(1):49–55.
27. Cabrejo R, etal. Functional network development in
sagittal craniosynostosis treated with whole vault cranioplasty. J Craniofac Surg. 2021;32(5):1721–6.
28. Tandon D, etal. Morphologic severity of craniosynostosis: implications for speech and neurodevelopment.
Cleft Palate Craniofac J. 2021;58(11):1361–9.
29. Chuang C, etal. Normalization of speech processing
after whole-vault cranioplasty in sagittal synostosis. J
Craniofac Surg. 2018;29(5):1132–6.
Part III
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Diseases: Orofacial Clefts
History ofCleft Treatment
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UlrichMeyer
Documentation ofCleft, Lip,
andPalate Malformations inArts
andScience
The history of the description of cleft lip and palate deformities and the description of cleft
patients in arts can be traced back many centuries. The variety of techniques used in cleft lip
and palate treatment approaches has grown considerably from ancient times to now.
Isolated archaeological evidence from the
ancient Schönwerda and Peruvian (Fig. 5.1)
civilizations has described persons with
untreated cleft deformities who lived until they
were adults [1]. Although the early Egyptians,
Mesopotamians, Indians, Greeks, and Romans
were pioneers in their respective medical and
surgical elds, no descriptions of cleft operations were recorded [2].
A terracotta statuette found in 1969 in the
Potters’ Quarter of Corinth is an early portray of a
patient with cleft, lip, and palate [3]. Dating from
700–300BC, the gurine portrays a clown with a
complete cleft lip modelled in such meticulous
detail that secondary defects of the premaxilla and
the alae of the nose are clearly visible. Statuettes
U. Meyer (*)
Center for Jaw-, Face- and Skull Surgery,
Münster, Germany
e-mail: praxis@mkg-muenster.de,
meyer@kieferklinik-muenster.de
5
Fig. 5.1 Sculpture of a patient with a cleft lip from the
Peruvian civilization (Sailko, Pre-Columbian art in the Art
Institute of Chicago, from https://commons.wikimedia.
org/wiki/File:Per%C3%B9,_moche,_contenitore_a_
forma_di_ragazzo_con_labbro_leporino_e_tatuaggi,_100_ac- 500_dc_ca.jpg , licensed under CC-By-3.0)
of gures with facial clefts can be seen at the
Musée Guimet in Paris (Wagner Collection), the
Museum für Völkerkunde in Munich, and in a
© Springer Nature Switzerland AG 2023
U. Meyer (ed.), Fundamentals of Craniofacial Malformations,
https://doi.org/10.1007/978-3-031-28069-6_5
57
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Fig. 5.2 Painting of a cleft lip and palate patient, displaying the typical signs of the cleft (head of a boy with a cleft
palate and hare lip; St. Bartholomew’s Hospital Archives
& Museum, from https://wellcomecollection.org/works/
xbbzquvg, licensed under CC BY 4.0)
group known as Los Danzantes at the archaeological site of Monte Albán in Mexico.
The treatment of cleft lip and palate patients
can also be traced back for centuries, but most of
them were single reports. A more standardized
approach to treat these deformities started in the
Renaissance and became more scientic during
the nineteenth century. All developments tried to
reconstruct and normalize the typical anatomical
deviations of the face (Fig.5.2): the lip, the nose,
the premaxilla, and the palate. From a historical
perspective (reected in this chapter), three steps
can be differentiated in a conceptualized manner:
(1), the closure of the lip, (2) the closure of the
palate, and (3) the approaches to handle the anterior jaw nose complex (premaxilla), especially in
bilateral clefts. The development of surgical techniques interdigitated over time to a state, where
the reconstruction is in some centers tried to
establish in a single-stage approach. Advances in
U. Meyer
the surgery of cleft lip and palate deformities
were paralleled by improvements in child anesthesia. This is of special relevance in the closure
of the palate, since bleeding and aspiration are
major and life-threatening complications.
History ofCleft Lip Surgery
The rst report of an operation for a cleft lip was
an impressive medical breakthrough at these
early times, as a Chinese surgeon successfully
closed a cleft lip in 390 BC [4]. An 18-year-old
youth, Wey Young Chi, who was born in the city
of Jen in the province of Hupeh was the rst
patient. The surgery was performed in Nanking
under the watchful eyes of Ying Chung-Khan, the
governor of the province, and was a success.
After his surgery, Wey Young-Chi was recruited
into the imperial army and quickly impressed
General Lin-Yu, by helping to suppress a revolt.
In due course of time, Wey himself rose to the
rank of general and later became Governor of the
Province of Yee. He eventually became Governor
General of the six provinces. Throughout his life,
he afrmed that he would never have achieved so
much if his cleft lip had not been repaired.
No operations for clefts were carried out in
ancient Egypt, but a mummy with a cleft palate
has been found. George Dorrance discussed the
case of a mummy that had been reported in 1929
by Smith and Dawson in their work Egyptian
Mummies published in London [5]. Thus,
Egyptians knew about the deformity. In the
ancient Mediterranean civilizations, these children were said to possess supernatural powers
[6]. In the great epoch of Greek and Roman medicine, no cleft operations were described,
although operations were performed in the mouth
and on the uvula. In Sparta, the unfortunate newborns were abandoned on Mount Tagete, while in
Rome, they were drowned in the Tiber River or
thrown off the Tarpeian Rock. The noted philosopher Plato, far from opposing this practice, justied it in one of his dialogues in the Republic,
explaining that it was a means of removing evil
omens and preserving the soundness of the race.
Not a single reference of this deformity is found
5 History ofCleft Treatment
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59
in the Corpus Hippocraticum, which represented
a compendium of the medical knowledge of the
period.
In Europe, Saxon surgeons described an operation for cleft lip in the Leechbook of Bald [7] in
950AD: “For harelip, pound mastic very small, add
the white of an egg and mingle as thou dost vermilion, cut with a knife the false edges of the lip, sew
fast with silk, then smear without and within with
the salve ere the silk rot. If it draw together, arrange
it with the hand; anoint again soon.”
The period between the Renaissance and the
nineteenth century was the beginning of a more
scientic oriented medicine. There are some surgical treatment principles reported (e.g.,
Albucasis of Arabia and his fellow surgeons used
the cautery instead of the scalpel). The technique
of suturing of the lips became more standardized
(Fig. 5.3). Fabricius ab Aquapendente (1537–
Fig. 5.3 Drawing of suturing a cleft lip (a discourse of
the whole art of chirurgie, wherein it is exactly set down
the denition, causes, accidents, prognostications, and
cures of all sorts of diseases ... whereunto is added the rule
of making remedies which chirurgeons commonly use:
with the presages of divine Hippocrates/[Peter Lowe]
from https://wellcomecollection.org/works/n5ef2byt/
images?id=bcdmjpps, Public Domain)
1619) was the rst to suggest the embryological
basis of such clefts [8]. Later on, studies on the
normal embryological development of the face
(Fig.5.4) were conducted and gave insight also
into orofacial cleft development.
Ambroise Paré was one of the greatest surgeons of the sixteenth century; he conducted
detailed studies on the anatomy of the lips and
palate and introduced signicant improvements
in the technique of suturing. He is credited with
the rst illustration of an operation on a cleft lip,
and it appears in a work by Paré in Les Oeuvres
showing the suturing method for cleft lip repair
[9]. The knowledge of cleft lip biology, treatment
concepts, and surgical corrections was accelerated by the publication of Pierre Franco’s Petit
Traité and Traité des Hernies in which he
described the condition as “lièvre fendu de nativité” (cleft lip present from birth). Pierre Franco, a
pupil of Ambroise Paré, never received a formal
medical education but wrote two surgical texts
based on his many years of experience, Petit
Traité and Traité des Hernies [10]. The latter was
published in 1561, and in it, Franco discusses the
cleft lip in ample detail devoting two chapters to
the subject. He was the rst to state the congenital nature of the malformation clearly and referred
to the unilateral harelip as the “lièvre fendu de
nativité” (cleft lip present from birth). He provides a meticulous classication of various types
of clefts, calling the bilateral harelip the “dent de
lièvre” (hare’s tooth) presumably because this
condition was frequently accompanied by a
marked protrusion of the premaxilla bone with its
teeth. There were no reports of cleft palate operations in the sixteenth and seventeenth centuries,
probably because surgeons were frightened of
the copious bleeding and the severe pain caused
by the operation.
In 1844, Germanicus Mirault devised, in a signicant advancement in cleft closure technique
towards the method of Pierre Franco, to an ingenious method to circumvent the problem of shortening the vertical lip height, by introducing a
triangular ap from the lateral side into a gap created by making a horizontal incision on the
medial side [11]. This broke up the linear scar
and introduced some extra tissue in an attempt to
lengthen the lip. It also helped create a nostril
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U. Meyer
Fig. 5.4 Early drawing (1852) of the embryologic development of the face. [Image from page 244 of “Operative
surgery illustrated: containing more than nineteen hundred engravings: including two hundred original, and 50
colored drawings: with explanatory text” (1852) from
https://www.flickr.com/photos/internetarchivebookimages/14579138277/, Public Domain]
5 History ofCleft Treatment
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61
oor. More than a century later, Victor Veau
stated, “Mirault is the genius of cleft lip surgery”;
and indeed his contribution was the most important since Franco’s description of his two-step
procedure [12].
Johan Fredrick Dieffenbach, who was a specialist in urethral operations, had gained considerable experience in atraumatic techniques and
was equally emphatic on the subject [13]. In the
absence of safe and reliable general anesthesia,
surgeons were forced to acknowledge whatever
results were obtained as satisfactory but all that
changed soon after “Mirault’s operation” was
widely adopted [11]. It is interesting to note that
during this early times of scientically oriented
cleft surgery, Hendrik van Roonhuyze (1622–
1672) advised that cleft lips should be operated
upon between 3 and 4months of age [14]. In his
opinion, if they were operated earlier, the results
would not be favorable.
Surgeons since then considered different
approaches to close the lip cleft. Early pioneers
of cleft lip surgery were Hagedorn [15], who
used a quadrangular ap to increase the thickness
of the medial part of the lip, and Blair and Brown
[16], who attempted to correct the anterior nares
by shifting the tissues towards the middle line.
Veau [12] was impressed by this procedure, and
his method is very similar. Le Mesurier reintroduced Hagedorn’s method and published his
experience extending over a period of 13years in
1949 [17]. The modern era of cleft surgery is
often credited to C. W. Tennison in 1952 and
Peter Randall in 1959 [18, 19]. Later on, Ralph
Millard gave advancement to the surgical techniques [20]. He reported the “rotation advancement principle” using an exchange ap in the
region of the anterior nares. This method has also
been modied several times. The advantage of
the procedure is improved nasal form and better
anatomical joining of the musculature compared
with that achieved by the Tennison operation.
The modern surgery is based on all these basic
principles but has seen multiple renements of
technical approaches. Another original operative
method is the so-called wave-shaped incision of
Pfeifer [21]. It is said that this method joins the
musculature better than any other. However, only
a few surgeons outside Germany have used this
technique. Delaire improved the technique of
cleft lip and palate closure based on a more complex approach, including the facial muscles in the
surgical reconstruction approach [22]. The functional repair of cleft lip as described by Delaire
adheres to the principle of “embryological surgery” in which the situation that existed at birth is
recreated. There is no true hypoplasia of tissues
in cleft lip, and normal tissues exist on either side
of the cleft. Taking this into account, Delaire
described a technique which places importance
on lifting the entire functional matrix by subperiosteal dissection up to the nasal septum. Also, the
skin which appears to be short is only because it
is retracted due to lack of activity of the underlying muscle. Once correct apposition of muscle is
performed and activity is resumed, the skin distends and lengthens, thus avoiding the need for
skin-lengthening incisions which have the disadvantage of creating a longer lip on the cleft side.
Surgery oftheCleft Palate
The introduction of chloroform as an anesthetic
was of course of enormous importance. Cleft palate surgery thus became much easier, especially
in children. Renements in the basic principles of
repair and greater attention to the details of anatomy and function marked the beginning of modern cleft palate treatment. The surgical techniques
of modern palatoplasty address the concerns of
speech development and midfacial skeletal
growth. Today’s child anesthesia with oral intubation makes the closure of cleft lip and palate
deformities much easier and safer, so that singlestage closures of a complete cleft lip and palate
deformity can be done.
Historically, the rst detailed description of a
cleft palate was done by Franco in 1556 [10];
there were no reports of cleft palate operations in
older times. Detailed descriptions of the different
forms of palatal clefts were documented later on
(Fig.5.5). Even in the sixteenth and seventeenth
centuries, descriptions of cleft palate operations
are missing, probably because surgeons were
frightened of the copious bleeding and the severe
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U. Meyer
Fig. 5.5 Drawing of different types of unilateral and
bilateral clefts (1895). [Image from page 493 of “An
American text-book of the diseases of children” (1895)
Fig. 5.6 Early depiction (1910) of the cleft palate closure
approach, the technique according to Graefe [Image from
page 614 of “Transactions of the Southern Surgical and
pain caused by the operation [23]. Additionally,
syphilitic perforations of the palate were, however, frequently encountered in those times, so
that surgeons believed that cleft palates were the
result of syphilis. For this reason, operations for
cleft palates were not advised.
In 1766, Le Monnier [24] described a cleft
palate operation for the rst time. He used a
measure to freshen the margins of the gap in the
hope that healing will close the gap. Eustache is
said to have closed the soft palate as early as
1779, but this has not been proven [5]. In 1798,
Desault and Bichat [25] published an article
stressing the importance of approximation of the
margins of the cleft palate. The literature recognizes Graefe [26] and Roux [27] as the rst
authors of a proper cleft palate operation. Graefe
from https://www.ickr.com/photos/internetarchiveboo-
kimages/14595883917/, Public Domain]
Gynecological Association” (1910) from https://ickr.
com/photos/126377022@N07/14582263107, Public
Domain]
rst cauterized the margins and then sewed them
together. After failure to achieve healing, he
freshened the wound margins and xed the
stitches to the cheeks, so that they could not rip
out (Fig.5.6). Small palatal clefts were operated
by narrowing and suturing the cleft margins
(Fig.5.7), but the resulting tension was a signicant problem in wound healing. Improvements in
the clinical results were seen, when lateral incisions were introduced in the surgical technique.
In 1826, Dieffenbach [28], a famous surgeon in
Berlin, recommended the dissection of the
mucosa from the hard palate, combined with lateral incisions to relieve tension. This operation
was rst carried out in 1828. Velpeau [29] thought
that the rst to introduce lateral incisions to
relieve tension was Krimer of Aix La Chapelle in
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