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1 Milestones Contributing totheHistory ofCraniofacial Surgery

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performed in an oblique way starting from just above the lingula and reaching the buccal cortex 1cm more caudally without touching the intraalveolar nerve. The Schuchardt operation could be performed intraorally and led to a more sufcient medullary bone attachment. However, without xation of the proximal and distal segments, this procedure only led to a minor reduction of complications. Trauner and Obwegeser [31, 32] further developed Schuchardt’s technique by increasing the gap between the horizontal cuts to 25mm, requiring the surgeon to address the intra-alveolar nerve. Obwegeser revolutionized oral and maxillofacial surgery by introducing the BSSO as a standardized and safe procedure [33], which is performed worldwide to date in the originally described manner. Following the foundation of the “Arbeitsgemeinschaft für

Fig. 1.7 Tessier classication of complex orofacial clefts (Plastische erasmusmc, from https://commons.wikime-

dia.org/wiki/File:Picture_Tessier_classification.jpg,

licensed under CC BY-SA 3.0)

Osteosynthesefragen” (AO) at Biel, Switzerland, in 1958, the next revolution started affecting the BSSO technique. In 1976, Spiessl [34] published their book New Concepts in Maxillofacial Bone Surgery in which they introduced rigid internal xation in the form of interfragmentary bone screws.

such as orbital hypertelorism, correction of the facial deformity of Treacher Collins-Franceschetti syndrome, and correction of oro-ocular clefts.

While the history of craniofacial surgery can be traced back to earlier advancements in various medical branches, it was formally established and recognized as a distinct medical and surgical specialty in 1967. This milestone occurred as a

History ofCraniofacial Surgery

result of the collaboration among renowned international surgeons, with Paul Tessier at the fore-

The rst PubMed citation with the use of the term craniofacial was in 1876 by T. H. Huxley [35]. Advancements in correcting pediatric craniofacial abnormalities were pioneered by plastic surgeons such as Court Cutting, Ralph Millard, Paul Tessier, and Joseph McCarthy. Tessier was the rst to give a classication for complex orofacial clefts (Fig.1.7). As patients with inborn craniofacial malformations have to be treated in infancy, and structures have to be constructed, craniofacial surgery is closely connected to the term pediatric plastic or pediatric craniofacial surgery. During the late 1960s and the 1970s, Dr. Tessier [36] developed all of the procedures that are currently used in performing craniofacial surgery: fronto-orbital advancements (Fig.1.8), transcranial and subcranial correction of orbital dystopias

front. Norman Rowe and J. C. Mustardé from Britain, Joseph Converse from the United States, Hugo Obwegeser from Switzerland, and Zur Hausen from Germany were members of the group. Dr. Tessier’s work has impacted many surgical specialties, including plastic surgery, otorhinolaryngology, ophthalmology, neurosurgery, trauma surgery, and oral and maxillofacial surgery. Many of his techniques have found signicant places in the performance of plastic surgery, where Dr. Tessier’s methods of autogenous bone grafts have often been seen as an improvement over the traditional use of silicone or acrylic.

The distraction technique introduced and scientifically elaborated by the genius Russian orthopedic surgeon Ilizarov [37] (Fig. 1.9a) was used for the elongation of short limbs in

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Fig. 1.8 Principle of fronto-orbital advancement (Xxjamesxx, from Bone_segments_removed_in_fronto- supraorbital_ advancement.png, licensed under CC BY-SA 3.0)

U. Meyer

Fig. 1.9 (a) Dimitri Ilizarov (Prof. Dr. med. Bernd- Dietmar Partecke, Archiv des Berufsgenossenschaftlichen Unfallkrankenhauses Hamburg, licensed under CC

children (Fig.1.9b). This technique was later applied to the lower jaw with first arch deformity by McCarthy etal. [38] (Fig.1.10). The paper authored by John McCarthy was a groundbreaking contribution that revolution-

BY-SA 3.0). (b) Clinical example of limb distraction by external distractor. (Figure source: Viapastrengo at

English Wikipedia)

ized the treatment of patients with craniofacial malformations. It remains one of the most cited papers in the craniofacial literature to this day.

1 Milestones Contributing totheHistory ofCraniofacial Surgery

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Fig. 1.10 Mandibular distraction osteogenesis, rst performed and described by Joseph McCarthy. a) Clinical view of the patient during the distraction period. b)

Future Directions

In terms of modern pediatric craniofacial surgery, in addition to soft tissue reconstruction, plastic surgeons address all aspects of the bony skeleton including entering the cranial vault. While these lists are nowhere near exhaustive, they are presented to demonstrate the dynamic eld that is pediatric craniofacial plastic surgery and the incredible advancements in surgical technique that have evolved throughout history. Currently, high-resolution prenatal ultrasound technology, genetic aspects, distraction osteogenesis, microsurgery, tissue engineering, and computer-aided surgery by patient-specic implants (PSI implants) and custom-made bone reconstruction biomaterials, fabricated by CAD/CAM measures, will again rene and redene craniofacial pediatric surgery, and plastic surgeons will undoubtedly lead the way in traversing that path in multidisciplinary cooperation with pediatricians and neonatologists.

Schematic drawing of the distraction approach. (Figure source: McCarthy, JF Craniofacial Distraction, Springer

2017)

References

1. Graefe CF. Rhinoplastik oder die Kunst den Verlust der Nase organisch zu ersetzen. In: In ihren früheren Verhältnissen erforscht und durch neue Verfahrungsweisen zur höheren Volkommenheit gefördert. Berlin: Realschulbuchhandlung; 1818.

2. Graefe CF, Hecker JFC, editors. De rhinoplastice sive arte curtum nasum ad vivum restituendi commentatio qua prisca illius ratio iterum experimentis illustratur, novisque methodis ad majorem perfectionem perducitur. Berlin: Apud Reimerum; 1818.

3. Zeis E.Handbuch der plastischen Chirurgie. Berlin: Reimer; 1838.

4. Restak R. Fixing the brain. mysteries of the mind. Washington, DC: National Geographic Society; 2000.

5. Andrushko VA, Verano JW. Prehistoric trepanation in the Cuzco region of Peru: a view into an ancient Andean practice. Am J Phys Anthropol. 2008;137(1):4–13.

6. Norris S. Inca skull surgeons were “highly skilled,” study nds. National Geographic; 2008.

7. Tubbs RS, Riech S, Verma K, Chern J, Mortazavi M, Cohen-Gadol AA.China’s rst surgeon: Hua Tuo (c. 108–208 AD). Childs Nerv Syst. 2011;27(9):1357–60.

8. Al-Rodhan NR, Fox JL. Al-Zahrawi and Arabian neurosurgery, 936–1013 AD. Surg Neurol. 1986;26(1):92–5.

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U. Meyer

9. Aciduman A, Arda B, Ozaktürk FG, Telatar UF. What does Al-Qanun Fi Al-Tibb (the Canon of Medicine) say on head injuries? Neurosurg Rev. 2009;32(3):255–63.

10. LeFanu W. A primitive anatomy: Johann Peyligk’s “Compendiosa Declaratio”. Ann R Coll Surg Engl. 1962;31(2):115–9.

11. Vesalius 1543, pp.605–609.

12. Lannelongue M. Dela craniectomie dans la microcephalie. Compt Rend Séances Acad Sci. 1890;1890(50):1382–5.

13. Lane LC. Pioneer craniectomie for relief of mental retardation due to premature sutural closure and microcephalus. JAMA. 1892;1892(18):49–50.

14. Faber HK, Towne EB.Early craniectomie as a preventive measure in oxycephaly and allied conditions with special reference to prevent blindness. Am J Med Sci. 1927;1927(173):701–11.

15. Shiffman M. Cosmetic surgery: art and techniques. Berlin: Springer; 2012. p.20.

16. MSN Encarta. Plastic surgery. 2008.

17. Lock S, et al. The Oxford illustrated companion to medicine. NewYork: Oxford University Press; 2001.

18. Dwivedi G, Dwivedi S. History of medicine: Sushruta—the clinician–teacher par excellence. New Delhi: National Informatics Centre (Government of India); 2007.

19. McCarthy JG. Introduction to plastic surgery. Philadelphia: W.B.Saunders Company; 1990.

20. Maniglia AJ. Reconstructive rhinoplasty. Laryngoscope. 1989;99(8 Pt 1):865–7.

21. Rogers BO. Nasal reconstruction 150 years ago: aesthetic and other problems. Aesthet Plast Surg. 1981;5(1):283–327.

22. Gnudi MT, Webster JP. The life and times of GasparoTagliacozzi. New York: Herbert Reichner;

1950.

23. Tagliacozzi G.De Curtorumchirurgia per Insitionem. Venice: GaspareBindoni; 1597.

24. Dieffenbach JF. The operative surgery of J. F. Dieffenbach. Br Foreign Med Rev. 1846;21(42):285–333.

25. Chambers JA, Ray PD.Achieving growth and excellence in medicine: the case history of armed conict and modern reconstructive surgery. Ann Plast Surg. 2009;63(5):473–8.

26. Hullihen SP.Case of elongation of the underjaw and distortion of the face and neck, caused by a burn, successfully treated. Am J Dent Sci. 1849;9:157–61.

27. Blair V.Operations on the jaw-bone and face. Surg Gynecol Obstet. 1907;4:67–78.

28. Blair VP. Reconstructive surgery of the face. Surg Gynecol Obstet. 1921;33(261):9.

29. Kazanjian VH, Converse JM. Surgical treatment of facial injuries. 3rd ed. Williams and Wilkins Co: Baltimore; 1974.

30. Schuchardt K. Ein Beitrag zur chirurgischen Kieferorthopädie unter Berücksichtigung ihrer für die Behandlung angeborener und erworbener Kieferdeformitäten bei Soldaten. Dtsch Zahn Mund Kieferheil. 1942;9:73–89.

31. Trauner R, Obwegeser HL. Zur Operationstechnik bei der Progenia und anderen Unterkieferanomalien. Dtsch Zahn Mund Kieferheilkd. 1955;23:11–25.

32. Trauner R, Obwegeser H. The surgical correction of mandibular prognathism and retrognathia with consideration of genioplasty. I.Surgical procedures to correct mandibular prognathism and reshaping of the chin. Oral Surg Oral Med Oral Pathol. 1957;10:677–89.

33. Hågensli N, Stenvik A, Espeland L. Extraoral vertical subcondylar osteotomy with rigid xation for correction of mandibular prognathism. Comparison with bilateral sagittal split osteotomy and surgical technique. J Craniomaxillofac Surg. 2013;41:212–8.

34. Spiessl B. Rigid internal xation after sagittal split osteotomy of the ascending ramus. In: Spiessl B, editor. New concepts in maxillofacial bone surgery. Berlin: Springer; 2011.

35. Huxley TH.The nature of the craniofacial apparatus of Petromyzon. J Anat Physiol. 1876;10(Pt 2):412–29.

36. Tulasne FN.Paul Tessier (1917–2008). Rev Stomatol Chir Maxillofac. 2008;109(5):291–5.

37. Spiegelberg B, Parratt T, Dheerendra SK, Khan WS, Jennings R, Marsh DR. Ilizarov principles of deformity correction. Ann R Coll Surg Engl. 2010;92(2):101–5.

38. McCarthy SJ, Karp N, Thorne CH, Grayson BH.Lengthening of the human mandible by gradual distraction. Plast Reconstr Surg. 1992;89(1):1–8.

Part II

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Diseases: Craniosynostoses

History ofCraniosynostosis

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Treatment

UlrichMeyer

The history of modern craniosynostosis surgery can be understood through the evolution of cranial and maxillofacial surgery. Cranial surgery can be traced back to neolithic period, whereas maxillofacial surgery developed much later. Both surgeries developed into different surgical specialties: neurosurgery and maxillofacial/plastic surgery. The skull base as the separating structure between the cranium and the face was surgically touched by both disciplines. The wall separating the face and the cranium was broken by Paul Tessier and Gérard Guiot [1] in the 1960s, making it possible to perform a combined operation around the orbits and forehead and opening up close cooperation between maxillofacial/plastic surgeons and neurosurgeons, especially for the treatment of major craniofacial malformations. The distraction of craniofacial bone and the subsequent gain in soft tissue structures (introduced by Ilizarov for the limbs and applied to the face at the mandibular level by McCarthy [2]) have overcome one of the problems of skull shaping: the retraction of soft tissues with the risk of relapse of facial retrusion. Computer-aided planning and execution of craniosynostosis surgeries are now on the way to rene surgical results. The way

U. Meyer (*) Center for Jaw-, Face- and Skull Surgery, Münster, Germany e-mail: praxis@mkg-muenster.de,

meyer@kieferklinik-muenster.de

from the beginning of skull surgery to modern craniofacial procedures gives insight into milestone developments in this surgical specialty.

History ofCraniotomy andCranioplasty

Craniotomy inAncient Times

The evolution of craniotomy and cranioplasty can be traced back over thousands of years. Craniotomy procedures and also cranioplasty procedures were done out of cultural, religious, or medical reasons. The surgical procedure of a craniotomy has been practiced longer than any other and certainly that for which we have, by far, the oldest tangible evidence. The rst ndings related to perforation of the skull date back to the neolithic period (8000–5000 BC) and were found in France already in 1685 [3]. According to the most popular theory, the very rst cases of craniotomy were probably performed, by prehistoric man, for reasons related to magic or religious rituals (Fig.2.1), as an initiation practice, or as part of a ritual related to exorcism. As proof of the great religious importance attached to those who had been subjected to drilling, it is worthwhile recalling that, from some of these skulls, diskettes of bony tissue were removed postmortem (Fig. 2.2), which

https://doi.org/10.1007/978-3-031-28069-6_2

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Fig. 2.1 Illustration of neolithic trephination making a hole in the skull to let the devils out (Behind the doctor/ Logan Clendening from https://wellcomecollection.org/

images?query=ueceazt9, licensed under CC-BY-4.0)

U. Meyer

omy operations. The earliest cranioplasty operation is dated to 3000BC in the Inca civiliza-

tion, where precious metals, gourds, and shells

were found next to trepanned skulls in graveyards, suggesting that cranioplasty had been performed [6]. In the Paracas region of present-day

Peru, a skull from 2000BC with a thin plate of

gold covering a cranial defect was found [7]. Moreover, defective skulls were found covered with coconut shells or palm leaves in ancient tribes of the Polynesian Islands [8]. Sanan and Haines stated that the materials being used for cranioplasty were associated with the status of the patient [9]. Research and practice on trephi-

nation were documented in ancient Greece and Rome, but cranioplasty procedures are not

emphasized among early surgical authors in ancient Asia, Egypt, Greece, and Rome.

Evolution ofMedically Driven Craniotomies

Fig. 2.2 Human skull with sections removed (Illustrations

of the great operations of surgery, trepan, hernia, amputation, aneurism, and lithotomy/[Sir Charles Bell], from

https://wellcomecollection.org/works/nx7xdmf5/ images?id=xxd8rtdk, Public Domain)

were then worn as amulets around the neck (the so-called rondelles, described for the rst time, by Prunières, in 1783) [4, 5].

Cranioplasty inAncient Times

It is not denitively solved when and why cranioplasty operations were done instead of craniot-

Indications and surgical approaches changed during the centuries. Whereas early craniotomy procedures were based on cultural or religious reasons, opening of the skull was later used for treatment purposes: the nding that this procedure could, thanks to encephalic decompression, lead to an improvement in certain preexisting neurological symptoms probably led to it being employed in the presence of these symptoms and, in particular, in traumatic lesions. The techniques used for such surgeries evolved over time (Fig.2.3). Even the medicine men of neolithic times had an incredible technical ability in performing a craniotomy procedure despite the fact that they only had primitive tools [10, 11]. The most ancient technique of craniotomy consisted of thinning down the bony wall with abrasive instruments; later, circular incisions were progressively made deeper, or a series of small holes were made in a circle, after which the bony bridges between them were broken down. The Egyptian physician Imhotep performed craniotomy, presumably related to head injuries that would have resulted from the numerous battles fought by the ancient Egyptians. The Edwin Smith Surgical Papyrus,

2 History ofCraniosynostosis Treatment

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Fig. 2.3 Edinburgh skull, showing trepanning hole in the back of the skull (Edinburgh Skull, trepanning showing hole in back of skull from https://wellcomecollection.org/

works/hk22bxbp, licensed under CC-BY-4.0)

which provided a scientic approach to craniotomy, dates between the sixteenth and seventeenth centuries BCE but is believed to have been originally written by Imhotep around 2900BCE [12]. Much later were metallic instruments used, made of copper or bronze, such as gouges, curettes, scalpels, and knives of various forms, some of which very special, such as the “tumi,” or scalpel, in ancient Peru [13]. The ancient Greek civilization saw a further understanding of pathologies of the central nervous system. By the fth century BCE, Hippocrates codied guidelines on the use of craniotomy for the treatment of intracranial pathology [14]. When craniotomy was performed, the crown drill (“trupanon”) and perforating drill were employed, instruments that Hippocrates does not describe but only mentions, as if it was in common use at that time. Hippocrates advised performing the craniotomy without delay, in fact within the rst 3days of the trauma, in the case of severe contusions or of simple fractures, whereas in the case of the comminute type or with embedded fragments, he suggested that they be removed, paying particular attention to preserve the meninx.

During the Middle Ages, the Arabic surgeon Abul-Qasim Al Zahrawi, known in Western literature as Abulcasis, wrote extensively on early

depictions of neurosurgical diagnosis and treatments, including the treatment of head injuries, skull fractures, hydrocephalus, and subdural collections [15].

Following a long period of decline, surgery involving craniotomy began to be performed again on a vast scale during the Renaissance period due to the widespread use of rearms, which greatly increased the incidence of fractures and trauma involving the skull. Moreover, it should also be pointed out that in the second half of the seventeenth century, studies performed by Vieussens, Malpighi, and Willis led to a better understanding of the neurophysiological aspects and, in particular, stressed the importance of the cerebral cortex, which had not been clearly understood until that time, inasmuch as the humoral theory took into consideration only the ventricles as essential structures of the brain [16–

18]. From the end of the eighteenth century

onwards, the use of craniotomy gradually decreased, mainly on account of the increase in the incidence of complications due to infections. Infections in hospital surroundings and suppuration of wounds had become so frequent.

Cranioplasty inModern Times

The earliest modern description of cranioplasty was written by the surgeon Ibrahim bin Abdullah of the Ottoman Empire, in his surgical book

Alâim- i Cerrâhîn in 1505. The book mentioned

the use of xenografts from Kangal dogs or goats as materials for cranioplasty. Such materials were used due to the accessibility of these animals near battleelds, where the procedure is likely to be performed [19]. The rst true description of cranioplasty in Europe was made by Fallopius in the sixteenth century (Fig.2.4), stating that the fractured cranium should be removed and be reinserted with a gold plate if the dura was damaged. This was questioned by other practitioners at his time, concerning that surgeons may keep the gold instead of using it for surgery. The rst cranioplasty was reported by the Dutch surgeon Job

Janszoon van Meekeren. The report described the

use of a segment of a canine cranium as a mate-

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Fig. 2.4 Portrait of Gabriele Fallopius, 1523–1562 (Proli bio-bibliograci di medici e naturalisti celebri italiani dal sec. XVo al sec. XVIIIo/[P. Capparoni] from

https://wellcomecollection.org/images?query=v3f8dc3a,

licensed under CC-BY-4.0)

rial for cranioplasty on a nobleman in Moscow. The operation was successful; however, the use of canine bone in the operation was not accepted by the church and the man was forced to leave Russia [20]. Since the rst operation, bones from more animal species were used as xenografts for cranioplasty. These include dogs, apes, geese,

rabbits, calves, eagles, oxen, and buffalos. In

1917, William Wayne Babcock reported the use of “soup bone,” a piece of cooked and perforated animal bone as a xenograft [21, 22]. Autografts,

allografts, and synthetic materials are the main

types of materials used for cranioplasty.

During the second half of the nineteenth century, after the advent of antisepsis and general anesthesia, the use and technique of cranioplasties evolved (Fig.2.5). By the twentieth century, neurosurgery became an autonomous discipline, and the modern era began [23, 24]. At rst, neurosurgical approaches were performed with

U. Meyer

Fig. 2.5 Drawing of skull trephination in the operation theatre (Le chirurgie françoise recueillie des antiens médecins et chirurgiens. Avec plusieurs gures des instrumens necesseres pour l’opération manuelle/Par Iacques Guillemeau, from https://wellcomecollection.org/works/

x7j3tpvn/images?id=anyvs5a6, Public Domain)

extended craniotomies. The development of neurosurgical techniques and approaches was greatly aided by the evolution of advanced diagnostic imaging. The ability to combine high-denition computed tomography images (developed in the 1970s) with magnetic resonance imaging (MRI) (developed in the 1980s) has improved preprocedural planning.

Detection ofCraniosynostosis Biology

The aberrant congenital deformities of the skull have been known to exist for centuries and were well recognized and described as early as the time of antiquity. In the Iliad, Homer describes the warrior Thersites as “the ugliest man who came before Troy … his head ran up to a point …,” a description characteristic of oxycephaly [25]. The recognition of cranial vault deformities by the ancient physician Galen, and some early understanding of the role of cranial sutures by Hippocrates, has also been reported [26, 27].

By the sixteenth century, it appears that anatomists appreciated the existence of cranial sutures (Fig.2.6) and had documented a broad range of characteristics of the deformity, across an appre-

2 History ofCraniosynostosis Treatment

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Fig. 2.6 Sutures of a child’s head. (Sobotta’s Atlas and Text-book of Human Anatomy 1909)

ciation of suture pattern and premature suture fusion in a variety of congurations by Hundt [28], specic abnormal varieties of sagittal and coronal sutures by Dryander [29], and what would now be described as oxycephaly and brachycephaly by della Croce [30] and Vesalius [31]. However, von Sömmerring [32] in the late 1790s was the rst to go beyond simple descriptions and apply scientic principles to the study of abnormal cranial suture growth.

In 1851, Virchow [33] published a landmark paper in the history of craniosynostosis in which he described the fundamental aberrant growth patterns in this condition, which he termed Virchow’s law (Fig. 2.7). Virchow’s law stated that the observed deformities occurred as a result of “cessation of growth across a prematurely fused suture,” with “compensatory growth” along non-fused sutures in a direction parallel to the affected suture, causing obstruction of normal brain growth [34].

By the early 1900s, craniosynostosis was recognized as one component of complex syndromic deformities, most notably by Apert [35] in 1906 and Crouzon [36] in 1912, whose names bare two of the most well-known syndromic deformities. Moss stated that the active growth of the underlying brain dictated the passive cranial growth along the suture lines [37]. He termed this the “functional matrix theory,” and it would later form part of the justication for the minimally invasive approach early in life.

Fig. 2.7 Virchow, observing a skull operation at a Paris clinic (from https://wellcomecollection.org/works/pnc-

n9yjq, licensed under CC-BY-4.0)

History ofCraniosynostosis Surgery

Craniectomies andSuturectomies

Patients with craniosynostosis were not properly treated until the twentieth century, so that disgured persons lived their lives under special circumstances (Fig.2.8). The rst reported surgical interventions for craniosynostosis were strip craniectomies, rst by Lannelongue in Paris in 1890 [38] followed shortly by Lane in San Francisco in 1892 [39]. Lannelongue performed bilateral strip craniectomies for sagittal synostosis and strongly advocated for release, not resection, of the fused suture. Lane performed a strip craniectomy with

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