Journal of Cranio-Maxillo-Facial Surgery 42 (2014) 1023e1047

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Pioneer steps in correcting secondary cleft lip and palate deformities: My philosophy and procedures Hugo L. Obwegeser* Zürich, Switzerland

1. Introduction In September 2012 my nephew, Professor Joachim Obwegeser, organized an international meeting on the correction of secondary cleft deformities at my former Department of Cranio-maxillofacial Surgery at the University Hospital, Zürich. He had kindly invited me to deliver an introductionary paper on the subject as I have been the first to report new possibilities on that subject at an international meeting in 1969. As the audience was very enthusiastic about my lecture and had expressed the suggestion to publish the lecture in full length, I decided to do so. First of all I want to mention my intention to present what I have done for these poor cleft patients to make them free from their disfigurement. What I am going to demonstrate is old stuff, because of my high age (93 years). Nevertheless it was a very important invention to our specialty and I am reporting on it with some pride. At the “First International Conference on Cleft Lip and Palate” from 14e17 April 1969 at Houston/Texas, I had the opportunity to present for the first time to an international audience my philosophy and techniques in my paper “Surgical Correction of Deformities of the Jaws in Adult Cleft-Cases”. Plastic surgeons and orthodontists have been the major part of the audience. It was new for all of them. My presentation was rather revolutionary at those days and was accepted with astonishment and enthusiasm by the majority of the audience. After that presentation Dr. Samuel Perkowitz, the chief orthodontist at the famous Ralf Millard Cleft Centre in Miami, has asked me to record my philosophy and procedures in the correction of secondary cleft deformities, so that these facts should be stored in the national library and will not be forgotten. I have done this in the form of a DVD. As a prerequisite for the correction of secondary cleft deformities it is essential that the surgeon is familiar with all aspects of the repair of primary clefts. In addition the surgeon must also be very experienced in the field of orthognathic surgery. As a pupil of Richard Trauner I was well trained in all aspects of primary cleft work and I had already acquired all the experience needed in * Zürich, Switzerland. Tel.: þ41 44 825 32 93; fax: þ41 44 887 18 35.

orthognathic surgery, mainly due to my own development of the necessary surgical procedures. In this publication I will use the same cases which I presented at that meeting at Houston in 1969 and I will add a few special cases which I feel necessary for teaching purposes. Finally I will end my publication with some important conclusions which I have drawn from my extensive experience with this subject. First of all, I want to pay my high respect and gratitude to my teachers (Fig. 1). Without them I would not have become able to produce ideas for procedures to correct the often very severe facial disfigurement of cleft patients. I also want to thank my former coworkers and staff. They have also been a part of my activity. And my special gratitude I owe my nephew Joachim Obwegeser, as without

Fig. 1. My teachers: Prof. Hermann von Chiari, Chief of the Institute for Pathology and Microbiology of the University of Vienna, Austria, 1945. Prof. Richard Trauner, Chief of Dentistry and Maxillofacial Surgery. University of Graz, Austria, 1949. Prof. Eduard Schmid, Chief of the Dept. für Gesichtschirurgie, Marien hospital Stuttgart, Germany, 1952. Sir Harold Gillies, International Founder of Plastic and Reconstructive Surgery, Basingstoke, England, 1951. Mr. Norman Rowe, Chief Dept. of Oral Surgery, Basingstoke, England, 1951 and Paul Tessier, Chief Dept. Plastic Surgery, Military Hospital, Paris, 1954, have been good friends of mine. We exchanged knowledge and experience and learned from each other.

http://dx.doi.org/10.1016/j.jcms.2014.08.003 1010-5182/© 2014 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

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his help I could not transfer the illustrations into the computer for digital handling for a publication like this. 2. My professional background After my publication of the sagittal splitting technique I was frequently invited for lecturing in the USA. Many young colleagues asked me for my explanation that I have been able to produce several new ideas in our specialty. They expressed their opinion that I must have no time left for hobbies because of my professional engagement. I told them that without time for hobbies I would not be so productive. I would not imagine only working all day and every day without interruption. I told them that enjoy five hobbies. They depend of the seasons of the year and my age. In wintertime and spring I am skiing. I liked skiing in the beautiful mountains of western Austria that is my home area. In fall I enjoyed hunting for red dear and chamois. From spring till fall I was a enthusiastic river fisherman. And all year I was fond of antiquity exhibitions. My fifth hobby e I must have forgotten it e it was a hobby all young man enjoy. All I know in my profession I owe my teachers. No doubt, it is my professional background that let me produce new ideas. I always told them the more pre-specialization in nearby field a person has the more will he have his mind open for recognizing a real problem in his final specialty and also the solution for it. One of my many principle says: It is not difficult to find a solution to a problem, it is only difficult to identify the problem. My professional background is as follows: M.D. degree in April 1945 at the University of Innsbruck, Austria, at my age of 24 and a half. I had my training in general surgery each six months during my military services and after the war in a general hospital in my native town. Following this I had the privilege to train for two years with Professor Herman von Chiari at his Viennese Institute for Pathology and Microbiology. After that I trained six years in Dentistry and Maxillofacial Surgery with my main teacher Richard Trauner at the Maxillofacial Unite of the Dental School of the University of Graz. During these six years I passed my Dental Examine and spent each six months in the various sections of the dental school, a very valuable training in retrospect. After these six years I spent five months each in Plastic and Reconstructive Surgery with Sir Harold Gillies at Basingstoke, England and later with Eduard Schmid in Stuttgart. Norman Rowe and Paul Tessier were very good friends and colleagues of mine, and we learned from each other. All I know I owe my teachers.

Fig. 2. Unilateral short lip after primary lip closure according to the Veau technique. Reoperation by Ralf Millards technique has produced a symmetrical lip.

results. Both sides become equal in height as seen in the illustration of my case. 4.2. Fig. 3: Whistling deformity of the upper lip In bilateral cleft lip cases a whistling deformity of the upper lip may result. For the repair of a pronounced deformity I like to use a flap of the mucosa of the lower lip, in particular when simultaneously an ectropion of the inferior lip exists. The illustration presents a typical whistling defect of the upper lip and a slight ectropion of the lower lip and its repair by the insertion of a mucosal flap from the lower lip. I have learned this from Eduard Schmid.

3. What is the subject? In secondary cleft deformities we have to distinguish between deformities of the soft tissues including the nose and deformities of the facial skeleton. 4. Soft tissue deformities Soft tissue deformities may either involve the lips or the palatal covering or even the soft palate. Shortness of the upper lip is quite common in unilateral as well in bilateral cleft lip cases, often found as a result of the primary closure in the area of the former cleft. In bilateral cleft cases we often find a whistling deformity of the upper lip and an ectropion of the lower lip. In both cases of clefts, in unilateral as well as bilateral, we find typical deformities of the nose.

Fig. 3. Typical whistling defect deformity of the upper lip after primary closure of bilateral CLP. Good correction after insertion of a mucosal flap from the inferior protruding lip.

4.3. Deformities of the nose 4.1. Fig. 2: Shortness of the upper lip It is often observed in unilateral cleft cases after primary cleft lip closure by the Veau-technique Reoperation of the lip using the Millard procedure produces in my experience very satisfying

Every cleft lip and palate case is accompanied by a typical nasal deformity. The deformity of the nose in unilateral cases is much more difficult to correct than the correction of the nose in a bilateral case. In adult cases it is more easily done together with the Le Fort-I

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advancement than as a separate operation. When the maxilla is completely mobilized there is open access to the floor of the nose, the septum, the turbinates and the floor of the nose. A deviated septum and a deformed naso-palatine crest and also a hyperplastic turbinate are easily corrected. Also in this problem following Sir Harold Gillies principle “Replace into normal position what is normal and retain it there” brings perfect results. For the elongation of a too short a columella in a bilateral cleft case Ralf Millards fork flap procedure brought excellent results in my hands. I disclaim describing all details of nose correction here, as they do not differ very much from ordinary nasal corrections. 4.4. Fig. 4a,b: It is a case with a extremely wide primary cleft of the palate In a non-operated case the cleft in the hard palate can be that large that its closure may produce a real problem for not very experienced surgeons. Illustration Fig. 4a shows the widest cleft of the hard and soft palate I have ever seen in an adult, non-operated upon before. After measuring the width of the cleft and that of the two palatal artery flaps I repaired it according to the Veautechnique, additionally with simultaneous reconstruction of the missing hard palate by the insertion of decorticated flattened pieces of a split rip. Some of the stitches of the nasal layer are left long and pulled through the bony reconstruction and the united

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palatal artery flaps. Using these stitches, the nasal as well as the oral mucosal flabs are adapted to the inserted pieces of bone, thereby avoiding empty spaces and a haematoma. Fig 4b shows that even in such extremely wide clefts a perfect result can be achieved for the soft as well as the hard palate, when using perfect surgical techniques. 4.5. Fig. 5: Secondary defects of the hard palate Secondary defects of the hard palate include automatically its covering palatal mucosa and its part of the nasal layer. In reconstructing any defect of the hard palate it is a must to mobilize the nasal mucosa rather radically and also that of the nasal septum or even of the vomer. Safe results will definitely be achieved when not only the soft tissues are united but also the missing bone of the hard palate is reconstructed. Fig. 5 shows a large unilateral defect of the hard palate and the technique and the result of its repair. This awkward large defect was closed by mobilization and sewing up of the usual nasal layers. Some of the stitches are left long and pulled through burr holes of an inserted bone graft and a covering oral layer. That was build by rotation of the intact palatal artery flap from the other side over the defect plus a wide flap from the vestibular mucosa. The long left stitches from the nasal layer have been gently knotted for adaptation of the nasal and oral covering onto the bone graft for avoidance of an empty space and haematoma. In addition, a vaseline gauze, hold in place by a plate, covered the free part of the bone graft and of the hard palate till granulation tissues, growing out of the bones, created the necessary prerequisites for self-epithelialization.

Fig. 5. Disgusting unilateral defect of the hard palate. Three layer reconstruction created a good result.

4.6. Fig. 6aec: Nothing but wholes and scars on the hard palate

Fig. 4. a,b: The widest cleft palate I have ever seen in a non-operated palate situation in adult patient. Three layer closure by reconstructing the hard palate with decorticated pieces of split ribs. The stitches of the nasal layer were pulled through the pieces of bone and also the oral mucosal layer, thusly avoiding empty spaces and haematomas. b: Perfect result of hard and soft palate after reconstruction.

If such a situation is found then we know an insufficient surgeon has produced it by several attempts to close the palate. In such a situation tissues from a distance must be brought into the defect. In 1963 I used a tubed pedicle flap raised from the chest and then attached to the reopened lip. The skin flap was then attached to the pharyngeal wall and to the edges of the defect in the soft and the hard palate, as can be seen at the illustration of Fig. 6a on the bottom on the left side. In the next operation (Fig. 6b) the tube was defatted and a cancellous bone graft was inserted for the reconstruction of the missing hard palate and an additional one also for building the alveolar process. After some weeks the skin must be

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completely thinned or replaced by a skin graft. Now, the situation is ready for proper prosthodontic work as can be seen on the bottom of the right side of the illustration. Fig. 6c shows the reopened lip and the situation after it has been closed again, using Millard's technique. Normal function and normal appearance resulted.

5. Jaws only In most cases of secondary cleft deformities the deformity and retrodisplacement of the maxilla is the main problem. Before we were able to advance the maxilla the mandible only was retropositioned for the correction of a mandibular-maxillary discrepancy, mostly using my sagittal splitting technique (Fig. 7), sometimes achieving an acceptable result (Fig. 8aec), mostly some improvement only (Fig. 9aeb). However, the main problem is the maxilla, its retro-position, its small seize (micro- and retromaxillism) and the malposition of its segments. The patient shown in Fig. 8a has almost a circular nonocclusion, in addition an open bite and a missing second incisor. She also suffers from a prognathic appearance. According to the model planning, by retropositioning of the mandible an acceptable intermaxillary relationship should be achievable and also the outer appearance would improve (Fig. 8c). As at those days I was not able to reposition the maxilla anteriorly I decided to use my sagittal splitting procedure of the ramus for the best I could do. In addition the department of crown and bridge work would also be able to help to improve the occlusal picture (Fig. 8b). Movement of parts or of a whole jaw should never be executed without prior accurate model and splinting planning.

6. Mobilization of the maxilla Many have tried to mobilize the maxilla for the correction of its retroposition. Axhausen published in 1934, 1936, 1939 that he corrected the retro-displaced maxilla in post-traumatic as well as in cleft cases. However, his technique has never become popular. K. Schuchardt published in 1942 a war case in which he had pulled the maxilla forward by weight traction. He stated that that procedure would have a wide indication in corrective cleft surgery, but for that purpose it will probably never come into use, he wrote. J.M. Converse and H. Shapiro in 1952, and also I. Cupar in 1954 (Fig. 10) have suggested a circular vestibular incision for the osteotomy of the anterior surface of the maxilla plus, after raising the palatal flaps, a transpalatal osteotomy for its mobilization. In my opinion this will result with complete necrosis of the maxilla. Some visitors confirmed that I am right. H.D. Gillies tried hard to improve the cleft patients appearance by lateral rotation of the maxillary segments. Together with N.

Fig. 6. aec: a: Nothing but holes and scars in the hard palate. Reconstruction of the defect by insertion of a tube pedicle and bone grafts for the hard palate and the alveolus. b: Perfect result was achieved. C: The reopened lip was closed by the Ralf Millard technique.

Fig. 7. Drawing of my sagittal splitting procedure of the mandibular rami.

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Fig. 9. a,b. a: In lateral skull radiographs a severe dish face deformity after closure of an unilateral CLP in childhood and after repositioning of the mandible only. b: The result were much better when the maxilla had also been advanced.

Fig. 8. aec: a: A typical open bite situation in a 19 year old female after primary closure of a unilateral CLP. b: Occlusal situation after mandibular set back and closure of the open bite by the sagittal splitting procedure of the mandible and some crown and bridge work. c: Patients appearance before and after correction: It is an acceptable improvement although the flat face is not corrected.

Rowe he published in 1954 what he had achieved. During my training with him in 1951e52 I had the privilege to assist him several times. He reopened the cleft and rotated both halves laterally and fixed them with a cap splint in the planned position to find occlusion with the mandibular teeth. However, he never advanced the segments anteriorly. But he did the first important step for the final success for the Le Fort I e mobilization, by placing cancellous bone grafts onto the steps in the canine fossae. In spite the fact that he could not cover the graft completely with mucosa on its side to the maxillary sinus and nasal cavity, the bone grafts healed in perfectly. This fact was the key for me to successful advancing the maxilla in cleft cases up to 20 mm and also more. Another important step was the separation of the maxilla from the pterygoid processes and filling the gap between the tuberosity and the pterygoid process with bone grafts after the maxilla has been advanced. That was suggested by myself (Obwegeser 1962, 1964, 1965 and in 2001). Fig. 11 shows that I secured the necessary blood supply by undermining the vestibular mucosa and performing the osteotomy via three vertical incisions. Many authors reported some or total relapse after maxillary advancement. For achieving stable results the maxilla must be that loose that it can be overcorrected with a pair of tweezers only and that its stability is guaranteed by filling the gaps of bone with bone

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Fig. 10. In drawings the ConverseeCupar procedure for advancing the maxilla. I wonder from where the blood supply will be secured. I have never tried it, however, some visitors have informed me that they experienced necrosis.

grafts. If that is carried out properly, then the maxilla has fewer tendencies to a relapse than the mandible has after its repositioning with the sagittal splitting technique. R. Drommer has reported in 1979 that the maxilla will even survive after a circular vestibular incision and ligation of the palatine artery when the palatal covering has not been mutilated. He also published an excellent paper in 1986 on the history of the Le Fort I e osteotomy.

6.1. Fig. 12a,b: It is a case of inferior position and rotation of the premaxilla and slight collapse of both lateral segments Illustration Fig. 12a shows the occlusal situation in this case of inferior position and rotation of the premaxilla and slight collapse of both lateral alveolar segments with bilateral palatal fistulas in a bilateral CLP-case before surgery (operated upon in 1961e62). In a first step the cleft palate was reopened through the fistulas and the lateral segments were osteotomized via a vestibular approach and rotated laterally. The segments were then repositioned as planned on the model operation and stabilized with a wire splint. In a second step after three months the malpositioned premaxilla was osteotomized from its palatal aspect at its bony strut via an incision on its palatal covering muco-periosteum. The reopened cleft was closed and the three segments were stabilized by bone grafts which were covered with nasal and oral mucosal flaps. Fig. 12b shows the very acceptable occlusion that has been achieved and the bone grafts in the alveolar clefts. In Fig. 12c the pre- and post-operative occlusion is shown and also the prosthodontic reconstruction. 6.2. Fig. 13a,b: It is a case with collapse of both segments in an unilateral CLP-case

Fig. 11. Drawing of H Obwegeser's technique to mobilize the maxilla through vertical incisions in the undermined vestibular mucosa (1962).

The photo demonstrates the collapse of both, the small and the large segment, in an unilateral CLP-case (operated upon in 1965e66) and its correction according to the model-operation and the result of our efforts. This case is a typical sample of a very deformed maxilla in an unilateral cleft lip and palate case. Illustration Fig. 13b demonstrates the situation of the bone grafts with the perforating stitches of the closed nasal layer. The alveolar process required a separate piece of bone. The picture on the bottom of the right side shows the final result of the formerly collapsed

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Fig. 12. a,b: A bilateral CLP deformity is corrected via the undermined vestibular mucosa. a): In a first intervention the two lateral segments are repositioned. Three months later the premaxilla was repositioned and the palatal cleft was closed in three layers. Simultaneously bone grafts secured the position of all three segments. b) The result was very pleasing.

maxilla. It is now a normal upper arch with a good condition for final prosthodontic work. That was achieved by a removable bridge reconstruction with the Dolder bar system, performed by the Prosthodontic Department of our Dental School.

6.3. Fig. 14aec: A collapse of all three segments in a bilateral CLPcase Fig. 14a shows the collapse of all three segments in a bilateral cleft and the steps in the reconstruction (operated upon in 1966e67). This situation is very typical for a bilateral cleft jaw deformity, as seen on the upper left picture. The model operation shows that by repositioning of all three segments with simultaneous reopening of the cleft good prerequisites for the final prosthodontic work can be achieved. In a first operation reopening of the cleft with lateral rotation of both lateral segments was done as seen on the bottom on the left side of this illustration. The next picture (Fig. 14b) demonstrates by drawings the second operation: osteotomy and repositioning of the premaxilla and three layer closing with bone grafting. It also demonstrates the radiographic situation before, during and after the reconstruction and Fig. 14c shows the final palatal and occlusal situation. The prosthodontic

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Fig. 13. a,b: A typical unilateral CLP- deformity with severe collapse of both segments. a): The model operation informed us what we can achieve and the drawings tell how we close the defect. b): The pictures taken during the operation show the bone grafts for the reconstruction of the hard palate and the alveolus and also the final result.

work was done by the department for crown and bridge work at our Dental school. 7. H. Pichler's law It says: “In facial reconstructive surgery first the bone, then the soft tissues”. This principle requests that the maxilla and the mandible must be completely free for repositioning the three segments wherever wanted. A posttraumatic case caused me to find the solution for that problem. That posttraumatic case (Fig. 15a,b) caused me to use a circular vestibular incision for cutting the lateral aspects of the maxilla. In that case the trauma has split the maxilla in two halves. They have been telescoped into the maxillary sinuses, with a medial palatal split and loss of three front teeth (see Fig. 15a on the left side of the top). There was a scar running around almost the whole vestibulum, as shown on the right side of the top. That included several oro-nasal and oro-sinus fistulas. I had no other chance but to cut carefully in the vestibular scar, step by step checking the circulation on the palatal mucosa. As the palatal mucosa proofed to remain well vascularized, the circular vestibular incision became to me routine for cutting the anterior maxillary wall. When I trained with Sir Harold Gillies and Mr. Norman Rowe then I had seen that they

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cut the compressed maxillary segments through a vestibular incision and rotated them laterally. But they did not reposition them anteriorly. Fig. 15a: It shows on the top on the left side the preoperative severe open bite, but no occlusion, on the bottom the model operation. It tells that the maxilla has to come forward for 9 mm and in the front region downwards for 15 mm. In the same illustration the mobilized maxilla is seen. Fig. 15b: shows the occlusion, before and after prosthodontic replacement of the missing three incisor teeth. In the radiographs the skeletal situation, before and after replacement of the segments is seen. This case taught me that a circular incision in the vestibulum is not doing any harm to the blood supply of the maxilla. Because of this experience resulted my final procedure for the mobilization of the maxilla (Fig 16). 7.1. Fig. 17aed: A unilateral CLP-case with severe retromaxillism and collapse of both alveolar segments This was the first CLP-case in which I could reposition into normal what was still normal (one of Sir Harold Gillies main principles). It was the first case of severe retromaxillism in an unilateral CLP-case, that gained from the fact that I now could mobilize the whole maxilla and reposition the segments into the planned new position (operated upon in 1968): Fig. 17a shows the patients profile and his lateral cephalograms and Fig. 17b the typical class III occlusion of such cases. Both, the patient's profile appearance as well as its skeletal background are very typical for the consequences of radical primary surgery of a unilateral cleft lip and palate situation. The model planning (Fig. 17b) suggested reopening of the cleft and advancing the maxillary segments and narrowing the two sections of the maxilla. Because of the large amount of advancement of the two segments of the maxilla (up to 18 mm) bone grafting at the steps in the canine fossae and between the tuberosities and the pterygoid processes became necessary as shown in the drawing of the planned surgery. In Fig. 17c the lateral pre- and post-operative cephalograms demonstrate the result of the advancement of the maxilla. The large bone block behind the tuberosity (20 mm) is easily recognizable on the right side cephalograms. The new position of the maxillary segments permitted a satisfying prosthodontic restoration as can be seen on the occlusal photograph. The next illustration (Fig. 17d) presents the aesthetic improvement of the patients profile view through this corrective surgery, performed on the maxilla only. There was no other additional nasal correction done. The nasal hump was automatically corrected by the advancement of the maxilla. 7.2. Fig. 18aeg demonstrates the circular nonocclusion and severe retromaxillism and ectropion of the lower lip in a case of unilateral CLP

Fig. 14. aec: A case of a bilateral CLP with very severe deformity of the palate. a): All three segments are compressed. The plan of treatment the same again: in a first intervention the lateral segments are repositioned. b): In a second operation the premaxilla is repositioned and the palate is closed in three layers as shown in the drawings. Bone grafts stabilize the segments in their new position. c) In spite the severe deformity the final result after additional bridge work is very satisfying.

This case shows a typical occlusal and profile appearance of a bilateral cleft lip and unilateral alveolus and palate with severe retro- and micro-maxillism with circular nonocclusion and ectropion of the lower lip. The patient had orthodontic treatment from babyhood till adolescence, when the orthodontist was not any longer able to prevent the mandible to overgrow the maxilla. It is a interesting case of complex planning and treatment problems (operated upon in 1970). Fig. 18a shows the profile view of the 17 years old girl and the skeletal cause of it while the illustration Fig. 18b shows the circular nonocclusion and the model planning for its correction. According to profile planning and model operation the maxilla must be moved anteriorly, in two segments for occlusal reasons, and the anterior

Fig. 15. a,b: A posttraumatic case was referred to me almost two months after the accident: a)The maxilla was split in two halves, compressed and telescoped into the nasal and sinus cavities. Three incisor teeth have been lost. In the vestibulum a circular scar was running all the way around. There were fistulas into the nose as well as into the maxillary sinuses. Because of that situation undermining the vestibular mucosa was impossible. I had to cut in the scar all he way around, but step by step carefully checking the blood supply of the palatal mucosa. The result was my first circular incision for proper access to the maxilla. It was the all decisive case for the final Le Fort I osteotomy for mobilisation of the maxilla. b): The result was perfect after dental bridge work.

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Fig. 16. This drawing shows my final Le Fort I-osteotomy. The maxilla is completely detached from the surrounding skeleton. That includes separating the tuberosity from the pterygoid processes. All defects of bone must be filled with bone grafts.

alveolar segment of the mandible must be repositioned after extraction of the first bicuspid on each side, for correction of the ectropion of the lower lip. In illustration Fig. 18c the planned operation is shown in drawings. Bone grafts will have to be placed at the steps in the canine fossae as well as between the tuberosities and the pterygoid processes. At the beginning of our maxillary advancement we did this almost routinely. For small steps we used deep frozen bank bone. For steps from 10 mm and more we used autologous bone grafts, as we had experienced pseudarthrosis of the maxilla because of non-union when we had used bank bone or none. With this operation a very acceptable intermaxillary relationship was achieved as seen on the right side of Fig. 18c. The next Figure (Fig. 18d) shows that an almost normal alveolar arch has been created, a good prerequisite for the orthodontist (Prof. Paul € ckli) to create a nice occlusion and appearance in an unilateral Sto CLP-case. Fig. 18e shows the occlusion before and after surgery and after orthodontic treatment. Without touching the nose or the lower lip, just by moving the skeletal framework into normal position, a very good profile was achieved as seen on the cephalometric radiographs and on the photographs taken before and eighteen months after surgery (Fig. 18f,g).

Fig. 17. aed: A case of typical facial deformation after repair of a unilateral CLP. a): There is pronounced retromaxillism and a typical humpy nose. b): The occlusion is also very typical. The model operation tells that the maxilla must be advanced in two pieces. Bone grafting is unavoidable. c): The lateral radiographs show the difference before and after and also the huge bone block in the space between tuber maxillae and pterygoid process. After replacement of the missing incisor the occlusion looks perfect. d): The postoperative profile of the patient shows that also the nasal hump was corrected without touching the nose at all.

Fig. 18. aeg: A 17 year old female was referred to me as the orthodontist who had taken care of her since babyhood, could not prevent the mandible of forward growth. a): I found a severe retromaxillism and a slight humpy nose. The lateral cephalograms demonstrates the skeletal cause. b): There is circular nonocclusion and the model operation tells that the maxilla must come forward in two pieces for 5 mm. Simultaneously the inferior mandibular alveolus must be retropositioned, the best procedure to correct the ectropion of the lower lip. c): The drawings show what we will have to do and the occlusal picture demonstrates the result. d, e): These two pictures show the maxillary arch before and after surgery and after final orthodontic .treatment. f, g): Show in cephalograms and in profile the change through this surgery.

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Fig. 19a,b: It is a case of a unilateral CLP with a gap in the maxillary dental arch because of a missing second incisor and a moderate retromaxillism. The plan was to correct the slight retromaxillism and to close the gap of the missing incisor by advancing the two alveolar segments independently and simultaneously closing the gap of the missing second incisor, another step forward in the correction of CLP-deformity. (I do not know why it took me that long to use that chance). This case demonstrates another important step forward in achieving normal occlusion and appearance by advancing and narrowing the maxillary segments in an unilateral cleft case (operated upon in 1976). Illustration Fig. 19a shows the presurgical occlusion and the model operation for evaluating the necessary skeletal surgery and in the drawing the surgical details of the maxillary procedure is shown. Whenever possible the advancement of the two cleft segments should be planned so that the gap created by a missing tooth can be closed. A remaining palatal cleft fistula can then simultaneously be closed without difficulty. The final occlusion should be equal as planned on the model operation as seen in Fig. 19a on the right side of the bottom.

If necessary, as it was in this case for the chin prominence, some additional profile improvement has to be planned and performed at the same operation. The final profile views (Fig. 19b) demonstrate the profile improvement after that single surgical intervention. 8. Jaws plus lip and nose 8.1. Fig. 20aee: A bilateral cleft case presents a complex situation with some soft tissue and maxillary but mainly mandibular deformities (operated upon in 1966) The profile view of the patient (Fig. 20a) demonstrates a very massive chin prominence and a vertical elongation of the inferior third of the face. The cephalometric radiograph shows the main cause of this rather severe deformity: It is the retropositon of the premaxilla and the rather very large mandible. Fig. 20b shows the patients terrible occlusal situation. This horizontal as well as vertical occlusal discrepancy required, according to the model operation, a forward positioning of the premaxilla and a remarkable reduction of the seize of the mandible by removing an inferior bicuspid on each side, followed by a retro-positioning of the anterior alveolar segment and simultaneously a repositioning of the whole mandible by the sagittal splitting procedure. All this was performed in one operation. Thereby an acceptable mandibularemaxillary relationship could be achieved, which permitted the patients dentist to supply the patient with a fixed bridgework after the fistula in the left alveolar cleft region was also closed. There existed also a shortness of the upper lip as often seen in bilateral cleft lip cases. The illustrations Fig. 20cee show that by an additional reoperation of the bilateral lip situation finally a very acceptable functional and aesthetic situation resulted. 8.2. Fig. 21aed: A bilateral CLP-case with circular non-occlusion and dish face deformity and a dropping nose due to severe microretromaxillism

Fig. 19. a, b: This case demonstrates an other important pioneer step in the correction of secondary cleft deformities. It is a unilateral CLP-case with slight retromaxillism only, but missing the second incisor on the cleft side. a) This photograph shows the preoperative occlusion. The model operation indicates: the maxilla has to be advanced in two sections, simultaneously closing the gap of the missing incisor and for profile improvement advancing the prominence of the chin. b) That ends with an excellent result.

This case shows the situation of a bilateral cleft with extremely collapsed maxilla and protruding mandible and with dropping tip of nose (operated upon in 1962e63), before I was able to advance the maxilla. There was a severe dish face deformity due to retro- and micromaxillism and there was circular nonocclusion as seen on the top right picture of Fig. 21a. According to the model planning the maxilla had mainly to be widened. But the premaxilla had to be rotated anteriorly and the mandible had to be retropositioned for 10 mm. The operation at the maxilla was carried out accordingly, with reopening of the cleft and the mandible was repositioned by the use of the sagittal splitting procedure. In a first intervention the lateral maxillary segments were rotated laterally with simultaneous reopening of the cleft. A splint and bone grafts in the canine fossae secured them in the new position. In a second operation the premaxilla was repositioned and the reopened palate was closed with bone grafts interpositioning for stabilization of all three segments. In an additional intervention the mandible was osteotomized by the sagittal splitting and fixed in the planned position. Fig. 21b shows on the right side that the first three steps of corrective surgery achieved a good intermaxillary relationship. That is also seen in the cephalometric pictures. Its facial contour proofed the planning. The same illustration (Fig. 21b) shows the change in the occlusion, of course, with prosthodontic work and it shows in the lateral cephalometric radiographs the wanted change of the facial skeleton. The following illustration (Fig. 21c) shows on the left side the patients appearance after the maxillo-mandibular discrepancy had

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Fig. 20. aee: A bilateral CLP-case presents a maxillary anomaly, but mainly an anomaly of the mandible. The outer appearance is difficult to diagnose. The lateral cephalograms suggests rather a pronounced micro-retromaxillism, but the evaluation of the case shows that the main cause for this patients anomaly is the macro- and ante-mandibulism. This becomes obvious when the occlusion is inspected and the model operation tells that only the premaxilla has to be repositioned while the mandible has to be reduced in size very remarkably by simultaneously repositioning the whole mandible and also its anterior alveolar segment after extraction of the two first bicuspids. After that skeletal surgery the lip needs to be reconstructed. d, e) That ended in a good frontal appearance as well as a good profile.

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Fig. 21. aed: This case is a typical facial deformity after a bilateral CLP. a): The patient presents a severe dish face deformity with a dropping nose and a circular nonocclusion. I had to correct that deformity in 1962e63, before I was able to reposition the maxilla. The model operation showed that an acceptable intermaxillary relation can be gained by rearranging the upper arch and retro positioning the mandible for 10 mm. In a first intervention I rotated the lateral segments into proper occlusion with the mandibular teeth. In a second intervention I repositioned the premaxilla and closed the cleft palate in the typical three layer way, that means that bone grafts secured the three segments in their position. b): Finally the mandible was repositioned by the sagittal splitting technique of the rami as planned on the models. c): After skeletal surgery the bilateral cleft of the lip became obvious. The reoperation of the bilateral lip gave me the chance to reconstruct the missing columella by the use of Ralf Millards fork flap technique a) The result was quite acceptable although the maxilla had not been advanced.

been corrected through corrective surgery on both jaws. Now the lip was reoperated on and simultaneously the missing columella was reconstructed by Millard's fork flap procedure and also the nasal deformity was corrected, altogether ending up with a functionally and aesthetically pleasing result, as can be seen on the right side of Fig. 21c and on the Fig. 21d with the patient's pre-and post surgical profile views. This case was operated upon before I had experience in simultaneously repositioning the whole maxilla and the mandible. 8.3. Fig. 22aee: This case is a situation of unilateral cleft lip and palate with severe retro- and micro-maxillism plus some degree of asymmetric antemandibulism (operated upon in 1969) In unilateral as well as in bilateral cleft cases it can become necessary not only to reposition the collapsed alveolar segments of the maxilla and bring them forward but also to reposition the mandible by the sagittal splitting procedure or parts of it in order to achieve good skeletal relationship. After proper model planning

and preparation of splints this can often be executed in one operation. This was my first case of simultaneous repositioning of the whole maxilla as well as the whole mandible. Fig. 22a shows the patients preoperative profile appearance and its skeletal background in the lateral cephalograms. Illustration Fig. 22b shows on the models the preoperative occlusion and the model operation proofs that by advancing the maxilla in two segments and retropositioning and slight rotating the mandible a good intermaxillary relationship could be achieved. The model planning (on the right side of Fig. 22b) showed that, according to the profile planning, both maxillary segments had to be advanced and narrowed independently and the mandible had to be repositioned anteriorly and shifted for 3 mm to the left side. Drawings of the planned operation (Fig. 22c) show in details what the model operation suggested according to the profile planning. Bone grafts were planned to be placed onto the steps in the canine fossae in order to cope with the rather flat infraorbital areas. In a second stage the shortness of the upper lip had to be corrected as well as the nasal deformity. As seen on illustration Fig. 22d a good profile has been

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Fig. 22. aee. An other typical secondary unilateral CLP- deformity in a young adult. a): Severe dish face deformity and a protruding mandible seem the main cause for this deformity. b): The model operation suggests that a good intermaxillary relation can be achieved by advancing the maxilla in two sections and simultaneously repositioning the mandible by a sagittal split procedure. c): The drawings tell clearly what the surgery has to do. d): The two cephalograms show clearly the skeletal back ground of the anomaly before and after the correction. e): After the nasal deformity was also corrected the final result was very satisfying.

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Fig. 23. aeg: This 21 year old female with her facial deformity after an unilateral CLP presented a typical anomaly of the maxilla but also a severe anomaly of the mandible. a): The dish face and the dropping nose cannot be overlooked. But there is also a asymmetry of the mandible and a certain amount of antemandibulism. The cephalograms proof the diagnosis. b): The orthopantomogram of the mandible shows the classical anomaly of hemimandiular elongation as first published by Obwegeser and Makek (1986). The model planning demonstrates the necessity for advancing the maxilla in two sections and repositioning and rotation of the mandible by the sagittal splitting technique. c): The drawings show what the model planning suggests and the occlusal picture show the situation before and after the surgery. d): After the skeletal correction has been performed the soft tissues are waiting to be improved. Lip and nasal correction are done simultaneously. f) The nose has become symmetric and so also looks the skeleton. g, h): The comparison of the preoperative situationwith the final result proofs my goal in the correction of secondary cleft deformities: The cleft patient has a right to have an outer appearance as normal as any other person.

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Fig. 24. aei: A case of unilateral CLP with a complex facial anomaly after orthodontic treatment for several years. a): This young grown up lady suffers from hypoplasia of the facial halve of the cleft side plus an ectropion of the lower lip. b): She has a good upper dental arch with a missing second incisor. In addition she has a palatal fistula behind the incisors. Such a hypoplasia of the cleft side of the face is not rare, as can be seen on the skull of an other case. c): The profile planning intends to produce a slight ante profile. d): The model operation suggests advancing the maxilla as well as the mandible, but primarily reposition the inferior alveolar front segment for permitting to advance the mandible as much as necessary (13 mm), together with the maxilla in two segments. That permits the closure of the gap of the missing second incisor. The drawings illustrate the planned operation. e): After finishing the orthodontic treatment the planned surgery created a perfect occlusal result. f): There was still the need to correct the hypoplasia of the cleft side. I did it transorally with some lyocartilage onlays. The patient wanted to have the nasal correction done by her plastic surgeon in the States, although it could have been executed at the same operation. g): The lateral cephalograms demonstrate the final skeletal result. h, i): The final profile and front views of the patient show the result of surgery. We have not achieved a slight ante profile face as we had intended, but still a good result.

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Fig. 24. (continued).

achieved through these two operations. There is a clear increase in vertical dimension as well as a forward shift in the infraorbital region recognisable. The last photograph (Fig. 22e) shows the patients frontal views of this case before and after these two operations. This was my first case of simultaneous repositioning of both jaws, an other important step in the development of corrective surgery of secondary cleft deformities. Since then it has become routine practice all over the world. 8.4. Fig. 23aeg: It is a case of simultaneous existence of a typical maxillary and also a typical mandibular anomaly in an unilateral CLP-case The maxillary anomaly is the result of a unilateral cleft lip and palate with severe micro- and retro-maxillism and the asymmetric antemandibulism is the result of a unilateral hemi-mandibular elongation. The front view proofs the cause of the asymmetry (Fig. 23a): The mandible is clearly too far anteriorly and shifted over to the left side while the anomaly of the middle face has its

cause in a asymmetric hypoplasia of the middle third of the face, in addition with the usual dropping nose, typical for a unilateral cleft lip and palate situation. The lateral cephalograms, on the right side of the illustration shows the skeletal background of the anomaly. In the orthopantomogram (Fig. 23b) the typical picture of a hemi-mandibular elongation on the right side of the mandible is seen and in the maxilla on the left side the cleft lip and palate deformity. The model operation proofs clearly that the maxillary halves have to be advanced unequally and the mandible must be shifted over to the right side for 6 mm. In illustration Fig. 23c the plan of surgery is shown by the drawings: the mandible will need a unilateral sagittal splitting of the right ramus only and the maxilla requires a sectioning in the cleft region and an unequal advancement of the two halves and medial rotation of both sections. With that amount of surgery on the skeleton a good intermaxillary relationship should result as seen on the right side of the Illustration Fig. 23c. Fig. 23d shows that after the skeletal rearrangement the lip and nose can be corrected in order to finish the surgical correction. The final result is seen on illustrations

Fig. 25. aeg: a): This 20 year old patient presents a severe micro- and retro-maxillism after the repair of an unilateral CLP in childhood. b): The teeth did not occlude. The goal was to create an acceptable profile and a good intermaxillary relation for the dentist to supply the patient with a good functioning dental appliance. The model operation suggested a Le Fort III þ I osteotomy for the necessary advancement of the face and also for some vertical increase of it. c): The drawings explain the surgery. That includes quite some bone grafting for bridging the bony defects. d): These pictures show the achieved intermaxillary relation and the final dental bridge work. e): The lateral cephalograms demonstrate the skeletal situation before and after surgery. f, g): After additional correction of the lip deformity and also of the nose the patient received an acceptable outer appearance that permitted him to lead a normal life.

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Fig. 26. aer). This last case became the case of my life. He was referred to me at his age of ten. a): He was born with two complete noses and a wide medial facial cleft, two rudimentary eyes and two complete premaxillae with four incisor teeth each and an exorbitant hypertelorism. A plastic surgeon has tried to close the medial cleft by suturing the two medial alae together. In aadirion he inserted an Abbe' flap for the same purpose. He was a charming boy and wanted badly the correction. He was 10 years and two months old when he was referred to me. b): The few of the maxilla showed the surplus of teeth with the medial cleft and the oblique few of the skull impressed by the wide lateral position of the eye sockets. c): I made my plan on the basis of Tessiers' hypertelerism operation without knowing how things are behind the face. There were no radio-tomograms nor MRI available at those days (1969). I planned to excise everything between the two nasal bridges. When the skeleton and the base of the skull will be freed I will have to adapt my surgery according to the local situation. The neurosurgeon was as much surprised as I was when he found two cristae Galli with a lamina cribrosa on each side after he had raised the brain from the bottom of the anterior cranial fossa. That finding made it clear to me how much I will have to excise from the anterior cranial fossa. d): The photographs taken during the surgery show clearly the two nasal frameworks and also the rudimentary orbital coni of the two rudimentary eyes, that had been removed in early childhood. They also show the gap between the two facial halves after the excision of the surplus of the anterior cranial fossa and the new nasal bony framework after the two sides were rotated

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Fig. 26. (continued). together. e): We had achieved a nice result, but the neurosurgeon had to remove the large cranial flap after a week because of heavy purulent infection. The children's hospital got him free of the infection by antibiotics. The patient could leaf for home with a helmet. Ten months later he returned with a severe facial deformity. The mandible had grown forward, but not the maxilla. The model planning showed that I will have to advance the maxilla and reposition the inferior alveolar segment to correct this unpleasant deformity. f): An acceptable appearance resulted. g): 13 months after the removal of the infected cranial bone flap the neurosurgeon reimplanted that piece of bone after its autoclaving. However, an infection again forced him to remove it. Ten months later I reconstructed the cranial defect by implantation of 14 halve decorticated pieces of ribs. h): With that new brain cover he left for home again. Two and a half year later we experienced again that the mandible had grown forward, but not the maxilla. I decided to wait till growth is finished. After that I should be able to make him a normal face that will not alter again. I): At his age of 18 and a halve he arrived with a most grotesque facial deformity, as can be seen in this illustration. j): The drawings of this illustration make it clear that I planned to advance the maxilla plus the infraorbital and paranasal areas and to reposition the anterior alveolar process of the mandible plus the whole mandible for 11 mm. k): A good facial skeleton resulted. Only the nasal cartilage and the columella have been missing. l): For the reconstruction of the

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Fig. 26. (continued).

columella I used a caterpillar flap from the nose and for the missing cartilage a L shaped piece of cartilage form the ribs. m): The final result was very convincing. It remained like that for more than 25 years when I saw him last. n): The final dental arch of the maxilla was o.k., in spite the missing incisors. o): The lateral cephalometrics before we started the correction at his age of ten and at the age of 22 demonstrate that it was worth while to go through all this efforts. p): He had finally a perfect nose and good nasal breathing and his speaking was good enough for his native town to employ him in the cities telephone business. q): I visited him in his town 25 years after I had finished his surgery and found a pleasant young man as happy as the surgeon himself.

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Fig. 23eeg. These photographs show the very pleasing result of that surgery. 8.5. Fig. 24aei: It is a case of unilateral cleft lip and palate with a rather pronounced hypoplasia of the cleft side of the face, with ectropion of the lower lip and a disturbing retroprofile line (operated upon in 1979) This complex anomaly of a unilateral cleft is clearly seen in Fig. 24a. It is not a rare condition in cleft cases that there is a remarkable hypoplasia of the midfacial area on the cleft side. In addition her lower lip ectropion and her retroprofile line were very disturbing. The patient had been referred after she had had already some orthodontic treatment. She had good dental arches and an oronasal fistula at the palate in its anterior region (Fig. 24b). Her hypoplasia of the right side of her face obviously had a skeletal background. That can also be observed at the skull of an unilateral cleft, as shown on the bottom of the same picture. My planning intended to produce a symmetrical face with a vertical or even an anteprofile line (Fig. 24c). In order to achieve this it will be necessary to advance the maxilla as well as the mandible. For the correction of the ectropion of the lower lip I find the surgical retropositioning of the inferior frontal segment more efficient than what can be achieved by orthodontic measures. Illustration Fig. 24d shows the model planning and the operation plan in drawings: The plan for the corrective surgery of the maxillo-mandibular complex included as a first step the extraction of the first inferior bicuspids and simultaneous retro positioning of the inferior frontal segment. This was felt necessary for the correction of the ectropion of the lower lip and for additional overjet for the necessary amount of advancement of the mandible. After this preliminary surgery the new orthodontist (Prof. Paul €ckli) arranged the teeth to such perfection that the surgical Sto correction became an easy job: advancing the maxilla in two segments with placing them into proper occlusion, on the right side simultaneously closing the gap of the missing second incisor, and also advancing the mandible by the sagittal splitting procedure. Fig. 24e shows the occlusion achieved through this combined orthodontic and surgical treatment in comparison to the occlusion when the patient had arrived. Illustration Fig. 24f shows that not only the occlusion but also the facial appearance had remarkably improved. There was still the hypoplastic region of the right zygoma area that required augmentation. In a further intervention I corrected that hypoplasia of the right midfacial region by transoral implantation of some slices of lyophilized bank cartilage and in addition I used my sliding transoral chin procedure to advance its prominence and increase the inferior facial height. She then went back to her plastic surgeon in the States, by whom she wanted to have the still necessary nasal correction done. Fig. 24g demonstrates in the lateral cephalograms the change in the facial skeleton and the two last figures (Fig. 24h,i) show the patients improvement in profile and in the frontal views. This case is a typical example for the necessity to follow the three important principles in treating these deformities. First: proper detailed diagnosis; second: consistent treatment plan; third: execution of the plan by very experienced specialists. 8.6. Fig. 25aeg: It is a case of bilateral cleft lip and palate with severe retromaxillism and hypoplasia of the middle third of the face (operated upon in 1972/73) The skeletal framework (Fig. 25a) of this very severe dish face deformity requests obviously that the whole middle third must be

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advanced and vertically increased, but the mandible should be left untouched. To achieving that goal a Le Fort III þ I will be indicated (H. Obwegeser, 1969). Fig. 25b demonstrates the patient's circular nonocclusion and the model planning: according to it the maxilla must be advanced for 20 mm in two pieces and it must be widened by reopening the cleft. In Fig. 25c the planned operation is shown in drawings. Bone grafts are a must since the advancement creates defects of 20 mm. There is not only an extensive advancement of the middle third of the facial skeleton by a Le Fort III osteotomy necessary as inaugurated by Tessier (1967) for the wanted improvement of the facial skeleton but also an increase of the vertical height. For that an additional Le Fort I osteotomy has to be performed, that will permit to place the tooth bearing maxilla into a planned intermaxillary relationship for the later prosthodontic work and also, very important, for the needed facial height. That will create a gap between the upper halve of the middle third and the inferior halve. That gap requires a massive bone graft. Consequently I performed a Le Fort III and an additional Le Fort I advancement with reopening the cleft in a first shot. In a second operation the reopened cleft was closed again and the nasal deformity was also corrected with a very satisfying result. From then on this simultaneous Fort III þ I operation has become routine in my clinic. Fig. 25d shows on the left side the occlusal situation before and after that surgery. The new intermaxillary relationship permitted the prosthodontist to construct a functionally and aesthetically very satisfying result, in particular by the use of the Dolder bar system. After that the columella was elongated and the nasal deformity corrected, as seen on the cephalometric radiographs (Fig. 25e) They also show that with the simultaneous LF III þ I advancement the anatomical cause of the severe dish face deformity was corrected and the height of the midfacial third was increased to normal as seen here on the lateral cephalometric radiographs. The last two photographs of this case (Fig. 25f,g) show that the patient is now ready to live a normal life. 8.7. Fig. 26aer: The last case is the case of Antonio. It is a case of facial duplication plus a large medial facial cleft (operated upon 1969e78) Antonio had come from southern Italy. He became the case of €henbühl, the Chief my life. He was referred to me by Prof. Hugo Kra of the University Clinic for Neurosurgery in Zürich. To him the patient was sent because he suffered from a congenital abnormal walking. In treating his congenital facial skeletal deformity I learned more about the influence of surgery on the growing facial skeleton than by collective experience. For the child's psychological support I wanted the parents to stay in town for the time of his hospitalization. As they were rather poor working people from the south of Italy they could not afford the child's hospitalization or any treatment costs. For that reason I personally felt responsible for the parent's full pension in a nearby hotel and I asked the government of the Canton of Zürich to permit to have the boy free of charge hospitalized and treated for scientific reasons at our department. The patient was born with two complete noses and a wide medial cleft as can be seen on Fig. 26a. He was referred to me in 1969, at his age of 10 years. In babyhood the medial cleft had been closed by a plastic surgeon in his home town with the utilization of  flap operation was the medial wings of the two noses and an Abbe used for closure of the very wide cleft lip. There was extreme hypertelorism. The tips of the two noses were 5 cm apart. As can be seen in Fig. 26b the large upper jaw had a fully developed premaxilla on each side. At those days only standard radiographs were

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available but no CT and no MRI. Nevertheless the oblique skull view shows very impressively the amount of hypertelorism. I made my plan for correcting this anomaly without knowing at those days how much damage surgery can do to the growing facial skeleton. Fig. 26c shows in drawings my treatment plan based on Tessier's hypertelorism operation technique (1967). Both halves of the midface, including the orbit and the maxillary half of each side, should be rotated medially, after removal of all tissues between the two nasal crests. As soon as the anatomy becomes clearer during surgery, surgical details will have to be adapted accordingly. A day before surgery, every person participating in the procedure, was given a written detailed plan, including the estimated time for each step. The operation was performed on the 4th of July 1969. When the neurosurgeon was raising the brain from the anterior cranial fossa he found, to our great surprise, two complete cristae galli with a lamina cribrosa on each side of them as shown in the drawings. That finding made it clear how much I had to excise in the anterior cranial fossa. Illustration Fig. 26d demonstrates the skeletal situation when the skull was freed. The two bony nasal frameworks were clearly recognizable and between them two rudimentary eye sockets. After the orbital coni wire sectioned and everything between the two halves of the facial skeleton, including the two premaxillae, had been excised, both halves could be rotated together. Bony defects were bridged with bone grafts from the iliac crest and ribs. Because of postoperative purulent meningitis the neurosurgeon had to remove the large cranial bone flap and I had to take away the bone grafts at the defects of the orbital walls. Under heavy antibiotic treatment at the children's hospital the infection was overcome. The patient had to wear a helmet. With that he left for home together with his parents: After 10 months a severe maxillomandibular discrepancy had developed clearly seen in the cephalometric radiograph of Fig. 26e which I corrected by advancing the maxilla and retro-positioning the anterior mandibular alveolar segment as seen on the model operation and demonstrated with the drawings of the planned procedure as seen in Fig. 26e. The next illustration (Fig. 26f) shows that in spite of the former removal of the bone grafts at the defects of the lateral orbital walls the result was quite pleasing again. The facial skeleton showed a somehow normal shape in the radiograph, particularly compared with the preoperative situation. Thirteen months after the removal of the infected cranial bone flap the neurosurgeon re-implanted the autoclaved cranial bone flap, but without success because of a new purulent infection. Ten months after the newly infected bone flap had been removed again, that is 23 month after the primary operation, I reconstructed the very large skull defect with 14 half ribs of the patient (Fig. 26g). Illustration Fig. 26h shows in lateral cephalograms on the left side the skeletal situation one month after the reconstruction of the cranial defect; this was one year and one month after I had corrected the newly developed maxillo-mandibular discrepancy. And on the right side the skeletal situation 2½ years later is shown: again, everything has grown forwards but not the middle third of the facial skeleton. Two more years later, when the skeletal growth had ceased, a large maxillo-mandibular discrepancy had developed again as shown in illustration Fig. 26i .It again asked for correction. I was now convinced that at this time the skeleton will remain as I place it. It has been so obvious that the middle third lacked of growth due to my surgery while the forehead and the mandible did not react negatively to the extensive denuding surgery. For the correction of this very severe facial skeletal anomaly again I performed a retropositioning of the protruded anterior mandibular alveolar

segment plus a sagittal splitting of the mandible for its push back and simultaneously I advanced the maxilla plus the paranasalinfraorbital regions including the anterior part of the zygomas. The drawings in illustration Fig. 26k demonstrate clearly my plan of surgery. As can be seen on the next illustration (Fig. 26l) it resulted in a good intermaxillary relationship. The missing columella and nasal framework still had to be reconstructed for a good profile. For the reconstruction of the missing columella I used a caterpillar flap from the nasal dorsum. For the missing nasal framework I inserted a L-shaped piece of rib cartilage, as I had learned it from Eduard Schmid. The procedure is nicely seen in details in Fig. 26m. This whole procedure produced a very acceptable final result (Fig. 26n). Regrettably, at those days we still used infraorbital incisions for the osteotomies of the orbital cones. The scars will remain visible for ever. The situation of the maxilla with the teeth, before we corrected this anomaly and the final stage of it, is seen in Fig. 26o. The lateral cephalograms (Fig. 26p) show the skeletal situation when I started the correction at age of 10 years and on the right one year and eleven months after the last operation, that is close to his age of 20 years. He had a good profile and a firm skull again. His final view from below at his age of 18 years and 7 months in comparison with his presurgical situation at the age of ten is seen in Fig. 26q. His nose was fairly normal and so was his nasal breathing. His speech quality was good enough that his native town could employ him in the city's telephone business. The last illustration shows the patient again, 25 years later, together with his surgeon. Both seem to be happy. 9. Conclusions 9.1. The aim The aim in any cleft case must be the achievement of normal appearance and normal chewing and speech function, not merely be the avoidance of secondary surgery, as formulated by some orthodontists; it must be, without any compromise, normal appearance and function, whatever may be needed for that. 9.2. Surgical basics It is a fact that any surgery on the maxilla before growth has ceased, even just elevating the palatal artery flaps (Herfert, 1954), causes lack of growth of that part. Scars can also influence the maxillary growth negatively. That means do as little surgery on the maxilla for the primary repair as possible. Lip closure: In babyhood, gentle lip closure or lip adhesion -flap operation, also in the adult operation only. Avoid any Abbe cases, whenever possible. I have never seen an undisturbed lower -flap operation even when it had been performed lip after the Abbe by the most experienced surgeon. Hard and soft palate: Closure of the soft palate only, just before the child starts talking. No surgical closure of the hard palate and the alveolar cleft before maxillary growth has finished. The palatal defect is covered with a plate, which must be adapted according to the growth of the maxilla and eruption of the teeth. After eruption of the permanent teeth the remaining alveolar and palatal fistula can easily be closed without causing growth impairment. In cases with congenital hypoplasia of the base of the maxilla and of the paranasal-infraorbital bony structures at the side of the cleft, as can be observed on skulls as well as on patients, the maxilla may require advancement after growth has finished. Then a residual palatal fistula can easily be closed and a missing tooth gap can be eliminated by simultaneous approximation of the cleft

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segments. The paranasal-infraorbital hypoplasia is corrected by contouring onlays or by surgical advancement of the affected areas. The nose: A nasal deformity can nowadays be corrected to such perfection that even the specialist may not recognise that a typical cleft nose deformity had existed, even in very severe unilateral cleft cases (Triaca, 1994). Palato-pharyngeal insufficiency: This may require a pharyngoplasty procedure. I got very good results only when I closed the naso-pharyngeal seal so much that the patient could not breathe through his nose. Then he was forced to lead the air through the mouth only. Thereby the patient could now produce normal articulation and pronunciation, however, with a typical closed nasality. When I reopened the connection of the soft palate with the pharyngeal wall 6e12 months later the patient produced a normal speech, with no further nasality. After a usual pharyngeal flap operation, a disturbing amount of open nasality often remained. 9.3. Orthodontic treatment In my experience, the goal of orthodontic treatment must be the arrangement of the permanent teeth in proper position to the respective base of the jaw, independent of the relation of the maxillary teeth to those of the mandible. Over-expanding the maxillary dentition or retruding the mandibular teeth cannot correct the skeletal anomaly. The surgeon can only produce a good aesthetic result when he brings the base of the maxilla into proper relation with the normal mandible. In my experience it is often necessary to extract a maxillary bicuspid on each side in order to be able to arrange the teeth in proper angulation. In craniostenosis cases it may even become necessary to extract two teeth on each side. There is no indication for orthodontic rearrangement of the deciduous teeth. In babyhood and up to the age of 14e15 years the orthodontist should feel responsible for providing the plate to cover the palatal defect and for caries prophylaxis and for the occasional need for the extraction of a tooth. Two years before the final surgical correction is needed he will, together with the surgeon, discuss the necessary procedure. Within one and a half or two years he has plenty of time to arrange the teeth with orthodontic methods so that they will fit into proper occlusion when the surgeon moves the skeletal parts for restoring normal appearance. 9.4. Logopaedic speech assistance This is suggested for every case. The logopedist has an easy job only when the surgeon and the orthodontist create proper prerequisites for normal articulation and pharyngeal closure. Without these two prerequisites even the best speech therapist may not succeed to achieve an undisturbed pronunciation. 9.5. Prosthodontic work It is natural that the patient prefers fixed crown and bridgework when some teeth are missing. From my point of view there is always a possibility for some change in the cleft region, even after years. For that reason and for hygienic purposes I found the Dolderbar construction with a removable superstructure the most ideal solution for the replacement of missing teeth. 9.6. Special instrumentation for maxillofacial surgery For good surgery good instruments are wanted. This is particularly true for our field of work on the facial skeleton. For the procedures which we had frequently used I had specially designed

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instruments made by different instrument making companies. I have experienced that during the process of new production of the instruments which finally had achieved the shape and quality I wanted, the new instruments had changed. Other instrument makers had produced them and other companies copied my original instruments. The results were often so bad that I had to go to the court for forbidding them to use my name for these instruments. That means that only those instruments are of accepted quality which I had the right to check their production in intervals. It is not easy to get every instrument made in the quality one wants. Regrettably that is true for my maxillary advancer. I have a sample piece but no further production of it, although it is a very important help for advancing the maxilla. The following companies are selling these checked instruments of my design: KLS in USA, Martin and Medicon in Europe and their distributing agencies anywhere in the world. These are the only companies which hold the right to place on the instruments “original Obwegeser”. All others are selling instruments as Obwegeser instruments, but their quality may be and often is not acceptable for me. 9.7. A special hand-piece for surgery on the facial skeleton A special hand-piece for burs to work on the facial skeleton was constructed for me by the W&H Company from Bürmoos, Austria. That company is well known for its hand-pieces for dental work. However, the usual straight hand-pieces are too short for our work in the depth of the mouth. Therefore I asked them to produce a longer hand-piece for our work. I also wanted that it should hold all bur type instruments with a shafts diameter of 2.35 mm. I did not want that we need special burs made for our work, except very few ones. I used that hand-piece constantly for the sagittal splitting procedure of the mandibular rami or whenever I had to work in the depth. It is also an excellent hand-piece for the removal of impacted wisdom teeth. I do not know any other company that produces such a special hand-piece. References Axhausen G. Dtsch Zahn Mund u Kieferhlk I: 334e342, 1934 Axhausen G. Dtsch Zschr Chir 248: 515e522, 1936 Axhausen G. Dtsch Zahn Mund u Kieferhlk 6: 582e600, 1939 Converse JM, Shapiro H. Plast Reconstr Surg 10: 473e510, 1952 Cupar J. Oesterr Z Stomat 51: 565e577, 1954 Drommer R. J Maxillofac Surg 7: 264e270, 1979 Drommer R. J Maxillofac Surg 14: 119e122, 1986 rieur enoisage  essentiellment Gillies HD, Rowe NL: L'osteotomie du maxillaire supe vre total. Rev Stomatol 55: 545e552, 1954 le cas de bec-de-lie Herfert O: Experimental contribution to the problem of injury to the maxillary growth by primature cleft palate surgery. Dtsch Zahn Mund u Kieferhlk 20(9e10): 369e381, 1954 Obwegeser HL: Cirugía del “mordex apertus”. Rev Asoc Odontol Argent 50: 430e441, 1962 Obwegeser HL: Der offene Biss in chirurgischer Sicht. Schweiz Monatsschr Zahnheilkd 74: 668e687, 1964 Obwegeser HL: Eingriffe am Oberkiefer zur Korrektur des progenen Zustandbildes. Schweiz Monatsschr Zahnheilkd 75: 365e373, 1965 Obwegeser HL: Surgical correction of small or Retrodisplaced maxillae. Plast Reconstr Surg 43: 352e365, 1969 Obwegeser HL, Makek M. J Maxillofac Surg 14: 183e208, 1986 €die unter BerSchuchardt K: Ein Beitrag zur chirurgischen Kieferothopa ücksichtigung ihrer Bedeutung für die Behandlung angeborener und erworb€ten. Dtsch Zahn Mund u Kieferhlk 9: 73, 1942 ener Kieferdeformita otoTessier P: Dysosotosis cranio-faciales (syndromes de Crouzon et d’Apert) oste mies totales de la face. In: Transaction of the Fourth international Congress of plastic and reconstructive surgery Rome, October 1967. Amsterdam: Excerpta Medica Fondation, 774e783, 1967 1969 Triaca A: Correction of unilateral cleft nose deformity. Paper read at the world Congress on Rhinoplasty, april 29eMay 1, Philadelphia; 1994

Pioneer steps in correcting secondary cleft lip and palate deformities: my philosophy and procedures.

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