nasal reconstruction for maxillonasal dysplasia

Copyright @ 2010 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

Nasal Reconstruction for Maxillonasal Dysplasia

Anthony D. Holmes, FRACS,*Þ Shu Jin Lee, MRCS, MD,*Þ Andrew Greensmith, FRACS,*ÞAndrew Heggie, FRACDS (OMS),*Þ and John G. Meara, MBA, FRACS*Þ

Background: Maxillonasal dysplasia, Binder type (Binder syn-drome and nasomaxillary hypoplasia), is a spectrum of deficientnasomaxillary osteocartilaginous framework, deficient nasal softtissues, and a short columella. The correction of these deformities ischallenging, and results are often disappointing. Tissue expansionwith multiple bone grafts for nasal augmentation from childhood hasbeen advocated as a means to address the constricted soft tissues.However, bone grafts in children have been associated with unpre-dictable growth and resorption. Agreeing with the principle of serialnasal augmentation that commences in childhood, we used allo-plastic material for tissue expansion followed by definitive recon-structive rhinoplasty at the completion of growth and orthognathicsurgery as required. Definitive rhinoplasty mainly used a 1-piececostochondral graft cantilevered to the frontal bone.Materials and Methods: Thirty-one patients over a period of27 years were reviewed. The patients were divided into 2 groupsbased on the age of presentation, namely, prepubertal and post-pubertal. The prepubertal group underwent serial tissue expansionof the constricted nasal envelope with customized silicone implantsand final reconstruction by costochondral rhinoplasty at the endof puberty. The postpubertal group underwent 1-stage costochon-dral rhinoplasty. The definitive rhinoplasty used a cantilevered 1-piece costochondral graft retaining the dorsal periosteum that wasdowelled into the frontal sinus wall.Results: In the prepubertal group (n = 20), 41 silicone implants wereplaced in the childhood years for tissue expansion of the nasal enve-lope. One patient developed implant infection, and another requiredreplacement after extrusion. Long-term follow-up showed minimalresorption of the costochondral graft in the pre-expanded prepubertalgroup and minimal to moderate graft resorption in the postpubertalgroup.Conclusions: Successful treatment of maxillonasal dysplasia is de-pendent on the following: an understanding of the underlying patho-logic anatomy, namely, that of the constricted nasal tissues, serial tissueexpansion of the nasal envelope in childhood, and definitive cos-tochondral rhinoplasty at the end of growth. Early tissue expansionwith the placement of alloplastic silicone implants effectively stretchesthe constricted nasal soft tissues in Binder syndrome to limit graft

resorption after definitive nasal reconstruction with costochondral ribgrafts. There is a possible role for similar tissue expansion in thepostpubertal patient with alloplastic material before costochondralgrafting if the soft tissues are inadequate. Long-term resorption ofcantilevered, 1-piece, periosteum-covered costochondral grafts wasminimal.

Key Words: Maxillonasal dysplasia, maxillonasal dysostosis,nasomaxillary hypoplasia, Binder syndrome, Binder association

(J Craniofac Surg 2010;21: 543Y551)

M axillonasal dysplasia results from hypoplasia of the osteo-cartilaginous framework of the nasomaxillary region1Y11 and

deficiency in anteroposterior growth of the nasomaxillary com-plex.12,13 This results in significant shortage of both the bonyframework and the soft tissues of the nose. The osseous deficiencyis evident in the deficient length and projection of the caudal por-tion of the nose with the pathognomonic feature being absenceof the anterior nasal spine.2,4,5,7,10,11 There is deficiency of thenasal soft tissue envelope and the nasal lining and, in partic-ular, a distinctive short columella (Fig. 1). The nasomaxillary hypo-plasia may be associated with a normal or class 3 dental occlusalrelationship.3,7,11,14

Correction of the nasal deformity in maxillonasal dysplasiais challenging, and results are often disappointing.3,15,16 Many sur-geons approach the correction of the nasal bony and soft tissuedeficiencies separately. Attempts to correct the foreshortened nosehave been unsatisfactory with obvious scarring resulting from V-Yor similar procedures to lengthen the nose,17Y19 forehead flaps toprovide additional skin covering,17 and skin grafts for nasal dor-sal inlays.20 Composite conchal grafts for nasal lengthening re-quire multiple operations and provide only small amounts of nasallengthening.3,21 The problem of deficient nasal lining has beenpreviously addressed with local turnover hinge flaps, nasolabialflaps,17 and intranasal epithelial inlays that require permanent sup-porting prostheses3,22,23 to overcome scar contraction.

Correction of the bony deformity was first attempted usingthe LeFort II osteotomy that was championed to provide simulta-neous correction of both the nasomaxillary hypoplasia and the fore-shortened nose.3,4,24Y27 The LeFort II osteotomy does not adequatelyaddress the retruded midface, and the nose was insufficientlylengthened.12,27 Others used additional bone and cartilage grafts in-serted via visible external incisions for paranasal augmentation andnasal lengthening, and local flaps are used for columella elongation.27

These procedures are often associated with visible scarring16 and un-predictable growth28 and resorption of bone grafts.16 Obvious scarringalso followed the use of the trapdoor incision over the nasal dorsumfor nasal lengthening29 and the paranasal incision for LeFort II os-teotomies.27 The LeFort II advancement can lead to the conversion ofnormal or mild class 3 occlusion to severe class 2 occlusion.4,27 TheLeFort I osteotomy alone was unsatisfactory because it accentuatesthe nasal retrusion.30

TECHNICAL STRATEGY

The Journal of Craniofacial Surgery & Volume 21, Number 2, March 2010 543

From the *Department of Plastic and Maxillofacial Surgery, Royal Children’sHospital, Melbourne, Victoria, Australia; and †Department of Plastic Sur-gery, Children’s Hospital Boston, Boston, Massachusetts.Received October 23, 2009.Accepted for publication November 15, 2009.Address correspondence and reprint requests to John G. Meara, MBA,

FRACS, Children’s Hospital Boston, Boston, MA 02115;E-mail: [email protected]

Copyright * 2010 by Mutaz B. Habal, MDISSN: 1049-2275DOI: 10.1097/SCS.0b013e3181d024b0


The approach to the Binder syndrome patient then became2 staged, with the correction of the maxillary deformity with or-thognathic treatment of the malocclusion first and, later, thecorrection of the nasal deformity with bone graft nasal augmenta-tion.11,30,31 As treatment commences after puberty, the limitations ofthis approach include school-age psychologic problems and that theconstricted nasal envelope remains unaddressed throughout child-hood. The deficient nasal envelope not only limits the size of thebone graft but has also been proposed to be constrictive and causebony resorption that is especially noticeable in the region of theforeshortened nasal tip.16

To overcome this limitation, Tessier32 proposed tissue expan-sion of the deficient nasal envelope and columella with serial bonegrafting, and Ortiz-Monasterio et al15 showed satisfactory correctionof the nasal deformity using multiple costochondral rib grafts fromearly childhood32 to achieve serial expansion before definitive cos-tochondral rhinoplasty.

We present 1 surgeon’s (A.D.H.) experience with the cor-rection of the nasal deformity in maxillonasal dysplasia in 31consecutive cases. This approach first addresses the problem of softtissue deficiency with serial alloplastic augmentation of the nasalenvelope from early childhood. Final nasal correction is performedwith costochondral grafts at the completion of growth. Orthognathicsurgery is performed at the time of final nasal correction or there-after, if indicated.

PATIENTS AND METHODSThe records of 31 consecutive cases of maxillonasal dys-

plasia treated in our institution since January 1, 1980, were re-viewed. There were 2 age group distributions: prepubertal andpostpubertal. The male to female ratio was 1:1. The anterior nasal

spine was hypoplastic or absent in all the patients. The age at thefirst nasal surgery ranged from 5 to 24 years, with a mean of12.3 years. In the prepubertal group (n = 20), the age range was from5 to 12 years, and in the postpubertal group (n = 11), the age rangewas from 14 to 24 years.

In the prepubertal group, all the patients underwent siliconenasal augmentation. Between 1 and 3 silicone nasal implants wereinserted while the patients passed in to their teenage years. Mostpatients required 2 implants (n = 15/20) to expand in tandem withfacial growth. Two patients had 1 implant placed. Three patientsrequired 3 implants. In this group, 1 patient had commenced treat-ment early at the age of 5 years, 1 patient had extrusion of theimplant after an altercation, and another had an infection thatrequired removal of the spacer, debridement, and intravenous anti-biotics (n = 2/18). The spacer was replaced after 6 months with nofurther infections.

In the prepubertal group, definitive costochondral rhinoplastywas performed from the ages of 19 to 22 years, with the mean age of19 years (n = 17). Two patients are still in their childhood years andawaiting definitive rhinoplasty. One patient refused definitive rhi-noplasty, being content with the silicone implant. The postpubertalgroup of patients had a mean age of 16.2 years at the time of nasalreconstruction (n = 11). In this group of patients with delayedpresentation, nasal reconstruction followed orthognathic surgerywith LeFort I maxillary advancement. Seventy percent of thepatients had class 3 dental occlusion (n = 21), and 30% of thepatients had class 1 dental occlusion (n = 10). Ninety percent(n = 17) of the patients underwent orthodontics, and 60% (n = 11)underwent orthognathic surgery to correct class 3 malocclusion viaLeFort I maxillary osteotomy. Follow-up periods after completionof the definitive nasal reconstruction ranged from 4 to 22 years,with a mean of 15 years.

RESULTSIn the prepubertal group of 19 patients, 41 implants were

placed in total. There was 1 case of implant infection that requiredremoval and antibiotics treatment. The patient underwent the place-ment of another implant 6 months later with no further problems.Another patient had exposure of the implant after a schoolyardaltercation requiring implant replacement.

Resorption of the bone graft was assessed from 3 yearspostoperatively. Those who had serial tissue expansion and sub-sequent adult or postpubertal costochondral grafting did bestwith minimal resorption noted. Prepubertal bone grafting wasnoted to be associated with overgrowth in 1 case. Those who pre-sented postpubertal costochondral and had a 1-stage costochon-dral reconstruction had minimal to moderate resorption (n = 3)

FIGURE 1. Classic Binder profile showing the classic shortnose with short columella.

FIGURE 2. Transbuccal insertion of customized nasal implant with the short limb of the L-shaped implant fashionedto be stouter than the long limb and allowing for extension from the pyriform margin to the proposed nasal tip.

Holmes et al The Journal of Craniofacial Surgery & Volume 21, Number 2, March 2010

544 * 2010 Mutaz B. Habal, MD


in the region of the nasal tip ranging from 3 to 5 mm. Thosepatients (n = 3) who had other bone grafts (iliac) had a large resorp-tion rate as compared with patients with costochondral grafts. In theinitial experience with the postpubertal group, 1 patient had a rhi-noplasty performed with an iliac bone graft that on follow-up, wasnoted to have complete resorption of the caudal end of the iliac bonegraft, requiring correction with a costochondral rib graft.

Treatment ProtocolSerial Silicone Nasal Spacers forTissue Expansion

The placement of silicone nasal spacers was performed in theprepubertal group. An L-shaped silicone implant is customized tothe dimensions required and carved from a block of medical-gradesilicone (Fig. 2).

The vertical limb is more substantial than the conventionalsilicone nasal implant and is carved to have a stout rectangularcross section. The vertical limb extends from the superior edge ofthe pyriform margin to project 3 to 5 mm higher and more anteriorto the nasal tip to stretch the columella vertically and anteriorly.The dimensions of the vertical limb are adjusted to provide themaximal length able to be accommodated within the soft tissuenasal pocket without blanching of the overlying skin. Thehorizontal dorsal limb extends to the nasofrontal junction but istapered cranially where the bony deficiency is less marked than inthe caudal portion of the nose.

Earlier cases in our chart review revealed that the siliconeimplants were inserted via an intra-oral superior alveolar incision,dissecting the nose via a membranous septal tunnel to the dorsum.Later, an open rhinoplasty technique was used to ensure that the alardomes were joined to cover the prosthesis in the region of the nasal

FIGURE 3. In the harvesting of the costochondral graft, it is important to choose a straight segment of rib and to maintainlateral periosteal flaps that are folded over the lateral aspects of the graft.

FIGURE 4. Lateral periosteal flaps are sutured to the upper lateral cartilages to maintain the integrity of the internal nasalvalves, and at the region of the nasal tip, the lower lateral cartilages are sutured above the costochondral graft.

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tip and reduce the risk of implant exposure. There was no significantdifference in the results, and the open rhinoplasty is now preferredbecause of improved visibility and access to upper and lower lateralcartilages.

Definitive Nasal Reconstruction WithCostochondral Graft

At the completion of growth, patients underwent definitivenasal reconstruction. The technique involves a fully cantilevered1-piece costochondral graft with the dorsal periosteum retained.The senior author has used this technique successfully for 29years, particularly for nasal reconstruction cases where strongnasal support is required and the dorsum is deficient. In max-illonasal dysplasia, the distal part of the nose has inadequatesupport and requires more buttressing than can be offered byseptal grafts or similar techniques. Preoperatively, skull x-raysare taken to confirm the presence of a pneumatized frontal sinusand to avoid dural penetration during preparation for stabiliza-tion of the graft.

Via a stepped columella incision and infracartilaginous rimincisions (open rhinoplasty), the nasal envelope is elevated fromthe nasal cartilages under the submuscular aponeurotic systemplane of the nose and then subperiosteally up to the level of thefrontal sinus. The medial crura are separated to approach the caudalseptum. The medial attachments of the upper lateral cartilages arecarefully dissected from the septum without breaching the nasalmucosa.

The nasal dorsum is rasped or burred to create a level surfacefor the placement of the graft, and a medium pineapple burr is thenused to create a 5- to 8-mm aperture through the anterior wall of thefrontal sinus. The desired length of graft is measured from the frontalbone to the proposed projection of the nasal tip to create a nasolabialangle of 90 degrees in the male and 110 degrees in the female. Extralength is harvested to allow for final trimming of the caudal endwhen the graft is fixed.

A straight segment of the costochondral cartilage is requiredand usually corresponds to the 9th or 10th rib (Fig. 3). The graft isharvested via a small Kocher incision, and the donor site is closed.The grafts are harvested with a sleeve of the periosteum and theperichondrium if possible (especially on the dorsal surface). Typi-cally, the grafts are two-thirds osseous and one-third cartilaginous.Care is taken not to disrupt the osseochondral junction.

Shaping of the graft was performed by drawing a pattern onthe straightest part of the rib, and then the periosteum was elevatedfrom the sides before burring to achieve the final size and shape. Theperiosteum is then flapped over the contoured edges at the side. Theperiosteum at the edges is then sutured to medial dorsal edges ofthe upper lateral cartilages, hence acting as a Bspreader[ graft byopening the internal nasal valves. The entire subcutaneous surface ofthe graft is therefore covered in periosteum.

The costochondral graft was then oriented with the bony endcephalad and the cartilaginous portion caudal (Fig. 4). In mostcases reviewed, the uppermost 10 mm of the grafts were trimmed tocreate a tapered cortical peg for stable insertion of the grafts intothe trephined basal bone of the frontal sinus. This technique wasascertained by the authors from Paul Tessier in 1977. Additionalfixation is usually not required. Occasionally, to avoid lateral

instability, the addition of percutaneous Kirschner wires is requiredand inserted at a right angle to the graft through the dorsal skinproximally and through to the nasal bone. In the absence of a

FIGURE 5. Patient 1. The patient presented as a child. First row, preoperative pictures. Second row, insertion of silicone nasalprosthesis. Third row, postoperative pictures after insertion. Fourth row, the patient 4 years after the silicone nasal implant.Fifth row, preoperative photos before the costochondral graft for nasal reconstruction at age 16 years. Last row, postoperativephotos after the final nasal reconstruction.

FIGURE 6. Patient 2. The patient presented as a child.Upper row, the patient as a child with features of Bindersyndrome. Middle row, improvement of the nasal formafter the silicone nasal implant. Lower row, 1 year afterthe costochondral graft for nasal reconstruction. She alsounderwent orthognathic surgery to correct a class 3malocclusion at the completion of growth.




frontal sinus, a bony ledge is burred in the cephalad end of thenasal dorsum and the graft is secured by 2 percutaneous Kirschnerwires left in situ for 2 weeks. Lateral nasal bone in-fractures areperformed where necessary to narrow the nasal width. Thecartilaginous portion of the costochondral graft is trimmed tolength and to a relatively thin width at the region of the nasal tip.The upper lateral cartilages are then connected to the periosteum ofthe lateral aspects of the graft with 4-0 Vicryl sutures to enhancethe opening of the internal nasal valves. The lower lateral cartilagesare then mobilized to allow elevation and attachment of the medialcrura and domes to the cartilaginous pole of the graft to provide foradequate projection and shape. In some cases, Sheen-type domeaugmentation grafts were added in thick-skinned individuals toget more tip definition. Extra cartilage was sometimes used toaugment the deficient caudal septum region to help with an aes-thetic columella nasolabial angle.

The columella skin incision is then closed with a 6-0 nylon;and the mucosa, with a 5-0 catgut. If necessary, alar base reductionsare performed to ensure that the interalar width is equal or less to that

of the medial canthii. A thermoplastic external dorsal splint isapplied and maintained for 10 to 14 days.

PatientsPatient 1 (Silicone Nasal Implant Placementin a Prepubertal Patient)

A 7-year-old girl presented with the classic nasal defor-mity for which she requested treatment (Fig. 5). She underwentthe insertion of a customized silicone nasal implant placed viathe transoral insertion at age 8 years (intraoperative picturesare those of a similar patient). The implant was placed underthe alar cartilages. There was satisfactory increase in tip projec-tion, nasal length, and columella length. Postoperative picturesare shown at ages 8, 12, and 16 years. At 17 years of age, amaxillary advancement followed by a costochondral rhinoplastywas performed.

Patient 2 (Prepubescent Presentation)A 12-month-old girl presented with a nasal deformity

(Fig. 6). She underwent silicone nasal augmentation at the ageof 8 years, placed via a transcolumellar incision. Correction ofthe class 3 dental malocclusion was performed with combinedorthodontic treatment and orthognathic surgery and consisted ofa LeFort I maxillary advancement (5 mm, anterior; 5 mm, anteriorimpaction; and 2 mm, rotation to the left), asymmetric mandibularreduction with bilateral sagittal section osteotomies, and anadvancement genioplasty (6 mm) at the age of 17 years. A de-finitive costochondral rhinoplasty, with the graft secured into thebase of the frontal sinus bone together with Sheen tip grafts, wasperformed at age 19 years. Minimal graft resorption was noted at10 years’ follow-up.

Patient 3 (Postpubescent Presentation)A 24-year-old woman presented without a diagnosis,

requesting correction of her poor nasal projection. She underwenta 1-stage costochondral reconstruction secured via a frontal bonetrephine. There was no obvious graft resorption on 8 years’ follow-up (Fig. 7).

Patient 4 (Complication of Graft Overgrowth)A 7-year-old boy presented having had a previous procedure

with another surgeon who performed a costochondral graft

FIGURE 7. Patient 3. The patient presented in adulthood.Upper row, preoperative pictures. Lower row, 2-yearpostoperative pictures showing satisfactory nasal projection.

FIGURE 8. Upper row, lateral cephalogram showing overgrowth of the costochondral graft in patient 4. Lower row,postoperative lateral cephalogram showing improvement of facial proportions and improved nasal form.




cantilevered to the frontal bone via a bicoronal approach. At thecompletion of puberty, he presented with graft overgrowth asevidenced by a long, curved graft; increased nasal length; and anacute nasolabial angle (Fig. 8). He had a class 3 malocclusion thatrequired orthognathic surgery, and the previous graft had to beremoved to facilitate surgical access for maxillary mobilization andadvancement. At the age of 19 years, orthognathic surgery wasperformed simultaneously with removal and replacement with a newcostochondral nasal graft (Fig. 9). At follow-up at 10 years, therewas minimal graft resorption.

Patient 5 (Postpubescent Presentation)This 14-year-old girl presented concerned about her

nasal deficiency. She had a mild maxillary deficiency and a class3malocclusion. A costochondral nasal reconstruction was per-formed primarily followed by maxillary advancement when shewas more mature. Postoperative pictures were taken at age 19 years(Fig. 10).

DISCUSSIONThe 3 main deformities in maxillonasal dysplasia are the

hypoplastic osteocartilaginous nasomaxillary region, constricted dis-tal nasal soft tissues, and a short columella. Some surgeons tendto commence treatment after puberty and to address the problemsseparately. Maxillary advancement via the LeFort II osteotomy doesnot adequately correct the foreshortened nose.11,27 Nasal soft tissuelengthening procedures often result in conservative nasal elongationand undesirable scarring,1,17Y21 and local columella-lengtheningflaps result in visible scarring.16,27 As the 3 main deformities are in-terrelated, it seems reasonable to address them with a staged proto-col. After our experience, treatment should ideally commence inchildhood with the key introduction of tissue expansion of the nasalenvelope in early childhood.15,26

The commencement of treatment in childhood also con-fers psychologic benefit through the alleviation of the stigmataof the syndrome and avoids teasing by peers. The timing of theinitial surgery is usually dictated by the patient. Nearly all of ourprepubertal patients requested surgery between the ages of 7 and9 years.

Our introduction of alloplastic implants for tissue expan-sion in Binder syndrome has been proven successful and welltolerated. The use of alloplastic material in childhood limitsdonor site morbidity and preserves the costal graft for finalreconstruction. In addition, costal osseochondral grafts in child-hood are more difficult because the donor site is not fully de-veloped and there is a higher risk of resorption or, occasionally,increased growth.

The results of early tissue expansion in our prepubertal groupconfirm the advantages of commencement of tissue expansion inchildhood. Early tissue expansion of the nasal envelope liningcapitalizes on the tissue elasticity of youth, allowing for expansionof the nasal envelope and lining, and final accommodation of a largerdefinitive costochondral graft at the completion of growth.15,16,32

The expanded nasal envelope may limit graft resorption. Theminimal graft resorption in this group contrasts favorably withreports of significant graft resorption in the absence of tissueexpansion.16 The finding of slightly more graft resorption in ourpatients presenting in the postpubertal group without tissueexpansion is consistent with previous reports.16 There may also bea role for tissue expansion in this postpubertal group with siliconeimplant placement before definitive costochondral grafting toattempt to limit graft resorption.

In the design of the silicone implant, it is important to create asizable columellar strut that extends from the pyriform margin to the

nasal tip. This allows the vertical limb of the implant to expand theshort columella and avoids columellar lengthening flaps andconsequent scarring. The standard commercial nasal prosthesis isinadequate. In the evolution of our treatment plan, we previouslytried the transoral approach for the placement of the siliconeimplants. We now prefer an open rhinoplasty approach because itallows direct access to the lower lateral cartilage.

Carved silicone nasal implants are well suited to the Binderpopulation who may require multiple-staged procedures. In our earlyexperience, we used costochondral graft for nasal augmentation inchildhood, but growth was unpredictable because some resorbed and

FIGURE 9. Patient 4. Upper row, the patient with featuresof Binder syndrome. Second row, the child after thecostochondral graft in childhood. Third row, showingfeatures of overgrowth of the costochondral graft. Fourthrow, after simultaneous orthognathic surgery and repeatedcostochondral graft to the nose.




1 overgrew. Similar experience has been reported by other authors.28

There is a long history of use of silicone for nasal augmentation inthe Asian population.33Y40 Silicone implants have been reported tobe well tolerated in up to 84% of patients.34,35,40 The key to avoidingextrusion and infection is aseptic technique,33,34 minimal ten-sion,33,40 and adequate soft tissue coverage especially in the regionof the nasal tip where the implant should ideally be placed deep tothe local lateral cartilages.38

For definitive reconstruction, our strong preference is forcantilevered costochondral grafts because of the ease of harvesting,the ability to obtain the correct 1-piece full-thickness shape, and themaintenance of a periosteal covering. In addition, the distal chondralsegment is easy to shape and connect with the lower lateralcartilages. The rigid peg and hole fixations seldom require screws orpins and, along with retaining the periosteal cover, probably accountfor the lack of resorption. The 9th or 10th rib are preferred becauseof their straighter shape.

Sixty percent of the patients in our series were noted to havemalocclusion. Orthognathic surgery was an important component ofmanagement to address the fundamental maxillary deficiency inmaxillonasal dysplasia. The definitive rhinoplasty was performedconcurrently with a small number of maxillary advancementsand was possible because the cantilevered graft does not rely onmaxillary platform support.

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FIGURE 10. Patient 5. Upper row, the patient with mild maxillary deficiency, class 3 malocclusion. Lower row, 5-yearpostoperative photos after the costochondral nasal reconstruction and the maxillary advancement.




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