CLINICAL STUDY

Use of Distraction Osteogenesis in Arrhinia Jose´ Rolando Prada, MD,*Þþ and Marı´a Bibiana Mendoza, MD* Abstract: Nasal malformations such as hemiarrhinia and arrhinia have a very low incidence, although many treatment protocols have been described. In this article, we describe 2 surgical techniques to treat arrhinia depending on the age at the beginning of treatment. In our practice, we use Le Fort III osteotomy with distraction osteogenesis as a pillar of the reconstruction because it allows to improve anteroposterior and vertical projections of the midface, giving a proper platform for nasal reconstruction, decreasing the number of interventions. We report a patient with a hemiarrhinia who has completed appropriate reconstruction results and a patient with total arrhinia in whom the distraction was achieved to create a nasal bone support and improve midface projection. Key Words: Arrhinia, Le Fort III osteotomy, distraction osteogenesis

Le Fort III (LF III) osteotomy with or without distraction osteogenesis (DO), have been described.5,13 Le Fort III osteotomy with DO allows normal midface projection and increases vertical and sagittal dimensions14Y18; for this reason, it has become the treatment of choice for patients with hypoplastic midface.18,19 The association of this osteotomy with DO decreases the relapse that occurs after orthognathic surgery20 and has the potential to be used several times to get appropriate facial anteroposterior and vertical projections.18,21 Distraction osteogenesis is a safe and effective technique for patients with midfacial hypoplasia,18,19 and when used with LF III osteotomy, it progresses 2 to 3 times more than conventional surgery,12,18,19 decreasing the postoperative morbidity, operative time, blood loss, postoperatively pain, hospital stay, and costs.22Y25 In this article, we present 2 patients who underwent include LF III osteotomy with DO for hemiarrhinia and arrhinia at different ages.

CLINICAL REPORT

(J Craniofac Surg 2014;25: 888Y891)

Among the previous surgeries the patients underwent, we can name cleft lip and palate repair, lacrimal sac resection, coloboma correction, and canthoplasty.

C

Patient 1

From the *Fundacio´n Universitaria de Ciencias de la Salud, †Hospital Infantil Universitario de San Jose´, Fundacio´n Santa Fe´ de Bogota´; and ‡FISULABYRehabilitation center for children with cleft lip and palate, Bogota´, Colombia. Received September 29, 2013. Accepted for publication October 28, 2013. Address correspondence and reprint requests to Maria Bibiana Mendoza, MD, Hospital Infantil Universitario de San Jose, Carrera 52 No. 67 A - 71, Bogota´, Colombia; E-mail: [email protected] The authors report no conflicts of interest. Copyright * 2014 by Mutaz B. Habal, MD ISSN: 1049-2275 DOI: 10.1097/SCS.0000000000000518

A boy was born at term with an uncomplicated vaginal delivery. The prenatal course was uncomplicated, and the mother was healthy. There was no family history of congenital malformations or other inherited disorders. The child was delivered in another institution, showing absence of half of the right nose. He was diagnosed with a right hemiarrhinia. He underwent a right nasal passage permeabilization and cheiloplasty; when he was referred to our department, he was 12 years old. On admission, the physical examination of the patient showed a cheiloplasty scar, right alveolar cleft, absence of right nasal structures, arched palate, and the reconstruct nasal passage. When he was 2 years old, he developed a nasal passage stenosis. At the age of 3 years, we began the nasal reconstruction (Fig. 1A) with an inferior-based open-book flap for the nostril reconstruction. The first surgery corresponded to the nasal reconstruction in which skin between the cheeks and medial frontal region was used to reconstruct the inner surface of the nasal vestibules. The cartilage nasal frame was reconstructed with auricular concha, and a pre-expanded forehead flap was used for external coverage. Cartilage framework was formed with concha cartilage, and a pre-expanded left forehead flap was used for external coverage (Figs. 1B, C). At the age of 5 years, we increased the dorsum with a rib graft. By the age of 12 years, a nasogenian flap was designed for columella coverage, and when he was 14 years old, he was scheduled for a modified subcranial LF III osteotomy and DO with an external rigid device. At this time, the patient had a concave profile and an open and crossed anterior bite with an overjet of 6 mm (Figs. 2AYC). We use a modified LF III osteotomy in which coronal incision is avoided to reduce operative time and minimize bleeding. The incisions are performed at the nasofrontal junction, subciliary, and vestibular areas. Through the nasofrontal incision, a subperiosteal dissection of the nasofrontal junction and the medial wall of the orbit behind the lacrimal crest is performed, then through a subciliary incision, the orbital floor is exposed, and the medial wall dissection

ongenital anomalies of the nose are a rare disorder with an incidence of approximately 1 in every 20,000 to 40,000 live births.1 These nose anomalies present different characteristics, over time, and despite the little casuistry, different classifications and protocols have been described.1Y6 Arrhinia refers to the absence or hypoplasia of nasal structures; thereof, it is commonly classified as total arrhinia, hemiarrhinia, and prosbosis.5 Total arrhinia has been defined as the complete absence of the nasal structures and the olfactory system,1,5 whereas hemiarrhinia corresponds to the absence of half of the nose the ala, nostril, columella, and choana.5 Anatomically, hemiarrhinia and arrhinia are characterized by a unilateral or bilateral maxillary defect, respectively. Typically, there is a small maxilla in 3 dimensions, and the absence of pneumatization perpetuates restriction growth.5Y9 Canine does not erupt, the elongation of the nasal cavity interrupts,10 and the absence of septum and vomer, which are considered growth midface centers, produces a restriction in the vertical maxillary development.9Y11 These abnormalities generate a short face and a skeletal class III, because of the difference between maxilla and mandible growth.1,5,9,12 To correct these maxillary features, different treatment protocols, which include Le Fort I osteotomy, Le Fort II osteotomy, or

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Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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Distraction Osteogenesis in Arrhinia

a pre-expanded right forehead flap. The osteocartilaginous framework was reconstructed again, using rib grafts (Figs. 3AYC).

Patient 2

FIGURE 1. A, Patient before the first nose reconstruction with tissue expander in forehead and the canalization of the nasal passage. B, Cartilaginous framework reconstruction with conchal cartilage. C, External coverage with left pre-expanded forehead flap.

is completed. In addition, the upper maxilla and zygomatic body are dissected; through the vestibular incision, the maxillary dissection is completed. Osteotomy starts with reciprocating saw at the nasofrontal junction to the medial orbital wall behind the lacrimal crest continuing through the orbital floor to the lower outer corner of the orbit and over the zygomatic body. Through the vestibular approach, a pterygomaxillary disjunction is done with a curved chisel; another osteotomy is done from the nasofrontal junction toward the posterior nasal spine with a cleft chisel. To verify segment release, we use Rowe maxillary disimpaction forceps. To position the external rigid device, we used retention plates below the infraorbital rim and over the maxilla. We waited for a latency period of 5 days, and the activation was performed for 12 days turning 0.5 mm every 12 hours until we obtained a distraction of 12 mm; the consolidation period corresponded to 12 weeks. Distraction osteogenesis was suspended when the proper malar prominence was achieved and when the occlusion was overcorrected (Figs. 2D, E). During the postoperative period, the patient had to use a facemask during the night to maintain the results (Figs. 2F, G), then he continued orthodontic treatment to align the arches. When he was 18 years old, he underwent definitive nose reconstruction with

FIGURE 2. A, Concave face profile with skeletal class III. B, Scar in his forehead, in the lip, and around the nose. C, Occlusion before distraction. D, Patient during consolidation phase with external distractor and overcorrection of the midface. E, Patient with bone-to-bone distraction system with infraorbital and maxillary retention plates. F, Preoperative cephalometry, which shows midface retrusion. G, Postoperative cephalometry, which shows overcorrection achieved with distraction.

This is a girl without family history of congenital malformations or inherited disorders. The patient was admitted to our department at the age of 8 years, with a diagnosis of total arrhinia. A right cheiloplasty was done in another center as well as choanal permeabilization. During the physical examination, a left microphthalmia, left telecanthus, complete absence of nose structures, and a decreased midfacial vertical height were evident (Fig. 4A), as well as poor malar projection, dental crowding in the upper arch with skeletal class III (Fig. 4B), and cross and open anterior bite with an inverted V shape of the maxilla (Fig. 4C) with an interincisor distance of 14 mm. The computed tomography scan showed a collapsed maxilla with no maxillary sinuses, superior arc crowding, absence of the nasal septum, and distorted ethmoid cells (Figs. 4D, E). Orthodontic treatment was initiated with a palatal transverse expansion using HYRAX, and then an LF III osteotomy without coronal incision was performed using a modified approached through the nasofrontal junction (Fig. 4F), subciliary, and vestibular areas. The retention plates were located in the upper level of the frontonasal edge with an angulation of 45 degrees and over the hypoplastic maxilla, leaving the 3 distraction screws on the same horizontal bar, resulting in a distraction force, which allowed a clockwise rotation of the entire midface. The latency period was 5 days; distraction was performed for 14 days, rotating 0.5 mm every 12 hours to achieve a 14-mm distraction distance. The consolidation period was 9 weeks. With this pattern of distraction, we achieved a better anteroposterior position of the maxilla and a clockwise rotation of the midface, which generated a partial bite closure with

FIGURE 3. A, Adequate midface, dorsum and tip projection. B, Patient with adequate nasal reconstruction dimensions. C, Final occlusion.

* 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

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Prada and Mendoza

FIGURE 4. A, Patient with total arrhinia and left microphthalmia. B, Midface retrusion and skeletal class III. C, Cross and open anterior bite and inverted VYshaped maxilla. D, Three-dimensional reconstruction of computed tomography with collapsed and inverted VYshaped maxilla. E, Three-dimensional reconstruction of computed tomography lateral view, evident midface retrusion. F, Incision design on skin fold at the nasofrontal area.

a decreased interincisal distance of 8 mm. In addition, an elongation of the nasofrontal bone was observed, creating a nasal dorsum (Figs. 5A, B), which at the time of the distractor’s removal was visualized as a consolidated bone (Figs. 5F, G).

DISCUSSION The goal of this treatment, which includes LF III osteotomy with DO, is to make a definitive nasal reconstruction with a maxilla in a correct position. The ideal time for nose reconstruction in hemiarrhinia or arrhinia is during adolescence; however, an initial reconstruction during preschool age is required to achieve social integration.5,9 Patient 1 arrived to our department before preschool age. We started treatment that included reconstruction during childhood with a second surgery when the facial growth is completed. Both patients required a previous forehead tissue expansion with special care during the first surgery to avoid injury to the contralateral pedicle. The cartilage was taken from the auricular concha or rib, preserving the other for the final reconstruction. The hemiarrhinia forehead flap is also used to reconstruct the ala rim or nasal tip. After the initial reconstruction, additional procedures may be required because of the nose’s growth. If the patient is admitted into our department during school age without nasal reconstruction, the first procedure performed is an LF III osteotomy with DO using an external rigid device, to begin the procedures in the maxilla obtaining a better position and dimensions. We think that this could allow a better and more stable aesthetic result. Le Fort III osteotomy was introduced by Paul Tessier and has been widely accepted for midface hypoplasia treatment and correction of functional and aesthetic problems not just for syndromic patients but also in different midface growth alterations.18 Tessier14Y17 described as LF III osteotomy objectives the following: restore normal facial projection, allow normal occlusion, increase vertical midface dimension, and correct exorbitism. He also described 3 modifications of this osteotomy varying the location of the osteotomy at the lateral orbital wall14Y16; since then, different modifications have been described.26Y28 In our group, we performed an osteotomy from the lower and external corner of the orbital floor to the zygoma body as shown in Figure 5F. This avoids the palpable step at the lateral wall level and allows to advance the body of zygoma providing adequate midface projection.

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After DO, patients may require orthognathic surgery or a new distraction to achieve an adequate profile and occlusion. Our first patient did not require any additional surgery when facial growth was completed because of the age in which the LF III osteotomy was performed and because overcorrection with DO prevented a pseudorelapse. External distractors allow a better control of the distraction vector, making it more effective and precise, and also have the possibility to change force vectors during the distraction period.18 For an LF III osteotomy, 2 fixing points allow a proper control and a more predictable result.19 For this reason, we always prefer to place a bilateral retention plate in the infraorbital rim and a lower attachment point in the maxilla bilaterally as was shown in our first patient. This fixation allows a better control of the center of resistance and an efficient distraction. Occasionally, in patients in whom there is an important alteration of the anatomy and density of the maxilla, as in patients with arrhinia, the vector management is a challenge.10 The nasofrontal area acts as a pivot point for counterclockwise rotation, which is an adverse movement in the distraction phase.12,19,29 In the second patient presented, a modification was made placing a retention plate angled at 45 degrees of the nasofrontal area to get a vertical control and achieve a clockwise rotation of sagittal midface advancement to correct not just anteroposterior and vertical dimensions, but also the open bite. This modification was previously used in patients with syndromic midface retrusion without nose abnormalities. In those patients, the complete bite closure was not achieved, and the nose elongated, which is an undesirable aesthetic result.30 In our patient, the elongation of the nasofrontal bone allowed a bone framework development, which formed the nasal dorsum. Full closure of bite was not achieved because of the maxilla inverted ‘‘V’’ shape. In these patients, it is not always possible to correct the dental arch discrepancies30 because of the canine’s absence.5 The result in our second patient confirms that varying the point of force’s vector application and direction with the external distractors, the control of the final midface position can be achieved.12 There is an important psychological impact when using an external distractor,18,29 but our patients had good adherence to treatment and no complications. In some protocols, the first step for nasal reconstruction is the nasal passage reconstruction, in an attempt to allow nasal ventilation and improve speech.5,31 We never canalized, reconstructed, or permeabilized nasal passage, because no nasal passage has been shown

FIGURE 5. A, Retention plate at the nasofrontal edge with an angle of 45 degrees. B, Scar over the newly formed bone, which corresponds to the nasal dorsum. C, Evident formation of nasion and projected nasal dorsum. D, Appropriate malar and nasal bone frame projection. E, Anterior open bite, inverted VYshaped maxilla, and decreased distance between the incisor edges. F, Retention plate displacement and nasofrontal bone segment distraction, the arrow shows osteotomy over the zygomatic body.

* 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

The Journal of Craniofacial Surgery

& Volume 25, Number 3, May 2014

to be functional with objective measurements, as per nasometry. Nasal physiology shows us that nasal passage is not just a duct for the passage of air but is also a cavity with a specialized epithelium and dynamic structures such as the turbinates. These kinds of structures are impossible to reconstruct. In contrast, a nonfunctional cavity with dryness and stenosis problems is obtained.5,12,32 During birth, these patients develop respiratory distress; in these cases, they have to be treated with tracheostomy and gastrostomy9,31; not all of the children showed respiratory distress. Apparently, the nasal airway absence appears to be compatible with life once the child develops an oral breathing adaptation.32

CONCLUSIONS Appropriate midface position before definitive nose reconstruction in patients with arrhinia could be achieve with LF III osteotomy with DO during childhood or adolescence, allowing a better and more stable aesthetic result.

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Distraction Osteogenesis in Arrhinia

14. Tessier P. Total facial osteotomy. Crouzon’s syndrome, Apert’s syndrome: oxycephaly, scaphocephaly, turricephaly. Ann Chir Plast 1967;12:273Y286 15. Tessier P. The definitive plastic surgical treatment of the severe facial deformities of craniofacial dysostosis. Crouzon’s and Apert’s diseases. Plast Reconstr Surg 1971;48:419Y442 16. Tessier P. Total osteotomy of the middle third of the face for faciostenosis or for sequelae of Le Fort 3 fractures. Plast Reconstr Surg 1971;48:533Y541 17. Tessier P, Guiot G, Rougerie J, et al. Cranio-naso-orbitofacial osteotomies. Hypertelorism. Ann Chir Plast 1967;12:103Y118 18. Nout E, Cesteleyn LL, van der Wal KG, et al. Advancement of the midface, from conventional Le Fort III osteotomy to Le Fort III distraction: review of the literature. Int J Oral Maxillofac Surg 2008;37:781Y789 19. Figueroa AA, Polley JW, Figueroa AL. Introduction of a new removable adjustable intraoral maxillary distraction system for correction of maxillary hypoplasia. J Craniofac Surg 2009;20:1776Y1786 20. Figueroa AA, Polley JW, Figueroa AD. Biomechanical considerations for distraction of the monobloc, Le Fort III, and Le Fort I segments. Plast Reconstr Surg 2010;126:1005Y1013 21. Mofid MM, Manson PN, Robertson BC, et al. Craniofacial distraction osteogenesis: a review of 3278 cases. Plast Reconstr Surg 2001;108:1103Y1114 22. Cohen SR, Holmes RE. Internal Le Fort III distraction with biodegradable devices. J Craniofac Surg 2001;12:264Y272 23. McCarthy JG, Stelnicki EJ, Mehrara BJ, et al. Distraction osteogenesis of the craniofacial skeleton. Plast Reconstr Surg 2001;107:1812Y1827 24. Phillips JH, George AK, Tompson B. Le Fort III osteotomy or distraction osteogenesis imperfecta: your choice. Plast Reconstr Surg 2006;117: 1255Y1260 25. Swennen G, Schliephake H, Dempf R, et al. Craniofacial distraction osteogenesis: a review of the literature: Part 1: clinical studies. Int J Oral Maxillofac Surg 2001;30:89Y103 26. Fearon JA. The Le Fort III osteotomy: to distract or not to distract? Plast Reconstr Surg 2001;107:1091Y1103 27. Kobus KF. New osteotomies for midface advancement in patients with Crouzon syndrome. J Craniofac Surg 2006;17:957Y961 28. Mavili ME, Tuncbilek G. Seesaw modification of the lateral orbital wall in Le Fort III osteotomy. Cleft Palate Craniofac J 2004;41: 579Y583 29. Gosain AK, Santoro TD, Havlik RJ, et al. Midface distraction following Le Fort III and monobloc osteotomies: problems and solutions. Plast Reconstr Surg 2002;109:1797Y1808 30. McGlone L. Congenital arrhinia. J Paediatr Child Health 2003;39: 474Y476 31. Meyer R. Total external and internal construction in arrhinia. Plast Reconstr Surg 1997;99:534Y542 32. Mu¨hlbauer W, Schmidt A, Fairley J. Simultaneous construction of an internal and external nose in an infant with arrhinia. Plast Reconstr Surg 1993;91:720Y725

* 2014 Mutaz B. Habal, MD

Copyright © 2014 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.

891

Use of distraction osteogenesis in arrhinia.

Nasal malformations such as hemiarrhinia and arrhinia have a very low incidence, although many treatment protocols have been described. In this articl...
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