357
Nasal Reconstruction in Children Helmut Fischer, MD1
Wolfgang Gubisch, MD, PhD1
1 Department of Facial Plastic Surgery, Centre for Plastic Surgery,
Marienhospital Stuttgart, Stuttgart, Germany Facial Plast Surg 2014;30:357–364.
Abstract
Keywords
► nasal reconstruction ► nasal growth ► pediatric rhinoplasty surgery ► children
Address for correspondence Dusanka Jurkovic, MD, Department of Facial Plastic Surgery, Centre for Plastic Surgery, Marienhospital Stuttgart, Böheimstr. 37, D-70199 Stuttgart, Germany (e-mail: [email protected]).
The nose represents the center of the face. The nasal shape changes with the progression from infancy to adulthood. In newborns, the main supporting structure of the nose is the dorsal septal cartilage; it is the facial growth center. The loss of septal cartilage at different ages leads to various facial syndromes involving the nose, maxilla, and orbita. Therefore, every surgical treatment can compromise not only the nasal growth but also the growth of the whole face. Childhood trauma or a malformation of the nose can also cause enormous functional impairments similar to those caused by surgical treatments. Our contribution represents different aspects of nasal deformities and their treatment. An individual concept is indispensable to reach the best compromise in all cases. The postulate of an early treatment gives priority to physical and mental damage control. The late therapy concept uses the inimitable potential of the growth of different tissues needed for a septoplasty and should be protected from iatrogenic impairment. We recommend a first-line pediatric nasal reconstruction, an attentive follow-up, and finally, a secondary nasal reconstruction in the adult patient if required for achieving normalcy.
The nose represents the center of the face. In addition to the function of warming, cleaning, and moistening the respiratory air, it is important for the appearance. The nasal shape changes with the progression from infancy to adulthood. In newborns, the main supporting structure of the nose is the dorsal septal cartilage. This is a T-bar-formed complex made of septal and upper lateral cartilage, which is basically an external extension of the cartilage of the anterior cranial base. The anatomic structure gradually changes.1,2 The cartilaginous septum is the growth center. The loss of septal cartilage at different ages leads to various facial syndromes involving the nose, maxilla, and orbita.3 Therefore, every surgical treatment can compromise not only the nasal growth but also the growth of the whole face. A childhood trauma or a malformation of the nose can also cause enormous functional impairments similar to those caused by surgical treatments. Thus, nasal surgery in children requires special knowledge of the nasal growth and development, and also of the various surgical options that are available. Nasal reconstruction in children raises specific questions4,5: When should the reconstruction be initiated? Will
Issue Theme Nasal Reconstruction; Guest Editor, Helmut Fischer, MD
the reconstructed tissue and the transplants grow in proportion to the developing face? Is a final reconstruction planned in adulthood which raises the necessity of saving considerable future donor sites? When the available literature is analyzed, we see that no large studies have been made on this subject except for OrtizMonasterio and Olmedo.5 All the other authors presented single case reports without any long-term follow-up. Thus, all of these case reports should be considered carefully. The following list is an overview of the most frequent surgical problems involving nasal reconstruction in children. We want to present an overview of the most frequent surgical problems of nasal reconstruction in children: 1. Congenital malformations and acquired defects 2. Hemangioma and vascular malformations 3. Nostril stenosis.
Principles of Rhinoplasty Surgery in Children The shape of an infant’s nose is different from the shape of the nose of an adult. The neonates face demonstrates less frontal
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DOI http://dx.doi.org/ 10.1055/s-0034-1376879. ISSN 0736-6825.
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Dusanka Jurkovic, MD1
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projection of the nose, a shorter nasal dorsum and columella, a larger nasolabial angle and rounder nares. Until adolescence, not only are there changes in the nose but there are also changes in the whole profile of the face. The whole profile becomes prominent, and at the same time, the middle and lower third of the face become longer. However, the growth of the nose is not completed during adolescence, but for planning the surgical treatment, this is negligible.6–8 The septum represents the growth center of the face. There are two relevant zones for growth: the so-called sphenospinal zone and the sphenodorsal area.3,9 Growth in the sphenodorsal zone appears to be primarily responsible for the normal increase in length and height of the dorsum. The sphenospinal zone is responsible for the sagittal growth and is the driving force in forward outgrowth of the premaxillary region. A morphogenetic function in facial development for the thinnest, ventrocentral, part of the septum and the septal leading-edge has not been found. A nasal trauma of the sphenodorsal zone may cause a saddle nose deformity. If the sphenospinal zone gets harmed, the development of the premaxilla and the midface is deformed.10
Congenital Malformation and Acquired Defects Congenital malformations include nasal encephaloceles, gliomas, and dermoid cysts, which can appear in the area of the dorsum, nasal tip, and columella. They are internally located and can extend until the skull base and frontal brain. Our focus is on the congenital malformations of the external nose. Like cysts and fistulas, congenital malformations are the result of a fetal development disorder in the first 12 weeks of pregnancy. Denecke and Meyer created the first systemic characterization of the surgical treatment options in 1964.11 Losee et al12 developed a comprehensive classification scheme dedicated to congenital nasal anomalies on the base of the Whitaker classification of craniofacial anomalies.13 The classification is based on etiological, anatomical, and therapeutic principles. The congenital nasal deformities were classified into four categories and a 22-year retrospective review with 261 cases was performed. All congenital nasal anomalies were classified into following scheme12: Type Type Type Type
I. Hypoplasia and atrophy II. Hyperplasia and duplications III. Clefts IV. Neoplasms and vascular anomalies.
Type I was seen most frequently with hypoplasia and atrophy of the different tissues, represents paucity, atrophy, or underdevelopment of skin, subcutaneous tissue, muscle, cartilage, and/or bone often in combination with hemifacial hypoplasia, often in context with some craniofacial syndromes such as Apert syndrome or Fraser syndrome. The Binder syndrome, a malformative midface alteration, is also included (►Fig.1). Aplasia is a rarity. The reconstruction of the external and internal nose was described by Mühlbauer et al,14 Meyer,15 Burget,16 and Pittet and Montandon.17 Cho et al18 prefer, after an initial intubation or tracheotomy, an Facial Plastic Surgery
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Fig. 1 (A) A 15-year-old girl with Binder syndrome and a severe septal deviation with nasal breathing impairment. The nasal reconstruction was performed by reconstructing a stable L-shaped septal framework out of rib cartilage. In the same procedure, the maxilla was augmented. (B) A very satisfying result after 2 years.
osseointegrated prosthesis for achieving a satisfactory plastic result. Type II was represented with 1% of their clientele, including the proboscis lateralis. External duplications were related to communicating nasal cavity, pyriform aperture anomalies, or choanal dysplasia. In the type III category of clefts, the comprehensive and widely used Tessier classification of craniofacial clefts was applied.19 Facial clefts that only involve the nose are classified as Tessier 0, 1, 2, and 3, and their cranial extensions are classified as Tessier 11, 12, 13, and 14. Tessier 0 represents the most common cleft deformity. The microform of this cleft may present as a mild nasal bifidity dysplasia20,21 or the median facial cleft syndrome (including orbital hypertelorism).22,23 Tessier 2 contains, as microform, the common cleft lip and cleft lip nasal deformity with deformation of the ala (►Fig. 2) and the rare extended form with involvement of the ethmoid region resulting in telecanthus and orbital hypertelorism. Tessier 3 cleft obtains the orbita. Type 4 deformities consist of benign and malignant neoplasms including the vascular deformities (►Fig. 3). Patients with nasal anomalies should be therapeutically supervised until they are fully grown up. An accompanying choanal atresia requires an early surgical intervention. Oral and/or craniofacial surgical corrections have, in most of the cases, priority before the definitive nasal correction or reconstruction should be realized. These nasal corrections demand a treatment concept with the greatest effectiveness and the least damage like growth delay, secondary scar deformities, or custodial problems.
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Acquired Nasal Defects
Fig. 2 (A) Congenital partial cleft with a huge defect at the left ala in the area of the soft triangle. (B) Result of primary nasal reconstruction with a composite graft from the ear and a local flap performed elsewhere at the age of 4 months, the result still shows a defect of the soft triangle. At the age of 2 years, we performed a secondary ala reconstruction with a cartilage graft from the right concha, and the skin defect was reconstructed with a local transposition flap from the dorsum. Results 1 (C1–2) and 4 years (D1–2) after the operation. The reconstructed tissue showed a growth tendency. As we still expect a growth deficit of the left ala, we suggest a final ala reconstruction when the patient is grown up.
Acquired nasal defects after tumor resection or trauma in children require nasal reconstruction in preschool or early school age. According to the functional development, the growth aspect of the reconstructed structures, for example, a nasal wing or vestibule, cannot be predicted. Thin-walled, silicon olives to prevent shrinking in the course of scarring are helpful. The stents can also be used for widening step by step. In the case of scarring or impaired growth of the reconstructed structures, a second reconstruction is necessary in adolescence. The inner lining can be replaced with skin or a buccal mucosa grafts. The cartilaginous frame can be reconstructed with cartilage from the ear or rib in a second stage after stabilization of the lining graft. A paramedian forehead flap can be useful for reshaping the skin. If a forehead flap was used for the infantile nasal reconstruction and a second flap is necessary in adolescence, a previous expansion is essential. In convenient cases, a final reconstruction can be performed during school age. The rebuilt structures should be larger and anticipate nasal growth. Even rib cartilage can be used in school age, according to Gurley et al examination of 32 patients with a mean age of 8.8 years over 7.9 years.24 Our experience with prosthetic replacement is limited and less promising. Young patients’ tolerance of foreign bodies in their faces is less than that of adults.
Fig. 3 (A) A young boy was born with a giant pigmented nevus affecting the whole nose, small parts of the left and a big area of the right cheek. (B) The first nevus resection was performed at the age of 6 months. At the age of 3 years, the nevus excision was completed. (C) A nonexpanded forehead flap was used for the reconstruction of the nasal skin. (D) The inconspicuous appearance 3 years later. We discussed a scar revision before school enrollment. Facial Plastic Surgery
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Median cleft noses with a wide dehiscence of the nasal tip and the medial crura can eventually, in combination with a wide bony broad nose or subcutaneous split nasal bone, be corrected via an open or closed rhinoplasty with adaption of the cartilaginous, and if necessary, bony framework during the infancy. A skin excess can be resected vertically spindle shaped in a second step if required. Lateral cleft nose deformities may include a default of the nasolacrimal system, starting from the ala to the orbita, if the occlusion growth plate is absent. The microform just affects the nasal ala which can be corrected with an auricular composite graft. Here too, the final reconstruction should be performed when growth is completed.
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Surgical Treatment of Nasal Hemangioma and Vascular Deformities Hemangioma Hemangiomas are the most common tumors in infancy and childhood with an estimated prevalence of 10% by 1 year of age. They consist of a cellular proliferation of endothelial cells and associated pericytes.25 A characteristic of hemangioma is a tumor-like growth in the 1st year. Afterward, it regresses to a residual state or resolves spontaneously. The biological classification proposed by Mulliken and Glowacki was a groundbreaking step toward understanding the nature of hemangioma and vascular malformations.25 Thirty percent of hemangiomas will be evident at birth and others will present within the first few weeks of life.26 The proliferation is divided in two phases. If the tumors are located in the labial, nasal, and periorbital areas, they can cause a threatening effect on the closure of the mouth, breathing, and visual impairment. The second, and less dramatic growth period, has its peak in the middle of the 1st year of life and terminates at the end of the 1st year.27 These features distinguish hemangiomas from vascular malformations that neither involute nor proliferate. Most hemangiomas involute completely and therefore may require no treatment. There are two types of regression with a spreading of 50:50. The first form involutes until the 5th year of life and 80% of those have excellent long-term results without treatment. The second form involutes until the 10th year of life and reaches just 40% of good long-term results.28 The manifestation of hemangioma is divided into superficial, deep, and compound types. The superficial hemangiomas are in the papillary dermis and are also called cutaneous hemangiomas. Those within the reticular dermis or subcutaneous tissue are known as deep hemangiomas. If both components are presented simultaneously in a lesion, it is called a mixed or compound hemangioma.26,29 On the nose, hemangiomas can be located in every area. One distinctive localization, and primary example, is the nasal tip. The subcutaneous tissue is often involved. During proliferation, it expands, distracts, and deviates nasal cartilage leading to a bulbous nose; the so-called Pinocchio or Cyrano nose. It is still controversial how and when hemangioma should be treated. The risk of severe complications such as irreversible contour deformities, growth disturbance, ulcerations, and nasal obstruction may make a therapeutic intervention necessary. As nonsurgical treatments, therapy with propranolol, laser treatments, cryotherapy, or intralesional corticosteroid injections is mentioned. The first-line therapy for infantile hemangioma is oral propanolol, a nonselective β-adrenergic receptor antagonist. Kum and Khan show that propanolol inhibits growth of hemangioma-initiating cells but does not induce apoptosis.30 Potential explanations for the therapeutic effect of propranolol on infantile capillary hemangiomas include vasoconstriction, which is immediately visible as a change in color associated with a palpable softening of the hemangioma; decreased expression of vascular endothelial growth factor Facial Plastic Surgery
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and β fibroblast growth factor) genes through the downregulation of the Raf–mitogen-activated protein kinase pathway31 (which explains the progressive improvement of the hemangioma) and the triggering of apoptosis of capillary endothelial cells.32 Lou et al meta-analyzed 35 studies on the effectiveness of propanolol in treating infantile hemangiomas. Regarding efficacy, a strong evidence for propanolol as a first-line therapy for infantile hemangiomas has been provided.33 The interstitial Neodymium:YAG laser treatment causes a scarification by coagulation of the subcutaneous part of the hemangioma. In comparison to cryotherapy, total regression can be achieved on small superficial hemangiomas with less impairment of the epidermis with the use of the flashlamppumped pulsed dye laser.34 Intracutaneous hemangioma should be treated restrictively because of the danger of deforming scars which can make a total nasal tip reconstruction necessary. The subcutaneous hemangioma carries on growing. Many publications deal on the subject of nasal tip hemangioma and their treatment. Amer et al35 recommend a nonsurgical treatment with a laser or an intralesional corticosteroid injection in three to four steps until the end of the 2nd year of life after diagnosing a hemangioma. A total regression can be achieved in one-third of the cases, while the others have a good initial situation for surgical treatment. This can be started at the beginning of the 3rd year of life with an open rhinoplasty with the intention to complete the therapy before school age. This allows the patient to overcome the psychological complications associated with the lesions. The open rhinoplasty incision can be done over the columella and extended to bilateral alar rim incisions. There is a risk of a contour deficiency at the columella or infratip region. An alternative to the alar rim incisions is the incision at the margins of the nasal subunits; thus, laterally at the tip unit,36 there is a risk of retraction of the alar soft triangle. Most often hemangioma of the nasal tip expands the healthy surrounding skin, which makes the operative treatment easier. Hemangioma can be dissected optimally via an open approach. The expanded alar cartilage and the domes can be adapted and the skin containing the hemangioma can be resected carefully through adaptation of the incision lines. An optimal shape has first priority. Even a whole resection of the hemangioma tissue is possible. A laser therapy can optimize the outcome later on. In case of deforming skin and tissue damages after an aggressive primary treatment, a reconstruction using a forehead flap of the whole esthetic unit of the tip area is necessary, even in elementary school age.4 In the past, we favored an expander implantation before transposing a forehead flap in children and adults,37–39 but nowadays, in case of enough hairless frontal skin, we abandoned the forehead expansion. A main reason for further operation of combined hemangioma is a transformation of the mesenchymal stem cells into fibrofatty tissue.40 The plastic-surgical correction can be realized in two steps: first an improvement of the disfigurement
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Fig. 4 Patient with a hemangioma at the nasal tip and temple in the 1st weeks of life. Afterward, an excision of the temporal hemangioma and a magnesium wiring was applied four times at the nasal tip in the 1st year of life. (A) Thereby, a regression of the hemangioma could be achieved, but also a necrosis of the nasal tip and columella was caused. At the age of 6, the forehead skin was expanded and used for reconstruction of the nasal tip and the columella 3 months later. In the adolescence, an improvement of the tip projection was planned. (B) The patient, today 20 years old, unfortunately was lost for follow-up.
should be performed with the goal to preserve the shape and surface, even if hemangioma infiltrated skin is left. The second and final step of the reconstruction and aesthetic operation should be conducted after the end of the process of growth under optimal conditions (►Figs. 4 and 5).
Craniofacial Vascular Malformations Mulliken and Glowacki proposed a classification of vascular anomalies based on histology, histochemistry, and clinical
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behavior.25 The vascular malformations represent an endothelial cell proliferation and a developmental error with abnormally formed vascular channels.41 These lesions are usually noted at birth, grow proportionately with the child, and consist of abnormal capillary, arterial, venous, and lymphatic vascular elements. In a retrospective review of 81 extracranial arteriovenous malformations of the head and neck, 10% of which were in the area of the nose, a follow-up over 20 years was realized.42 A vascular anomaly was apparent at birth in 59% of the patients; in the others, it appeared in childhood (10%), in adolescence (10%), and in adulthood (21%). According to Schobinger clinical staging, 27% of the arteriovenous malformations were in the quiescence stage, 38 in the expansion stage, and 38% in the destruction stage. There was only one patient in the decompensation stage. Interdisciplinary teams are essential for the therapy of vascular malformations.43–46 Interventional radiological procedures have evolved to an essential element in an interdisciplinary treatment plan for vascular malformations. They include percutaneous sclerotherapy with ethanol and OK-432 for venous and lymphatic malformations and transarterial embolization for high-flow lesions.45 A transarterial embolization is often necessary to enable a surgical treatment. However, the embolization alone cannot solve the substantial aesthetic problem.47 After an embolization, the surgical treatment should be performed as soon as possible to prevent a growth of the nidus and make a complete resection feasible. Knowledge about the vascular anatomy, the options of the operative approach, and the best technique for the reconstruction are obligate requirements.
Case Report
Fig. 5 (A) A 9-year-old girl with loss of the nasal tip after laser therapy of a hemangioma. The nasal reconstruction was realized with short local hinge flaps for lining, rib cartilage for central L-strut and ala cartilages and a non-expended forehead flap. (B) The result 1 year after the reconstruction. Assuming nasal growth will be reduced for some degree, the neo tip was designed oversized.
At the time of the nose reconstruction, the patient was 7 years old. He was born with a hemangiopericytoma broadly connected to the right lateral nasal wall and to its base in the area of the right ethmoid sinuses and right frontal skull base. Although the nose and the anterior wall of the right maxillary sinus were compressed, the nasal pyramid, including its bony and cartilaginous parts, was developed. The huge vascular tumor was diagnosed during the sonographic examination of the mother. A primary cesarean section was performed during the 33rd week of pregnancy and after a stable cardiorespiratory state was established, the tumor was laser resected. This case was reported and published by Götte et al.52 Before school enrollment, the young patient presented for a nose reconstruction in our department. Defects caused by the primary resection were found as a cleft at the right lateral nasal wall, at the dorsum, and both alae. The right ala had a well-placed base, but the apical part was cephalically rotated and fixed at the medial angle of the right eye. A preschool reconstruction was necessary because of psychological reasons. The cartilaginous framework of both alae, the right nasal wall, and the dorsum was reconstructed with conchal cartilage from both ears. The septum was extended with septal cartilage from the thin central part, local skin pivot flaps were used for the reconstruction of the inner lining. A nonexpanded forehead flap was necessary to cover the new nose. The intention, according to the age of the patient, was to Facial Plastic Surgery
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create an oversized nose to compensate for an expected growth deficit of the reconstructed structures, which could possibly be caused by scarring. The intermediate result at the age of 11 years was satisfying. Social integration and school development were fine. Ten years after the reconstruction, a growth of the rebuilt structures could be noted. A further operation will be necessary to optimize the symmetry and correct the scars (►Fig. 6).
Nostril Stenosis Nostril stenosis in children is an infrequent finding that often has an iatrogenic cause. A pressure ulcer can be caused by therapy with oversized rigid packing or supportive care in neonates. The healing of the ulcer results in scars. The tissue shrinks with the process of scarring.48 Such scars lead to nasal airway obstruction and breathing difficulties during feeding. The growth of the external nasal valve area is impaired. We recommend an early surgical correction, optimally, before school enrollment. There are many treatment strategies, such as the correction with flaps based on the principle of a threedimensional z-plasty, but with insufficient results. We prefer an excision of the scarred tissue and a reconstruction with a
Fig. 7 (A-1,2) A 1-year-old girl with a severe stenosis of the left nostril after resection of a benign tumor (hamartoma). The correction was performed by cutting the circular scar, and inserting a local flap according to a modified z-plasty. (B-1) After 1 year, a very satisfactory result could be obtained. (B-2) After the treatment, a stent and steroid ointment were used.
local flap or a skin graft. An alternative to skin grafting is auricular composite grafting, which is more resistant against shrinking but prone to heal incompletely. Moreover, a deformity can be caused at the donor site on the ear. There are no long-term reviews confirming a growing of the composite graft with the growth of the nose. How to involve a “boomerang flap” for reconstruction was described by Bozkurt et al. It was used in combination with a nasolabial flap, a vestibular rotation flap, and a conchal cartilage graft to achieve a satisfying repair.49 Blandini et al recommend a stable correction by using a “piercing” flap taken from the labial vestibule transferred to the nasal floor.50 In case of surgical treatment of vestibular stenosis, the use of a custom-made vestibular device such as thinly walled stents with increasing diameter and intermediate sponge packing with an expanding effect may help prevent restenosis.50,51 Supportive local application of cortisone or mitomycin C should be conducted48 (►Fig. 7).
Conclusion
Fig. 6 (A) Nasal defect after perinatal resection of a huge congenital hemangiopericytoma at the age of 4. (B) Four years after the nasal reconstruction. (C) The 16-year-old patient with a harmonic proportion of the nose to the residual face. Facial Plastic Surgery
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In our contribution, we represent different aspects of nasal deformities and their treatment. In all cases, an individual concept is indispensable to reach the best compromise. The postulate of an early treatment gives priority to physical and mental damage control. The late therapy concept attempts to use the inimitable potential of the growth of the different tissue which is needed for a septoplasty, and should be protected from iatrogenic impairment. One can find benefits for a treatment in both phases, during infancy and after
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18 Cho CH, Shakibaei M, Merker HJ, Klein M. The rare malformation of
19 20
Acknowledgment We thank all of the patients for the permission to use their photographs to illustrate the treatment concepts discussed in this article.
Conflict of Interest The authors declare that they have no conflict of interest as defined by the guidelines of the International Committee of Medical Journal Editors.
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completion of growth, to reach a clearly defined target. So, we recommend a first-line pediatric nasal reconstruction, an attentive follow-up, and finally, a secondary nasal reconstruction in the adult patient if required for achieving the normal.
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40 Yu Y, Fuhr J, Boye E, et al. Mesenchymal stem cells and adipo-
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genesis in hemangioma involution. Stem Cells 2006;24(6): 1605–1612 Folkman J, D’Amore PA. Blood vessel formation: what is its molecular basis? Cell 1996;87(7):1153–1155 Kohout MP, Hansen M, Pribaz JJ, Mulliken JB. Arteriovenous malformations of the head and neck: natural history and management. Plast Reconstr Surg 1998;102(3):643–654 Jackson IT, Carreño R, Potparic Z, Hussain K. Hemangiomas, vascular malformations, and lymphovenous malformations: classification and methods of treatment. Plast Reconstr Surg 1993; 91(7):1216–1230 Kubiena H, Cejna M, Kreuzer S, Schoder M, Frey M. Interdisciplinary management of craniofacial vascular malformations. Eur J Plast Surg 2007;30:81–86 Ernemann U, Kramer U, Miller S, et al. Current concepts in the classification, diagnosis and treatment of vascular anomalies. Eur J Radiol 2010;75(1):2–11 Ernemann U, Hoffmann J, Breuninger H, Reinert S, Skalej M. Interdisciplinary concept for classification and treatment of vas-
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cular anomalies in the head and neck [in German]. Mund Kiefer Gesichtschir 2002;6(6):402–409 Lopez-Gutierrez JC, Ros Z, Perez-Higueras A. Giant vascular malformation of the face in a premature infant: complete resolution by embolization. J Pediatr Surg 1995;30(10):1519–1520 Smith LP, Roy S. Treatment strategy for iatrogenic nasal vestibular stenosis in young children. Int J Pediatr Otorhinolaryngol 2006; 70(8):1369–1373 Bozkurt M, Kapi E, Kuvat SV, Selçuk CT. Repair of nostril stenosis using a triple flap combination: boomerang, nasolabial, and vestibular rotation flaps. Cleft Palate Craniofac J 2012;49(6):753–758 Blandini D, Tremolada C, Beretta M, Mascetti M. Iatrogenic nostril stenosis: aesthetic correction using a vestibular labial mucosa flap. Plast Reconstr Surg 1995;95(3):569–571 Menger DJ, Lohuis PJ, Kerssemakers S, Nolst Trenité GJ. Postoperative management of nasal vestibular stenosis: the custom-made vestibular device. Arch Facial Plast Surg 2005;7(6):381–386 Götte K, Hörmann K, Schmoll J, Hiltmann WD. Congenital nasal hemangiopericytoma: intrauterine, intraoperative, and histologic findings. Ann Otol Rhinol Laryngol 1999;108(6):589–593
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Nasal Reconstruction in Children Helmut Fischer, MD1
Wolfgang Gubisch, MD, PhD1
1 Department of Facial Plastic Surgery, Centre for Plastic Surgery,
Marienhospital Stuttgart, Stuttgart, Germany Facial Plast Surg 2014;30:357–364.
Abstract
Keywords
► nasal reconstruction ► nasal growth ► pediatric rhinoplasty surgery ► children
Address for correspondence Dusanka Jurkovic, MD, Department of Facial Plastic Surgery, Centre for Plastic Surgery, Marienhospital Stuttgart, Böheimstr. 37, D-70199 Stuttgart, Germany (e-mail: [email protected]).
The nose represents the center of the face. The nasal shape changes with the progression from infancy to adulthood. In newborns, the main supporting structure of the nose is the dorsal septal cartilage; it is the facial growth center. The loss of septal cartilage at different ages leads to various facial syndromes involving the nose, maxilla, and orbita. Therefore, every surgical treatment can compromise not only the nasal growth but also the growth of the whole face. Childhood trauma or a malformation of the nose can also cause enormous functional impairments similar to those caused by surgical treatments. Our contribution represents different aspects of nasal deformities and their treatment. An individual concept is indispensable to reach the best compromise in all cases. The postulate of an early treatment gives priority to physical and mental damage control. The late therapy concept uses the inimitable potential of the growth of different tissues needed for a septoplasty and should be protected from iatrogenic impairment. We recommend a first-line pediatric nasal reconstruction, an attentive follow-up, and finally, a secondary nasal reconstruction in the adult patient if required for achieving normalcy.
The nose represents the center of the face. In addition to the function of warming, cleaning, and moistening the respiratory air, it is important for the appearance. The nasal shape changes with the progression from infancy to adulthood. In newborns, the main supporting structure of the nose is the dorsal septal cartilage. This is a T-bar-formed complex made of septal and upper lateral cartilage, which is basically an external extension of the cartilage of the anterior cranial base. The anatomic structure gradually changes.1,2 The cartilaginous septum is the growth center. The loss of septal cartilage at different ages leads to various facial syndromes involving the nose, maxilla, and orbita.3 Therefore, every surgical treatment can compromise not only the nasal growth but also the growth of the whole face. A childhood trauma or a malformation of the nose can also cause enormous functional impairments similar to those caused by surgical treatments. Thus, nasal surgery in children requires special knowledge of the nasal growth and development, and also of the various surgical options that are available. Nasal reconstruction in children raises specific questions4,5: When should the reconstruction be initiated? Will
Issue Theme Nasal Reconstruction; Guest Editor, Helmut Fischer, MD
the reconstructed tissue and the transplants grow in proportion to the developing face? Is a final reconstruction planned in adulthood which raises the necessity of saving considerable future donor sites? When the available literature is analyzed, we see that no large studies have been made on this subject except for OrtizMonasterio and Olmedo.5 All the other authors presented single case reports without any long-term follow-up. Thus, all of these case reports should be considered carefully. The following list is an overview of the most frequent surgical problems involving nasal reconstruction in children. We want to present an overview of the most frequent surgical problems of nasal reconstruction in children: 1. Congenital malformations and acquired defects 2. Hemangioma and vascular malformations 3. Nostril stenosis.
Principles of Rhinoplasty Surgery in Children The shape of an infant’s nose is different from the shape of the nose of an adult. The neonates face demonstrates less frontal
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DOI http://dx.doi.org/ 10.1055/s-0034-1376879. ISSN 0736-6825.
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Dusanka Jurkovic, MD1
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projection of the nose, a shorter nasal dorsum and columella, a larger nasolabial angle and rounder nares. Until adolescence, not only are there changes in the nose but there are also changes in the whole profile of the face. The whole profile becomes prominent, and at the same time, the middle and lower third of the face become longer. However, the growth of the nose is not completed during adolescence, but for planning the surgical treatment, this is negligible.6–8 The septum represents the growth center of the face. There are two relevant zones for growth: the so-called sphenospinal zone and the sphenodorsal area.3,9 Growth in the sphenodorsal zone appears to be primarily responsible for the normal increase in length and height of the dorsum. The sphenospinal zone is responsible for the sagittal growth and is the driving force in forward outgrowth of the premaxillary region. A morphogenetic function in facial development for the thinnest, ventrocentral, part of the septum and the septal leading-edge has not been found. A nasal trauma of the sphenodorsal zone may cause a saddle nose deformity. If the sphenospinal zone gets harmed, the development of the premaxilla and the midface is deformed.10
Congenital Malformation and Acquired Defects Congenital malformations include nasal encephaloceles, gliomas, and dermoid cysts, which can appear in the area of the dorsum, nasal tip, and columella. They are internally located and can extend until the skull base and frontal brain. Our focus is on the congenital malformations of the external nose. Like cysts and fistulas, congenital malformations are the result of a fetal development disorder in the first 12 weeks of pregnancy. Denecke and Meyer created the first systemic characterization of the surgical treatment options in 1964.11 Losee et al12 developed a comprehensive classification scheme dedicated to congenital nasal anomalies on the base of the Whitaker classification of craniofacial anomalies.13 The classification is based on etiological, anatomical, and therapeutic principles. The congenital nasal deformities were classified into four categories and a 22-year retrospective review with 261 cases was performed. All congenital nasal anomalies were classified into following scheme12: Type Type Type Type
I. Hypoplasia and atrophy II. Hyperplasia and duplications III. Clefts IV. Neoplasms and vascular anomalies.
Type I was seen most frequently with hypoplasia and atrophy of the different tissues, represents paucity, atrophy, or underdevelopment of skin, subcutaneous tissue, muscle, cartilage, and/or bone often in combination with hemifacial hypoplasia, often in context with some craniofacial syndromes such as Apert syndrome or Fraser syndrome. The Binder syndrome, a malformative midface alteration, is also included (►Fig.1). Aplasia is a rarity. The reconstruction of the external and internal nose was described by Mühlbauer et al,14 Meyer,15 Burget,16 and Pittet and Montandon.17 Cho et al18 prefer, after an initial intubation or tracheotomy, an Facial Plastic Surgery
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Fig. 1 (A) A 15-year-old girl with Binder syndrome and a severe septal deviation with nasal breathing impairment. The nasal reconstruction was performed by reconstructing a stable L-shaped septal framework out of rib cartilage. In the same procedure, the maxilla was augmented. (B) A very satisfying result after 2 years.
osseointegrated prosthesis for achieving a satisfactory plastic result. Type II was represented with 1% of their clientele, including the proboscis lateralis. External duplications were related to communicating nasal cavity, pyriform aperture anomalies, or choanal dysplasia. In the type III category of clefts, the comprehensive and widely used Tessier classification of craniofacial clefts was applied.19 Facial clefts that only involve the nose are classified as Tessier 0, 1, 2, and 3, and their cranial extensions are classified as Tessier 11, 12, 13, and 14. Tessier 0 represents the most common cleft deformity. The microform of this cleft may present as a mild nasal bifidity dysplasia20,21 or the median facial cleft syndrome (including orbital hypertelorism).22,23 Tessier 2 contains, as microform, the common cleft lip and cleft lip nasal deformity with deformation of the ala (►Fig. 2) and the rare extended form with involvement of the ethmoid region resulting in telecanthus and orbital hypertelorism. Tessier 3 cleft obtains the orbita. Type 4 deformities consist of benign and malignant neoplasms including the vascular deformities (►Fig. 3). Patients with nasal anomalies should be therapeutically supervised until they are fully grown up. An accompanying choanal atresia requires an early surgical intervention. Oral and/or craniofacial surgical corrections have, in most of the cases, priority before the definitive nasal correction or reconstruction should be realized. These nasal corrections demand a treatment concept with the greatest effectiveness and the least damage like growth delay, secondary scar deformities, or custodial problems.
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Acquired Nasal Defects
Fig. 2 (A) Congenital partial cleft with a huge defect at the left ala in the area of the soft triangle. (B) Result of primary nasal reconstruction with a composite graft from the ear and a local flap performed elsewhere at the age of 4 months, the result still shows a defect of the soft triangle. At the age of 2 years, we performed a secondary ala reconstruction with a cartilage graft from the right concha, and the skin defect was reconstructed with a local transposition flap from the dorsum. Results 1 (C1–2) and 4 years (D1–2) after the operation. The reconstructed tissue showed a growth tendency. As we still expect a growth deficit of the left ala, we suggest a final ala reconstruction when the patient is grown up.
Acquired nasal defects after tumor resection or trauma in children require nasal reconstruction in preschool or early school age. According to the functional development, the growth aspect of the reconstructed structures, for example, a nasal wing or vestibule, cannot be predicted. Thin-walled, silicon olives to prevent shrinking in the course of scarring are helpful. The stents can also be used for widening step by step. In the case of scarring or impaired growth of the reconstructed structures, a second reconstruction is necessary in adolescence. The inner lining can be replaced with skin or a buccal mucosa grafts. The cartilaginous frame can be reconstructed with cartilage from the ear or rib in a second stage after stabilization of the lining graft. A paramedian forehead flap can be useful for reshaping the skin. If a forehead flap was used for the infantile nasal reconstruction and a second flap is necessary in adolescence, a previous expansion is essential. In convenient cases, a final reconstruction can be performed during school age. The rebuilt structures should be larger and anticipate nasal growth. Even rib cartilage can be used in school age, according to Gurley et al examination of 32 patients with a mean age of 8.8 years over 7.9 years.24 Our experience with prosthetic replacement is limited and less promising. Young patients’ tolerance of foreign bodies in their faces is less than that of adults.
Fig. 3 (A) A young boy was born with a giant pigmented nevus affecting the whole nose, small parts of the left and a big area of the right cheek. (B) The first nevus resection was performed at the age of 6 months. At the age of 3 years, the nevus excision was completed. (C) A nonexpanded forehead flap was used for the reconstruction of the nasal skin. (D) The inconspicuous appearance 3 years later. We discussed a scar revision before school enrollment. Facial Plastic Surgery
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Median cleft noses with a wide dehiscence of the nasal tip and the medial crura can eventually, in combination with a wide bony broad nose or subcutaneous split nasal bone, be corrected via an open or closed rhinoplasty with adaption of the cartilaginous, and if necessary, bony framework during the infancy. A skin excess can be resected vertically spindle shaped in a second step if required. Lateral cleft nose deformities may include a default of the nasolacrimal system, starting from the ala to the orbita, if the occlusion growth plate is absent. The microform just affects the nasal ala which can be corrected with an auricular composite graft. Here too, the final reconstruction should be performed when growth is completed.
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Surgical Treatment of Nasal Hemangioma and Vascular Deformities Hemangioma Hemangiomas are the most common tumors in infancy and childhood with an estimated prevalence of 10% by 1 year of age. They consist of a cellular proliferation of endothelial cells and associated pericytes.25 A characteristic of hemangioma is a tumor-like growth in the 1st year. Afterward, it regresses to a residual state or resolves spontaneously. The biological classification proposed by Mulliken and Glowacki was a groundbreaking step toward understanding the nature of hemangioma and vascular malformations.25 Thirty percent of hemangiomas will be evident at birth and others will present within the first few weeks of life.26 The proliferation is divided in two phases. If the tumors are located in the labial, nasal, and periorbital areas, they can cause a threatening effect on the closure of the mouth, breathing, and visual impairment. The second, and less dramatic growth period, has its peak in the middle of the 1st year of life and terminates at the end of the 1st year.27 These features distinguish hemangiomas from vascular malformations that neither involute nor proliferate. Most hemangiomas involute completely and therefore may require no treatment. There are two types of regression with a spreading of 50:50. The first form involutes until the 5th year of life and 80% of those have excellent long-term results without treatment. The second form involutes until the 10th year of life and reaches just 40% of good long-term results.28 The manifestation of hemangioma is divided into superficial, deep, and compound types. The superficial hemangiomas are in the papillary dermis and are also called cutaneous hemangiomas. Those within the reticular dermis or subcutaneous tissue are known as deep hemangiomas. If both components are presented simultaneously in a lesion, it is called a mixed or compound hemangioma.26,29 On the nose, hemangiomas can be located in every area. One distinctive localization, and primary example, is the nasal tip. The subcutaneous tissue is often involved. During proliferation, it expands, distracts, and deviates nasal cartilage leading to a bulbous nose; the so-called Pinocchio or Cyrano nose. It is still controversial how and when hemangioma should be treated. The risk of severe complications such as irreversible contour deformities, growth disturbance, ulcerations, and nasal obstruction may make a therapeutic intervention necessary. As nonsurgical treatments, therapy with propranolol, laser treatments, cryotherapy, or intralesional corticosteroid injections is mentioned. The first-line therapy for infantile hemangioma is oral propanolol, a nonselective β-adrenergic receptor antagonist. Kum and Khan show that propanolol inhibits growth of hemangioma-initiating cells but does not induce apoptosis.30 Potential explanations for the therapeutic effect of propranolol on infantile capillary hemangiomas include vasoconstriction, which is immediately visible as a change in color associated with a palpable softening of the hemangioma; decreased expression of vascular endothelial growth factor Facial Plastic Surgery
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and β fibroblast growth factor) genes through the downregulation of the Raf–mitogen-activated protein kinase pathway31 (which explains the progressive improvement of the hemangioma) and the triggering of apoptosis of capillary endothelial cells.32 Lou et al meta-analyzed 35 studies on the effectiveness of propanolol in treating infantile hemangiomas. Regarding efficacy, a strong evidence for propanolol as a first-line therapy for infantile hemangiomas has been provided.33 The interstitial Neodymium:YAG laser treatment causes a scarification by coagulation of the subcutaneous part of the hemangioma. In comparison to cryotherapy, total regression can be achieved on small superficial hemangiomas with less impairment of the epidermis with the use of the flashlamppumped pulsed dye laser.34 Intracutaneous hemangioma should be treated restrictively because of the danger of deforming scars which can make a total nasal tip reconstruction necessary. The subcutaneous hemangioma carries on growing. Many publications deal on the subject of nasal tip hemangioma and their treatment. Amer et al35 recommend a nonsurgical treatment with a laser or an intralesional corticosteroid injection in three to four steps until the end of the 2nd year of life after diagnosing a hemangioma. A total regression can be achieved in one-third of the cases, while the others have a good initial situation for surgical treatment. This can be started at the beginning of the 3rd year of life with an open rhinoplasty with the intention to complete the therapy before school age. This allows the patient to overcome the psychological complications associated with the lesions. The open rhinoplasty incision can be done over the columella and extended to bilateral alar rim incisions. There is a risk of a contour deficiency at the columella or infratip region. An alternative to the alar rim incisions is the incision at the margins of the nasal subunits; thus, laterally at the tip unit,36 there is a risk of retraction of the alar soft triangle. Most often hemangioma of the nasal tip expands the healthy surrounding skin, which makes the operative treatment easier. Hemangioma can be dissected optimally via an open approach. The expanded alar cartilage and the domes can be adapted and the skin containing the hemangioma can be resected carefully through adaptation of the incision lines. An optimal shape has first priority. Even a whole resection of the hemangioma tissue is possible. A laser therapy can optimize the outcome later on. In case of deforming skin and tissue damages after an aggressive primary treatment, a reconstruction using a forehead flap of the whole esthetic unit of the tip area is necessary, even in elementary school age.4 In the past, we favored an expander implantation before transposing a forehead flap in children and adults,37–39 but nowadays, in case of enough hairless frontal skin, we abandoned the forehead expansion. A main reason for further operation of combined hemangioma is a transformation of the mesenchymal stem cells into fibrofatty tissue.40 The plastic-surgical correction can be realized in two steps: first an improvement of the disfigurement
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Fig. 4 Patient with a hemangioma at the nasal tip and temple in the 1st weeks of life. Afterward, an excision of the temporal hemangioma and a magnesium wiring was applied four times at the nasal tip in the 1st year of life. (A) Thereby, a regression of the hemangioma could be achieved, but also a necrosis of the nasal tip and columella was caused. At the age of 6, the forehead skin was expanded and used for reconstruction of the nasal tip and the columella 3 months later. In the adolescence, an improvement of the tip projection was planned. (B) The patient, today 20 years old, unfortunately was lost for follow-up.
should be performed with the goal to preserve the shape and surface, even if hemangioma infiltrated skin is left. The second and final step of the reconstruction and aesthetic operation should be conducted after the end of the process of growth under optimal conditions (►Figs. 4 and 5).
Craniofacial Vascular Malformations Mulliken and Glowacki proposed a classification of vascular anomalies based on histology, histochemistry, and clinical
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behavior.25 The vascular malformations represent an endothelial cell proliferation and a developmental error with abnormally formed vascular channels.41 These lesions are usually noted at birth, grow proportionately with the child, and consist of abnormal capillary, arterial, venous, and lymphatic vascular elements. In a retrospective review of 81 extracranial arteriovenous malformations of the head and neck, 10% of which were in the area of the nose, a follow-up over 20 years was realized.42 A vascular anomaly was apparent at birth in 59% of the patients; in the others, it appeared in childhood (10%), in adolescence (10%), and in adulthood (21%). According to Schobinger clinical staging, 27% of the arteriovenous malformations were in the quiescence stage, 38 in the expansion stage, and 38% in the destruction stage. There was only one patient in the decompensation stage. Interdisciplinary teams are essential for the therapy of vascular malformations.43–46 Interventional radiological procedures have evolved to an essential element in an interdisciplinary treatment plan for vascular malformations. They include percutaneous sclerotherapy with ethanol and OK-432 for venous and lymphatic malformations and transarterial embolization for high-flow lesions.45 A transarterial embolization is often necessary to enable a surgical treatment. However, the embolization alone cannot solve the substantial aesthetic problem.47 After an embolization, the surgical treatment should be performed as soon as possible to prevent a growth of the nidus and make a complete resection feasible. Knowledge about the vascular anatomy, the options of the operative approach, and the best technique for the reconstruction are obligate requirements.
Case Report
Fig. 5 (A) A 9-year-old girl with loss of the nasal tip after laser therapy of a hemangioma. The nasal reconstruction was realized with short local hinge flaps for lining, rib cartilage for central L-strut and ala cartilages and a non-expended forehead flap. (B) The result 1 year after the reconstruction. Assuming nasal growth will be reduced for some degree, the neo tip was designed oversized.
At the time of the nose reconstruction, the patient was 7 years old. He was born with a hemangiopericytoma broadly connected to the right lateral nasal wall and to its base in the area of the right ethmoid sinuses and right frontal skull base. Although the nose and the anterior wall of the right maxillary sinus were compressed, the nasal pyramid, including its bony and cartilaginous parts, was developed. The huge vascular tumor was diagnosed during the sonographic examination of the mother. A primary cesarean section was performed during the 33rd week of pregnancy and after a stable cardiorespiratory state was established, the tumor was laser resected. This case was reported and published by Götte et al.52 Before school enrollment, the young patient presented for a nose reconstruction in our department. Defects caused by the primary resection were found as a cleft at the right lateral nasal wall, at the dorsum, and both alae. The right ala had a well-placed base, but the apical part was cephalically rotated and fixed at the medial angle of the right eye. A preschool reconstruction was necessary because of psychological reasons. The cartilaginous framework of both alae, the right nasal wall, and the dorsum was reconstructed with conchal cartilage from both ears. The septum was extended with septal cartilage from the thin central part, local skin pivot flaps were used for the reconstruction of the inner lining. A nonexpanded forehead flap was necessary to cover the new nose. The intention, according to the age of the patient, was to Facial Plastic Surgery
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create an oversized nose to compensate for an expected growth deficit of the reconstructed structures, which could possibly be caused by scarring. The intermediate result at the age of 11 years was satisfying. Social integration and school development were fine. Ten years after the reconstruction, a growth of the rebuilt structures could be noted. A further operation will be necessary to optimize the symmetry and correct the scars (►Fig. 6).
Nostril Stenosis Nostril stenosis in children is an infrequent finding that often has an iatrogenic cause. A pressure ulcer can be caused by therapy with oversized rigid packing or supportive care in neonates. The healing of the ulcer results in scars. The tissue shrinks with the process of scarring.48 Such scars lead to nasal airway obstruction and breathing difficulties during feeding. The growth of the external nasal valve area is impaired. We recommend an early surgical correction, optimally, before school enrollment. There are many treatment strategies, such as the correction with flaps based on the principle of a threedimensional z-plasty, but with insufficient results. We prefer an excision of the scarred tissue and a reconstruction with a
Fig. 7 (A-1,2) A 1-year-old girl with a severe stenosis of the left nostril after resection of a benign tumor (hamartoma). The correction was performed by cutting the circular scar, and inserting a local flap according to a modified z-plasty. (B-1) After 1 year, a very satisfactory result could be obtained. (B-2) After the treatment, a stent and steroid ointment were used.
local flap or a skin graft. An alternative to skin grafting is auricular composite grafting, which is more resistant against shrinking but prone to heal incompletely. Moreover, a deformity can be caused at the donor site on the ear. There are no long-term reviews confirming a growing of the composite graft with the growth of the nose. How to involve a “boomerang flap” for reconstruction was described by Bozkurt et al. It was used in combination with a nasolabial flap, a vestibular rotation flap, and a conchal cartilage graft to achieve a satisfying repair.49 Blandini et al recommend a stable correction by using a “piercing” flap taken from the labial vestibule transferred to the nasal floor.50 In case of surgical treatment of vestibular stenosis, the use of a custom-made vestibular device such as thinly walled stents with increasing diameter and intermediate sponge packing with an expanding effect may help prevent restenosis.50,51 Supportive local application of cortisone or mitomycin C should be conducted48 (►Fig. 7).
Conclusion
Fig. 6 (A) Nasal defect after perinatal resection of a huge congenital hemangiopericytoma at the age of 4. (B) Four years after the nasal reconstruction. (C) The 16-year-old patient with a harmonic proportion of the nose to the residual face. Facial Plastic Surgery
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In our contribution, we represent different aspects of nasal deformities and their treatment. In all cases, an individual concept is indispensable to reach the best compromise. The postulate of an early treatment gives priority to physical and mental damage control. The late therapy concept attempts to use the inimitable potential of the growth of the different tissue which is needed for a septoplasty, and should be protected from iatrogenic impairment. One can find benefits for a treatment in both phases, during infancy and after
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18 Cho CH, Shakibaei M, Merker HJ, Klein M. The rare malformation of
19 20
Acknowledgment We thank all of the patients for the permission to use their photographs to illustrate the treatment concepts discussed in this article.
Conflict of Interest The authors declare that they have no conflict of interest as defined by the guidelines of the International Committee of Medical Journal Editors.
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24
25
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completion of growth, to reach a clearly defined target. So, we recommend a first-line pediatric nasal reconstruction, an attentive follow-up, and finally, a secondary nasal reconstruction in the adult patient if required for achieving the normal.
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Nasal Reconstruction in Children
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