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A rare location for fibrous dysplasia. The middle turbinate
G Model ANORL-514; No. of Pages 2
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Letter to the Editor A rare location for fibrous dysplasia. The middle turbinate To the editor A 17-year-old female referred to our clinic with a history of growing difficulty in breathing through the nose for 5 years. Physical examination revealed a hypertrophic left middle turbinate. A computerized tomography (CT) was performed which revealed a homogeneous mass with intensive contrast uptake in the left nasal cavity, 2.5 × 4 cm in diameter. The mass was involved the middle turbinate extending through the skull base and deviating the nasal septum to right side. (Fig. 1) Biopsy was performed via endoscopic approach, which revealed fibrous dysplasia. As she did not have serious symptoms, waiting until adulthood was preferred for treatment. Fibrous dysplasia is an uncommon developmental anomaly of bone characterized by replacement of medullary bone by fibroosseous tissue. It accounts 7% of all benign bone neoplasm, which indicates slow progression [1]. According to the number of bones involved and accompanying symptoms, three forms of the disease are described: monostotic, polyostotic, and McCune-Albright syndrome. The most common form is monostotic in which single bone is involved. It accounts for approximately 70% of all cases. This form generally occurs at ribs and craniofacial bones and is being diagnosed at 2–3 decades. The polyostotic form accounts for 30% of all
cases in which multiple bones are involved. Half or more of the cases with polyostotic fibrous dysplasia have involvement of craniofacial bones. The lesion may involve any facial bone. The most common origin in the head neck region is reported as maxilla [2]. The appearance of the disease in the nasal cavity is rare [3]. The most severe form of the disease is called McCune-Albright syndrome, which is characterized by accompanying systemic complications such as abnormal skin pigmentation, premature sexual development and hypo-hyperthyroidism. It accounts for approximately 3% of all cases [2,4]. Fibrous dysplasia is generally asymptomatic at the onset, according to the bone involved it can cause various symptoms at further periods. The involvement of long bones may result in pathological fractures. Craniofacial involvement causes symptoms such as facial asymmetry, headache and nasal obstruction due to the site of bony involvement. The condition usually develops until adulthood. The growth of the monostotic type arrests in adulthood in most of cases. Some cases with polyostotic form and McCune-Albright syndrome continue growing even after adulthood. The reason of the continuing of bone growth after adulthood could not been established [5]. The diagnosis of fibrous dysplasia depends on physical examination, radiographic imaging and histological examination. CT is useful to diagnose the disease, to plan the treatment options and to measure the size of the mass in follow-up period. The treatment of fibrous dysplasia of the craniofacial region varies according to the clinical situation of the patient. As fibrous dysplasia usually stops growing after puberty, following-up the patients with imaging must be concluded in asymptomatic patients [3]. In patients with symptoms such as headache or facial asymmetry, surgical treatment may be thought. The aim of the surgical treatment is correcting or preventing functional problems and composing normal facial aesthetics.
Disclosure of Interest The authors declare that they have no competing interest.
References
Fig. 1. Paranasal CT showed a homogeneous mass with intensive contrast uptake in the left nasal cavity (asterisk).
[1] Delap TG, Kaberos A, Dowling P, Rees J, Golding-Wood D. Craniofacial fibroosseous dysplasia. A case report and overview of the management of the condition. Int J Pediatr Otorhinolaryngol 1996;37:179–91. [2] Erdem LO, Erdem CZ, Kargi S. A case of monostotic fibrous dysplasia of the maxillary sinus. Kulak Burun Bogaz Ihtis Derg 2003;10:208–11. [3] Galvan O, Gassner EM, Neher A, Gunkel AR. Fibro-osseous lesion of the middle turbinate: ossifying fibroma or fibrous dysplasia? J Laryngol Otol 2007;121:1201–3. [4] Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001;127:1239–47. [5] Kusano T, Hirabayashi S, Eguchi T, Sugawara YJ. Treatment strategies for fibrous dysplasia. Craniofac Surg 2009;20:768–70.
http://dx.doi.org/10.1016/j.anorl.2015.06.009 1879-7296/© 2016 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Sitki Gozeler M, et al. A rare location for fibrous dysplasia. The middle turbinate. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.06.009
G Model ANORL-514; No. of Pages 2 2
ARTICLE IN PRESS Letter to the Editor / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
M. Sitki Gozeler a K. Kilic b M. Sedat Sakat a H. Ucuncu a,∗ a Department of Otorhinolaryngology, Faculty of Medicine, Atatürk University, 25240 Erzurum, Turkey
b
Otorhinolaryngology Clinics, Palandöken State Hospital, 25080 Erzurum, Turkey
∗ Corresponding
author. Tel.: +90 442 344 7133; fax: +90 442 236 1301. E-mail address: [email protected] (H. Ucuncu)
Please cite this article in press as: Sitki Gozeler M, et al. A rare location for fibrous dysplasia. The middle turbinate. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.06.009
ARTICLE IN PRESS European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
Available online at
ScienceDirect www.sciencedirect.com
Letter to the Editor A rare location for fibrous dysplasia. The middle turbinate To the editor A 17-year-old female referred to our clinic with a history of growing difficulty in breathing through the nose for 5 years. Physical examination revealed a hypertrophic left middle turbinate. A computerized tomography (CT) was performed which revealed a homogeneous mass with intensive contrast uptake in the left nasal cavity, 2.5 × 4 cm in diameter. The mass was involved the middle turbinate extending through the skull base and deviating the nasal septum to right side. (Fig. 1) Biopsy was performed via endoscopic approach, which revealed fibrous dysplasia. As she did not have serious symptoms, waiting until adulthood was preferred for treatment. Fibrous dysplasia is an uncommon developmental anomaly of bone characterized by replacement of medullary bone by fibroosseous tissue. It accounts 7% of all benign bone neoplasm, which indicates slow progression [1]. According to the number of bones involved and accompanying symptoms, three forms of the disease are described: monostotic, polyostotic, and McCune-Albright syndrome. The most common form is monostotic in which single bone is involved. It accounts for approximately 70% of all cases. This form generally occurs at ribs and craniofacial bones and is being diagnosed at 2–3 decades. The polyostotic form accounts for 30% of all
cases in which multiple bones are involved. Half or more of the cases with polyostotic fibrous dysplasia have involvement of craniofacial bones. The lesion may involve any facial bone. The most common origin in the head neck region is reported as maxilla [2]. The appearance of the disease in the nasal cavity is rare [3]. The most severe form of the disease is called McCune-Albright syndrome, which is characterized by accompanying systemic complications such as abnormal skin pigmentation, premature sexual development and hypo-hyperthyroidism. It accounts for approximately 3% of all cases [2,4]. Fibrous dysplasia is generally asymptomatic at the onset, according to the bone involved it can cause various symptoms at further periods. The involvement of long bones may result in pathological fractures. Craniofacial involvement causes symptoms such as facial asymmetry, headache and nasal obstruction due to the site of bony involvement. The condition usually develops until adulthood. The growth of the monostotic type arrests in adulthood in most of cases. Some cases with polyostotic form and McCune-Albright syndrome continue growing even after adulthood. The reason of the continuing of bone growth after adulthood could not been established [5]. The diagnosis of fibrous dysplasia depends on physical examination, radiographic imaging and histological examination. CT is useful to diagnose the disease, to plan the treatment options and to measure the size of the mass in follow-up period. The treatment of fibrous dysplasia of the craniofacial region varies according to the clinical situation of the patient. As fibrous dysplasia usually stops growing after puberty, following-up the patients with imaging must be concluded in asymptomatic patients [3]. In patients with symptoms such as headache or facial asymmetry, surgical treatment may be thought. The aim of the surgical treatment is correcting or preventing functional problems and composing normal facial aesthetics.
Disclosure of Interest The authors declare that they have no competing interest.
References
Fig. 1. Paranasal CT showed a homogeneous mass with intensive contrast uptake in the left nasal cavity (asterisk).
[1] Delap TG, Kaberos A, Dowling P, Rees J, Golding-Wood D. Craniofacial fibroosseous dysplasia. A case report and overview of the management of the condition. Int J Pediatr Otorhinolaryngol 1996;37:179–91. [2] Erdem LO, Erdem CZ, Kargi S. A case of monostotic fibrous dysplasia of the maxillary sinus. Kulak Burun Bogaz Ihtis Derg 2003;10:208–11. [3] Galvan O, Gassner EM, Neher A, Gunkel AR. Fibro-osseous lesion of the middle turbinate: ossifying fibroma or fibrous dysplasia? J Laryngol Otol 2007;121:1201–3. [4] Lustig LR, Holliday MJ, McCarthy EF, Nager GT. Fibrous dysplasia involving the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001;127:1239–47. [5] Kusano T, Hirabayashi S, Eguchi T, Sugawara YJ. Treatment strategies for fibrous dysplasia. Craniofac Surg 2009;20:768–70.
http://dx.doi.org/10.1016/j.anorl.2015.06.009 1879-7296/© 2016 Elsevier Masson SAS. All rights reserved.
Please cite this article in press as: Sitki Gozeler M, et al. A rare location for fibrous dysplasia. The middle turbinate. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.06.009
G Model ANORL-514; No. of Pages 2 2
ARTICLE IN PRESS Letter to the Editor / European Annals of Otorhinolaryngology, Head and Neck diseases xxx (2016) xxx–xxx
M. Sitki Gozeler a K. Kilic b M. Sedat Sakat a H. Ucuncu a,∗ a Department of Otorhinolaryngology, Faculty of Medicine, Atatürk University, 25240 Erzurum, Turkey
b
Otorhinolaryngology Clinics, Palandöken State Hospital, 25080 Erzurum, Turkey
∗ Corresponding
author. Tel.: +90 442 344 7133; fax: +90 442 236 1301. E-mail address: [email protected] (H. Ucuncu)
Please cite this article in press as: Sitki Gozeler M, et al. A rare location for fibrous dysplasia. The middle turbinate. European Annals of Otorhinolaryngology, Head and Neck diseases (2016), http://dx.doi.org/10.1016/j.anorl.2015.06.009