Brief Clinical Studies The Journal of Craniofacial Surgery Volume 28, Number 3, May 2017

general medical history or nasal trauma was unremarkable for the Conchae Bullosis in a Pediatric patient. The rhinoscopic and endoscopic evaluation of the Patient revealed hypertrophied middle and inferior conchae with the sep- tum in the midline. The was normal and there was no Omer Erdur, MD, Kayhan Ozturk, MD, and Ceren Aksoy, MD secretion. Flexible endoscopic nasopharyngeal evaluation revealed no particular pathologic signs of mass or tissue. The Abstract: Pneumatization of the turbinates called concha bullosa is paranasal computerized tomography (CT) scans of the patient one of the most frequent anatomic variations of the nasal cavity. We revealed a surprisingly unique , with bilateral inferior report the first case of computed tomography findings of bilateral and middle CB and pneumatization of the crista galli (Fig. 1A). middle and inferior concha bullosa in a 13-year-old child with nasal As the pneumatization of middle and inferior turbinates was obstruction. Here we describe a patient with extreme bilateral expansive, it was possible to see in same coronal CT image. The bullosa of inferior and middle conchas, as well as crista galli. pneumatization extending to the entire length of the inferior turbi- The patient was treated successfully with endoscopic surgery of nate was related to . None of the sinuses or cells was diseased. conchas. Nasal obstruction secondary to a bilateral turbinate bul- For the treatment the resection of the lateral lamella of the losis in a child has not been described before. The clinicians should middle turbinates and submucous bony resection and out-fracture of take this entity into consideration when evaluating the pediatric the inferior turbinates were done endoscopically under general patients with nasal obstruction. anesthesia (Fig. 1B). As the patient was a child, no other structures were touched. The child was discharged 1 day after the operation Key Words: Concha bullosa, nasal obstruction, turbinate and no complication was detected during the operation and after the pneumatization operation. In the follow-up period, the patient remained free of symptoms. he turbinates are the outgrowths that develop from the lateral T nasal wall and they are usually identified during the 8th to 10th DISCUSSION weeks of fetal life.1 Anatomic variations of these turbinates reduce The anatomic variations of nasal and paranasal area and the the quality of life as these have important functions for normal nasal associations with diseases were not described well in pediatric physiology. and nasal obstruction, anosmia, post- patients.4 In addition, nature and management of sinonasal vari- nasal drip, headache, and epiphora are the symptoms that may be ations is not known well in this population. Underlying mechanisms seen due to pathologies of the turbinates. Although the aeration of of the nasal and sinus pneumatization process are not yet known the turbinates is called concha bullosa (CB), it is generally used for well, and it is considered an active process during fetal development describing the air in the middle turbinate. and adolescence. Concha bullosa continues to develop until the end Conchae bullosis is used for multiple pneumatization of the of adolescence and can be apparent after the age of 7 to 8.5 It has conchae.2 The exact mechanism of pneumatization of the turbinates been shown that 82% of CB was unilateral and 18% of CB has not been defined well. Sinonasal anomalous bony pneumatiza- was bilateral. tion is not common in the pediatric age group. Although middle CB Anatomic skeletal pathologies such as pneumatization of turbi- is a rare finding in pediatric patients, pneumatization of the inferior nates can also obstruct the nasal passage and may have an influence turbinate is extremely rare, as only a few cases have been previously on nasal patency.6 Postnasal drip, anosmia, and epiphora may also presented in the literature.3 Hence, there is no adequate information be faced.7 In addition, middle CB may reduce ventilation and on the symptoms, diagnosis, and treatment management for inferior drainage of osteomeatal drainage area. Enlargement of the turbi- CB. To the best of our knowledge, this is the first extreme nates causes mucosal contact and it can be the reason of headache pneumatization that presents bilateral middle and inferior CB in even in the absence of sinonasal pathology and epiphora due to a pediatric patient. We wanted to draw attention of the readers to the nasolacrimal duct blockage.7,8 Our patient’s primary symptom was etiology of nasal obstruction with bilateral middle and inferior CB nasal obstruction. He also had headache and postnasal drip without with pneumatization of crista galli in a pediatric patient. a sign of paranasal inflammation. The advancement of radiologic CT techniques has allowed us CLINICAL REPORT more information about the paranasal area, nasal cavity, as well as turbinates. The difference between a hypertrophied turbinate and its A13-year-oldboyreferredtoourdepartment for nasal obstruction, pneumatization can be distinguished only by radiologic imaging intermittent facial pain, and postnasal drip that had been occurring techniques. These findings help the surgeons to be aware of the continuously for the past 3 years. For these complaints, he anatomic variations. In our patient, the anterior rhinoscopic and had consulted different pediatricians and otolaryngologists. endoscopic views of the nasal cavity were not distinctive for He had been evaluated for allergic diseases and adenoid hyper- pneumatization. As the patient was not an adult, the other clinicians trophy, but all tests including skin prick test were negative. Other who evaluated him earlier did not ask for paranasal CT. The patient was generally prescribed local decongestants and local steroids. The From the Department of Otolaryngology, Selcuk University, Konya, CT scans of the patient revealed a unique and rare pathology: Turkey. bilateral inferior and middle CB and pneumatization of crista galli. Received November 3, 2016. Crista galli can be pneumatized, usually coming from anterior Accepted for publication December 12, 2016. ethmoid; however, this anatomic variation has no clinical import- Address correspondence and reprint requests to Omer Erdur, MD, Selcuk ance and is a rare clinical situation. University Medical School, Alaeddin Keykubad Campus, 42075 Yeni, Yang et al evaluated 59,238 patients with sinonasal CT for Istanbul Caddesi, Konya, Turkey; E-mail: [email protected] their study, and found inferior CB only in 16 patients, and only 2 of The authors report no conflicts of interest 9 Copyright # 2017 by Mutaz B. Habal, MD them had bilateral CB. Giourgos et al first described a child ISSN: 1049-2275 having CB of inferior turbinate and coexisting pneumatized DOI: 10.1097/SCS.0000000000003543 anatomic structures such as uncinate process and the Haller cells.3 e266 # 2017 Mutaz B. Habal, MD Copyright © 2017 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited. The Journal of Craniofacial Surgery Volume 28, Number 3, May 2017 Brief Clinical Studies

A Rare Case of Malignant Fibrous Histiocytoma (Undifferentiated High-Grade Pleomorphic Sarcoma) of Malar Region E´der A. Sigua-Rodriguez, DDS, MSc, FIGURE 1. (A) Coronal section of paranasal computed tomography showing bilateral inferior and middle concha bullosa and pneumatization of crista galli. Douglas Rangel Goulart, DDS, PhD, (B) The endoscopic view of turbinates during surgery. Afonso Celso de Moraes Manzano, MD, MSc,y and Luciana Asprino, DDS, PhD

Although the asymptomatic patients do not require treatment, Abstract: Malignant fibrous histiocytoma is a sarcoma with rare outfracture, resection of lateral lamella, and submucous resection occurrence in the oral and maxillofacial region; surgery is the 10,11 are the surgical treatment options for inferior CB. Our patient most reliable treatment. Inadequate resection of the sarcoma on was 13 years old and for bilateral bullosa of the middle and inferior the oral and maxillofacial region is associated with a high conchas we resected the lateral lamella of the middle conchas and incidence of local recurrence and a poor prognosis. Only few performed submucous resection and lateralization for the inferior conchas. patients of malignant fibrous histiocytoma of the malar region Concha bullosa of both middle and (very rarely) inferior turbi- have been previously reported in the literature. The authors nates may take up too much space due to their enlargement with air. report a new patient of malignant fibrous histiocytoma on the They may well contribute to obstruction and impaired nasal breath- right malar region that treated a complete tumor surgical ing. Clinicians do not consider CB for the etiology for nasal excision without node dissection. Examination of the obstruction in children. For this reason, our patient was misdiag- resected specimen revealed that the tumor was completely nosed for 3 years and all clinicians gave the diagnosis as adenoid removed. hypertrophy or concha hypertrophy. Therefore, the pneumatization of nasal structures should be evaluated well on the scans for any resistant nasal obstruction etiology or before sinus surgery is Key Words: Head and neck neoplasms, malignant fibrous undertaken in children. histiocytoma, sarcoma

alignant fibrous histiocytoma (MFH) was first reported as CONCLUSION M malignant fibrous xanthoma by O’Brien and Stout1 in In the patients presenting with nasal obstruction who do not respond 1964. It is a high grade and aggressive sarcoma and is considered to local treatments, turbinate pneumatizations should be kept in the most common soft tissue sarcoma in adults,2 being most mind even in pediatric patients. Computed tomography imaging is a commonly located in the extremities and retroperitoneum and valuable tool in making the diagnosis. relatively uncommon in the head and neck region, when detected, it has been observed in the nasal sinus, salivary gland, oral cavity, 3 REFERENCES mandible, larynx, auricula, and eyelid and occurs most com- monly in accessory nasal sinuses, followed by the salivary 1. Bolger WE. Anatomy of the . In: Kennedy DW, Bolger glands.4 WE, Zinreich SJ, eds. Disease of the Sinuses. London, UK: BC Decker; Surgery is the most reliable treatment for MFH, adjuvant 2001:1–12 2. Ozturan O, Yenigun A, Degirmenci N, et al. ‘Conchae bullosis’: a rare chemotherapy is considered in high-grade tumors because these case with bilateral triple turbinate pneumatisations. J Laryngol Otol tumors may present subclinical or microscopic metastases at the 5 2013;127:73–75 time of diagnosis. Survival rate for MFH in the head and neck is 3. Giourgos G, Matti E, Carena P, et al. A unique case of multiple sites of low compared with MFH in the extremities and trunk.3 It is rarely pneumatization of the sinonasal bony framework in a pediatric patient. diagnosed before excision and has an unfavorable prognostic in Ear Nose J 2010;89:E10–E11 some patients.6 Here, we report a rare case of a woman with 4. Hebert RL, Bent JP. Meta-analysis of outcomes of pediatric functional cutaneous malignant fibrous histiocytoma on the right malar endoscopic sinus surgery. Laryngoscope 1998;108:796–799 region. 5. Unlu HH, Akyar S, Caylan R, et al. Concha bullosa. J Otolaryngol 1994;23:23–27 6. Hilberg O, Grymer LF, Pedersen OF, et al. Turbinate hypertrophy. Evaluation of the nasal cavity by acoustic rhinometry. Arch From the Department of Oral and Maxillofacial Surgery, Piracicaba Otolaryngol Head Neck Surg 1990;116:283–289 Dental School, State University of Campinas (UNICAMP), Piracicaba; 7. Ingram WA, Richardson BE. Concha bullosa of an inferior turbinate. and yDepartment of Head and Neck Surgery, Santa Casa of Limeira Ear Nose Throat J 2003;82:605–607 Hospital, Limeira, Brazil. 8. Kantarci M, Karasen RM, Alper F, et al. Remarkable anatomic Received November 7, 2016. variations in paranasal sinus region and their clinical importance. Accepted for publication December 12, 2016. Eur J Radiol. 2004;50:296–302 Address correspondence and reprint requests to Douglas Rangel Goulart, 9. Yang BT, Chong VFH, Wang ZC, et al. CT appearance of pneumatized DDS, PhD, State University of Campinas, Piracicaba, Brazil; inferior turbinate. Clin Radiol 2008;63:901–905 E-mail: [email protected] 10. Braun H, Stammberger H. Pneumatization of turbinates. Laryngoscope The authors report no conflicts of interest. 2003;113:668–672 Copyright # 2017 by Mutaz B. Habal, MD 11. Woolford TJ, Jones NS. A concha bullosa crusher for use in endoscopic ISSN: 1049-2275 sinus surgery. J Laryngol Otol 2000;114:205–206 DOI: 10.1097/SCS.0000000000003566

# 2017 Mutaz B. Habal, MD e267 Copyright © 2017 Mutaz B. Habal, MD. Unauthorized reproduction of this article is prohibited.