Ethmoid Research Articles

New Perspectives in the Etiology of Chronic Rhinosinusitis—Haller Cells

New Perspectives in the Etiology of Chronic Rhinosinusitis—Haller Cells

Anatomical Variations on NCCT Nose and Paranasal Sinuses and Their Relation with Symptoms of the Patients: A Retrospective Analysis

Background: There are various bones around the nasal cavity that pneumatize to form the paranasal sinuses; namely, maxilla, ethmoid bone, frontal bone, and sphenoid bone. The CT scan of nose and paranasal sinus is the gold standard investigation for the patients of chronic rhino-sinusitis (CRS). Objective: This study was conducted with aim to determine the prevalence anatomical variation of Nose and Paranasal Sinuses on CT-scan and their relation with symptoms. Methods: A retrospective study was conducted among 200 patients over a period of one year (2022-2023). Patients with various symptoms of chronic rhinosinusitis were subjected to non-contrast enhanced Computed Tomography of nose and paranasal sinuses. Results: In our study 56% patients had septal deviation with most common C shaped deviation followed by S shaped deviation. 99.5% patients have agger nasi cells. The attachment of uncinate process was to the lamina papyracea in 83.0%, followed by the base of skull in 13.0%. The least common types were free uncinate process in 1.5%. The prevalence of concha bullosa was found 16.5%. However, paradoxical turbinate was present in 2.0% subjects. The incidence of Haller cells was found to be 5.0% and Onodi cells were found in 4.5%. The type of frontal cell was Agger Nasi followed by a Supra-bullar. In Sphenoid sinus, commonest type was pre-sellar type of pneumatisation. Conclusion: Our study concluded these various anatomical variations and its prevalence. The relation of variations with disease symptomatology is inconclusive.

Biphenotypic Sinonasal Sarcoma: Literature Review of a Peculiar Pathological Entity—The Neurosurgical Point of View

Background: Biphenotypic sinonasal sarcoma (BSNS) is a low-grade tumor of the sinonasal tract with frequent extension to the orbit and skull base. Due to its rare incidence and recent histopathological and molecular characterization, little data are available in regard to its natural history, treatment and surveillance protocol. Methods: A comprehensive literature review in Embase online electronic databases on BSNS was made. The analyzed factors included the patients’ sex and age, presenting symptoms and signs, anatomical origin and pattern of growth of the tumor, immunohistochemical and molecular features, time to treatment, type of treatment, surgical approach, extent of resection, peri- and post-operative complications, adjuvant therapies, clinical outcome, recurrence and overall survival rates. Results: This literature review involved 34 studies for an overall series of 149 cases of BSNS. The female (66.9%) and middle-aged populations (median 54.88 years old) were mainly affected. The most frequent clinical onset was nasal obstruction (81%), followed by facial discomfort (44%), epistaxis (15.5%) and ocular impairment (14.3%). Ethmoid sinus (67.8%) and nasal cavity (45.4%) were the most common anatomical site of tumor origin, while an extension to the orbit and skull base was registered in 28.7% and 24.5% of cases. Surgery was the main treatment, especially in the form of endoscopic endonasal approach (56.9%), and allowed for gross total resection in 79% of cases. The recurrence rate was 26.2%; three cases of tumor-related death were reported. Median follow-up was 4.6 years. Conclusions: Biphenotypic sinonasal sarcoma is a rare and unique tumoral entity in terms of biological and clinical behavior. Based on the current knowledge, surgery plays the leading role in treatment, accounting for gross total tumor resection in most cases, allowing for clinical symptom and sign resolution and presenting a low rate of perioperative complications. The type of approach and the aim of surgery should be assessed case by case according to patient and pathology features and the surgeon’s experience, as well as the aim of the treatment. Further studies including large surgical series and with long follow-up are required to define prognostic factors and guidelines of treatment for this peculiar pathological entity.

“Deciphering The Enigma Of Nasal & Paranasal Anatomy Through Computed Tomography (CT) Imaging”

Introduction: the paranasal sinuses are mucosa-lined structures physically contiguous with the nasal cavity that aid in insulation, reducing cranial weight, heating & humidifying the air, imparting resonance to the voice, providing airway defense and simply to replace functionless bone. Sinusitis is rarely life threatening, but the complex anatomy of the facial planes, the associated venous and lymphatic spread and the close location to the central nervous system can lead on to serious complications. It is, therefore, important to understand the normal anatomy of the paranasal sinuses in order to understand the pathogenesis of sinus disease. Hence, the aim of the present study was deciphering the role of ct as the investigation of choice for the preoperative evaluation of the nose & paranasal sinuses and for delineation of obstructive inflammatory sinus disease to aid in the diagnosis and management of recurrent & chronic disease and to define the bony anatomy before surgery. Materials and methods: prospective cross-sectional study was conducted on 100 asymptomatic patients who were getting investigated by computed tomography for non-sinus indications in whom the paranasal sinus region could be included in the field of study referred to department of radio-diagnosis and imaging from indoor and outdoor departments of jan sewa hospital of dr. S. S. Tantia medical college, sri ganganagar. Results: the majority of patients were in the age group of 21-40 years (42.3%) with 70% being male. Patients in the age group equaling or more than 61 years & with diabetes had the most number of incidental findings (68.2 %) agger nasi was the commonest anatomical paranasal sinus variant (82.9 %) followed by concha bullosa (39.3 %), deviation of the nasal septum (35.4 %), onodi cells ( 12.2 %) & haller cells ( 11.8 %). Incidental paranasal sinus abnormality in the form of mucosal thickening, sinus secretions or polyps were detected in 51.9 % patients, mucosal thickening being most common (40.1 %) followed by secretions (31.9 %) and mucosal polyps ( 3.0 %). Incidental paranasal abnormalities were most commonly noted in the ethmoid sinuses (36.7 %) followed by the maxillary sinuses, sphenoid sinuses and then frontal sinuses in descending order of involvement. Conclusion: computed tomography (ct) provides a “road map” for diagnosis and management of recurrent & chronic paranasal sinus disease and to delineate the bony anatomy before sinus surgery and to get an insight into anatomical variants apropos of their clinical relevance & impact on sinus disease.

“Deciphering The Enigma Of Nasal & Paranasal Anatomy Through Computed Tomography (CT) Imaging”

Introduction: the paranasal sinuses are mucosa-lined structures physically contiguous with the nasal cavity that aid in insulation, reducing cranial weight, heating & humidifying the air, imparting resonance to the voice, providing airway defense and simply to replace functionless bone. Sinusitis is rarely life threatening, but the complex anatomy of the facial planes, the associated venous and lymphatic spread and the close location to the central nervous system can lead on to serious complications. It is, therefore, important to understand the normal anatomy of the paranasal sinuses in order to understand the pathogenesis of sinus disease. Hence, the aim of the present study was deciphering the role of ct as the investigation of choice for the preoperative evaluation of the nose & paranasal sinuses and for delineation of obstructive inflammatory sinus disease to aid in the diagnosis and management of recurrent & chronic disease and to define the bony anatomy before surgery. Materials and methods: prospective cross-sectional study was conducted on 100 asymptomatic patients who were getting investigated by computed tomography for non-sinus indications in whom the paranasal sinus region could be included in the field of study referred to department of radio-diagnosis and imaging from indoor and outdoor departments of jan sewa hospital of dr. S. S. Tantia medical college, sri ganganagar. Results: the majority of patients were in the age group of 21-40 years (42.3%) with 70% being male. Patients in the age group equaling or more than 61 years & with diabetes had the most number of incidental findings (68.2 %) agger nasi was the commonest anatomical paranasal sinus variant (82.9 %) followed by concha bullosa (39.3 %), deviation of the nasal septum (35.4 %), onodi cells ( 12.2 %) & haller cells ( 11.8 %). Incidental paranasal sinus abnormality in the form of mucosal thickening, sinus secretions or polyps were detected in 51.9 % patients, mucosal thickening being most common (40.1 %) followed by secretions (31.9 %) and mucosal polyps ( 3.0 %). Incidental paranasal abnormalities were most commonly noted in the ethmoid sinuses (36.7 %) followed by the maxillary sinuses, sphenoid sinuses and then frontal sinuses in descending order of involvement. Conclusion: computed tomography (ct) provides a “road map” for diagnosis and management of recurrent & chronic paranasal sinus disease and to delineate the bony anatomy before sinus surgery and to get an insight into anatomical variants apropos of their clinical relevance & impact on sinus disease.

Radiographic Variants Associated With Allergic Fungal Rhinosinusitis: Key Differences for Pre-Operative Planning.

Allergic fungal rhinosinusitis (AFRS) often results in expansion of disease beyond the paranasal sinuses, which may put important structures, such as the anterior ethmoid artery (AEA) or lateral lamella of the cribiform, at risk of injury during endoscopic sinus surgery (ESS). This study aims to compare the AEA to skull base (AEA-SB) length in patients with AFRS versus chronic rhinosinusitis with nasal polyps (CRSwNP), as well as additional anatomic variants. A single institutional retrospective chart review of patients undergoing ESS for AFRS and CRSwNP was performed. AEA-SB length were compared between the 2 groups. Other anatomic variants, including Keros measurement and presence of supraorbital ethmoid air cells (SOEC), concha bullosa (CB), sphenoethmoidal, and infraorbital ethmoid cells were measured and compared between the 2 groups. Twenty-one patients were included in each cohort. The AFRS group was younger in age (P = .015) and had a significantly longer AEA-SB length (P = .014) compared to the CRSwNP group. No significant differences were observed between the 2 groups regarding Keros measurement, presence of concha bullosa, infraorbital ethmoid, sphenoethmoidal, or SOEC. No association was seen between AEA-SB length and Keros class in either group. AFRS harbors anatomical differences when compared to CRSwNP, with the former associated with a longer AEA-SB length. This key difference should be considered in preoperative planning to prevent injury to the AEA in patients undergoing ESS for AFRS.

Intracranial Off-Midline Mature Teratoma and Pneumosinus Dilatans: A Unique Clinical Report

AbstractTeratomas typically arise as midline lesions in the suprasellar and pineal regions. Pneumosinus dilatans is a rare condition characterized by the expansion of one or more of the paranasal sinuses and thinning of their bony walls with a normal covering mucosa. It usually involves the sphenoid and posterior ethmoid sinuses and has been associated with meningiomas and arachnoid cysts. Off-midline mature teratomas are uncommon, and no reports have described an association with pneumosinus dilatans. We present a rare association between an intracranial off-midline mature teratoma and pneumosinus dilatans in an 18-year-old male patient who presented with a second episode of a left-sided seizure, which has not yet been reported in the literature.

Variations of Anterior Skull Base Seen on Computed Tomography Scans of the Paranasal Sinuses

Abstract Context: The anterior skull base (ASB) and the paranasal sinuses are known for anatomical variations. Therefore, a preoperative assessment for the assessment of these variations is a necessity. This study aims to determine the profile of variations of the ASB using computed tomography of the paranasal sinuses (CT-PNS) scans. Settings and Design: Observational Study. Materials and Methods: Forty CT-PNS scans were studied with measurement of different parameters. Statistical Analysis: Observational study analysis – Descriptive stats, t-tests, Chi-square, nonparametric tests, analysis of variance, regression, P < 0.05 significance level. A significance level of P < 0.05 was used for all tests. Results: Seventeen male and 23 female CT-PNS scans were studied. Different measurements on the right and left sides, as well as between genders, were examined and compared. The supraorbital ethmoidal cell is the most often seen anatomical variation. Variations in the ASB are equally visible on both sides in men and on the right in females (Type-II > Type-1). Type I was more common among female, whereas Type II was more common. The existence of the anterior ethmoidal foramen is a dependable marker for the anterior ethmoidal artery. In the group under investigation, there was no lamina papyracea abnormality. Conclusion: This study aims to assess anatomical variations in the Indian population undergoing ASB surgeries, contributing to the current understanding of Indian paranasal sinuses and skull base dynamics.

Sinonasal Phosphaturic Mesenchymal Tumors Without Any Nasal Symptoms: A Case Report and Literature Review.

We present a case of phosphaturic mesenchymal tumor (PMT) in the left ethmoid without any nasal symptoms in a 63-year-old woman. Initially diagnosed with postmenopausal osteoporosis, 2-year history of hypophosphatemia and a significantly higher uptake of Fluorine-18 (18F)-AlF-NOTA-octreotide (18F-OC) in the left ethmoid sinus, provided crucial information for accurate diagnosis. We presented a case with chart review and conducted review of the literature. The patient endured 1-year history of weakness and bone pain but without any nasal symptoms before a tissue diagnosis was eventually reached. It is a challenging diagnosis to make-patients present with non-specific clinical symptoms and the culprit neoplasm is often tiny in size and difficult to detect. It emphasizes the importance of thorough patient history-taking and the whole-body functional imaging. Sinonasal PMTs are rare, and because of this most otolaryngologists are unfamiliar with its clinical presentation. This case highlights the importance of early diagnosis to enable prompt intervention and reduce the burden of associated symptoms.

Approach to frontal sinus via five frontal sinus drainage pathways.

This study aimed to validate a method for successful frontal sinus surgery. The method classifies the frontal sinus drainage pathway (FSDP) into five categories based on three bony walls of the anterior ethmoid sinus, including the uncinate process (UP), accessory uncinate process (UPa), and basal lamella of the ethmoid bulla (BLEB), which was tested in actual surgical procedures. This study analyzed 53 sides of 48 patients who underwent frontal sinus surgery between October 2022 and March 2023. We classified the FSDPs using preoperative computed tomography (CT) and multiplanar reconstruction (MPR). During surgeries for FSDPs located anterior to the BLEB, we used a two-step method involving resection of the turbinal UP, followed by upward resection from the lower edge of the ethmoidal UP. For FSDPs located posterior to the BLEB, we resected the BLEB at the superior semilunar recess. We confirmed the origin of each of the five types of FSDP during surgery. These origins, which were located at the lowest part of the anterior ethmoid, could be identified in the early stages of ethmoid sinus surgery before proceeding to the frontal recess area. The origins of the five types of FSDP, classified based on the bony walls, can be clearly and accurately identified during surgery. This provides a reliable method for preoperatively predicting and locating the inferior end of the FSDP (origin), without extensive manipulation of the cells formed in the frontal fossa.

Endoscopic surgery for squamous cell carcinoma in the nasal cavity and ethmoid sinus: A retrospective observational study

ObjectiveReports of endoscopic surgery for squamous cell carcinoma of the nasal cavity and ethmoid sinus are limited. Herein, we present a comprehensive account of the results obtained from performing endoscopic surgery based on the concept of en bloc resection. MethodsThis was a retrospective observational study of patients who underwent nasal endoscopic surgery for squamous cell carcinoma of the nasal cavity and ethmoid sinus at a hospital between July 2018 and December 2021. Primary endpoints were overall survival, relapse-free survival, and local recurrence-free survival. Data on tumor stage, tumor site of origin, postoperative treatment, and papilloma-related status were reviewed. Statistical analyses included the Kaplan–Meier method, Cox regression analysis, and Fisher's exact test. ResultsTwenty-two patients with a median age of 62 (range 27–84) years, comprising 15 male and 7 female, were included in this study, and the median duration of observation was 2.1 (range 0.6–4.3) years. The 2-year overall survival, relapse-free survival, and recurrence-free survival rates were 91.0%, 69.9%, and 79.0%, respectively. Cancer of the nasal cavity was significantly superior to that of the ethmoid sinus in terms of local recurrence-free survival (p = 0.03). The patients who underwent postoperative treatment had significantly worse recurrence-free survival rates than those who did not receive postoperative treatment (p = 0.03). ConclusionsThis study examined the results of the endoscopic treatment for squamous cell carcinoma of the nasal cavity and ethmoid sinus with the concept of en bloc resection. Patients with ethmoid sinus carcinoma had a greater risk for local recurrence, and the prognosis for patients requiring postoperative treatment was poor.

Extent of Endoscopic Sinus Surgery for Odontogenic Sinusitis of Endodontic Origin with Ethmoid and Frontal Sinus Involvement.

Background/Objectives: Odontogenic sinusitis (ODS) is the most common cause of unilateral maxillary sinus opacification. Initial treatment consists of intranasal steroids and antimicrobial therapy. In case of persistence of the disease, endoscopic sinus surgery (ESS) is advised. It is still not clear what extension of ESS is required and whether frontal sinusotomy or ethmoidectomy is justified in ODS with frontal sinus involvement. Methods: Adult patients presented with uncomplicated recalcitrant bacterial ODS due to endodontic-related dental pathology were evaluated by an otolaryngologist and a dentist and scheduled for ESS. Sinus CT scan demonstrated opacification of maxillary sinus and partial or complete opacification of extramaxillary sinuses ipsilateral to the side of ODS. Patients were undergoing either maxillary antrostomy, antroethmoidectomy, or antroethmofrontostomy. Preoperative and postoperative evaluations were done with nasal endoscopy, dental examination, subjective and radiological symptoms. Results: The study group consisted of 30 patients. Statistically significant decreases in values after surgery were found for SNOT-22, OHIP-14, Lund-Mackay, Lund-Kennedy, and Zinreich scale. Tooth pain was present in 40% cases during the first visit and in 10% during the follow-up visit. Foul smell was initially reported by 73.3% and by one patient during follow-up visit (3.3%). Significantly longer total recovery time and more crusting was marked for antroethmofrontostomy when compared to maxillary antrostomy. Conclusions: ESS resolved ODS with ethmoid and frontal involvement in almost every case. Minimal surgery led to improved overall clinical success in the same way as antroethmofrontostomy without risking the frontal recess scarring and stenosis.

Cancer of the paranasal sinuses in Germany: Data on incidence and survival from a population-based cancer registry

PurposeThis study aims to provide a broad overview of the epidemiology of cancer of the paranasal sinuses (PSC) in Germany. The data include information on incidence, staging, clinicopathological features and survival from one of the largest cancer registries in Europe. MethodsPopulation-based data on PSC diagnosed from January 1st, 2009 until December 31st, 2019 were retrieved from the German Centre for Cancer Registry Data (ZfKD). Age standardized incidence was calculated and relative survival estimates were computed by sex, histological subtype, age group and T-, N-, and M-Stage. ResultsIn total, 3975 cases were included in this study. The age-adjusted incidence rate (ASR) for PSC was 0.3/100,000 which remained stable during the observation period. The most frequent tumor localization was the maxillary sinus (41.9 %) and the most common histological subtype was keratinizing squamous cell carcinoma (kSCC) (44.3 %). All subtypes were predominantly found in the maxillary sinus except for adenocarcinoma and neuroendocrine carcinomas (SNEC), which were most frequently located in the ethmoidal sinus. The majority of the patients with a known T stage was diagnosed in tumor stage T4 (60.8 %). The overall 5-year relative survival (RS) for all patients with PSC was 52 %. RS dropped from 93 % for T1 stage tumors to 39 % for T4 tumors. RS was 58 % for N0 and 31 % for N+ cases, 54 % for M0 and 27 % for M1 cases. ConclusionAge-adjusted incidence for PSC is low and has been stable for the observed 11-year period. RS decreases continuously with increasing T-, N- and M-stage.

Orbital injuries: aspects of forensic medical examination in assessing the severity of harm caused to human health

Background: Forensic examination plays a key role in establishing the severity of injuries, especially of orbital trauma, which can lead to serious consequences, including vision loss. Examination of forensic reports associated with orbital trauma provides valuable information about the nature of the injuries, their prevalence, and factors influencing the severity of the injury. Aim: Analysis of the possibilities of an interdisciplinary approach based on the presence of a full ophthalmological status and computed tomography data of the skull in conducting a forensic medical examination of living persons and in the final qualification of the degree of harm to health in orbital injuries. Materials and methods: An analysis of 37 completed forensic medical examinations of living persons with orbital injuries who were treated in multidisciplinary hospitals in Moscow was carried out. The forensic medical examination was carried out in the Bureau of Forensic Medical Examination of the Moscow Health Department. In 23 cases, the ophthalmological status was assessed at periods from 1 week to 6 months after the injury. In all cases (n = 37; 100%), computed tomography of the facial and cerebral skull was performed. The age of the victims at the time of injury ranged from 12 to 82 years (average 39.7 ± 9.2 years). There were 29 adults among the victims (78.3%), 8 children (21.6%). In terms of gender distribution, there was a significant male predominance — 27 men (73%) versus 10 women (27%). Results: According to the results of the analysis of forensic medical reports, polytrauma with the simultaneous presence of several severe injuries to various organs and systems, combined with orbital trauma, was recorded in 12 victims (32.4%). A combination of traumatic brain injury and orbital injury without involvement of other organs and systems was detected in 9 victims (24.3%), isolated orbital trauma — in 13 people (35.1%), isolated injury of two orbits simultaneously — in 3 victims (8.1%). From the conclusions of forensic experts, it follows that in 89% of cases, the bone walls of the orbits, formed by the frontal, ethmoid and sphenoid bones, as well as the upper jaw, were damaged, which could subsequently lead to damage to the globe, optic nerve and other orbital structures. Damage to the soft tissue of the orbits with globe contusion was noted in 11% of cases. In 3 cases (n = 3; 8.1%), moderate harm to health was determined based on significant persistent loss of general ability to work. In 14 cases (n = 14; 37.8%), it was not possible to focus on the acuteness of the injured globe before the traumatic episode, due to the fact that the victims had no documented visits to an ophthalmologist before the injury. Conclusions: To objectively assess of the orbital trauma and determine the degree of harm to human health, it is necessary to have a full ophthalmological status, including such clinical and instrumental criteria as visual acuteness, presence or absence of ophthalmoplegia and globe dystopia, as well as computed tomography data of the skull, which must be presented in the primary medical documentation.

8403 A Rare Presentation: Mucosal Melanoma with Pituitary Gland Involvement

Abstract Disclosure: L.R. Sepulveda-Garcia: None. L. El Musa Penna: None. W. Medina-Torres: None. J. Segarra-Villafane: None. Z. Maisonet-Feliciano: None. I. Arroyo-Gomez: None. L.A. Gonzalez-Rodriguez: None. M. Alvarado-Santiago: None. M. Ramirez-Vick: None. Introduction: Sinonasal mucosal melanoma (SNMM) is a rare form of aggressive melanoma with poor prognosis, compromising less than 1% of all melanoma. The most frequently reported symptoms include nasal obstruction and epistaxis, but the manifestation of symptoms may occur late and be nonspecific, causing delays in diagnosis and resulting in a less favorable prognosis. Here we report a rare case of mucosal melanoma with pituitary gland involvement initially thought to be a pituitary adenoma. Clinical Case Description: A 22-year-old male with no previous medical history was found with a biopsy proven pigmented epithelioid melanocytoma (PEM) in the palate. Three weeks after the biopsy, the patient presented with a severe headache, prompting the performance of a CT scan that revealed a pituitary macroadenoma. Several days later he developed acute right eye ptosis requiring emergency evaluation. The patient reported mild epistaxis, headaches, and nausea of 2 weeks duration associated with lack of erections and diminished libido. Physical exam was remarkable for sinus bradycardia, right eye ptosis, and a palate bluish pigmentation. Hormonal work up revealed a prolactin of 23 ng/mL (3.46-19.40), TSH of 2.22 uIU/mL (0.27-4.20), fT4 of 0.37 ng/dL (0.93-1.70), total testosterone <0.007 ng/mL (1.88-6.84), free testosterone of 0.001 ng/mL (0.032-0.168), FSH of 0.41 mIU/mL (2.6-11.0), and LH of 0.27 mIU/mL (0.4-7). Serum cortisol and ACTH levels were found to be low at <1.00 µg/dL (3.7-19.40) and 5.98 pg/mL (0.00-46.00), respectively, but these were taken while patient was given dexamethasone by neurosurgery for headaches. Brain MRI was remarkable for 3.4 cm x 3.3 cm x 3.5 cm sellar mass extending inferior into the sphenoid sinus, posteriorly into the right ethmoid air cells, superiorly into the suprasellar cistern with upward displacement of the optic chiasm, as well as bilateral cavernous sinus invasion. Case outcomes: A diagnosis of hypopituitarism was made. Physiologic doses of hydrocortisone were started after being found with central adrenal insufficiency. This replacement unmasked an arginine vasopressin deficiency also requiring desmopressin therapy. Central hypothyroidism was treated with levothyroxine and treatment for the hypogonadotropic hypogonadism was deferred in the meantime. Biopsy of right sphenoid sinus revealed a mucosal melanoma. Revised pathology slides led to the conclusion that palate PEM was an independent entity to the mucosal melanoma Case discussion: Prognosis is poor in patients with SNMM since this tumor is usually diagnosed at advanced stages. In this case, the sellar mass was initially thought to be a pituitary adenoma that later was found to be an extension of a mucosal melanoma. This case serves as a reminder of the critical role that differential diagnosis plays in evaluating sellar masses. Presentation: 6/1/2024

The continuous rise in orbital subperiosteal abscess incidence in the Iranian pediatric population

In this study, we aimed to follow up on the surprising increase in the incidence of orbital subperiosteal abscesses (SPA) reported in the Iranian pediatric population in the post-COVID era. Pediatric patients (≤ 18 years old) diagnosed with orbital SPA following acute sinusitis who were hospitalized between October 1st, 2023, and March 31st, 2024 in Farabi Eye Hospital, Tehran, Iran, were included. Demographic, clinical, microbiologic, and radiological characteristics of patients were reviewed. During the 6-month study period, 39 patients were admitted to our tertiary referral hospital. The average age of patients was 7.29 ± 4.13 years and 30 patients (76.92%) were male. The ethmoid sinus was involved in 100% of patients, and SPA involvement was in the medial wall in 27 patients (69.23%). The mean volume of SPA was 1.45 ± 1.77 cm3. Nineteen patients (48.72%) underwent surgical interventions, however, 20 patients (51.28%) received only medical treatment. The best corrected visual acuity of patients increased from 0.63 (0.20 LogMAR) on the first day of admission to 0.84 (0.07 LogMAR) according to the Snellen chart in the first month after treatment. It seems that the outbreak of orbital SPA in the Iranian children population in the post-COVID era is an increasing trend.

7999 Tumor Induced Osteomalacia Caused By Glomangiopericytoma

Abstract Disclosure: N. Kabir: None. E. Campbell: None. S. Singh: None. Introduction: Tumor induced osteomalacia (TIO) is a rare paraneoplastic syndrome caused primarily by FGF-23 secreting tumors. Excessive production of FGF23 causes hypophosphatemia, decreased phosphate resorption in the gut, increased renal phosphate wasting, and decreased activation of Vitamin D. This leads to decreased bone density, bone pain, proximal muscle weakness, impaired gait, and recurrent fractures. Case Presentation: 44 year old male with a history of recurrent insufficiency fractures and weakness. At the time of evaluation, he reported fracture of tibial growth plate, sacral fracture, and metatarsal stress fracture. Other symptoms included bone pain, fatigue, muscle weakness, and intercostal muscle spasms with exertion. His pain was debilitating, requiring crutches to ambulate. Available labs at that time were significant for hypophosphatemia (1.3 mg/dL), elevated ALP (160 U/L), low 25OH-vitamin D (26 ng/mL), and normal calcium (8.7 mg/dL). Urine studies demonstrated phosphaturia (fractional excretion of phosphate, 62.4%). FGF23 level resulted at the upper limit of normal (210 RU/mL). Pending further evaluation, burosumab was started. Further work up included a 68Ga-PET-DOTATATE scan revealing a somatostatin receptor expressing soft tissue mass in the left sphenoid sinus and posterior left ethmoid sinus. The scan also demonstrated impending bilateral femur fractures, prior rib fractures, and sacral insufficiency fractures. Based on these findings, he was admitted to the hospital and underwent prophylactic, intramedullary nail fixation of his bilateral femur fractures. He underwent resection of skull base tumor with the final pathology revealing a sinonasal glomangiopericytoma. Immunohistochemical stain was positive for CD99 and cyclin D1, and focally positive for SMA, consistent with histopathologic diagnosis. Patient required prolonged inpatient rehabilitation to recover independent ambulation. Discussion: Sinonasal glomangiopericytoma (SNGP) is a mesenchymal tumor that accounts for <0.5% of all nasal and sinus tumors. TIO due to SNGP is extremely rare, with only a few cases reported since the 1990s. As seen with our patient, TIO from SNGP rarely presents with sinonasal symptoms, and can often be overlooked. Our patient hadb significant progression of disease and disability due to numerous delays in diagnosis and management. Definitive cure for TIO can be achieved with complete surgical resection of the tumor, but failure to diagnose in a timely manner can result in decline in quality of life. Our takeaway from this case is that providers should obtain FGF23 levels, especially in the setting of hypophosphatemia and phosphaturia, to complete the work up for osteomalacia. Presentation: 6/1/2024

9375 Acth-Producing Pituitary Carcinoma in the Falx Cerebri and Other Unusual Locations

Abstract Disclosure: J.E. Esquivel Vargas: None. A. Cob Guillén: None. A.B. Santos Rojo: None. A. Hong Lo: None. ACTH-producing Pituitary Carcinoma in the Falx Cerebri and other unusual locations Background: Pituitary carcinoma is a condition defined by metastasis of a pituitary tumor to a distant location. Pituitary carcinomas are very rare and account for approximately 0.12% of adenohypophyseal tumors (1). Clinical case: We present a case of a 29-year-old female who was followed up in our Endocrinology Department. Past medical history included the diagnosis of Cushing Disease and transsphenoidal tumor resection at 12 years of age, followed by transcranial resection two years later because of persistently elevated adrenocorticotropic hormone (ACTH). Despite the surgical management, the patient persisted with increased ACTH and hypercortisolism, so bilateral adrenalectomy was performed a year later. Two years after the procedure, the patient presented with a newly diagnosed pituitary macroadenoma, so the diagnosis of Nelson Syndrome was made. Linear accelerator radiotherapy was given, which reduced the size of the tumor. Later, multiple imaging studies showed multiple lesions on the falx cerebri, posterior clinoid process, retroclival region, cerebellopontine angle, pterygopalatine fossa, infratentorial region, and posterior ethmoidal cells. Biopsy and immunohistochemistry of the falx cerebri lesions described ACTH-producing pituitary adenocarcinoma. Treatment was given with intramuscular octreotide, dabrafenib, and trametinib. Despite persistently elevated ACTH levels, the patient has since remained clinically stable, without new development or worsening of symptoms. Conclusion: There are 3 unique aspects of our case. First, we reported an unusual presentation of this disease, because the patient in our case was a female with an early age of onset, in contrast with the reported literature. Second, this is the first reported case demonstrating pituitary carcinoma in the falx cerebri. Third, the prognosis of pituitary carcinoma is very poor, and mortality is extremely high, however, the patient in our case has been followed up for 7 years since the diagnosis of the metastatic lesions and has remained clinically stable.

Biphenotypic Sinonasal Sarcoma- A case Series with a Review of Literature.

Biphenotypic sinonasal sarcoma (BSNS) is a rare malignant tumor that affects the upper nasal cavity and ethmoid sinuses. It is more commonly found in middle-aged women and is characterized by the infiltration and hypercellular proliferation of spindle cells. These cells exhibit specific immunoreactivity. We add seven cases diagnosed as BSNS, to the handful of cases already available in the literature.BSNS is a malignant disease of the sinonasal tract that requires prompt and accurate diagnosis, followed by surgical resection and consideration of radiotherapy. Our analysis of seven cases supports previous research that confirms the aggressive nature of the disease but also shows that it is treatable with the right approach.

Achieving a Strong and Straight Septal Extension Graft: A Novel Four-step Surgical Approach in Rhinoplasty.

Septal extension grafts allow the precise elongation of the nasal septum to attain facial harmony and optimize aesthetic outcomes. In this context, septal surgery requires meticulous design to address both functional and aesthetic goals. Traditional septoplasty techniques often overlook aesthetic considerations and postoperative nasal structural stability, resulting in unresolved or recurrent deviations, nose tip drooping, and obstruction recurrences. Despite advancements in surgical techniques, achieving simultaneous septal alignment and long-term stability remains a significant challenge. This study introduces a systematic 4-step technique. The procedure encompasses caudal septum liberation, placement of the septum extension cartilage graft, positioning of an ethmoidal bone graft, and redefinition through an intermediate cartilage. A retrospective analysis of outcomes involving 753 patients was conducted with a minimum follow-up of 6 months. During follow-up visits, 1.73% of cases exhibited residual columellar asymmetry, and 0.66% showed tip asymmetries. The infection rate was 0.40%, with no cases of tip drop identified. The overall satisfaction rating on the Rhinoplasty Outcomes Evaluation questionnaire at 12 months postoperative was 9.3. The technique provides a systematic approach to integrate functional and aesthetic objectives, emphasizing septal stability and alignment while concurrently addressing tip projection and stability. The study provides comprehensive insights into the principles, methodology, and advantages of this rhinoplasty technique. The outcomes underscore the efficacy of the technique, presenting a reliable and long-term stable solution.