Endoscopically managed giant frontoethmoidal osteoma with orbital extension.

A patient in his 20s presented with a change in the appearance of his left eye with evidence of relative afferent pupillary defect. Imaging revealed a giant frontoethmoidal osteoma, a benign sinonasal tumour, invading three-quarters of the orbit. Multidisciplinary discussion involving opthalmology, maxillofacial surgery, neurosurgery and otolaryngology resulted in the decision to attempt entirely endoscopic excision of this lesion, which was performed with successful outcomes. This case demonstrates how a sinonasal osteoma should be considered in the differential diagnosis for a patient presenting with proptosis or other eye signs suggestive of compression of the orbital compartment. This case report and literature review highlights the possibility of managing giant sinonasal osteomas with orbital extension through a completely endoscopic approach.

Anterior Skull Base Abnormalities in Congenital Anosmia.

INTRODUCTION: The structures of the skull and the brain are related to each other. Prior work in individuals with isolated congenital anosmia (ICA) showed that these individuals were characterized by olfactory bulb (OB) defects. The aim of this study was to compare the morphological pattern of the anterior skull base surrounding the OB between individuals with ICA and normosmic controls. We meant to investigate whether these features can help distinguish abnormalities from normal variation. METHODS: We conducted a retrospective study to acquire T2-weighted magnetic resonance images from individuals diagnosed with ICA (n = 31) and healthy, normosmic controls matched for age and gender (n = 62). Between both groups, we compared the depth and width of the olfactory fossa, the angle of the ethmoidal fovea, as well as the angle of the lateral lamella of the cribriform plate. Within the ICA group, we further performed subgroup analyses based on the presence or absence of the OB, to investigate whether the morphology of the anterior skull base relates to the presence of OBs. The diagnostic performance of these parameters was evaluated using receiver operating characteristic analysis. RESULTS: Individuals with ICA exhibited a flattened ethmoid roof and shallower olfactory fossa when compared to controls. Further, the absence of the OB was found to be associated with a higher degree of flattening of the ethmoid roof and a shallow olfactory fossa. We reached the results in the following areas under the receiver operating characteristic curves: 0.80 - angle of fovea ethmoidalis, 0.76 - depth of olfactory fossa, 0.70 - angle of lateral lamella of the cribriform plate for significant differentiation between individuals with ICA and normosmic controls. CONCLUSION: Individuals with ICA exhibited an unusual anterior skull base surrounding the OB. This study supports the idea of an integrated development of OB and anterior skull base. Hence, the morphological pattern of the anterior skull base surrounding the OB helps distinguish individuals with ICA from normosmic controls and may therefore be useful for the diagnosis of ICA, although it is certainly not an invariable sign of congenital anosmia.

Incidental seromucinous hamartoma of the anterior nasal cavity presenting after episode of vestibular neuritis.

A woman in her 70s presented to primary care clinic complaining of acute onset dizziness for 1 day that was initially diagnosed as vestibular neuritis and treated with steroids. The next day, she presented to the emergency department with worsening symptoms. Imaging revealed no intracranial process; however, non-contrast CT imaging revealed a soft-tissue mass in the posterior ethmoid sinus. The vertigo completely resolved before an otolaryngologist surgically removed the nasal mass, which actually originated from the right cribriform plate and extended to the anterior middle turbinate head. The final pathology was consistent with seromucinous hamartoma.

Glioma with cribriform plate involvement in 6 dogs.

Distinct patterns of local infiltration are a common feature of canine oligodendroglioma and astrocytoma, and typically involve the surrounding neuroparenchyma, ventricles, or leptomeninges. Infiltration of adjacent extraneural sites is rare and has not been well documented in veterinary medicine. Here we describe 6 canine gliomas with cribriform plate involvement (compression or infiltration) and caudal nasal invasion confirmed by neuroimaging, autopsy, and/or histology. All affected dogs were adults (9-12-y-old), and 3 were brachycephalic. Clinical signs were associated with the brain tumor, with no respiratory signs reported. Magnetic resonance imaging in 2 patients revealed a rostral intraparenchymal telencephalic mass with extension into the cribriform plate. All dogs were euthanized. Gross changes consisted of poorly demarcated, white or pale-yellow, soft, and, in oligodendrogliomas, gelatinous, intraparenchymal masses that expanded the rostral portions of the telencephalon and adhered firmly to the ethmoid bone and cribriform plate. Gliomas were classified as high-grade oligodendrogliomas (4 cases) and high-grade astrocytomas (2 cases) based on histology and immunohistochemistry for OLIG2 and GFAP. In all cases, there was evidence of cribriform plate invasion and, in one case, additional invasion of the caudal nasal cavity.

MRI and CT characteristics of a grade I meningioma with concurrent cribriform plate lysis in a dog.

A 6-year-old female spayed German Shepherd mixed-breed dog was presented for treatment of a frontal lobe mass diagnosed on MRI, after an acute onset of generalized seizures and behavior changes. Computed tomography of the head was performed for radiation therapy planning and revealed concurrent cribriform plate lysis without nasal sinus invasion, and focal lysis of the left ventrolateral cranial fossa. Histopathology of the mass obtained via surgical excision was consistent with a grade I fibrous meningioma. The dog had a good outcome following surgery and radiation therapy.

Ethmoidal osteoma in children: Literature review and presentation of a case report / Osteoma etmoidal en niños: revisión de la literatura y presentación de un caso

No disponible

Ethmoidal meningoencephalocele in a C57BL/6J mouse.

An otherwise healthy two-month-old female C57BL/6J mouse presented with a left-sided head tilt. Differential diagnoses included idiopathic necrotizing arteritis, bacterial otitis media/interna (Pasteurella pneumotropica, Pseudomonas aeruginosa, Streptococcus sp., Mycoplasma pulmonis and Burkholderia gladioli), encephalitis, an abscess, neoplasia, a congenital malformation and an accidental or iatrogenic head trauma. Magnetic resonance imaging (MRI) revealed a large space-occupying right olfactory lobe intra-axial lesion with severe secondary left-sided subfalcine herniation. Following imaging, the animal was euthanized due to poor prognosis. Histopathologic examination revealed a unilateral, full-thickness bone defect at the base of the cribriform plate and nasal conchae dysplasia, resulting in the herniation of the olfactory bulb into the nasal cavity. There was also a left midline-shift of the frontal cortex and moderate catarrhal sinusitis in the left mandibular sinus. The MRI and histopathologic changes are consistent with a congenital malformation of the nasal cavity and frontal aspect of the skull known as an ethmoidal meningoencephalocele. Encephaloceles are rare abnormalities caused by herniation of contents of the brain through a defect in the skull which occur due to disruption of the neural tube closure at the level anterior neuropore or secondary to trauma, surgical complications, cleft palate or increased intracranial pressure. The etiology is incompletely understood but hypotheses include genetics, vitamin deficiency, teratogens, infectious agents and environmental factors. Ethmoidal encephaloceles have been reported in multiple species including humans but have not been reported previously in mice. There are multiple models for spontaneous and induced craniofacial malformation in mice, but none described for ethmoidal encephaloceles.

Development of Rift valley fever encephalitis in rats is mediated by early infection of olfactory epithelium and neuroinvasion across the cribriform plate.

The zoonotic emerging Rift Valley fever virus (RVFV) causes sporadic disease in livestock and humans throughout Africa and the Saudi Arabian peninsula. Infection of people with RVFV can occur through mosquito bite or mucosal exposure during butchering or milking of infected livestock. Disease typically presents as a self-limiting fever; however, in rare cases, hepatitis, encephalitis and ocular disease may occur. Recent studies have illuminated the neuropathogenic mechanisms of RVFV in a rat aerosol infection model. Neurological disease in rats is characterized by breakdown of the blood-brain barrier late in infection, infiltration of leukocytes to the central nervous system (CNS) and massive viral replication in the brain. However, the route of RVFV entry into the CNS after inhalational exposure remains unknown. Here, we visualized the entire nasal olfactory route from snout to brain after RVFV infection using RNA in situ hybridization and immunofluorescence microscopy. We found widespread RVFV-infected cells within the olfactory epithelium, across the cribriform plate, and in the glomerular region of the olfactory bulb within 2 days of infection. These results indicate that the olfactory tract is a major route of infection of the brain after inhalational exposure. A better understanding of potential neuroinvasion pathways can support the design of more effective therapeutic regiments for the treatment of neurological disease caused by RVFV.

Chondroma in the Area of the Spheno-Ethmoid Junction.

Chondroma in the area of the spheno-ethmoidal junction is very rare. A 29-year-old male patient with chronic rhinosinusitis with nasal polyps was arranged for a preoperative computed tomography scan, and a lesion was accidentally found in his spheno-ethmoidal junction and involved the skull base. Combined with MRI, the lesion was misdiagnosed as fungal sinusitis. However, no fungal lesions were found during the operation, and cartilage tissue was confirmed only after some bone was ground away under the guidance of a surgical navigation system. Our case indicates that chondroma is easily misdiagnosed as fungal sinusitis when it appears in the sinuses and should be carefully distinguished from fungal sinusitis. Moreover, when lesions involve the skull base, surgical navigation systems are useful in accurately locating lesions.

Pneumatized Crista Galli: A Histopathologic Study.

OBJECTIVE: This is the first histopathologic study that investigates the incidence of the pneumatized crista galli. STUDY DESIGN: A prospective histopathologic study. SETTING: Tertiary academic medical center. SUBJECTS AND METHODS: A total of 109 specimens of crista galli were obtained postmortem during 2018 from randomly chosen patients who died at the University Hospital Centre Zagreb and had an autopsy at our Department of Pathology and Cytology. Specimens were surgically resected during the autopsy and then fixed, decalcinated, dehydrated, and embedded in paraffin. All slides were cut into 5-µm-thin sections and stained with a standard method (hematoxylin and eosin) for light microscope analysis. Specimens were histopathologically analyzed for the existence of pneumatization inside crista galli. The criterion to declare a specimen pneumatized was the presence of mucosa inside the cavity. RESULTS: Pneumatized crista galli was found in 5 of 109 specimens (4.59%). In 5 of 5 cases (100%) of pneumatized crista galli, there was evidence of chronic inflammation. CONCLUSION: We found that the incidence of pneumatized crista galli is significantly lower in our histopathologic study in comparison with the majority of previous radiologic studies. Our study also found that all 5 specimens with pneumatized crista galli had chronic inflammation in the mucosa, which is a considerably higher incidence than in the previous studies (7.7%-44%). Due to the emerging evidence of pneumatized crista galli being of clinical importance, we suggest that a larger study be conducted before the results are generalized to the general population.

Fibroblast Growth Factor 23-Producing Phosphaturic Mesenchymal Tumor with Extraordinary Morphology Causing Oncogenic Osteomalacia.

A possible cause of hypophosphatemia is paraneoplastic secretion of fibroblast growth factor 23 (FGF-23). Tumors secreting FGF-23 are rare, mostly of mesenchymal origin, usually benign, and may be located anywhere in the body, including hands and feet, which are often not represented in conventional imaging. A 50-year-old woman presented with diffuse musculoskeletal pain and several fractures. Secondary causes of osteoporosis were excluded. Laboratory analysis revealed hypophosphatemia and elevated alkaline phosphatase, parathyroid hormone, and FGF-23. Thus, oncogenic osteomalacia due to neoplastic FGF-23 secretion was suspected. FDG-PET-CT and DOTATATE-PET-CT imaging demonstrated no tumor. Cranial MRI revealed a tumorous mass in the left cellulae ethmoidales. The tumor was resected and histopathological examination showed a cell-rich tumor with round to ovoid nuclei, sparse cytoplasm, and sparse matrix, resembling an olfactory neuroblastoma. Immunohistochemical analysis first led to diagnosis of olfactory neuroblastoma, which was later revised to phosphaturic mesenchymal tumor. Following the resection, FGF-23 and phosphate levels normalized. In conclusion, we here describe a patient with an FGF-23-secreting phosphaturic mesenchymal tumor with an unusual morphology. Furthermore, we emphasize diagnostic pitfalls when dealing with FGF-23-induced hypophosphatemia.

A rare differential for sinusitis and visual loss.

Erdheim-Chester disease is a rare infiltrative histiocytic disorder with around 800 cases being reported worldwide. Patients most commonly present with skeletal pain, but the condition has been shown to affect multiple other organs. We describe a rare presentation in which the disease infiltrated the sinuses and affected an ex-RAF pilot's vision. After extensive investigation of the elusive diagnosis, repeating of a molecular test using polymerase chain reaction analysis allowed for identification of a mutation (BRAF V600) ultimately leading to the diagnosis of Erdheim-Chester disease.

Optic Neuropathy, Secondary to Ethmoiditis, and Onodi Cell Inflammation during Childhood: A Case Report and Review of the Literature.

Optic neuropathy consists of several etiological events. The primary etiologies of its acute form include optic neuritis, ischemic optic neuropathy, inflammatory (nondemyelinating) disorders, and trauma. Its subacute and chronic forms are most often linked to compressive, toxic, nutritional, or hereditary-genetic causes. Visual loss, dyschromatopsia, and visual field defects are the presenting symptoms. The Onodi cell (sphenoethmoidal air cell) is an anatomic variant located laterally and superior to the sphenoid sinus; it is closely related to the optic nerve. Onodi cell disorders are rare and may be unnoticed in differential diagnoses of patients with ocular and neurological manifestations. Here, we present the case of a 12-year-old boy with headache and acute loss of sight characterized by hemianopsia in the left eye and retrobulbar optic neuropathy caused by left sphenoethmoidal sinusitis with the presence of Onodi cell inflammation. The diagnosis was confirmed by multilayered paranasal computed tomography and cerebral magnetic resonance imaging. Therapeutic treatment resulted in gradual improvement: at the 2-week follow-up, the patient no longer had headaches and his visual acuity returned to normal. Inflammation of Onodi cells should be considered in children with headache and abnormal vision.

Death Related to Elective Nasal Polypectomy: Case Report.

The investigation of deaths that are suspected to be related to medical therapy present several challenges for a forensic pathologist. We present a case of an otherwise healthy 58-year-old woman with multiple nasal polyps who underwent nasal polypectomy. The operation was initially considered successful. However, the patient had never recovered from general anesthesia and was declared deceased 24 hours after the surgery.The autopsy revealed a basilar subarachnoid hemorrhage. The examination of the basilar skull showed a perforation of approximately 15 by 7 mm in the right cribriform plate. Above the bone perforation, there was a disruption of the dura and a 20-mm-long penetrating wound within the right frontal lobe parenchyma of the brain, with associated intraventricular hemorrhage. The subsequent sectioning of the formalin-fixed brain revealed extensive parenchymal destruction. The cause of death was certified as complications of nasal/sinus surgery, with a perforation of the skull base with hemorrhagic tissue destruction, whereas the manner of death was considered accidental.Common nasal surgical procedures and known complications are discussed.

Anatomic variations associated with antrochoanal polyps.

OBJECTIVE: Although more than a century has passed since antrochoanal polyps (ACPs) were first defined, etiopathogenesis still remains unclear. The aim of this study was to investigate the relationship between ACPs and sinonasal cavity variations. SUBJECTS AND METHODS: One hundred and forty-four patients with ACP on paranasal sinus computed tomography scans (ACP group) and 160 paranasal sinuses without ACP (control group) were included into the study. The study group was evaluated in respect of the presence of retention cyst in the contralateral maxillary sinus and sinus bone wall sclerosis thickening. Both groups were also compared with respect to the frequency of sinonasal anatomic variations, nasal septal deviation, variations of the uncinate process insertion, concha bullosa, paradoxical middle turbinate, and accessory maxillary sinus ostium. In the ACP group, the cases with septal deviation (SD) were also evaluated whether the deviation convexity was towards the polyp side or the opposite side. In addition, the posterior extension of ACPs were evaluated in three groups as middle meatus, nasopharynx, and oropharynx extension. RESULTS: The prevalence of retention cyst, sinus wall sclerosis thickening, SD, and accessory maxillary ostium was significantly higher in the ACP group. A negative directional correlation was determined between the SD side and ACP side. When the ACP extensions were examined, middle meatus extension was seen in 32.6%, nasopharynx in 56.3%, and oropharynx in 11.1%. CONCLUSION: Accessory ostium may be an accelerating factor in the transformation of retention cyst to ACP. Furthermore, the changes in the nasal passage airflow on the opposite side suggest that SD contributes to this process.

Does paranasal sinus development affect olfactory fossa depth and lateral lamella length?

OBJECTIVES: To investigate the olfactory fossa depths and lateral lamella lengths of patients with different types of developmental disorders of paranasal sinuses in comparison with normal controls. STUDY DESIGN: Retrospective, archival, radio-anatomical study. METHODS: We included 58 patients with maxillary sinus hypoplasia, 50 patients with frontal sinus hypoplasia/aplasia, 50 patients with sphenoid sinus hypoplasia/aplasia, and 40 normal controls. Reviewing paranasal computerized tomography scans, we noted the olfactory fossa depths and lateral lamella lengths of all the groups and compared between the hypoplasia groups and the control group. RESULTS: Compared with the normal controls, the maxillary hypoplasia group (P < 0.001), frontal hypoplasia/aplasia group (P = 0.004), and sphenoid hypoplasia/aplasia group had significantly deeper olfactory fossa (P = 0.003). The mean lateral lamella lengths in the type 1, type 2, and type 3 hypoplastic maxillary sinus groups were significantly greater compared with that in the control group (P < 0.001). Additionally, the mean lateral lamella lengths in the hypoplastic frontal sinus, aplastic frontal sinus, and hypoplastic sphenoid sinus groups were significantly greater compared with that in the control group (P < 0.001). CONCLUSION: The patients with pneumatization defects of the maxillary, frontal, and sphenoid sinuses had deeper olfactory fossa and longer lateral lamella related to increased risk of skull base injury during endoscopic sinus surgery. LEVEL OF EVIDENCE: 3 Laryngoscope, 129:2458-2463, 2019.

More than meets the eye.

We report an extremely rare case of a hybrid tumour of the maxillary sinus. A 51-year-old man presented with a 6-week history of nasal congestion and epiphora. Radiological imaging demonstrated a maxillary sinus tumour, with extensive local invasion. Surgical excision included maxillectomy, left eye exenteration and free flap closure. Histology of the excised specimen showed a rare hybrid tumour containing adenoid cystic carcinoma, salivary duct carcinoma, epithelial-myoepithelial carcinoma and basal cell adenoma. Hybrid tumours are very rare tumour entities which are composed of at least two distinct tumour types. Each tumour entity conforms with a defined tumour type. The tumour entities of a hybrid tumour are not separated but have an identical origin within a definite topographical area. Diagnosis and appropriate management requires high index of suspicion, pathological endeavour to look for a more aggressive accompanying tumour and adequate oncological treatment according to the highest grade of tumour.

Is there a relationship between Onodi cell and optic canal?

OBJECTIVES: We investigated the relationship between Onodi cells and optic canal by paranasal sinus computed tomography (PNSCT). METHODS: In this retrospective study, 508 PNSCT (265 males and 243 females) was examined. Onodi cell presence, pneumatization types, optic canal types; and also sphenoid sinusitis and anterior clinoid process pneumatization were evaluated. RESULTS: The prevalence of Onodi cells was 21.2% of the patients. Onodi cells were observed 40.7% on the right side and 25.9% on the left side. In 33.4% of the patients, bilateral Onodi cells were present. Male/Female ratio was 24.5%/17.6%. Onodi cell types were detected as Type I > Type II > Type III bilaterally. There was a positive correlation between the right and left Onodi cell types (p < 0.05). Optic canal types were detected as Type IV > Type I > Type II > Type III. bilaterally. There was a positive correlation between right and left optic canal types. Onodi cell presence and ACP pneumatization were found as statistically significant (p < 0.05). In 65.5% of the patients, Onodi cells and ACP pneumatization were absent. ACP pneumatization was present in 35.4% of the cases. In nine cases, bilateral Onodi cells and ACP pneumatization were detected. Sphenoid sinusitis was detected in 11.4% of Type I and 13.8% of the Type II Onodi cells on the right side. On the left side, it was detected in 12.9% of the Type I and 19.0% of Type II Onodi cells. CONCLUSION: Identification of Onodi cell is very important clinically because of its proximity to optic nerve canal. We concluded that type IV Onodi-optic canal relationship was the most common finding in our study. Onodi cell presence and their patterns of pneumatization must be evaluated on PNSCT preoperatively to avoid optic canal damage.

Giant Frontal Sinus Osteomas: Demographic, Clinical Presentation, and Management of 10 Cases.

BACKGROUND: Osteomas are rare benign and slow-growing osteogenic tumors mainly involving frontal and ethmoid sinuses. OBJECTIVES: The primary objective of our study is to present the management of cases of giant frontal sinus osteomas. Secondarily, we describe our modified unilateral osteoplastic flap approach without obliteration to remove these osteomas. METHODS: Retrospective chart review at a tertiary academic center ("Hôpital de l'Enfant-Jésus") from July 2006 to October 2016. Demographics characteristics, tumor characteristics, presenting symptoms, frontal sinus surgery technique (osteoplastic flap, endoscopic surgery, or a combination of both), and outcomes of giant frontal sinus osteomas (≥30 mm) were recorded. For laterally placed osteomas, tumors with posterior wall involvement, orbital roof involvement, or intracranial extension, the modified unilateral osteoplastic flap approach was used. A decision-making algorithm is proposed for the choice of surgical approach. RESULTS: Ten giant frontal osteomas were analyzed (7 men and 3 women). The mean age at diagnosis was 38 years old (range, 24-55 years; median, 39 years; standard deviation, 11 years). The most common presenting symptom was headache (43% of symptomatic patients). Five patients had complications preoperatively due to tumoral extension (sinusitis, cellulitis, mucocele, optic nerve compression, and convulsions). One patient was treated endoscopically, 3 patients had an open approach and 6 patients had a combined technique. One patient experienced a postoperative complication (local infection treated with oral antibiotics). Six patients had minimal residual tumor with one patient needing reoperation. CONCLUSION: Osteomas are rare paranasal sinus tumors. Due to the proximity to noble structures, a giant frontal osteoma should be managed surgically. The modified unilateral osteoplastic flap without obliteration offers good long-term surgical and aesthetic results. Osteomas are not known for malignant transformation and recurrences are rare; thus, subtotal resection is warranted and safe when a cleavage plan is not found.