Multidisciplinary approach to severe intracranial, intraorbital allergic fungal sinusitis.

BACKGROUND: Allergic fungal sinusitis (AFS) is a form of paranasal mycosis that often involves bone destruction and can extend into the orbit and anterior skull base. Intracranial and intraorbital involvement are published but not both in each included patient of a series. The purpose of the present study was to review cases of extensive AFS with orbital or/and skull base erosion, including the presenting symptoms, patient socioeconomic background, imaging features, surgical technique, and post-operative outcomes. METHODS: The records of 30 patients with the histological diagnosis of AFS and both intracranial and intraorbital involvement were reviewed. RESULTS: The average age of the patients was 25.2 years. 83 % of patients were male. 70 % were African American. 66 % of patients had Medicaid or were uninsured. Presenting symptoms were headaches (80 %), nasal obstruction (33 %), proptosis (40 %), vision change (23 %), facial pressure (10 %), and decreased sense of smell (7 %). 100 % of patients had bone erosion observed on computerized tomography scans with disease extending intracranially through the anterior skull base or posterior wall of the frontal sinus along with disease that eroded through the lamina papyracea. All patients had failed medical management. All patients underwent surgery by Otolaryngology, Ophthalmology, and Neurosurgery with transnasal endoscopic approaches and orbitotomy. 80 % also underwent a bifrontal craniotomy for removal of intracranial extradural disease with cranialization. 53 % of patients had orbital implants remaining after surgery; 23 % had nasal stents. There were no postoperative cerebrospinal fluid leaks. Average hospital stay was 4.8 days. Preoperatively, 13 % of patients underwent allergy testing. 94 % saw an Allergist postoperatively. 21 % required follow up surgery. CONCLUSIONS: A team approach of Otolaryngologists, Ophthalmologists, and Neurosurgeons is recommended for this slowly growing but locally destructive disease. Most patients with AFS will present with headaches and nasal obstruction. While most cases of AFS can be successfully managed with transnasal endoscopic techniques, orbitotomy and craniotomy represents an effective treatment for severe AFS cases with extensive intracranial and intraorbital involvement. This disease is found most commonly in young African American males and in the underinsured.

Intracranial extension of an intramuscular haemangioma of superior rectus: case report and literature review.

BACKGROUND: Intramuscular haemangiomas are rare, benign vascular tumours that represent < 1% of all haemangiomas. When involving the extraocular muscles, haemangiomas are extremely rare, with only nine cases reported in the literature; to date there are no reported cases of extraocular muscle haemangiomas extending into the brain. CASE PRESENTATION: A 6-year-old boy with a two-week history of a painless swelling and erythema on the upper eyelid. On examination, the patient had restricted extraocular motility in all directions of gaze. In addition, the eye appeared proptotic, with chemosis and hyperaemia of the conjunctiva. Visual acuity and intraocular pressure were normal. Orbital computed tomography imaging showed a mass in the left superior rectus, with heterogeneous enhancement following contrast administration. Incisional biopsy revealed an intramuscular haemangioma of the superior rectus muscle with capillary-type vessels. The patient received an intralesional steroid, which improved the condition for a few months; however, the lesion later recurred and included an intracranial extension. CONCLUSION: This case represents the first reported case of intracranial extension of intramuscular haemangioma of extraocular muscle.

Infantile Atypical Teratoid Rhabdoid Tumor of the Spine Presenting with Acute Hydrocephalus.

INTRODUCTION: Central nervous system atypical teratoid rhabdoid tumors (ATRTs) are aggressive lesions usually presenting during the first 3 years of life. These tumors have a dismal prognosis with most patients dying within 1 year from presentation. Primary spinal location in infants is very rare. CASE PRESENTATION: We report a case of a 4-month-old boy who presented with a history of hypotonia, poor head control, and gradually reduced level of consciousness, over the past week. Computed tomography (CT) showed acute hydrocephalus with no underlying intracranial pathology. A ventriculoperitoneal shunt was inserted acutely. Postoperatively, ventilator weaning was unsuccessful. MRI of the brain and whole spine revealed an intraspinal extradural contrast-enhancing heterogenous mass in the subaxial cervical spine extending to the thoracic cavity. A biopsy was taken through a transthoracic approach, and histopathology confirmed the diagnosis of ATRT. Several cycles of radiation therapy and chemotherapy were given but the tumor progressed both locally and intracranially. Eventually, pupils became dilated and fixed. Brain CT scan showed widespread ischemic lesions and an extensive intracranial tumor extension with massive bleeding. The child eventually died 110 days after admission. CONCLUSIONS: In infants presenting with acute hydrocephalus where an obvious intracranial cause is not detected, the whole neuraxis should be screened. However, despite aggressive measures and advances in multimodality treatment, prognosis of ATRT remains dismal.

Median Cleft Lip with Nasal Dermoid: A Rare Occurrence.

Median cleft lip with nasal dermoid is a very rare occurrence. Midline nasal dermoid results from the abnormal fusion of embryological processes. They can present as asymptomatic midline nasal swelling, infection, or meningitis due to intracranial extension. Median cleft lip also results from failure of fusion of embryological processes and can be complete or incomplete or associated with other congenital anomalies or as a part of syndrome. We present a rare combination of both in a 7-month-old female child who was surgically treated.

Diagnostic performance of CT, MRI, and their combined use for the assessment of the direct cranial or intracranial extension of malignant head and neck tumors.

BACKGROUND: Preoperative radiological evaluation of the cranial or intracranial extension of malignant head and neck tumors is critical in the planning of curative surgery. PURPOSE: To assess the diagnostic accuracy of computed tomography (CT) combined with magnetic resonance imaging (MRI), compared to CT or MRI alone in diagnosing the direct cranial or intracranial extension of malignant head and neck tumors, using histopathologic results as the reference standard. MATERIAL AND METHODS: CT and MRI images in 41 patients with malignant head and neck tumors abutting the skull were retrospectively reviewed. The images were evaluated for the presence or absence of skull invasion (erosion/destruction of the skull), dural invasion (nodular dural enhancement), and brain invasion (enhancing brain lesion with or without brain swelling/edema). The results of the CT alone, MRI alone, and CT combined with MRI were compared with the histopathologic findings. RESULTS: Of the 41 patients studied, ten had no invasion, eight had skull invasion, 17 had dural invasion, and six had brain invasion by tumor. The sensitivity/specificity/accuracy of CT alone, MRI alone, and CT combined with MRI for diagnosing intracranial extension were 78.0%/100%/94.5%, 85.4%/80.5%/93.9%, and 95.1%/100%/98.8%, respectively. The sensitivity of CT combined with MRI was significantly higher than those of CT alone ( P = 0.0156) and MRI alone ( P = 0.0313). CONCLUSION: CT combined with MRI is a more sensitive tool for the diagnosis of the direct cranial or intracranial extension of malignant head and neck tumors than CT alone and MRI alone.

Management of congenital midline nasofrontal dermoid cysts in two identical twins: Case report.

Midline dermoid cysts are uncommon lesions that can lead to severe complications when an intracranial extension exists. We report the cases of two twin sisters referred to surgery for removal of the masses and the intracranial extension. They represent an additional example of familial nasal dermoid cysts, providing further support for a genetic basis for the disorder.

Intracranial complications of midline nasal dermoid cysts.

BACKGROUND: Nasal dermoid cysts are common tumors in children. Due to anomalies in embryologic development of the nasal complex, sometimes an intracranial extension exists. When these cysts become infected they can lead to meningitis, brain abscess and death. METHODS: We report the case of a 1.5-year-old girl admitted to the paediatric intensive care unit after infection of a nasal dermoid cyst. RESULTS: The infant had a spiking fever and epileptic seizures. She was stabilized, intubated and a CT scan showed a subcutaneous mass with an adjacent zone of encephalitis and brain abscess formation. Neurosurgical interventions were necessary to lower intracranial pressure and control infectious spread. After a hospital stay of 69 days the child could be discharged. Due to her young age, irreversible brain damage is expected. CONCLUSION: Nasal midline dermoid cysts are considered benign swellings. When an intracranial extension exists, infection can lead to deleterious complications. It is important for health care practitioners to be aware of this imminent risk. Suspicion of a nasal midline dermoid cyst should prompt a careful clinical work-up with an ultrasound followed by CT or MRI imaging. The treatment is complete excision to avoid disastrous complications and recurrences.

Nasopharyngeal carcinoma with intracranial extension in the era of intensity-modulated radiotherapy: case-control study using propensity score matching method.

The objective of the study was to evaluate long-term survival outcomes and toxicity of T4 classification nasopharyngeal carcinoma (NPC) with intracranial extension (IE group) or without intracranial extension (non-IE group) after intensity-modulated radiotherapy (IMRT) using the propensity score matching method. After generating propensity scores given the covariates of age, sex, N classification, and concurrent chemotherapy, 132 patients in each group were matched. The 5-year local failure-free survival rate and the 5-year overall survival rate in the IE group were lower than the patients in the non-IE group (74.6 vs. 88.9 %, p = .008; 51.1 vs. 71.9 %, p = .005). Grade 2 hypothyroidism was more common in the IE group (13.2 vs. 3.4 %, p = .029). For patients with T4 classification NPC after IMRT, patients with intracranial extension need more attention to the thyroid gland function and are more likely to experience local failure and death than patients without intracranial extension.

A giant frontoethmoid mucocele with intracranial extension.

Mucoceles are mucus-containing cysts lined by epithelium. Although benign, they may show expansive growth and remain undiagnosed until symptoms due to compression of surrounding structures arise. We report a rare case of frontoethmoid mucocele with intracranial extension in an 80-year-old woman with complaints of headache, right diplopia and proptosis. A right frontoorbital craniotomy was performed, and a mucocele in the frontal sinus extending into the frontal lobe and orbit was totally removed. The patient was successfully treated without any complication. The two-year follow-up results were satisfactory. Magnetic resonance imaging excluded any recurrence of the mucocele. Combined intranasal and transcranial approach is necessary to treat giant frontoetmoid mucoceles with intracranial extension.

Primary spinal oligodendroglioma with intracranial extension: a case report.

Introduction: Primary spinal cord oligodendrogliomas (PSO) are sporadic tumors that arise from oligodendrocytes in the central nervous system (CNS). They can affect adults and children and make up about 2% of all intramedullary (IM) spinal tumors. Here, the authors present the second case in the literature of a primary spinal oligodendroglioma with intracranial extension. Presentation: A 28-year-old right-handed female presented to our emergency room severely malaised with left-sided hemiparesis, numbness, tingling, and urinary retention with positive Babinski and negative Hoffmann. MRI showed a widespread heterogeneous mass extending from the medulla to C7 with syringomyelia inferior to the mass. The mass was removed surgically, and her neurological condition improved rapidly. The gross, pathological exams, and immunohistochemistry confirmed the diagnosis of oligodendroglioma. Discussion: Up until 2017, there have been 60 documented cases of PSO in the literature and we have found two more cases in our search between 2017 and 2023. Also, there has been only one case recorded with an intracranial extension, making our case the 63rd PSO case and the second one with cranial extension. Conclusion: The golden standard for imaging is MRI. Surgical excision is the main treatment in the literature. Single-stage laminectomy showed promising results and surgical resection was the critical intervention to which the patient responded. This matches what was stated in the literature that surgery is the primary mode of treatment in PSO patients.

The Role of Preoperative Imaging for Midline Congenital Upper Lip Sinus Tracts.

OBJECTIVE: To describe 3 cases of midline congenital upper lip sinus (MCULS) and review current literature to inform risk of intracranial involvement in the context of this rare congenital facial anomaly. MATERIALS AND METHODS: A limited case series with chart review is presented. A literature search was conducted to review proposed theories of the embryology of MCULS and to determine the relative frequency of cephalic extension. RESULTS: Including the 3 new cases presented herein, there have been 42 cases of MCULS described in the literature over the past 53 years. Thirty-nine cases (93%) underwent surgical excision, with 2 of these cases (4.7%) demonstrating cephalic extension of the fistula tract beyond the maxillary crest with termination at the anterior skull base. However, 95% (37/39) of surgically excised MCULS cases demonstrated a more limited depth of extension, with termination of the tract at or below the anterior nasal spine. CONCLUSIONS: The MCULS anomaly is rare, with fewer than 50 cases reported in the literature. Only 2 cases have been described with extension of the MCULS superior to the anterior nasal spine and into the nasal septum. It is the authors' opinion that preoperative neuroimaging is not routinely required for MCULS. However, if extension of the sinus tract beyond the anterior nasal spine is noted intraoperatively, the surgeon should consider aborting the case and obtaining appropriate neuroimaging.

Exploring Sociodemographic Factors in Allergic Fungal Rhinosinusitis in a Northern California Patient Population.

BACKGROUND: Allergic fungal rhinosinusitis (AFRS) is a subtype of chronic rhinosinusitis (CRS) that has previously been associated with younger age and Black patients. However, the role of demographic and socioeconomic factors in AFRS severity remains to be fully elucidated. OBJECTIVE: The objective of this study was to determine whether demographic and socioeconomic factors are associated with incidence of AFRS, as well as with disease severity in Northern California. METHODS: A retrospective cohort study was conducted of adult patients with AFRS and CRS from 2010 to 2019. AFRS was determined by the Bent and Kuhn criteria, and severity was assessed by radiographic evidence of cranioorbital invasion and other clinical parameters. Chi-square and t-test were used to assess demographic and socioeconomic differences between AFRS and CRS cohorts, and multivariable logistic regression was used to assess risk factors for severe AFRS. RESULTS: Black patients represented 26.2% (55/210 patients) of the AFRS group and 4.9% (842/17,300 patients) of the CRS group, with pairwise comparison of race/ethnicity categories showing that the AFRS group had significantly higher proportions of Black race/ethnicity compared with other race/ethnicities (p < 0.01). AFRS and CRS groups differed significantly by age, with mean ages of 48.7 and 51.0 years, respectively (p = 0.04). There were no significant differences in gender, Medicaid status, comorbidities, and socioeconomic status measures. Multivariate logistic regression showed that Black patients had higher odds of having severe AFRS (adjusted odds ratio = 2.29; 95% confidence interval: 1.18-4.45). CONCLUSION: AFRS has a unique predilection for Black patients, and severe disease is also more likely in this population.

Fatal Orbital Cellulitis With Intracranial Abscess: A Case Report.

Orbital cellulitis is a sight- and life-threatening condition. Prompt diagnosis and immediate intervention are required. We report a case of fatal odontogenic orbital cellulitis complicated with intracranial abscesses in a 55-year-old gentleman. The patient presented with painful swelling of the left eye associated with reduced eye movement, blurry vision, and a headache. There was generalized periodontitis. Initial imaging was suggestive of left eye orbital cellulitis with intracranial abscess, and intensive systemic antibiotic therapy was initiated. After six weeks of antibiotic therapy, there was an improvement in ocular signs and symptoms. However, the patient developed signs of meningism with a persistent fever. Serial brain imaging demonstrated worsening intracranial abscesses. The patient died eight weeks after the initial onset of presentation. This case emphasized that brain abscesses could be a fatal complication of odontogenic orbital cellulitis. A high index of suspicion is important in diagnosing orbital cellulitis and its complications. Early consideration of surgical intervention is necessary in cases not responding to antibiotic therapy.

Giant ethmoid mucocele presenting as a nasal region mass with intracranial extension, a case report.

Background: Mucocele is expansile slowly growing benign cystic lesion that occurs as a result of occlusion of the ostia of the paranasal sinuses. It mostly involves the frontal sinus followed by ethmoid and rarely sphenoid and maxillary sinuses. Case presentation: Authors present a rare case of giant ethmoid mucocele in 34-year-old adult male presenting as nasal region mass with intracranial extension. Conclusions: Authors highlight that giant ethmoid mucoceles are rare and may have intracranial or extracranial extension resulting in craniofacial disfigurement which should be considered in patient presenting with nasal region mass.

Congenital midline upper lip sinuses with intracranial extension - A variant of nasal dermoid? An embryology-based concept.

OBJECTIVE: The study aimed to present the comparative analysis of midline congenital upper lip sinuses (MCULS) and nasal dermoids (NDs). The clinical similarity of congenital midline pathologies of the midface was interesting subject for further studies. Therefore, histopathological, and embryological background were also analyzed to verify a hypothesis that NDs and MCULSs are the different variants of the same entity. MATERIAL AND METHODS: The study group included 27 surgically treated pediatric cases with the congenital midline sinus of the nose (n = 25) or upper lip (n = 2). Seven children presented intraoperatively confirmed intracranial extension of the abnormality, 6 in NDs group and 1 in MCULS group. Apart from clinical characteristics also histopathological results were compared in both groups. An analysis of the literature concerning the proposed theories of origin of NDs as well as the current classification systems of MCULSs and NDs were conducted. RESULTS: The analysis revealed that MCULSs present the same clinical characteristics as NDs. No differences were noticed in the histopathological results. The embryological theories presented so far are insufficient to convincingly explain the precise process of origin of abnormalities such as ND and MCULS. Nevertheless, the developmental embryological processes of the midface shed a new light on the common origin of NDs and MCULSs. The proposal of an update of the existing classification of midline congenital midface sinuses was presented. CONCLUSIONS: The comparative analysis of clinical, histopathological, and embryological characteristics confirms that NDs and MCULSs are different forms of the same entity. Embryological implications result in the possibility of intracranial extension in each case of MCULS. Therefore, CT and MR imaging which provide irreplaceable information are recommended for all patients with MCULS. The updated classification of midline congenital midface sinuses should be taken into consideration.

Squamous Cell Carcinoma Scalp With Intracranial Extension: Challenging Case of Multidisciplinary Care.

Squamous cell carcinoma (SCC) of the scalp is the second most common non-melanoma cancer of the skin. The incidence of squamous cell carcinoma on the scalp is on the rise, but the intracranial extension is rare. Cranial invasion is rare in SCC of the scalp, but when present, it is associated with a poor prognosis. A 62-year-old female presented with complaints of swelling over the back of her scalp for three months, which rapidly increased in size. She also had a throbbing headache, alopecia in that area, and multiple episodes of pustules in that area. On examination, she had an ulceroproliferative lesion measuring 5*5*3 cm with an irregular surface and varying consistency over the occipital area in the midline surrounded by ulcerations and crusted discharge and fixed to the bone. Contrast-enhanced magnetic resonance imaging (MRI) showed an irregular lesion with the destruction of the right parietal and occipital bones involving both inner and outer tables with intracranial and extracranial components, and the lesion was abutting the superior sagittal sinus. The treatment is surgical resection of the tumor with margin clearance. The treatment plan was designed using a multidisciplinary approach with the collaboration of oncosurgery, neurosurgery, and plastic surgery. The patient underwent wide local excision of the tumor with adequate skin and cranial bone clearance. The tumor was found to have infiltrated the dura mater overlying the superior sagittal sinus. The defect was then closed using a vault prosthetic cover and a scalp transposition flap from the left parietal area. This case report intends to highlight the need for a multidisciplinary approach to the proper management of advanced squamous cell carcinoma to decrease the morbidity and mortality in patients.

A Real Headache: Intracranial Extension and Epidural Abscess As Complication of Chronic Mucocele.

Mucoceles are benign lesions of salivary glands typically originating from the paranasal sinuses. Intracranial extension and superinfection of these lesions are rare but serious complications of chronic mucoceles. Here, we discuss a patient with a known mucocele, initially lost to follow-up, who presented three years later with headache, purulent rhinorrhea, and intracranial extension of his mucocele with development of an epidural abscess. This case highlights the potential complications of chronic, large mucoceles and emphasizes the importance of thorough evaluation in patients with facial abscesses in the setting of known sinus pathology. Any mucocele with signs of superinfection such as purulent rhinorrhea, abscess near the sinuses, or refractory symptoms should warrant cranial imaging. Mucoceles with evidence of intracranial extension require neurosurgical and/or otolaryngologic evaluation for evacuation and debridement to avoid neurologic injury or devastating infection.

Pituitary adenoma with cavernous sinus compartment penetration and intracranial extension: surgical anatomy, approach, and outcomes.

Objectives: To understand the different characteristics and growth corridors of knosp grade 4 pituitary adenomas (Knosp4PA) with cavernous sinus (CS) compartments penetration and intracranial extension, aiming to improve the safety, effectiveness, and total resection rate of surgery. Methods: A case series of 120 Knosp4PA patients with 187 invaded compartments were retrospectively reviewed. A novel surgery-relevant grading system was proposed according to the CS penetrating features. The details of approach drafting, risk prediction, and complication avoidance were analyzed and integrated through illustrated cases. Results: All enrolled tumor was Knosp4PA which was derived from Knosp subgrades 3A(62.5%) and 3B(37.5%). Based on the tumor growth pathway and its relevant features, five subclassifications of intracranial extension(n=98,81.7%) were classified, which derived from the superior (Dolenc's and Oculomotor subtype, 5% and 24.2%), lateral (Parkinson's subtype,18.3%), and posterior (cerebral peduncle and Dorello's subtype, 5.8% and 1.7%) CS compartment penetration. The size of intracranial extension is assessed by Lou's scale proposed here based on preoperative MRI characteristics. Under Lou's scale, the gross total rate (GTR) decreased (82%, 53%, 22%, and 19%) with grades increased (grade 0,1,2,3, respectively), and presents significant difference between the four groups (p=0.000), as well as between single and multiple compartments involved (p=0.001). Preoperative cranial nerve deficits included the optic nerve (53%), oculomotor nerve (24.2%), and abducent nerve (4.2%), with an overall rate of visual function improvement in 68.1%. Postoperative complications of transient diabetes insipidus, cerebrospinal fluid (CSF) leakage, and cranial nerve deficits were 6.7%, 0.8%, and 0%. No new cranial nerve deficits occurred. The mortality rate was 0.8%. Conclusion: The concept of "penetration" refines the extracavernous growth pattern, and the five intracranial subclassifications help to understand the potential extension corridors, enhancing adequate exposure and targeted resection of Knosp4PA. This grading system may benefit from its predictive and prognostic value, from which a higher GTR rate can be achieved.

Neurofibromatosis Type 1-Associated Malignant Peripheral Nerve Sheath Tumor in the Nasal Cavity.

Malignant peripheral nerve sheath tumors (MPNSTs) are rare soft tissue sarcomas, with 50% of cases associated with type 1 neurofibromatosis (NF-1). A 27-year-old male patient was referred to our department with an extended right nasal cavity MPNST. The lesion extended to the skull base, intracranial, parapharyngeal space, and infratemporal fossa. NF-1 was also confirmed by a neurologist. The patient was negative for distant metastases. Due to the tumor's proximity to vital structures, it was decided to treat it with chemotherapy and radiotherapy. Nasal cavity MPNSTs are particularly uncommon, with few reported cases. They should be included in the differential diagnosis of nasal masses or recurrent nosebleeds, particularly in patients with NF-1. Careful follow-up is essential to detect early recurrence, which contributes to a better prognosis.

Nasoethmoidal Schwannoma as a Mimicar of Esthesioneuroblastoma: A Case Report and Literature Review.

Nasoethmoidal schwannomas are rare lesions and their presentation with intracranial extension is even rarer. Here, a patient presenting with rhinorrhea, epistaxis, and proptosis of left eye was diagnosed with giant nasoethmoidal schwannoma extending to frontal lobe and orbit, which was managed with bifrontal craniotomy with endoscopic transnasal gross total excision. This being predominantly a benign lesion has good prognosis if total excision is achieved.