Pott's puffy tumor: An unusual complication of rhino-orbito-cerebral mucormycosis.

Objective: To describe clinicoradiological features and surgical outcomes in a series of nine patients with rhino-orbito-cerebral mucormycosis (ROCM) who presented with Pott's puffy tumor (ROCM-PPT). Methods: The records of nine patients with ROCM-PPT seen between March 2020 and December 2021 were analysed. Clinical features, radiology, histopathology, operative findings, management and outcome were noted. Frontal sinus pneumatisation and outflow tract configuration was compared between patients and controls with ROCM and no PPT. Results: ROCM-PPT was diagnosed in 9 of 284 (3.2 %) patients with ROCM seen during the study period. There were six (66.7 %) males and the median age was 54 (IQR 46-60) years. Eight (88.9 %) patients had diabetes mellitus and seven (77.8 %) had been COVID-19 positive. Radiological features of osteomyelitis, subperiosteal abscess formation and dural enhancement were seen in all patients. No significant differences in pneumatisation or frontal sinus outflow tract configuration were noted between patients and controls. All patients underwent a craniectomy with frontal bone debridement and frontal sinus exteriorisation. All patients were treated with anti-fungal agents for several months. All patients had symptomatic improvement at a median follow-up of 21 (IQR 18-23) months. Repeat CT/MRI scans showed disease regression/resolution in six out of eight (75 %) patients with follow-up imaging, and stable disease in two others. Conclusions: ROCM-PPT is a rare, delayed complication of mucormycosis that was seen in larger numbers during the recent COVID-19 pandemic. Aggressive debridement of osteomyelitic bone and antifungal therapy results in a good outcome.

Correlation between Monocyte Gene Expression and Inflammation on Brain Imaging in Patients with Solitary Cerebral Cysticercus Granuloma.

Prior work has shown that 14 monocyte genes are upregulated in patients with different forms of parenchymal neurocysticercosis, including solitary cysticercus granuloma (SCG). The aim of this study was to investigate whether changes in inflammation associated with SCG seen on follow-up brain imaging are also reflected in changes in expression of these 14 genes. Peripheral blood CD14+ monocytes were isolated from 20 patients with SCG at initial diagnosis and at clinical and imaging follow-up of 6 months or more. Expressions of 14 target monocyte genes were determined by quantitative polymerase chain reaction at each visit. At a median follow-up of 14 months, the SCG had resolved in 11 patients, was persistent in four patients, and had calcified in five patients. Edema seen in the initial imaging in 17 patients had resolved in 15 patients and was markedly reduced in two patients. The expression levels of the monocyte genes LRRFIP2, TAXIBP1, and MZB1 were significantly lower at follow-up, regardless of the status of SCG on follow-up imaging. Our findings show that expression levels of monocyte genes involved with inflammatory processes decrease in patients with SCG concomitant with follow-up imaging that reveals a reduction in inflammation as revealed by complete or near-complete resolution of edema, as well as resolution or reduction in the enhancement of the granuloma.

Correlation Between Post-Radiosurgery Perinidal Hyperintensity and AVM Obliteration Following LINAC-Based Stereotactic Radiosurgery.

OBJECTIVE: We studied the correlation between new-onset perinidal hyperintensity (PH) on T2-weighted magnetic resonance imaging and obliteration of intracranial arteriovenous malformation (AVM) after stereotactic radiosurgery (SRS). METHODS: A retrospective study of 148 patients with an intracranial AVM who underwent SRS between September 2005 and June 2018 and had ≥1 radiological follow-up (early magnetic resonance imaging) 12-18 months after SRS was performed to analyze the correlation between PH (graded from 0 to 2) and AVM obliteration. RESULTS: Of the 148 patients, 95 were male. The mean patient age was 27.7 ± 12.4 years. Of the 148 AVMs, 105 (70.9%) were obliterated at a median follow-up of 27 months (interquartile range, 14-48 months). The cumulative 3-, 5-, 10-year obliteration rate was 51.8%, 70.8%, and 91.8%, respectively. New-onset PH was observed in 58 AVMs (39.2%; 50 obliterated and 8 not obliterated). No association was found between the pretreatment variables or dose delivered and the development of PH. Grade 2 PH was associated with the risk of symptoms developing compared with grade 1 PH (37.5% vs. 4%; P = 0.002). Symptomatic PH was more likely to develop in patients with a larger AVM (P = 0.05). On multivariate analysis, the presence of a single draining vein (odds ratio [OR], 2.9; 95% confidence interval [CI], 1.3-6.8), a lower median AVM volume (OR, 0.97; 95% CI, 0.6-0.89), a mean marginal radiation dose (OR, 1.29; 95% CI, 1.02-1.64), and the presence of PH (OR, 3.16; 95% CI, 1.29-7.71) were independent predictors of AVM obliteration. CONCLUSIONS: The incidence of PH after SRS for AVM was 39.2%. PH was an independent predictor of AVM obliteration after SRS. Grade 2 PH and a larger AVM volume were associated with symptomatic PH.

Preventing Postoperative Apoplexy in Giant Pituitary Adenomas using Combined Trans-Sphenoidal and Cranial Surgery.

Background: Combined surgery consisting of endoscopic trans-sphenoidal surgery (ETSS) and transcranial (TC) surgery for giant pituitary adenomas (GPAs) has been recommended to prevent lethal postoperative apoplexy. Based on our experience, we attempt to rationalize the indications for such surgery. Materials and Methods: We report the magnetic resonance (MR) characteristics of the tumor and the outcomes in patients with GPAs who underwent ETSS only and combined surgery. Total tumor volume (TTV), tumor extension volume (TEV), and suprasellar extension of tumor (SET) were calculated based on the lines drawn on MR images and compared between those who underwent ETSS only and those who underwent combined surgery. Results: Of 80 patients with GPAs, eight (10%) underwent combined surgery (seven in the same sitting and one had staged surgery). All eight patients (100%) who underwent combined surgery had tumors with multilobulations, extensions, and encasement of the vessels in the circle of Willis (COW). Of 72 patients who underwent ETSS alone, 21 (29.1%) had a multilobulated tumor, 26 (36.2%) tumors had anterior/lateral extensions, and 12 (16.6%) had encasement of the COW. The mean TTV, TEV, and SET for the combined surgery group were significantly higher than those in the ETSS group. None of the patients who underwent combined surgery suffered postoperative residual tumor apoplexy. Conclusion: Patients with GPAs in whom there are significant lateral intradural or subfrontal tumor extensions should be considered for combined surgery at the same sitting to avoid devastating postoperative apoplexy in the residual tumor, which can occur when ETSS alone is performed.

Chronic Granulomatous Invasive Fungal Sinusitis in Patients With Immunocompetence: A Review.

OBJECTIVE: We aimed to study the literature on chronic granulomatous invasive fungal sinusitis to elucidate the changing trends in the management of the disease. DATA SOURCES: Using specific keywords, we searched the PubMed, PubMed Central, and Scopus databases over the past 50 years, which yielded 938 articles in the English language. REVIEW METHODS: Scrutiny of 147 relevant articles revealed 15 homogenous case series (255 cases of histologically proven chronic granulomatous fungal sinusitis alone) and 8 heterogeneous case series (patients with other types of fungal sinusitis included), which were analyzed in detail (all with >5 cases each). CONCLUSIONS: The disease typically affected middle-aged adults with immunocompetence. Most reports were from Sudan, India, and Saudi Arabia. A slowly progressive orbital, cheek, or palatal mass with proptosis (88.2%) or sinonasal symptoms (39.2%) was typical. Ethmoid (57.2%) and maxillary (51.4%) sinuses were chiefly affected with intracranial extension in 35.1%. Aspergillus flavus (64%) was the most frequent isolate reported. Endoscopic excision (78.8%) followed by azole therapy was the preferred treatment in recent reports. Orbital exenteration and craniotomy were infrequently performed. Complete resolution or improvement was reported in 91.3% of patients. Mortality ranged from 5.9% to 22.2%. There is a trend in the literature toward less radical and disfiguring surgery and preferential use of azoles, with good outcomes even in advanced cases. IMPLICATIONS FOR PRACTICE: Chronic granulomatous fungal sinusitis should be diagnosed on the basis of well-defined histopathologic features. A combination of endoscopic sinus surgery and azole therapy usually yields good outcomes.

Risk Factors for Postoperative Brain Contusion and Early Outcomes in Patients with Anterior Skull Base Meningiomas Undergoing Unifrontal Surgery: Single Center Experience with 110 Patients.

Background: Though frontal lobe contusion is a major cause for morbidity and prolonged hospitalization following excision of anterior skull base meningiomas, there is only limited literature on this complication. This study aimed to find out the incidence of postoperative frontal lobe contusion, identify the risk factors for its development and its impact on early postoperative outcome. Methods: Data from 110 patients who underwent excision of anterior skull base meningiomas through a unilateral supraorbital craniotomy from 2001 to 2018 were retrospectively analyzed. The risk factors analyzed for development of postoperative contusion were tumor location, size, volume, peritumoral edema, tumor consistency, extent of resection, tumor grade and type of retraction used. Results: Simpson grade II excision was achieved in ninety-two patients (83.6%). There was no frontal lobe contusion in eighty-two patients (74.5%). Frontal lobe contusion was noted in twenty-eight patients (25.5%), but was severe in only four patients (3.6%). On multivariate analysis, fixed retractor use (OR 11.56 [1.21-110.03]; P =0.03) and WHO grade II tumor (OR 3.29 [1.11-9.77]; P =0.03) were independently associated with postoperative frontal lobe contusion. Patients with higher contusion grade had significantly longer postoperative hospitalization (P =.02) and lower KPS score at discharge (P =.01). Conclusions: Unilateral supraorbital craniotomy and lateral subfrontal approach is an excellent procedure for excision of anterior skull base meningiomas with minimal postoperative complications related to frontal lobe retraction. Frontal lobe contusion should be avoided with the use of dynamic retraction, since postoperative contusion prolongs hospitalization and worsens the functional outcome at discharge.

Characterizing syringomyelia and its clinical significance in 140 patients with lipomyelomeningocele.

OBJECTIVE: The aims of this study were to characterize syringomyelia in patients with lipomyelomeningocele (LMMC) and investigate the association between the presence of a syrinx and patient neurological deficits. METHODS: Patients with LMMC who had been admitted between 2007 and 2020 were included in the study. Syringomyelia on magnetic resonance imaging (MRI) was classified into three groups: true syrinx, early syrinx, and presyrinx. The correlation of clinical deficits (at birth, new onset, and progressive) and type of lipoma with the presence and type of syrinx was studied. RESULTS: Among a total of 140 patients, the median age was 2.5 (IQR 1.3-9) years. Neurological deficits were present at birth in 75 (53.6%) patients, but 67 (47.9%) had new-onset deficits or progression of their birth deficits. The most common type of spinal lipoma was the transitional type (75.7%). Thirty-nine (27.9%) patients had a syrinx (37 with a true syrinx, 2 with an early syrinx), and 25 (17.9%) patients had a presyrinx. There was no significant correlation between the presence of a syrinx and patient neurological deficits. However, patients with a syrinx that was not adjacent to the lipoma (≥ 1 vertebral level away) had a higher incidence of deficits at birth (p = 0.045), whereas those with a syrinx spanning ≥ 5 vertebral levels had a higher incidence of progressive neurological deficits (p = 0.04). Six (75%) of 8 patients in whom serial MRI had shown syrinx progression had clinical worsening. CONCLUSIONS: Syrinx location with respect to the spinal lipoma, syrinx length, and progressive syringomyelia may have clinical significance in patients with LMMC.

Cerebrospinal Fluid Procalcitonin-A Potential Biomarker for Post-Craniotomy Bacterial Meningitis.

Background: The literature regarding the utility of cerebrospinal fluid (CSF) procalcitonin (PCT) in the diagnosis of post-craniotomy bacterial meningitis and differentiating it from aseptic meningitis is sparse. Materials and Methods: CSF total WBC count, sugar, protein, and PCT were measured in febrile patients with suspected post-craniotomy meningitis during the first 30 days following an intradural cranial procedure for non-trauma indications. Patients were diagnosed as postoperative bacterial meningitis if CSF culture was positive (PBM, n = 28) or postoperative aseptic meningitis if CSF culture was sterile and there was no evidence of systemic infection (PAM, n = 31). CSF cytochemical parameters and PCT values were compared between the groups. Normal values of CSF PCT were obtained from 14 patients with noninfectious indications with hydrocephalus. Results: There was no significant difference in CSF total WBC count, sugar, and protein levels between PAM and PBM groups. The median PCT level in CSF in the normal group was 0.03 ng/mL (interquartile range [IQR] 0.02-0.07 ng/mL). CSF PCT in the PBM group (median 0.37 ng/mL, IQR 0.2-1.4 ng/mL) was significantly higher than normal values as well as PAM group (median 0.12 ng/mL, IQR 0.07-0.26 ng/mL (P = 0.0004). The area under the receiver operating characteristic (ROC) curve for CSF PCT was 0.767. A cutoff value of 0.12 ng/mL yielded a sensitivity of 85.7% (95% CI: 67.3% to 96%), specificity of 51.6% (95% CI: 33% to 69.9%), positive predictive value of 61.5% (95% CI: 51.9% to 70.3%), and negative predictive value of 80% (95% CI: 60.3.8% to 91.3%). Conclusions: CSF PCT assay in patients who are febrile during the first 30 days post-non-trauma neurosurgical procedures has a role in the early diagnosis of bacterial meningitis.

ZFTA (Zinc Finger Translocation Associated) Fusion in Supratentorial Ependymomas: Low Prevalence in South Asians and No Correlation with Survival.

BACKGROUND: Supratentorial ependymomas (STEs) are an aggressive group of ependymomas, topographically distinct from their posterior fossa and spinal counterparts. Zinc finger translocation associated (ZFTA) fusion-positive cases have been reported to account for the majority of STEs, although data on its association with poorer outcomes are inconsistent. MATERIALS AND METHODS: We assessed the prevalence of the ZFTA fusion by reverse-transcription polymerase chain reaction and fluorescence in situ hybridization in a cohort of 61 patients (68 samples) with STE. Our primary outcome was to determine the role of the ZFTA fusion on progression-free and overall survival of patients with STE. Our secondary objectives were to assess the impact of ZFTA fusion on nuclear factor (NF)-kB pathway signaling via surrogate markers of this pathway, namely COX-2, CCND1, and L1 cell adhesion molecule. RESULTS: ZFTA fusion was noted in 21.3% of STEs in our cohort. The presence of this rearrangement did not significantly impact the progression-free or overall survival of patients with STEs and was not associated with upregulation of markers of the NF-kB pathway. Only gross total resection was significantly associated with better progression-free survival. CONCLUSIONS: In contradiction to previous reports from across the world, the ZFTA fusion is far less prevalent among our population. It does not appear to drive NF-kB signaling or significantly affect outcomes. Gross total resection must be attempted in all cases of STE and adjuvant radiation and/or chemotherapy employed when gross total resection is not achieved.

Risk factors for pre-operative functional deterioration in children with lipomyelomeningocele.

PURPOSE: To characterize the temporal profile of pre-operative deterioration in children with lipomyelomeningocele (LMMC) including those with congenital deficits and identify risk factors for clinical worsening. METHODS: Records of 87 children who underwent surgery for LMMC were retrospectively reviewed to study the temporal profile of pre-operative deterioration, defined by the onset of new neurological dysfunction or progression of a pre-existing deficit. Preoperative magnetic resonance imaging (MRI) studies were examined to identify radiological features associated with deterioration. In children with extra-spinal placodes, the angle subtended by the terminal placode with the cord at the level of the laminar-fascial defect ("J sign") was assessed. RESULTS: Pre-operative deterioration in function was seen in 37 children (43%), occurring at a median age of 36 months and was more frequent in children without congenital deficits (54% versus 27%; p = 0.016). On Cox regression analysis, extra-spinal location of the placode (p = 0.003) and presence of a congenital deficit (p = 0.009) were positively and negatively associated with deterioration respectively. On Kaplan-Meier analysis, the median deterioration-free survival time was 72 months and was positively associated with presence of congenital deficit (p = 0.026) and negatively associated with presence of an extra-spinal placode (p < 0.001) or a meningocele sac (p = 0.001). CONCLUSION: Extra-spinal location of the neural placode was associated with higher risk of clinical deterioration in children with LMMC, whereas the presence of a congenital deficit conferred a decreased risk. Risk stratification based on clinical and radiological features can be used to guide decisions regarding early prophylactic surgery in children with LMMC.

MR findings suggestive of idiopathic intracranial hypertension in 117 patients with spontaneous cerebrospinal fluid rhinorrhea.

PURPOSE: The purpose of this study is to document the prevalence of MR findings suggestive of idiopathic intracranial hypertension (IIH) in patients undergoing endoscopic repair of spontaneous CSF rhinorrhea (SCSFR). METHODS: In a retrospective study, MR images of 117 consecutive patients who had undergone endoscopic repair of SCSFR were evaluated for features suggestive of IIH (empty sella, widened optic nerve sheath, tortuous optic nerve, flattened posterior globe, and enlarged Meckel's cave). Pituitary height was used to diagnose partial and complete empty sella. MR images were independently evaluated by two of the authors without knowledge of the clinical findings. Consensus method was used to resolve differences between the two evaluators. RESULTS: Empty or partially empty sella was diagnosed in the MR of 105 (89.7%) patients. In 38/105 (36.2%) patients with empty/partial empty sella, no additional MR findings were present. In 43/105 (41%) patients, one or more of the MR features with high specificity for diagnosis of IIH (flattened posterior globe and enlarged Meckel's cave) were seen. In the other 24 (22.9%) additional MR findings, less specific for IIH (widened optic nerve sheath, tortuous optic nerve) were noted. Papilledema was seen in 11 of 60 (18.3%) patients who underwent funduscopic examination. All patients with papilledema had empty/partial empty sella, and 9/11 (81.8%) had an additional MR finding suggestive of IIH. CONCLUSION: The majority of patients with SCSFR have MR imaging features of IIH. These imaging features should be a major component of previously published modified diagnostic criteria for IIH in patients with SCSFR.

Imaging correlates of serum enzyme-linked immunoelectrotransfer blot (EITB) positivity in patients with parenchymal neurocysticercosis: results from 521 patients.

BACKGROUND: The presence of perilesional edema among patients with parenchymal neurocysticercosis (pNCC) of various lesion subtypes has not been correlated with results of serum enzyme-linked immunotransfer blot (EITB) for cysticercal antibodies. METHODS: In total, 521 patients with pNCC were classified into solitary cysticercus granuloma (SCG), multiple lesions, at least one of which was an enhancing granuloma (GMNCC), solitary calcified cysticercal lesion (SCC) and multiple calcified cysticercal lesions (CMNCC). The proportion of EITB positivity among each lesion subtype and its association with perilesional edema were determined. RESULTS: There were significantly higher positive EITB results in patients with GMNCC (90/111, 81.1%) compared with other lesion types. Perilesional edema was associated with positive EITB in patients with CMNCC. On univariate analysis, perilesional edema and GMNCC were associated with EITB positivity. On multivariate analysis, only GMNCC (OR 7.5; 95% CI 3.5 to 16.2) was significantly associated with EITB positivity. CONCLUSIONS: In patients with pNCC, the presence of perilesional edema is associated with a higher probability of a positive EITB result in patients with CMNCC, suggesting a synchronicity in the mechanisms associated with formation of perilesional edema and the antibody response in this subtype. In patients with enhancing granulomas, edema is not an independent predictor of a positive EITB, suggesting that the enhancement itself is associated with a strong antibody response.

Posterior fossa morphometry in 170 South Asian children and adults with Chiari malformation and its correlation with tonsillar descent.

AIM: Patients with Chiari I malformations (CM1) have posterior fossa hypoplasia with crowding of the neural structures. We aimed to study the posterior fossa measurements to analyse the posterior fossa morphology, presence of basilar invagination (BI) and correlated the measurements with the degree of tonsillar descent. METHODS: We retrospectively reviewed data in170 patients who underwent foramen magnum decompression (FMD) for CM1. The posterior fossa measurements were compared with 30 controls. Patients were divided into those with and without BI. The variables measured included clival length, posterior fossa height, supraocciput length, posterior fossa diameter, foramen magnum width, Boogard's angle, clival angle, clival slope and the newly introduced foramen magnum (FM) angle. RESULTS: The average clivus length and posterior fossa height were significantly shorter with a significant increase in the Boogard's and FM angle in the patient groups. Tonsillar descent showed a negative correlation with posterior fossa height (r = -0.498, p ≤ 0.001) and clivus length (r = -0.325, p ≤ 0.001) and a positive correlation with Boogard's angle (r = 0.469, p ≤ 0.001) and FM angle (r = 0.330, p ≤ 0.001). Patients with BI had statistically significant reduced posterior fossa height (p ≤ 0.001) and increased extent of tonsillar herniation (p = 0.001) compared to patients without BI. CONCLUSION: Patients with CM1 have significantly shorter clival length and posterior fossa height with smaller posterior fossa in support of published data. The presence of BI shortens the posterior fossa height and worsens the extent of tonsillar herniation. An increased Boogard's angle and FM angle result in a more horizontally placed suboccipital bone compared to a slanting bone in normal persons.

Changes in pattern of presentation of patients with unilateral vestibular schwannoma over two decades: Influence of cell phone use in early diagnosis.

We aimed to study whether the increased use of cell phone in south Asia over the past two decades has impacted presentation of patients with vestibular schwannoma (VS). In this observational cohort study, data on cell phone use and severity of hearing loss and tumor size was collected using a questionnaire through a patient interview (n = 62) in consecutive patients with VS managed between 2017 and 2020. Association between cell phone use and severity of hearing loss and tumor size were studied and compared with our data published earlier when telephone use was not widely prevalent. 71% of the 62 patients (aged between 15 and 81 years) had large or giant VS. Pure tone audiometry showed moderately severe or profound hearing loss in77.4%. Sixty (96.7%) patients used cell phones. 50% of patients complained of difficulty in using their cell phone because of hearing loss. Compared to the earlier era, a higher proportion consulted an ENT surgeon first for hearing loss (59.7% vs 26%; p = 0.0005) and had small or medium sized tumors (29% vs 8%; p = 0.008) with a smaller mean tumor size (3.3 vs 3.9 cm; p = 0.03). Increased cell phone use has led to earlier diagnosis of VS and smaller tumours in recent years when compared to an earlier era. However, the vast majority of patients continue to present with severe to profound hearing loss and large tumors.