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.

Intracranial posterior fossa proliferating trichilemmal tumor: first case report.

Proliferating trichilemmal tumors (PTT) are rare benign lesions that predominantly occur in elderly women and usually affect the scalp. They originate from the outer root sheath of hair shafts and have trichilemmal differentiation on histopathological examination. Compared to trichilemmal cysts, PTTs show increased cell proliferation and variable cytological atypia. We report the first case of a patient with an intracranial PTT in the posterior fossa. The clinicoradiological presentation and surgical management of the patient as well as the possible pathogenesis of this tumor are discussed.

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.

Long-Term Follow-up of Patients Managed Conservatively for Acute Traumatic CSF Rhinorrhea.

OBJECTIVE: Conservative management of acute traumatic cerebrospinal fluid rhinorrhea (TCR) results in cessation of the leak in most patients. The objective of this study was to estimate the incidence of recurrent cerebrospinal fluid (CSF) rhinorrhea and meningitis in these patients on long-term follow-up and to determine the risk factors associated with them. METHODS: Data on 50 patients with acute TCR who were successfully treated with conservative management between 2013 and 2015 and had long-term follow-up was retrieved from our head injury database. Patient variables were analyzed to determine the risk factors associated with recurrence of CSF rhinorrhea and meningitis. RESULTS: All patients in our series developed CSF rhinorrhea within 48 hours of trauma. The mean duration of follow-up was 6.3 ± 1.3 years. CSF rhinorrhea recurred in 16 (32%) patients, 15 (93.8%) of whom developed it within 3 years of trauma. Meningitis occurred in 5 (10%) patients and 1 died. Sphenoid sinus fractures and features of raised intracranial pressure on computerized tomography of the brain at admission were significantly associated with the development of meningitis. There were no risk factors identified for the recurrence of CSF rhinorrhea. CONCLUSIONS: Patients with acute TCR in whom rhinorrhea subsides with conservative therapy have the highest risk for recurrence of leak or meningitis within 3 years of the trauma. Therefore, we recommend that these patients be counselled about the need for periodic follow-up for several years.

USP8, USP48, and BRAF mutations differ in their genotype-phenotype correlation in Asian Indian patients with Cushing's disease.

PURPOSE: To estimate the prevalence of USP8, USP48, and BRAF mutations in patients with Cushing's disease (CD) from the Indian subcontinent, and determine their genotype-phenotype correlation. METHODS: We prospectively recruited 46 patients with CD who underwent surgery between September 2015 and July 2019 at our institute. Fresh frozen tumour tissue was obtained in all patients. Using Sanger sequencing, the presence of somatic USP8 mutations was documented and the frequency of USP48 and BRAF mutations in USP8 wild-type corticotroph adenomas was determined. Clinical, hormonal, and surgical data were then compared between USP8-, USP48- and BRAF-variant carriers and patients with wild-type tumours. RESULTS: Signature USP8 mutations were detected in 17 (37%) patients. Of the 29 USP8 wild-type adenomas, 4 (13.8%) harboured USP48 mutations, one of them being a splice-site mutation that has previously not been described. BRAF mutations were not found in any of the 29 patients. Corticotroph adenomas with USP8 mutations had a higher incidence of Crooke's hyaline change than wild-type tumours (70.6 vs. 37.9%, p = 0.032). Adenomas with USP48 mutations had a higher rate of cavernous sinus invasion than their wild-type counterparts (50 vs. 4%, p = 0.042). No other significant phenotypic difference could be established between mutant and wild-type tumours. CONCLUSIONS: The prevalence of USP8 mutations in our series of patients with CD was 37%. The prevalence of USP48 mutations in USP8 wild-type adenomas was 13.8%, including a novel splice-site mutation. BRAF mutations were not found in any USP8 wild-type tumour. USP8-mutants showed significantly more Crooke's hyaline change and USP48-mutants were more likely to demonstrate cavernous sinus invasion.

Selective perioperative steroid supplementation protocol in patients undergoing endoscopic transsphenoidal surgery for pituitary adenomas.

BACKGROUND: There is no consensus regarding the use of perioperative steroids for transsphenoidal pituitary surgery. We audited the effectiveness and safety of our selective perioperative steroid supplementation protocol in patients with pituitary adenomas. METHODS: Two hundred ninety-seven patients underwent 306 endoscopic transsphenoidal surgeries for removal of their pituitary tumors. Steroids were given to those with an impaired hypothalamic-pituitary-adrenal (HPA) axis, age ≥ 60 years, clinical apoplexy, hyponatremia, or if the pituitary gland was not preserved at surgery. We excluded 111 patients in whom the integrity of the HPA axis could not be determined. We compared the incidence of early postoperative adrenal insufficiency and complications in 135 patients with intact HPA axes who underwent surgery without steroids (group A) with 60 patients who had compromised preoperative HPA axes and received perioperative steroids (group B). In addition, we audited the total number of protocol violations during this period. RESULTS: Five patients (3.7%) in group A developed postoperative hypocortisolemia. There was no significant difference in the incidence of cerebrospinal fluid leak, diabetes insipidus, or hyponatremia between both groups. There were protocol deviations in 47 (15.4%) patients. Twenty one of these patients did not receive perioperative steroids in violation of the protocol, of whom 4 (19%) developed postoperative hypocortisolemia. CONCLUSIONS: Our steroid sparing protocol was both safe and effective. The 15% incidence of protocol deviations is a reminder that the rigorous usage of checklists is mandatory for successful clinical practice.

Secondary Bacterial Infection of a Solitary Cysticercus Granuloma.

The most common presenting form of neurocysticercosis in the Indian subcontinent is a solitary cysticercus granuloma (SCG). Patients with typical SCGs almost never require any form of surgical intervention. Herein, we report an extremely rare case of bacterial superinfection of a left frontal SCG in a 23-year-old female, resulting in severe perilesional edema and mass effect. The patient had to undergo an emergency left decompressive hemicraniectomy and excision of the infected granuloma. Serum enzyme-linked immunoelectrotransfer blot (EITB) for cysticercal antibodies was positive and histopathological examination of the lesion revealed a cysticercus. The culture of the pus from within the lesion grew vancomycin-resistant Enterococcus spp. for which she was treated with linezolid for 6 weeks. At 6 months follow-up, she had residual motor dysphasia, right homonymous hemianopia, and right hemiparesis but was steadily improving. Secondary bacterial infection of an SCG is very uncommon and can be devastating. A high index of suspicion is therefore required when there is disproportionate perilesional edema and mass effect.

The Prevalence of Physiological Anisocoria and its Clinical Significance - A Neurosurgical Perspective.

AIM: We aimed to estimate the prevalence of physiological anisocoria and also evaluate the accuracy of clinical assessment of anisocoria employed in routine bedside examination. MATERIALS AND METHODS: A total of 708 voluntary subjects between the ages of 20-69 years who had no history of ophthalmologic or neurological disease other than refractive error were included in the study. In a closed room with uniform ambient lighting, the subjects' pupils were examined clinically and the presence or absence of anisocoria was recorded. This was followed by photography of the subjects' pupils using a digital camera secured on a tripod at a fixed distance from the subject's face. A difference in pupillary size of 0.4 mm or more was considered anisocoria. RESULTS: Of the 708 subjects, 361 (51%) were males. The average pupillary diameter of the subjects was 4.99 mm. Ninety-seven (13.7%) had measured anisocoria on photography. Ninety-seven subjects (13.7%) also had anisocoria on clinical examination, however, only 45 of them had measured anisocoria. The clinical measurement of anisocoria, therefore, showed a specificity of 0.91 and a sensitivity of 0.46. With a prevalence of anisocoria of 13.7%, the positive predictive value was 0.46, and the negative predictive value was 0.91. CONCLUSIONS: The prevalence of physiological anisocoria was 13.7%, which is less than what has previously been reported. The sensitivity of clinical examination in detecting early anisocoria is poor. Patients at risk of developing uncal herniation may, therefore, benefit from routine bedside pupillary assessment with a portable device such as a pupillometer.

Postoperative intraventricular blood: a new modifiable risk factor for early postoperative symptomatic hydrocephalus in children with posterior fossa tumors.

OBJECTIVE: To analyze factors associated with the development of early symptomatic hydrocephalus following posterior fossa tumor (PFT) surgery in children. METHODS: In this retrospective study, data from 148 children (age < 18 years) who underwent primary resection of their PFTs without preoperative permanent CSF diversion procedures were collected. The incidence of symptomatic hydrocephalus within 30 days of tumor resection was studied and its association with various demographic, tumor-related, and surgery-related risk factors was analyzed. RESULTS: At presentation, 131 (89%) of the 148 patients had symptomatic hydrocephalus. There were 99 males and 49 females (mean age 8.7 years; range 1 to 17 years). Postoperatively, 14 (9.4%) patients required shunt placement for symptomatic hydrocephalus. The indications for shunt surgery were persistent symptoms of raised intracranial pressure (n = 6, 43%), CSF leak from the wound (n = 7, 50%), and tense pseudomeningocele (n = 1, 7%). On multivariate analysis, age < 6 years (OR 5.9, 95% CI 1.6-22.6, p = 0.009) and the presence of intraventricular blood (IVB) on postoperative CT (OR 6.4, 95% CI 1.7-23.7, p = 0.006) were independent risk factors for developing symptomatic hydrocephalus. CONCLUSIONS: The incidence of postoperative symptomatic hydrocephalus in our series (9.4%) is lower than that reported in most previous studies. Age < 6 years and the presence of postoperative IVB were independent risk factors for developing symptomatic hydrocephalus. Of these, postoperative IVB is probably the only modifiable risk factor.

EEG lateralization and seizure outcome following peri-insular hemispherotomy for pediatric hemispheric epilepsy.

OBJECTIVE: To determine whether preoperative non-lateralizing scalp electroencephalography (EEG) influences seizure outcome following peri-insular hemispherotomy (PIH) in pediatric hemispheric epilepsy. METHODS: Retrospective data was collected on all 45 pediatric patients who underwent PIH between 2005 and 2016. All underwent a basic pre-surgical evaluation consisting of detailed history and examination, neuropsychological assessment, MRI, and EEG. SPECT/PET, fRMI, or Wada testing were done in only eight patients. Seizure outcome was assessed using the Engel classification. RESULTS: Among those who underwent hemispherotomy, 20 (44%) were females. Mean age at surgery was 8 ± 4.3 years and mean duration of symptoms was 5.2 ± 3.7 years. The most common etiologies of hemispheric epilepsy were hemiconvulsion-hemiplegia epilepsy syndrome, Rasmussen encephalitis, and post-encephalitic sequelae, together comprising 27 (60%) patients. Among the 44 patients with follow-up data (mean duration 48 ± 33 months), seizure freedom (Engel class I) was attained by 41 (93.2%). Anti-epileptic medications were stopped or decreased in 36 (82%). Seventeen (38.6%) patients had non-lateralizing EEG. Seizure outcome was not related to lateralization of EEG activity. CONCLUSIONS: PIH provides excellent long-term seizure control in patients despite the presence of non-lateralizing epileptiform activity, although occurrence of acute postoperative seizures may be higher. Routine SPECT/PET may not be required in patients with a non-lateralizing EEG if there is good clinico-radiological concordance.

Malignant Peripheral Nerve Sheath Tumour of the Small Bowel Presenting with Intussusception and Perforation: a Double Jeopardy?

Malignant peripheral nerve sheath tumours (MPNST) are rare soft tissue sarcomas which largely occur in the extremities and the head and neck region. The tumours are aggressive with a high rate of recurrence. Radical surgical resection remains the treatment of choice with adjuvant radiation therapy and chemotherapy still failing to demonstrate a clear benefit. The gastrointestinal tract is an exceedingly rare site for these tumours. We report an unusual case of a young male with an MPNST of the small bowel who presented with an ileocolic intussusception and sigmoid perforation.

Surface electromyography activity in the upper limbs of patients following surgery for compressive cervical myelopathy.

BACKGROUND: Surface electromyography (EMG) is a noninvasive, accurate method to measure electrical activity produced in muscles. AIM: To assess the improvement of spasticity after decompressive surgery for compressive myelopathy using surface EMG. SETTING AND DESIGN: Neurophysiology laboratory of a tertiary care center. Before-after trial. Both EMG and Modified Modified Ashworth Scale (MMAS) were utilized. MATERIALS AND METHODS: Thirty-one nonconsecutive patients (28 males; age 25-72 years) with compressive cervical myelopathy and spasticity (MMAS score ≥1) were recruited. Patients with lower motor neuron findings, Nurick grade 5, and those with joint deformities, contractures, or thrombophlebitis of the upper limbs were excluded. EMG activity was measured from the pronator teres and biceps brachii for 31 age-related controls (25 males) as well as for the patients both pre- and post-operatively. STATISTICAL ANALYSIS: Student's t-test for comparison of continuous variables and Pearson correlation co-efficient for assessing the significance of associations. RESULTS: EMG recording done 1-week postoperatively showed a reduction in baseline activity in the pronators and supinators by 21% and 36%, respectively. There was a decrease in co-activation of the pronators during active supination by almost 62% and of the supinators during active pronation by around 33% (P < 0.05). On passive movement, there was a decrease in co-activation of the pronators during supination by approximately 23%, and the supinators during pronation by 35% (P < 0.05). EMG activity was significantly reduced in the pronators during supination in all patients, including those in whom the MMAS scores remained the same postoperatively. CONCLUSION: Surface EMG is an objective tool to detect improvement in spasticity following decompressive surgery for compressive cervical myelopathy even in those patients who showed no improvement on the MMAS.

Phenytoin toxicity in patients with traumatic brain injury.

BACKGROUND: We observed that in patients with traumatic brain injury (TBI) who did not improve as expected, serum levels of phenytoin were in the toxic range and that their sensorium improved with modification of the dose. This led us to study the usage of phenytoin in patients with TBI. AIMS: To determine the prevalence of phenytoin toxicity in TBI patients and to study the suitability of using ideal body weight (IBW) to guide phenytoin dosing. SETTING AND DESIGN: Neurotrauma unit of a tertiary care centre in India. Prospective data collection from an already established protocol of drug level monitoring. MATERIALS AND METHODS: The study cohort included 100 consecutive adult patients with mild or moderate TBI who were administered phenytoin based on IBW. Trough serum phenytoin and albumin levels were measured on day 4 after administration of the loading dose and actual body weight obtained when it was possible. STATISTICAL ANALYSIS: Chi-square was used for comparing categorical variables, student's t-test for continuous variables and multivariate regression analysis to obtain independent risk factors. RESULTS: Clinical toxicity was observed in 15% of patients and biochemical toxicity in 36%, with a significant association between the two (P < 0.01). Using multivariate analysis, abdominal girth ≤75 cm (P = 0.07), neck circumference ≤34 cm (P = 0.025) and IV dose proportion ≥80% (P = 0.003) were independent risk factors for biochemical toxicity. The plot between actual weight and IBW showed that toxicity occurred when IBW was higher than actual weight. CONCLUSION: The prevalence of biochemical phenytoin toxicity was high, with independent risk factors being a higher proportion of IV administration and overestimation of weight by IBW. Clinical suspicion of phenytoin toxicity was a good predictor of biochemical toxicity.