An Analysis of Temporal Trend of Incidence of Post-Resection Cerebrospinal Fluid Diversion in Pediatric Posterior Fossa Tumor Patients and the Predictive Factors.

Objective: We aim to find the temporal trend of incidence of post-resection cerebrospinal fluid (CSF) diversion (ventriculoperitoneal [VP] shunt/endoscopic third ventriculostomy [ETV]) in pediatric posterior fossa tumor (pPFT) patients with no pre-resection CSF diversion and the possible clinical predictors. Methods: We reviewed 108 operated children (age ≤16 years) with PFTs, from 2012 to 2020, at a tertiary care center. Patients with preoperative CSF diversion (n = 42), lesions within cerebellopontine cistern (n = 8), and those lost to follow-up (n = 4) were excluded. Life table, Kaplan-Meier curve, univariate and multivariate analyses were used to determine CSF-diversion-free survival and independent predictive factors, with significance defined as P < 0.05. Results: The median (IQR) age was 9 (7) years (M: F: 2.5:1). Mean (±SD) duration of follow-up was 32.43 ± 21.3 months. 38.9% of patients (n = 42) needed post-resection CSF diversion. Of these, 64.3% (n = 27) were done in early (≤ 30 days), 23.8% (n = 10) in intermediate (>30 days to ≤6 months), and 11.9% (n = 5) in late (≥6 months) postoperative period (P-value < 0.001). Preoperative papilledema (HR: -5.8, 95%CI: 1.7-5.8), periventricular lucency (PVL) (HR: 6.2, 95%CI: 2.3-16.6), and wound complication (HR: 3.8, 95%CI: 1.7-8.3) were found on univariate analysis as significant risk factors for early post-resection CSF diversion. On multivariate analysis, PVL on preoperative imaging (HR: -4.2, 95%CI: 1.2-14.7, P = 0.02) was identified as an independent predictor. Preoperative ventriculomegaly, raised intracranial pressure and intraoperative visualization of CSF egress from the aqueduct were not found to be significant factors. Conclusion: Significantly high incidence of post-resection CSF diversion in pPFTs occurs in early (≤30 days) postoperative period, with preoperative papilledema, PVL, and wound complication being its significant predictive factors. Postoperative inflammation, causing edema and adhesion formation can be one of the important factors for post-resection hydrocephalus in pPFTs.

Cerebellopontine Angle Epidermoids: Comparative Results of Microscopic and Endoscopic Excision Using the Retromastoid Approach".

Background Densely packed neurovascular structures, often times inseparable capsular adhesions and sometimes a multicompartmental tumor extension, make surgical excision of cerebellopontine angle epidermoids (CPEs) a challenging task. A simultaneous or an exclusive endoscopic visualization has added a new dimension to the classical microscopic approaches to these tumors recently. Methods Eighty-six patients (age: 31.6 ± 11.7 years, M:F = 1:1) were included. Nineteen patients (22.1%) had a multicompartmental tumor. Tumor extension was classified into five subtypes. Sixty-two patients underwent a pure microscopic approach (72%) out of which 10 patients (16%) underwent an endoscope-assisted surgery (11.6%) and 24 patients (28%) underwent an endoscope-controlled excision. Surgical outcomes were retrospectively analyzed. Results Headache (53.4%), hearing loss (46.5%), and trigeminal neuralgia (41.8%) were the leading symptoms. Interestingly, 21% of the patients had at least one preexisting cranial nerve deficit. Endoscopic assistance helped in removing an unseen tumor lobule in 3 of 10 patients (30%). Pure endoscopic approach significantly reduced the hospital stay from 9.2 to 7.3 days ( p = 0.012), and had a statistically insignificant yet a clearly noticeable lesser incidence of subtotal tumor excision (0 vs. 10%, p = 0.18) with comparable cranial nerve deficits but with a higher postoperative cerebrospinal fluid (CSF) leak rate (29% vs. 4.8%, p = 0.004). Conclusion Endoscope assistance in CPE surgery is a useful addition to conventional microscopic retromastoid approach. Pure endoscopic excision in CPE is feasible, associated with a lesser duration of hospital stay, better extent of excision in selected cases, and it has a comparable cranial nerve morbidity profile albeit with a higher rate of CSF leak.

C3 segmental vertebral artery and its surgical implication in craniovertebral junction anomalies: Insights from two cases.

A spectrum of vertebral artery (VA) anomalies have been described with or without an associated congenital craniovertebral junction (CVJ) anomalies. C3 segmental VA, where the VA enters the dura at the level of C2/3 intervertebral foramen is an extremely rare anomaly. We report two cases of congenital CVJ anomaly (irreducible in one with C2/3 fusion and reducible in the other; without any subaxial fusion but with articular agenesis at C2/3 joint on the anomalous artery side). Computed tomographic angiography revealed intraspinal intradural entry of VA through the C2/3 intervertebral foramen on the right side with the contralateral artery found crossing the atlanto-axial joint. Both the patients underwent posterior approach and C2 was spared from instrumentation in both cases. Postoperatively, the patient with irreducible dislocation recovered well while the patient with reducible dislocation expired, possibly secondary to the thrombosis of the dominant VA from C2/3 foraminal encroachment. C3 segmental VA may be advantageous in aggressively exposing the C1/2 joint but instrumentation of C2 or C3 needs caution in view of the possibility of VA injury. Our experience shows that VA may be endangered even while exposing and protecting the artery. For such cases, we recommend posterior decompression of the C2/3 neural foramen during instrumentation in the absence of associated C2/3 fusion, as an abnormal joint morphology of C2/3 indicates a C2/3 instability.

Type I Chiari Malformation Without Concomitant Bony Instability: Assessment of Different Surgical Procedures and Outcomes in 73 Patients.

OBJECTIVE: Posterior fossa decompression is the treatment of choice in type 1 Chiari malformation (CM-1) without bony instability. Although surgical fixation has been recommended by a few authors recently, comparative studies to evaluate these treatment strategies using objective outcome tools are lacking. METHODS: Seventy-three patients with pure CM-1 (posterior fossa bony decompression [PFBD], n = 21; posterior fossa bony and dural decompression [PFBDD], n = 40; and posterior fixation [PF], n = 12) underwent a postoperative outcome assessment using Chicago Chiari Outcome Score (CCOS). Logistic regression analysis detected predictors of an unfavorable outcome. RESULTS: Minimally symptomatic patients generally underwent a PFBD while most of the clinically severe patients underwent a PFBDD (p = 0.049). The mean CCOS score at discharge was highest in the PF (12.0 ± 1.41) and lowest in PFBDD group (10.98 ± 1.73, p = 0.087). Patients with minimal preoperative clinical disease severity (adjusted odds ratio [AOR], 4.58; 95% confidence interval [CI], 1.29-16.31) and PFBDD (AOR, 7.56; 95% CI, 1.70-33.68) represented risks for an unfavorable short-term postoperative outcome. Though long-term outcomes (CCOS) did not differ among the 3 groups (p = 0.615), PFBD group showed the best long-term improvements (mean follow-up CCOS, 13.71 ± 0.95), PFBDD group improved to a comparable degree despite a poorer short-term outcome while PF had the lowest scores. Late deteriorations (n = 3, 4.1%) occurred in the PFBDD group. CONCLUSION: Minimally symptomatic patients and PFBDD predict a poor short-term postoperative outcome. PFBD appears to be a durable procedure while PFBDD group is marred by complications and late deteriorations. PF does not provide any better results than posterior fossa decompression alone in the long run.

Resistant Temporal Lobe Epilepsy: Initial Steps into a Bigger Epilepsy Surgery Program.

Background Among the patients of drug-resistant epilepsy, a subset which has focal impaired seizures localizes to the temporal lobe region (TLE). A majority of these cases are surgically amenable with anterior-medial temporal lobe resection or "lesionectomy." Objective In India, there is scarcity of "specialized centers" providing "comprehensive epilepsy care" and this dearth is further worse in populous states. In this article, we share our single center, observational, and retrospective experience of TLE in background of limited resources and utmost requirement. Methodology Our study is a retrospective analysis medically refractory epilepsy patients (2016-2019). Patients with medically refractory epilepsy were selected based upon our noninvasive protocol (clinical semiology, interictal scalp electroencephalography (EEG), long-term video EEG monitoring data, and magnetic resonance injury [MRI]). The follow-up was noted from the last out-patient visit record or through telephonic conversation (International League Against Epilepsy score). Results Of 23 cases of TLE ( n = 7, mesial temporal sclerosis; n = 16 temporal lobe like cavernomas, tumors, or arterio-venous malformations). Single photon emission computed tomography/positron emission tomography (SPECT/PET) was performed in five cases (three cases of ictal/interictal SPECT and two cases of PET scan) where there was discordance between EEG/clinical and MRI. The median follow-up was of 19 months with 18 cases being seizure free. Five cases were fully off the antiepileptic drug (AEDs) while in 15 cases, the AEDs dosages or the number were reduced. Average number of AEDs reduced from 2.9 in preoperative period to 1.2 postoperatively. Two cases had quadrantanopia and one case of cerebrospinal fluid leak. Conclusion A multidisciplinary and holistic approach is required for best patient care. The results of our initial surgical experience are encouraging.

Temporal lobe angiocentric glioma with oligodendroglioma-like areas: a rare association of an uncommon tumor. A case report with review of literature.

BACKGROUND: Angiocentric glioma (AG) is a relatively uncommon clinico-pathological entity that presents in childhood. Angiocentric glioma displays various histopathological features which resemble cortical ependymoma, astroblastoma, and pilomyxoid astrocytoma and schwannoma. The astrocytes in angiocentric glioma appear peculiarly elongated, bipolar in shape, and characteristically arranged around blood vessels. They resemble radial glia and tanycytes morphologically. Unlike ependymomas, AG is a diffusely infiltrating lesion and perivascular processes are often much thicker than those in classic ependymomas. CASE PRESENTATION: AG usually present clinically as seizures, often as medically intractable epilepsy. In the indexed case, apart from unusual presentation with features of raised intra-cranial tension, an unusual histological picture of a more cellular oligodendroglioma like component was also seen. CONCLUSION: The appropriate diagnosis is critical as AG is usually slowly growing and treatable by surgical excision alone.

Coexistent Cerebral Cavernous Malformation and Developmental Venous Anomaly: Does an Aggressive Natural History Always Call for Surgical Intervention?

Coexistence of cerebral cavernous malformations (CMs) and developmental venous anomaly (DVA) represents the most common form of mixed intracranial vascular malformations. Existing literature supports not only a possible causative role of DVA for de novo CMs but also a potentially detrimental effect on an associated CM, increasing the chances of hemorrhagic complications and growth in the latter. A 52-year-old gentleman presented to us with a 17-year long history of simple motor seizures on the left faciobrachial region. On magnetic resonance imaging (MRI) of the head, a 1.5 cm × 1.5 cm CM without any evidence of recent hemorrhage was identified in the left high frontal premotor area. There was a linear enhancement in the adjoining superior frontal sulcus on contrast MRI. On intra-arterial angiogram, this hyperintensity was confirmed to be a venous channel draining into the superior sagittal sinus. Thus, a diagnosis of cavernoma associated with a DVA was made. The patient was advised conservative treatment and he was doing well at follow-up. Unless diligently looked for, DVA associated with CM may be easily missed. The coexistence has pathophysiological and management implications. Despite the reported aggressive natural history, there is a scope for conservative treatment for these complex vascular malformations.

Pathologic Laughter as an Early and Unusual Presenting Symptom of Petroclival Meningioma: a Case Report and Review of the Literature.

BACKGROUND: Pathologic laughter is inappropriate, involuntary, and unmotivated laughter episodes that may or may not be associated with mirth or amusement. Although associated with many diffuse brain pathologies, its association with intracranial focal mass lesions causing ventrolateral brainstem compression, like petroclival meningioma, is very rare. The exact pathophysiology of this interesting and unusual clinical symptom is unknown, but probably involves disinhibition and release of the so-called coordination center located in the upper brainstem due to compression by the tumor. CASE DESCRIPTION: A 26-year-old woman presented with recurrent episodes of inappropriate and involuntary laughter, which significantly affected her quality of life, for 2 years. These episodes did not resolve, and a magnetic resonance imaging of the brain showed a giant petroclival meningioma causing upper brainstem compression. Near-total excision of the tumor was done using an extended middle fossa approach. To our surprise, the pathologic laughter subsided immediately after surgery. CONCLUSIONS: Pathologic laughter may be the only symptom of a focal mass lesion causing ventrolateral upper brainstem compression, like petroclival meningioma, well before other neurological sign/symptoms appear. Tumors causing ventral brainstem compression must be ruled out before the patient is sent for a psychiatric evaluation.

Intracranial Peripheral Primitive Neuroectodermal Tumor Arising from the Clivus with Intracranial Metastasis in an Elderly Woman: Case Report and Review of the Literature.

BACKGROUND: Intracranial peripheral primitive neuroectodermal tumors (pPNETs) are rare lesions, accounting for only 1% of all pPNETs, and usually arise from bone and soft tissue. These tumors primarily affect young patients (<30 years old) and rarely affect older patients. The common intracranial locations of these lesions are frontal dura and tentorium. These lesions rarely may involve the cerebellopontine angle, cavernous sinus, and jugular foramen. Involvement of clivus has not been reported to date. Intracranial metastasis from primary intracranial pPNET is a further rare occurrence. CASE DESCRIPTION: A 74-year-old woman presented with gradually progressive painless diminution of vision in both eyes alongwith headache for 6 months duration. Radiology demonstrated a clival mass as well as a suspected intracranial metastasis. Histopathology was suggestive of pPNET. We also present a brief review of the literature on pPNETs. CONCLUSIONS: pPNETs of the clival region are exceptionally rare. In elderly patients with clival mass lesions, the rare possibility of pPNET should be kept in mind.