Comparative Analysis of Hemispherotomy in Adults versus Children- A Prospective Observational Series.

BACKGROUND: Hemispherotomy (HS) is an effective treatment for unilateral hemispheric onset epilepsy. There are few publications for HS in adults, and there is no series comparing adults and pediatric patients of HS. OBJECTIVE: To compare the hemispherotomies done in adult patients with pediatric ones in terms of efficacy and safety. METHODS: Data was prospectively collected for HS patients (up to 18 years and more) from Aug 2014 to Aug 2018. Comparison between the groups was made for seizure onset, duration of epilepsy, frequency of seizures, number of drugs, intraoperative blood loss, postoperative seizure control, postoperative stay, postoperative motor functions, and preoperative and postoperative intelligence quotient. Follow-up was one year. RESULTS: A total of 61 pediatric and 11 adults underwent HS. The seizure onset was earlier in children, and the duration of epilepsy was longer in adults. The frequency of seizures per day was more in children being 14.62 ± 26.34 in children, and 7.71 ± 5.21 per day in adults (P - 0.49). The mean number of drugs was similar in the preoperative and postoperative periods in both. Class I seizure outcome was similar in both the groups being 85.24% in children and 90.9% in adults (P - 0.56). Blood loss, postoperative stay, was similar in both the groups. No patient had a new permanent motor deficit. Power worsened transiently in 1 pediatric patient and in 4 adult patients. The visual word reading and object naming improved in both the groups (no intergroup difference), and IQ remained the same in both groups. One adult patient had meningitis, and another had hydrocephalus requiring shunt placement. CONCLUSION: Hemispherotomy is a safe and effective procedure in adults as in children in appropriately selected patients.

Long-Term Functional Outcome Following Left Hemispherotomy in Adults and Pediatric Participants with Fmri Analysis.

Background and Objective: Hemispherotomy surgery in adults is shrouded in doubts regarding the functional outcome. The age at surgery alone should not be the deciding factor for surgery. Language paradigms were used in functional magnetic resonance imaging (fMRI) to confirm the role played by the age at the onset of seizures to predict the postoperative functional outcome. The objective of the study was to formulate an optimal strategy for patient selection for the left-sided hemispherotomy in adults, based on functional outcome analysis. Materials and Methods: A retrospective analysis of 20 participants (age at surgery 1-26 years) who underwent left hemispherotomy (over a 5-year period) was conducted. The language and motor functional assessments of 18 participants (13 pediatric and five adult participants; attrition of participants- two) were recorded at presentation and during follow-up visits. After approval was obtained from the Institutional Ethics Committee, 13 cooperative participants (eight pediatric and five adult participants) underwent language fMRI. Motor fMRI with both active and passive paradigms was done in 16 participants. Results: All 18 participants with a mean follow-up of 24 months had class I seizure-free outcome. Of these 18, five were adults (mean age = 21 years, range: 18-22 years) and 13 were in the pediatric age group (mean age = 8 years, range: 2-15 years). Postoperatively, four adults retained both verbal fluency and language comprehension at a mean follow-up period of 38 months (range: 24-48 months). Their pre- and post-op language fMRI showed word generation and regional activations for semantic comprehension in the right hemisphere. The motor area activations were seen in the right hemisphere in two and in the left hemisphere in two participants. Among the pediatric participants, four (group I [n = 4/13]) who had good language outcome showed activations in the right hemisphere. In two participants (group II [n = 2/13]) who deteriorated postoperatively, the activations were in the left hemisphere. Five participants (group III [n = 5/13]) who retained the telegraphic language postoperatively had bilateral activations of semantic comprehension areas in fMRI. All 13 pediatric participants had motor area activations seen in the left hemisphere, similar to controls. Conclusion: Left hemispherotomy can be advised to adults with comparably good postoperative language and motor outcome as in the pediatric age group, provided the weakness is acquired perinatally or below the age of 7 years. The fMRI is a valuable tool to aid in patient selection.

Mini Temporal Craniotomy Using Anatomical Surface Landmarks for Temporal Lobe Epilepsy: Technical Note and Clinical Outcomes.

Background: Patients with temporal lobe epilepsy are subjected to standard temporal lobectomy wherever indicated. This is performed using a reverse question mark flap and a standard frontotemporal craniotomy. We describe the technique of minitemporal craniotomy (3 × 3cms) for temporal lobe epilepsy (TLE) and analyze the clinical outcomes of patients operated using this approach. Objectives: To describe the technique of minitemporal craniotomy for TLE without navigation guidance and to analyze the clinical outcomes of patients operated using this approach. Materials and Method: This was a retrospective analysis of all consecutive TLE cases operated at our institute from 2014 to 2019, via minitemporal craniotomy, using surface landmarks only without navigation guidance. The surgical technique, indications for surgery, and their clinical outcomes were analyzed. Results: A total number of 48 patients underwent surgery for TLE. There were no complications except three patients who had transient hemiparesis. The average duration of hospital stay was 4 days following surgery. Out of 28 patients with mesial temporal sclerosis, 22 (82%) had international league against epilepsy, Class I seizure outcome, 4 (12.5%) had Class II outcome and 2 (5.5%) had Class III outcome. 9 patients with dysembryoplastic neurectodermal tumor (DNET), 4 gangliogliomas, 2 neurocystecercosis (NCC), all had Class I outcome. Out of the five patients with MTS and associated anterior temporal focal cortical dysplasia (FCD), four (80%) had a Class I outcome, whereas one (20%) had Class II outcome. Conclusion: Utilizing surface anatomical landmarks, minitemporal craniotomy can be performed in even peripheral centers without neuronavigation, with good cosmesis, seizure outcomes.

Robotic thermocoagulative hemispherotomy: concept, feasibility, outcomes, and safety of a new "bloodless" technique.

OBJECTIVE: The authors present a new "bloodless" technique for minimally invasive robotic thermocoagulative hemispherotomy (ROTCH). Such a method is being described in the literature for the first time. METHODS: A robotic system was used to plan five sets of different trajectories: anterior disconnection, middle disconnection, posterior disconnection, corpus callosotomy, and temporal stem and amygdalar disconnection. A special technique, called the "X" technique, allowed planar disconnection. Registration was performed with surface landmarks (n = 5) and bone fiducials (n = 1). Coregistration with O-arm images was performed one or two times to confirm the trajectories (once for middle disconnection, and once for disconnection of the temporal stem and amygdala or body of the corpus callosum). Impedance measured before ablation allowed for minor adjustments. Radiofrequency ablation was performed at 75°C-80°C for 60 seconds. Surgical procedures were performed with multiple twist drills. After removal of the electrode, glue was used to prevent CSF leak, and a single stitch was applied. Follow-up CT and MRI were immediately performed. RESULTS: The pathologies included Rasmussen's encephalitis (n = 2), hemispheric cortical dysplasia (n = 2), posttraumatic encephalomalacia (n = 1), and perinatal insult (n = 1). The mean ± SD (range) age was 6.7 ± 3.6 years (5 months to 10.2 years), and the right side was affected in 4 patients. The mean ± SD seizure frequency was 7.4 ± 5.6 seizures per day (1 patient had epilepsia partialis continua). The mean ± SD number of trajectories was 15.3 ± 2.5, and the mean ± SD number of lesions was 108 ± 25.8. The mean ± SD maximum numbers of trajectories and lesions required for middle disconnection were 7.1 ± 1.7 and 57.5 ± 18.4, respectively. All but 1 patient had class 1 outcomes according to the International League Against Epilepsy Outcome Scale at a mean ± SD (range) follow-up of 13.5 ± 1.6 (12-16) months; the remaining patient had a class 2 outcome. The estimated blood loss was < 5 ml for all patients. Complications included repeat surgery (after 2 weeks) for a "skip" area (n = 1) and a small temporal hematoma (n = 1), which resolved. CONCLUSIONS: ROTCH seems to be a safe, feasible, and bloodless procedure, with a very low morbidity rate and promising outcomes.

Early- and long-term surgical outcomes in 109 children with lipomyelomeningocele.

AIM: To determine the functional outcomes and risk factors for outcomes following surgery for lipomyelomeningocele (LMMC). METHODS: Data from 109 children with LMMC who underwent surgery from January 2008 to December 2017 were retrospectively studied to evaluate functional outcomes and possible risk factors for early- (at discharge from hospital or within 1 month of surgery) and long-term outcomes after surgery. RESULTS: There were 53 boys and 56 girls with median age of 36 months (IQR 12-90 months; range, 4 months to 18 years) at surgery. At presentation, neurological function was normal (asymptomatic group) in 28 (25.7%) children while there was neurological dysfunction (symptomatic group) in 81 (74.3%). Near total or radical excision of lipoma was done in 71 (65.1%) children. Six (5.8%) children had deterioration of neurological function in the post-operative period with recovery of function in three of them. There were no statistically significant risk factors for early outcomes. At a mean follow-up of 62.5 months (IQR 35-82 months; range, 12-146 months), 21 (27.6%) of the 76 symptomatic group children (including 59.3% with incomplete bladder dysfunction) showed improvement, 52 (68.4%) remained the same, while 3 (4%) deteriorated. In 31 children (symptomatic group) who developed symptoms after 1 year of age, early surgery after development of symptoms was associated with better chances of recovery (p = 0.0008). In the asymptomatic group, 23 (88.5%) had normal neurological function at mean follow-up of 57.5 months (IQR 30-77 months; range, 12-141 months) and 3 (11.5%) had neurological deterioration. The mean time to late deterioration (re-tethering) from surgery was 51.3 months (IQR 24-75 months; range, 24-84 months). Presence of syrinx on initial MRI (p = 0.008) and partial resection of the lipoma (p = 0.02) were independent risk factors for delayed deterioration. CONCLUSION: Radical resection of LMMC probably helps in preserving neurological function in > 90% of children at long-term follow-up. Fifty-five percent of children > 2 years of age with incomplete bladder dysfunction regained normal function following surgery. In children who develop symptoms after birth, early surgical intervention can reverse the neurological deficits.

Hypothalamic Hamartoma and Endocrinopathy: A Neurosurgeon's Perspective.

BACKGROUND: The management of hypothalamic hamartomas (HH) rests upon the type of presentation. These are rare congenital benign lesions presenting either with central precocious puberty (CPP), drug refractory epilepsy (DRE) or combination of both. We present here our experience in the management of these lesions from a neurosurgeon's perspective and review the pertinent literature. OBJECTIVE: To present a series of HH presenting with CPP and DRE managed in the neurosurgery department at our center with an emphasis on the associated endocrine abnormalities. MATERIALS AND METHODS: A prospective observational study over a period of five years included 16 patients of HH. All patients were evaluated with 3 Tesla Magnetic Resonance Imaging (MRI) brain, complete hormonal workup including gonadotrophins, testosterone (males) and estradiol (females), and video-electroencephalography (VEEG) as a part of epilepsy workup. All these patients were evaluated with postoperative hormonal workup and repeat MRI brain if repeat surgery was contemplated. RESULTS: Among the 16 patients of HH, there were 11 male and 6 female children. All the patients presented with DRE with four of these had associated CPP. All the patients underwent robotic-guided radiofrequency ablation (RFA), with 75% seizure freedom following 1st RFA surgery. Three of the four patients with CPP achieved both clinical and biochemical normalization. One patient had just a marginal reduction in the serum gonadotrophins. One patient was reoperated twice and three underwent RFA thrice. CONCLUSION: The management of HH should be individualized with DRE taking the precedence requiring early surgery. A multidisciplinary approach is therefore recommended for a successful outcome.

Left Temporal Lobectomy Using Functional MRI in a Math Genius: A Case Report.

Standard treatment of drug-refractory epilepsy, due to left mesial temporal sclerosis, is anterior temporal lobectomy with amygdalohippocampectomy (ATL). This carries a risk of cognitive deficits, including comprehension, verbal memory, and visual memory. Preoperative language lateralization and localization is important to preserve these functions. Often Wada testing is used for these, but it carries risk due to its invasive nature. In addition, it can lateralize but not localize and may not be readily available. We hereby present a mathematics genius who underwent left ATL under the guidance of functional MRI and neuropsychological assessment alone, resulting in the preservation of all of his cognitive abilities even in the immediate postoperative period. A video demonstration of his calendar likeability is also shown.

Enhancing outcomes of endoscopic vertical approach hemispherotomy: understanding the role of "temporal stem" residual connections causing recurrence of seizures.

OBJECTIVE: The authors sought to analyze the residual connections formed by the temporal stem as a cause for seizure recurrence following endoscopic vertical interhemispheric hemispherotomy and to review and compare lateral approach (perisylvian) with vertical approach surgical techniques to highlight the anatomical factors responsible for residual connections. METHODS: This study was a retrospective analysis of patients who underwent endoscopic hemispherotomy for drug-resistant epilepsy. Postoperative MR images were analyzed. Specific attention was given to anatomical 3D-acquired thin-section T1 images to assess the extent of disconnection, which was confirmed with a diffusion tensor imaging sequence. Cadaver brain dissection was done to analyze the anatomical factors responsible for persistent connections. RESULTS: Of 39 patients who underwent surgery, 80% (31/39) were seizure free (follow-up of 23.61 ± 8.25 months) following the first surgery. Thirty patients underwent postoperative MRI studies, which revealed persistent connections in 14 patients (11 temporal stem only; 3 temporal stem + amygdala + splenium). Eight of these 14 patients had persistent seizures. In 4 of these 8 patients, investigations revealed good concordance with the affected hemisphere, and repeat endoscopic disconnection of the residual connection was performed. Two of the 8 patients were lost to follow-up, and 2 had bihemispheric seizure onset. The 4 patients who underwent repeat endoscopic disconnection had seizure-free outcomes following the second surgery, increasing the good outcome total among all patients to 90% (35/39). Cadaveric brain dissection analysis revealed the anatomical factors responsible for the persistence of residual connections. CONCLUSIONS: In endoscopic vertical approach interhemispheric hemispherotomy (and also vertical approach parasagittal hemispherotomy) the temporal stem, which lies deep and parallel to the plane of disconnection, is prone to be missed, which might lead to persistent or recurrent seizures. The recognition of this limitation can lead to improved seizure outcome. The amygdala and splenium are areas less commonly prone to be missed during surgery.

Need of Immediate Drug Reduction after Epilepsy Surgery - A Prospective Observational Study.

BACKGROUND: Patients undergoing epilepsy surgery are on polytherapy. Drug tapering is usually done after 1 year in adults and after 6 months in children. Sometimes, drugs have to be altered during the perioperative period, which is more commonly seen in hemispherotomy (HS) patients. The present study was done to compare perioperative drug alterations between HS and temporal (TL) lobectomy patients. MATERIALS AND METHODS: Prospective analysis of postoperative HS and TL patients was done. Primary outcomes were drug number, dosage changes, and seizure outcome. Secondary outcome studied was a change in intelligence quotient (IQ) in the two groups. RESULTS: At total of 71 patients were included. Perioperative drug stopping (clobazam - CLB) was needed in 3/38 patients in the HS group, due to sedation. Dosage was reduced in 23/38 (60.52%) in HS group, and in 2/33 (6%) in TL group P < 0.001. The most common drug was CLB, with reduction in 21/27 (77.77%) patients, with a mean reduction of 41.21 ± 4.01%. Two patients required drug substitution in the HS group. About 64/71 (90.1%) patients achieved Class I outcome at a 1-year postoperative time point (TL - 90.9%, HS - 89.47%). There was no change in IQ in any of the groups. CONCLUSION: Perioperative drug alteration is often needed in the HS patients as compared to TL patients. Benzodiazepines have to be reduced to maintain alertness in the HS patients. The increased sedation postoperatively can be due to decreased cortical drive over the reticular activating system, gamma-aminobutyric acid (GABA) receptor denervation hypersensitivity, or increased activity of drugs over the remaining active hemisphere.

Endoscope-assisted (with robotic guidance and using a hybrid technique) interhemispheric transcallosal hemispherotomy: a comparative study with open hemispherotomy to evaluate efficacy, complications, and outcome.

OBJECTIVEEndoscope-assisted hemispherotomy (EH) has emerged as a good alternative option for hemispheric pathologies with drug-resistant epilepsy.METHODSThis was a prospective observational study. Parameters measured included primary outcome measures (frequency, severity of seizures) and secondary outcomes (cognition, behavior, and quality of life). Blood loss, operating time, complications, and hospital stay were also taken into account. A comparison was made between the open hemispherotomy (OH) and endoscopic techniques performed by the senior author.RESULTSOf 59 cases (42 males), 27 underwent OH (8 periinsular, the rest vertical) and 32 received EH. The mean age was 8.65 ± 5.41 years (EH: 8.6 ± 5.3 years; OH: 8.6 ± 5.7 years). Seizure frequency per day was 7 ± 5.9 (EH: 7.3 ± 4.6; OH: 15.0 ± 6.2). Duration of disease (years since first episode) was 3.92 ± 1.24 years (EH: 5.2 ± 4.3; OH: 5.8 ± 4.5 years). Number of antiepileptic drugs per patient was 3.9 ± 1.2 (EH: 4.2 ± 1.2; OH: 3.8 ± 0.98). Values for the foregoing variables are expressed as the mean ± SD. Pathologies included the following: postinfarct encephalomalacia in 19 (EH: 11); Rasmussen's syndrome in 14 (EH: 7); hemimegalencephaly in 12 (EH: 7); hemispheric cortical dysplasia in 7 (EH: 4); postencephalitis sequelae in 6 (EH: 2); and Sturge-Weber syndrome in 1 (EH: 1). The mean follow-up was 40.16 ± 17.3 months. Thirty-nine of 49 (79.6%) had favorable outcomes (International League Against Epilepsy class I and II): in EH the total was 19/23 (82.6%) and in OH it was 20/26 (76.9%). There was no difference in the primary outcome between EH and OH (p = 0.15). Significant improvement was seen in the behavioral/quality of life performance, but not in IQ scores in both EH and OH (p < 0.01, no intergroup difference). Blood loss (p = 0.02) and hospital stay (p = 0.049) were less in EH.CONCLUSIONSEH was as effective as the open procedure in terms of primary and secondary outcomes. It also resulted in less blood loss and a shorter postoperative hospital stay.

Endoscopic Transmaxillary Transposition of Temporalis Flap for Recurrent Cerebrospinal Fluid Leak Closure.

Objective To describe the technique of endoscopic transmaxillary temporalis muscle flap transposition for the repair of a persistent postoperative sphenoidal cerebrospinal fluid leak. Design The repair of a recurrent cerebrospinal fluid leak for a patient who had undergone endoscopic transsphenoidal excision of an invasive silent corticotroph Hardy C and Knosp Grade IV pituitary adenoma was undertaken. The patient had completed postoperative radiotherapy for the residual tumor and presented with cerebrospinal fluid leak, 1 year later. The initial two attempts to repair the cerebrospinal fluid leak with free grafts failed. Therefore, an endoscopic transmaxillary transposition of the temporalis muscle flap was attempted to stop the cerebrospinal fluid leak. Results The endoscopic transmaxillary transposition of the vascularized temporalis muscle flap onto the cerebrospinal fluid leak repair site resulted in successful closure of the cerebrospinal fluid leak. Conclusion Endoscopic transmaxillary transposition of the temporalis flap resulted in closure of recurrent cerebrospinal fluid leak in a patient with recurrent pituitary adenoma, who had undergone previous surgery and radiotherapy. This technique has advantages over the endoscopic transpterygoid transposition of the same flap and could be used as a complementary technique in selected patients.

Rapid-onset paraparesis and quadriparesis in patients with intramedullary spinal dermoid cysts: report of 10 cases.

OBJECT Intramedullary dermoid cysts are rare tumors of the spinal cord. Presentation with rapid onset of paraparesis or quadriparesis (onset within 2 weeks) is rarer still. The authors present their experience in the management and outcome of patients with such a presentation. METHODS Patient records between 2000 and 2014 were retrospectively reviewed to identify those with intraspinal dermoid cysts who presented with rapid-onset paraparesis or quadriparesis. Their clinical, radiological, operative, and follow-up data were analyzed. RESULTS Of a total of 50 patients with intraspinal dermoid cysts managed during the study period, 10 (20%) presented with rapid-onset paraparesis or quadriparesis; 9 patients ranged in age from 8 months to 2 years, and 1 patient was 25 years old. A dermal sinus was seen in the lumbar region of 4 patients, the sacral region of 3, and the thoracic region of 1, and in 1 patient no sinus was found. All except 1 patient presented with rapid-onset paraparesis secondary to infection of the intramedullary dermoid cyst. One patient presented with rupture of a dermoid cyst with extension into the central canal up to the medulla. Early surgery was done soon after presentation in all except 2 patients. Among the 9 patients who underwent surgery (1 patient did not undergo surgery), total excision of the intramedullary dermoid cyst was done in 3 patients, near-total excision in 4 patients, and partial excision in 2 patients. Of the 9 patients who underwent surgery, 8 showed significant improvement in their neurological status, and 1 patient remained stable. The 1 patient who did not undergo surgery died as a result of an uncontrolled infection after being discharged to a local facility for management of wound infection. CONCLUSIONS Early recognition of a dermal sinus and the associated intraspinal dermoid cyst and timely surgical intervention can eliminate the chances of acute deterioration of neurological function. Even after an acute onset of paraparesis or quadriparesis, appropriate antibiotic therapy and prompt surgery can provide reasonably good outcomes in these patients.