Maximizing outcomes in hypothalamic hamartoma surgery

Achieving favorable seizure outcomes is challenging in patients with seizures resulting from hypothalamic hamartoma. Although minimally invasive and non-invasive surgical procedures are used to treat this population, these procedures have limitations. Therefore, we analyzed the outcomes of patients with hypothalamic hamartoma following direct resection. We included 159 patients with hypothalamic hamartoma who underwent direct resection using the transcallosal interforniceal approach between 2011 and 2018. The relationships between clinical parameters and seizure outcomes were analyzed. In total, 55.3% achieved gross total resection and 25.2% underwent near-total resection. Of all patients, 79.2% were overall seizure-free at one year, but this number dropped to 77.0% at more than five years. Moreover, 88.4% (129/146) reached gelastic seizure (GS)-free status at one year and this number increased to 89.0% (97/109) at more than five years. Seventy-one patients took antiseizure medication (ASM) long-term, 68 took it for one year, and 11 took it for one-half year. The duration of ASM consumption (p < 0.001) and extent of hypothalamic hamartoma resection (p = 0.016) were significant independent predictors of long-term overall seizure-free survival, while the duration of ASM consumption (p = 0.011) and extent of hypothalamic hamartoma resection (p = 0.026) were significant independent predictors of long-term GS-free survival. Most patients' behavior, school performance, and intelligence were not affected after surgery. Direct resection is effective and safe strategy for patients with hypothalamic hamartomas. Hypothalamic hamartomas should be removed as completely as possible, and patients should take ASM long-term following surgery to reach long-term overall seizure-free or GS-free status.

We present a novel variable aspiration tissue resector for use with neuroendoscopy. Two patients, 4 and 14 years old, respectively, presented with intractable gelastic seizures refractory to maximal medical therapies. Magnetic resonance imaging showed mass lesions of the third ventricle consistent with hypothalamic hamartoma. The patients underwent magnetic resonance imaging wand-guided endoscopic resection of the tumor with the Suros novel variable aspiration tissue resector. There were no device-associated complications or adverse events. The hamartoma was disconnected in one patient, and gross total resection was achieved in the other. Endoscopy for tumor resection is still frustrated by the lack of surgical tools, such as ultrasonic aspirators, comparable with those available for use during open procedures. The variable-aspiration tissue resector reported here can be used to resect tumor tissue safely. These two cases demonstrate that gross total resection of small hypothalamic hamartomas is feasible with minimal morbidity through an endoscopic approach.

ABSTRACTBACKGROUND: Endoscopic transnasal surgery has gained rapid global acceptance over the last two decades. The growing literature and understanding of anterior skull base endoscopic anatomy, in addition to new dedicated endoscopic instruments and tools, have helped to expand the use of the transnasal route in skull base surgery.OBJECTIVE: Report our early experience in expanded endoscopic transnasal surgery (EETS) and approach to skull base neoplasms.DESIGN: Descriptive, retrospective case series.SETTING: Major tertiary care center.PATIENTS AND METHODS: A retrospective case review was conducted at King Saud University Medical City between December 2014 and August 2019. Cases with skull base neoplasms that underwent EETS were included. EETS was defined as endoscopic surgical exposure that extended beyond the sellar margins (prechiasmatic sulcus superiorly, clival recess inferiorly, cavernous carotid lines laterally). Routine transsphenoidal pituitary neoplasms, neoplasms of sinonasal origin and meningoencephaloceles were excluded.MAIN OUTCOME MEASURES: Preoperative clinical assessment, imaging results, surgical approach, and hospital course were all retrieved from the patient electronic charts. Clinical follow-up, perioperative complications, and gross residual tumor rates were documented and reviewed.SAMPLE SIZE AND CHARACTERISTICS: 45 cases of EETS, 13 males and 32 females with mean age of 39.0 (17.7) years (range 2–70 years).RESULTS: The series comprised a wide range of pathologies, including giant pituitary adenoma (8 cases), meningioma (23 cases), craniopharyngioma (4 cases), chordoma (4 cases), optic pathway glioma (2 cases), epidermoid neoplasms (2 cases), astrocytoma (1 case), and teratoma (1 case). For the entire series, gross total resection was achieved in 25/45 operations (55.5%). Postoperative cerebrospinal fluid leak was the most common complication observed in 9 patients (20%) which were all managed endoscopically. Major vascular complications occurred in 2 patients (4.4%) and are described. Other complications are outlined as well. No mortality was observed.CONCLUSIONS: EETS to the skull base can be done with results comparable to traditional approaches. More work is needed to expand our experience, improve outcomes, and educate the public and medical community in our region about the usefulness of this approach.LIMITATIONS: Sample size and study design.CONFLICT OF INTEREST: None.

We tested the hypothesis that widespread extralesional abnormalities of cerebral structure exist in association with apparently isolated hypothalamic hamartomata, providing a structural basis for the poor response of seizures to removal of the hamartoma or other apparently focal epileptogenic zones present. High-resolution magnetic resonance imaging (MRI) brain scans of 2 patients with hypothalamic hamartomata were quantified by determination of regional distribution and symmetry of distribution of cortical gray matter and subcortical matter volumes. The results were compared with normal ranges for the distribution of such tissues in 33 controls. Both patients had abnormalities of distribution of gray and subcortical matter, whereas control subjects did not. These abnormalities were beyond the hamartoma itself, in areas of cerebrum that on visual inspection alone appeared completely normal. Extralesional abnormalities of cerebral structure are present in the cerebrum of patients with hypothalamic hamartoma, as in most patients with other dysgeneses. These abnormalities may explain the poor outcome of epilepsy surgery in patients with this form of dysgenesis. These preliminary findings require further investigation.

Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children. The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (> 7 cm/year), bone age remained advanced (> +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery. Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot. The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.

Central neurocytomas (CN) are rare pediatric CNS tumors most often with a benign clinical course. Occasionally, these tumors occur outside the ventricles and are called extraventricular neurocytomas (EVN). We present a retrospective institutional analysis of children with neurocytoma with prolonged follow-up. Twelve patients were diagnosed with neurocytoma at our institution between 1993 and 2004. Six patients were male and the median age at diagnosis was 12 years (1.5 to 16 y). Seven patients had CN and 5 had EVN. Presenting symptoms included headaches (67%), vomiting (50%), nausea (33%), seizures (33%), and mental status changes (25%). Obstructive hydrocephalus was present at diagnosis in 42% of the cases. Younger age and seizures were more common in patients with EVN. Gross total resection (GTR) was achieved in 42% (5/12) of the patients. Patients with GTR received no adjuvant therapy upfront; 1 patient subsequently had recurrence with leptomeningeal disease. Patients with subtotal resection received additional treatment: 1 underwent reoperation (GTR), 2 patients received focal radiation, 2 patients received adjuvant chemotherapy, and 2 patients received craniospinal irradiation followed by chemotherapy. The 20-year overall survival for this cohort was 83% with event free survival of 56%. Overall survival for CNs was 100%, versus 40% for EVN. Event free survival for CNs was 57% and 53% for the EVNs. An MIB-1 fraction >2% was associated with worse prognosis. Neurocytomas are rare brain tumors in children usually cured with GTR. Adjuvant focal radiation therapy and/or chemotherapy may improve disease control in cases with subtotal resection, but case-by-case analysis should be done. EVNs might be associated with worse outcome due to a higher proliferative index.

From a historical perspective, frontal sinus surgery has evolved from radical, highly invasive, disfiguring approaches to function-preserving, minimally invasive, and non-disfiguring intranasal procedures. Most sinus surgeons would agree that a sound surgical procedure is one that relieves patients' symptoms and provides a safe sinus in which future intracranial and orbital complications will not occur. For the future, sinus surgeons are searching for the ideal procedure, ie, one that is minimally invasive, reversible, and ensures the patient a safe frontal sinus for the long term. The ideal surgery will also leave minimal morbidity, will leave no cosmetic defect, and will allow for easy postoperative surveillance. To achieve this new standard in frontal sinus surgery, continuous refinements are required in the medical management and understanding of the disease processes that undermine long-term surgical success. Further advancements in instrumentation and visualization techniques are also necessary to enhance surgical precision, spare mucosa, and prevent the scarring and neo-osteogenesis that may cause surgical failures. Perhaps the most important development may be in the selection criteria for appropriate candidates who will benefit most from frontal sinus surgery.

Hypothalamic hamartomas are often associated to a progressive epileptic encephalopathy. Non‐invasive data indicates that destruction or isolation of the hamartoma may stop seizures. We present data on patients who underwent stereotactic radiofrequency with or without endoscopy. Epilepsy improved in 60% of patients with minimal morbidity. This approach was less succesful in large lesions. These results indicate that this approach should be considered in patients with small/medium lesions.

IntroductionFluorescence-guided (FG) surgery enhances the extent of resection (EOR) in glioblastoma. While 5-aminolevulinic acid (5-ALA) is the current standard, fluorescein sodium (FS) has emerged as a promising, accessible alternative. This study evaluates the impact of FS-guided resection on surgical outcomes and survival compared to conventional white-light (WL) resection.Research questionDoes FS-guided surgery improve EOR, overall survival (OS), and progression-free survival (PFS) compared to WL-guided glioblastoma surgery?Material and methodsIn this retrospective single-center analysis, data from patients with newly diagnosed glioblastoma who underwent primary resection with gross total resection (GTR) intent between 2013 and 2023 were evaluated. Based on intraoperative technique, patients were allocated to either FS-guided or WL-guided surgery groups. Logistic and linear regression assessed FS impact on GTR and residual tumor volume. Multivariate Cox regression and Kaplan-Meier analysis were used to assess OS and PFS.ResultsAmong 128 patients, 85 (66 %) underwent FS-guided surgery. GTR was achieved in 69 % of FS cases versus 36 % with WL (p = 0.0004; OR = 1.379). FS significantly reduced residual tumor volume (1.82 ml vs. 8.83 ml; p = 0.0003). MGMT methylation, GTR, and Stupp-protocol therapy were identified as independent predictors of improved OS and PFS. FS use itself showed no significant association with survival outcomes.Discussion and conclusionFS-guided surgery significantly improved EOR and reduced residual tumor volume without impacting OS or PFS. It represents a safe, effective alternative to standard WL resection. Prospective studies should explore combining fluorophores for optimal resection strategies.

Objective. To identify predictive factors of favourable outcomes in children with drug resistant epilepsy (DRE) associated with structural brain changes, who received surgical treatment. Methods. We performed a retrospective analysis of surgical treatment in 239 children with drug resistant epilepsy treated at the Pediatric Neurosurgery Department of the A. L. Polenov Russian Neurosurgical Institute from 2004 to 2014. The age of the patients varied between 2 and 18 years. The follow-up time ranged from 1 year to 10 years (on average 4.3 years). Results. According to magnetic resonance imaging (MRI), 223 (93.3%) patients had different structural brain changes. Among the patients, 47 (21.1%) had cerebral cicatrix, gliosis, cysts and atrophic changes, 33 (14.8%) had malformations of the cortical development, 43 (19.3%) had benign brain tumors, 47 (21.1%) had vascular malformations, 21 (9.4%) had arachnoid cysts, 12 (5.4%) had hippocampal sclerosis, 11 (4.9%) had phacomatosis, 5 (2.2%) had Rasmussen’s encephalitis, and 4 (1.8%) had hypothalamic hamartoma. Positron emission tomography revealed the areas of gray matter hypometabolism in the brain, which correspond to the areas of a metabolic deficiency around the epileptic focus. Favorable long-term outcomes of epilepsy surgery (class I according to Engel scale) were registered in 67.6% of the cases. The most favorable results (class I according to Engel scale) were achieved after lesionectomy (81.9%), temporal resections (78.4%), and hemispherectomy (72.7%).Conclusions. The most important predictive factors of favorable outcomes after epilepsy surgery are as follows: detection of epileptogenic morphological changes of the brain, completeness of resection of an epileptic and epileptogenic areas, and absense of seizures in the early postoperative period.

Craniopharyngiomas (CP) are rare noncancerous brain tumors located in the skull base. To date, CP remain challenging-to-resect tumors, owing to their difficult location and invasive potential, with profound adverse effects for the patient if left to grow. Indeed, gross total resection may also be accompanied by unwelcome sequalae, underscoring the need for continued investigation. In the present work, we provide a scoping review of current CP management, with emphasis on our knowledge of their genesis, available treatment options, post-intervention clinical outcomes. Leading theories of CP development are (1) the embryonic theory, explaining the development of adamantinomatous CP from epithelial remnants of Rathke's pouch and (2) the metaplastic theory, which describes papillary CP development as a result of adenohypophyseal cell metaplasia. Treatment may include surgery, intracystic therapy, or irradiation depending on tumor size, history and location. However, whether a single ideal approach and timing for CP intervention exists remains debated. We appraise and critique these areas with priority for emerging basic results and innovation.

Ganglioglioma (GG) is a rare pediatric brain tumor (1-4 %) with neoplastic glial and neuronal cells. Posterior fossa GGs (PF GGs) occur less frequently than supratentorial GGs (ST GGs). The BRAF V600E mutation has been reported in GGs and carries therapeutic implications. We compare the presenting symptoms, magnetic resonance imaging, BRAF V600E mutation status, treatment, and prognosis in children with ST and PF GGs. The neuro-oncology database at a tertiary care Children's Hospital was retrospectively reviewed from 1995 to 2010 for patients with ST and PF GG. All available imaging was reviewed. Symptoms, BRAF V600E mutation status, treatment, and survival data were collected from the electronic medical record and analyzed. Our series consisted of 11 PF GG and 20 ST GG. Children with PF GG presented with ataxia, cranial nerve deficits and long tract signs whereas the majority with ST GGs presented with seizures. On imaging, PF GGs were infiltrative and expansile solid masses with dorsal predominant "paintbrush" enhancement whereas ST GGs were well circumscribed mixed solid and cystic masses with heterogeneous enhancement. Five of 11 (45%) PF GGs and 6 of 9 (67%) ST GGs expressed the BRAF V600E mutation. No unique imaging features were identified in BRAF V600E mutation positive tumors. The majority of ST GGs were treated with surgery alone, whereas the majority of PF GGs required multimodality therapy. PF GGs had worse progression-free survival and a higher mortality rate compared with ST GGs. Unlike ST GGs, PF GGs are expansile, infiltrative, show dorsal predominant "paintbrush" enhancement, are not amenable to gross total resection, and have worse progression-free survival and mortality.

To review outcomes and complications in a series of adults undergoing a frontal osteoplastic flap procedure without obliteration, for endoscopically inaccessible sinus disease. Retrospective case note review of patients treated at Glasgow Royal Infirmary between January 2004 and October 2008. Ten patients were identified (age range 19-81 years, mean age 46.3 years). No major intra- or post-operative complications occurred. There were three minor complications: superficial discharging wound, forehead swelling and haematoma. The frontal osteoplastic flap still has a role in frontal sinus surgery. With minor technical modifications, this procedure may be performed with minimal complication and morbidity for patients with endoscopically inaccessible frontal sinus disease.

Trigeminal neuromas are rare tumours that may involve any part of the Vth nerve complex, including extracranial peripheral divisions of the nerve. A series of eight patients with intracranial trigeminal neuromas who underwent surgical management are presented. The hospital records and radiological images were reviewed with regard to clinical presentation, surgical approach, operative findings, peri-operative morbidity and neurological outcome, and percentage of tumour recurrence. The principal presenting symptoms were those of involvement of the trigeminal nerve with sensory impairment in one or more of the three divisions. Tumour location was the prime determinant of surgical approach. Lateral skull base approaches were used as they are considered to be superior for identifying tumour origin, extension, and relationship to surrounding structures. Total excision of the tumour was carried out in three of the eight patients. In the remaining five patients some tumour was left purposely in order to minimize neurological deficit and optimize post-operative quality of life. There was no peri-operative mortality or major morbidity in our series. Five patients experienced symptomatic tumour recurrence and revision surgery was performed. Management of trigeminal neuromas is complex and requires a multidisciplinary approach. Pre-operative surgical planning allows tumour removal with preservation of important neural structures in the majority of cases. For large tumours occupying both the middle and posterior cranial fossae, the retrosigmoid/retrolabyrinthine/middle cranial fossa approach provides good exposure and results in minimal brain retraction. A Fisch type C approach is necessary for the largest tumours. Long-term follow up with interval imaging is mandatory to exclude long-term tumour recurrence.

Pineocytomas are rare pineal region tumors. The mainstay of treatment for pineocytomas is total surgical resection. To address the worthiness of endoscopy to achieve total macroscopic resection and whether or not this portends better progression-free and overall survival. A series of 15 patients (11 females, 4 males; mean age, 48 years) with histological diagnosis of pineocytoma were retrospectively reviewed. All patients underwent an attempt at total resection via an interhemispheric/transtentorial/retrosplenial approach. The endoscope was used at various times throughout the case and consistently at the end to evaluate any possible residual tumor. No patient in this series was given radiotherapy after surgery. There was no surgical mortality and all patients are currently alive without recurrence (mean follow-up, 4.28 years). A macroscopic and radiological complete removal was achieved in all cases. There was only one patient with permanent neurological deficit (disconjugate eye movements). Two patients (13%) had transient poor sleep pattern, 5 patients (33%) had transient disconjugate eye movements, and 2 patients (13%) had transient short-term memory disturbance. In 6 cases (40%), the endoscope was able to detect residual tumor located either behind the Vein of Galen or attached to the undersurface of the corpus callosum. Residual tumor was then resected using a 30 degrees endoscope and dedicated angled endoscopic instruments. This series demonstrates the advantage of endoscopic-assisted surgery in the total removal of pineocytomas. With total removal of these histologically benign tumors patients may enjoy extended progression-free survival without adjuvant radiotherapy.