Transcallosal Resection of Hypothalamic Hamartomas, with Control of Seizures, in Children with Gelastic Epilepsy

Neurosurgery ◽  
2001 ◽  
Vol 48 (1) ◽  
pp. 108-118 ◽  
Author(s):  
Jeffrey V. Rosenfeld ◽  
A. Simon Harvey ◽  
Jacquie Wrennall ◽  
Margaret Zacharin ◽  
Samuel F. Berkovic
Keyword(s):  
Third Ventricle ◽  
Intractable Epilepsy ◽  
Frameless Stereotaxy ◽  
Seizure Freedom ◽  
Gelastic Seizures ◽  
Complete Excision ◽  
Transcallosal Approach ◽  
Hypothalamic Hamartomas ◽  
Gelastic Epilepsy ◽  
Antiepileptic Medications

Abstract OBJECTIVE Hypothalamic hamartomas (HHs) are associated with precocious puberty and gelastic epilepsy; the seizures are often refractory to antiepileptic medications and associated with delayed development and disturbed behavior. The current opinion is that surgery to treat intrahypothalamic lesions is formidable and that complete excision is not technically achievable. We report our experience with a transcallosal approach to the resection of HHs. METHODS Five children (age, 4–13 yr) with intractable epilepsy and HHs underwent preoperative clinical, electroencephalographic, and imaging evaluations. Two patients experienced only gelastic seizures, and three patients experienced mixed seizure disorders with drop attacks; all experienced multiple daily seizures. Patients were evaluated with respect to seizures, cognition, behavior, and endocrine status 9 to 37 months (mean, 24 mo) after surgery. The HHs were approached via a transcallosal-interforniceal route to the third ventricle and were resected using a microsurgical technique and frameless stereotaxy. RESULTS Complete or nearly complete (>95%) excision of the HHs was achieved for all patients, with no adverse neurological, psychological, or visual sequelae. Two patients experienced mild transient diabetes insipidus after surgery. Two patients developed appetite stimulation, but no other significant endocrinological sequelae were observed. Three patients are seizure-free and two patients have experienced only occasional, brief, mild gelastic seizures after surgery, all with reduced antiepileptic medications. On the basis of parental reports and our own subjective observations, the children also exhibited marked improvements in behavior, school performance, and quality of life. CONCLUSION Complete or nearly complete resection of HHs can be safely achieved via a transcallosal approach, with the possibility of seizure freedom and neurobehavioral improvements.

Surgical management of hypothalamic hamartomas in patients with gelastic epilepsy

2008 ◽  
Vol 25 (3) ◽  
pp. E8 ◽  
Author(s):  
Bassam Addas ◽  
Elisabeth M. S. Sherman ◽  
Walter J. Hader
Keyword(s):  
Quality Of Life ◽  
Epileptic Encephalopathy ◽  
Clinical Syndrome ◽  
Seizure Freedom ◽  
Interstitial Radiotherapy ◽  
Hypothalamic Hamartomas ◽  
Surgical Options ◽  
Gelastic Epilepsy ◽  
Refractory Seizures

Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is a result of a progressive epileptic encephalopathy or secondary epileptogenesis, which is potentially reversible with treatment of the HH. A variety of surgical options for the treatment of HHs exist, including open and endoscopic procedures, radiosurgery, interstitial radiotherapy, and stereotactic radiofrequency thermocoagulation. Surgical treatment can result in seizure freedom in up to 50% of patients and can be accompanied by significant improvements in behavior, cognition, and quality of life. Partial treatment of HHs may be sufficient to reduce seizure frequency and improve behavior and quality of life with less risk. A component of reversible cognitive dysfunction may be present in some patients with an HH-GE syndrome.

Seizure outcome and complications following hypothalamic hamartoma treatment in adults: endoscopic, open, and Gamma Knife procedures

2012 ◽  
Vol 117 (2) ◽  
pp. 255-261 ◽  
Author(s):  
Cornelia Drees ◽  
Kevin Chapman ◽  
Erin Prenger ◽  
Leslie Baxter ◽  
Rama Maganti ◽  
...  
Keyword(s):  
Mental Retardation ◽  
Weight Gain ◽  
Gamma Knife ◽  
Third Ventricle ◽  
Intractable Epilepsy ◽  
Adult Patients ◽  
Neurological Deficits ◽  
Hypothalamic Hamartoma ◽  
Seizure Outcome ◽  
Seizure Freedom

Object This study aimed at identifying outcomes with respect to seizures, morbidity, and mortality in adult patients undergoing resective or Gamma Knife surgery (GKS) to treat intractable epilepsy associated with hypothalamic hamartoma (HH). Methods Adult patients undergoing surgical treatment for HH-related epilepsy were prospectively monitored at a single center for complications and seizure outcome by using a proprietary database. Preintervention and postintervention data for patients 18 years of age and older, and with at least 1 year of follow-up, were analyzed, with specific attention to seizure control, complications, hormonal status, and death. Results Forty adult patients were found in the database (21 were women). The median HH volume was 0.54 cm3. In 70% of patients, it was located inside the third ventricle, attached unilaterally and vertically to the hypothalamus (Delalande Type II). Most patients (26) underwent an endoscopic resection, 10 patients had a transcallosal or other type of open (pterional or orbitozygomatic) resection, and 4 patients chose GKS. Twenty-nine percent became seizure free in the long term, and overall a majority of patients (55%) reported at least > 90% seizure improvement. Only 3 patients were ultimately able to discontinue anticonvulsants, whereas most patients were taking an average of 2 antiepileptic drugs pre- and postoperatively. The only factor significantly correlated with seizure-free outcome was the absence of mental retardation. The HH volume, HH type, and amount of resection or disconnection were not correlated to seizure freedom. A total of 4 patients (10%) died, 2 immediately after surgery and 2 later. All of them had undergone a resection, as opposed to GKS, and still had seizures. Postoperatively, persistent neurological deficits were seen in 1 patient; 34% of patients had mild hormonal problems; and 59% experienced weight gain of at least 6.8 kg (average gain 12.7 kg). Conclusions Surgical or GKS procedures in adults with HH provided seizure freedom in one-third of patients. The only significant favorable prognostic factor was the absence of mental retardation. The overall mortality rate was high, at 10%. Other important morbidities were persistent hormonal disturbances and weight gain.

Long-term outcome after emergency resection of hypothalamic hamartomas for status gelasticus

2011 ◽  
Vol 30 (2) ◽  
pp. E5 ◽  
Author(s):  
Sandipan Pati ◽  
Reena G Rastogi ◽  
Adib A. Abla ◽  
Harold L. Rekate ◽  
Yu-Tze Ng
Keyword(s):  
Endoscopic Resection ◽  
Emergency Surgery ◽  
Seizure Freedom ◽  
Gelastic Seizures ◽  
Long Term Outcome ◽  
Hypothalamic Hamartomas ◽  
Long Term Follow Up ◽  
Emergency Resection

Object Gelastic seizures are epileptic events characterized by bouts of laughter. They are rare and mostly associated with hypothalamic hamartomas (HHs). Status gelasticus, a rare form of status epilepticus, is defined as a prolonged cluster of gelastic seizures (> 20–30 minutes) without necessarily involving loss of awareness between seizures. Emergency resection of the hamartoma is highly effective in these situations and should be considered as early as possible. The authors retrospectively reviewed their surgical cases to document the success, complications, and long-term follow-up after emergency resection of HHs for status gelasticus. Methods The authors report on a retrospective case series from a single tertiary care center. Three patients who presented with status gelasticus underwent emergency resection of HHs. Demographic details, seizure history, medical treatment, and postoperative follow-up data were evaluated. Long-term follow-up (minimum 2 years) data were obtained either from the last clinic visit notes or via telephone and e-mail contacts. The institutional review board at St. Joseph's Hospital approved this study. Results In the last 7 years, of 157 patients who underwent HH resection, the resection was performed on an emergency basis for status gelasticus in 3 cases. At emergency surgery, these 3 patients ranged in age from 9 months to 3.5 years. All of the patients were boys. Delalande and Fohlen Type II, III, and IV lesions were present in the 3 patients. Surgical approaches for resection of HH included an orbitozygomatic, transcallosal anterior interforniceal approach and endoscopic resection. Status gelasticus was terminated following emergency surgery in all cases, and 1 patient was seizure free. Postsurgical complications included, in 1 case, a small right thalamic infarct with mild transient left hemiparesis, which completely resolved within 2 days. Within 2 years of their original surgery, 2 patients underwent further elective surgeries (endoscopic resection and radiosurgery for persistent symptomatic seizures). Follow-up since their most recent surgery ranged from 8 months to 2 years. Two patients were seizure free and 1 patient had greater than 50% reduction in seizures. Conclusions Status gelasticus associated with HHs can be successfully terminated by emergency resection of the HH. Long-term follow-up in the present series suggests good seizure freedom results or at least greater than 50% reduction in seizures, although repeat operations were necessary.

scholarly journals Combined open microsurgical and endoscopic resection of hypothalamic hamartomas

2013 ◽  
Vol 11 (5) ◽  
pp. 491-494 ◽  
Author(s):  
Jonathan Roth ◽  
Marian M. Bercu ◽  
Shlomi Constantini
Keyword(s):  
Third Ventricle ◽  
Endoscopic Approach ◽  
Combined Approach ◽  
Foramen Of Monro ◽  
Transcallosal Approach ◽  
Mammillary Bodies ◽  
The Third ◽  
Hypothalamic Hamartomas ◽  
Interpeduncular Cistern ◽  
The Right

Hypothalamic hamartomas (HHs) are typically located within the vicinity of the third ventricle. They can be attached to the walls of the third ventricle, within the interpeduncular cistern (third ventricle floor), and/or attached to the mammillary bodies and hypothalamus. Depending on their location, resection is performed either through the third ventricle, approaching from above, or via a frontotemporal craniotomy (pterional or frontoorbital), approaching from below. “Above” approaches typically include the transcallosal–anterior interforniceal approach, and recently, purely endoscopic approaches performed transforaminally. The authors present a combined open and endoscopic approach for resection of HHs located within the third ventricle. They used this approach in 2 young girls with relatively small lateral and third ventricles. Following an interhemispheric, transcallosal approach and exposure of the right foramen of Monro, an endoscope was inserted through the foramen, which enabled safe resection of the HH. The main advantage of the combined approach is when the lateral and third ventricles are relatively small, making a purely endoscopic approach more challenging and possibly riskier.

scholarly journals Magnetic resonance imaging–guided laser interstitial thermal therapy for previously treated hypothalamic hamartomas

2016 ◽  
Vol 41 (4) ◽  
pp. E8 ◽  
Author(s):  
Anthony M. Burrows ◽  
W. Richard Marsh ◽  
Gregory Worrell ◽  
David A. Woodrum ◽  
Bruce E. Pollock ◽  
...  
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Minimally Invasive ◽  
Thermal Therapy ◽  
Seizure Freedom ◽  
Resonance Imaging ◽  
Gelastic Seizures ◽  
Laser Interstitial Thermal Therapy ◽  
Hypothalamic Hamartomas

OBJECTIVE Hypothalamic hamartomas (HHs) are associated with gelastic seizures and the development of medically refractory epilepsy. Magnetic resonance imaging–guided laser interstitial thermal therapy (MRg-LITT) is a minimally invasive ablative treatment that may have applicability for these deep-seated lesions. Here, the authors describe 3 patients with refractory HHs who they treated with MRg-LITT. METHODS An institutional review board–approved prospective database of patients undergoing Visualase MRg-LITT was retrospectively reviewed. Demographic and historical medical data, including seizure and medication histories, previous surgeries, procedural details, and surgical complications, along with radiological interpretation of the HHs, were recorded. The primary outcome was seizure freedom, and secondary outcomes included medication reduction, seizure frequency, operative morbidity, and clinical outcome at the latest follow-up. RESULTS All 3 patients in the multi-institutional database had developed gelastic seizures related to HH at the ages of 7, 7, and 9 years. They presented for further treatment at 25, 28, and 48 years of age, after previous treatments with stereotactic radiosurgery in all cases and partial hamartoma resection in one case. One ablation was complicated by a small tract hemorrhage, which was stable on postoperative imaging. One patient developed hyponatremia and experienced weight gain, which were respectively managed with fluid restriction and counseling. At the most recent follow-up at a mean of 21 months (range 1–32 months), one patient was seizure free while another had meaningful seizure reduction. Medication was reduced in one case. CONCLUSIONS Adults with gelastic seizures despite previous treatments can undergo MRg-LITT with reasonable safety and efficacy. This novel therapy may provide a minimally invasive alternative for primary and recurrent HH as the technique is refined.

Autonomic and hormonal ictal changes in gelastic seizures from hypothalamic hamartomas

1998 ◽  
Vol 107 (5) ◽  
pp. 317-322 ◽  
Author(s):  
A Cerullo ◽  
P Tinuper ◽  
F Provini ◽  
M Contin ◽  
A Rosati ◽  
...  
Keyword(s):  
Gelastic Seizures ◽  
Hypothalamic Hamartomas

scholarly journals Utility of diffusion tensor imaging studies linked to neuronavigation and other modalities in repeat hemispherotomy for intractable epilepsy

2016 ◽  
Vol 17 (4) ◽  
pp. 483-490 ◽  
Author(s):  
Erin N. Kiehna ◽  
Elysa Widjaja ◽  
Stephanie Holowka ◽  
O. Carter Snead ◽  
James Drake ◽  
...  
Keyword(s):  
Diffusion Tensor Imaging ◽  
Diffusion Tensor ◽  
Intractable Epilepsy ◽  
Seizure Freedom ◽  
Rasmussen’S Encephalitis ◽  
Initial Procedure ◽  
Surgical Options ◽  
Failed Surgery ◽  
Association Fibers

OBJECT Hemispherectomy for unilateral, medically refractory epilepsy is associated with excellent long-term seizure control. However, for patients with recurrent seizures following disconnection, workup and investigation can be challenging, and surgical options may be limited. Few studies have examined the role of repeat hemispherotomy in these patients. The authors hypothesized that residual fiber connections between the hemispheres could be the underlying cause of recurrent epilepsy in these patients. Diffusion tensor imaging (DTI) was used to test this hypothesis, and to target residual connections at reoperation using neuronavigation. METHODS The authors identified 8 patients with recurrent seizures following hemispherectomy who underwent surgery between 1995 and 2012. Prolonged video electroencephalography recordings documented persistent seizures arising from the affected hemisphere. In all patients, DTI demonstrated residual white matter association fibers connecting the hemispheres. A repeat craniotomy and neuronavigation-guided targeted disconnection of these residual fibers was performed. Engel class was used to determine outcome after surgery at a minimum of 2 years of follow-up. RESULTS Two patients underwent initial hemidecortication and 6 had periinsular hemispherotomy as their first procedures at a median age of 9.7 months. Initial pathologies included hemimegalencephaly (n = 4), multilobar cortical dysplasia (n = 3), and Rasmussen's encephalitis (n = 1). The mean duration of seizure freedom for the group after the initial procedure was 32.5 months (range 6–77 months). In all patients, DTI showed limited but definite residual connections between the 2 hemispheres, primarily across the rostrum/genu of the corpus callosum. The median age at reoperation was 6.8 years (range 1.3–14 years). The average time taken for reoperation was 3 hours (range 1.8–4.3 hours), with a mean blood loss of 150 ml (range 50–250 ml). One patient required a blood transfusion. Five patients are seizure free, and the remaining 3 patients are Engel Class II, with a minimum follow-up of 24 months for the group. CONCLUSIONS Repeat hemispherotomy is an option for consideration in patients with recurrent intractable epilepsy following failed surgery for catastrophic epilepsy. In conjunction with other modalities to establish seizure onset zones, advanced MRI and DTI sequences may be of value in identifying patients with residual connectivity between the affected and unaffected hemispheres. Targeted disconnection of these residual areas of connectivity using neuronavigation may result in improved seizure outcomes, with minimal and acceptable morbidity.

COLLOID CYSTS OF THE THIRD VENTRICLE: A REVIEW OF SIXTEEN CASES

2021 ◽  
pp. 62-64
Author(s):  
Y Srinivas Rao ◽  
Hemal Chheda ◽  
Ch Surendra ◽  
M V Vijayasekhar ◽  
K Satya Varaprasad
Keyword(s):  
Benign Lesion ◽  
Third Ventricle ◽  
Clinical Manifestations ◽  
Colloid Cyst ◽  
Surgical Approaches ◽  
Imaging Features ◽  
Complete Excision ◽  
Advantages And Disadvantages ◽  
The Third ◽  
Colloid Cysts

BACKGROUND : Colloid cysts are one of the rare brain tumours and are mostly located in the anterosuperior portion of the third ventricle, between the fornix and surround of Foramen of Monroe. OBJECTIVES: Ÿ 1.To review the demographic information & analyse clinical manifestations of patients presenting with colloid cyst of third ventricle. Ÿ 2.To analyze the advantages and disadvantages of various surgical approaches Ÿ 3.To assess the surgical outcome in colloid cyst patients operated by any method. MATERIALS AND METHODS: A retrospective study was performed on 16 patients who presented with a colloid cyst and underwent surgery at the Department of Neurosurgery, King George Hospital, Andhra Medical College between 2013-2018. They were evaluated based on clinical ndings and imaging features, surgical approaches used for resection and their outcomes. RESULTS: Sixteen cases of colloid cyst of the third ventricle were operated upon between 2013-2018. There were seven male and nine female patients with their ages varying between 9 and 62 years old. Nine patients were operated on by using a transcortical trans-ventricular approach, four using the anterior trans-callosal approach and, three patients by using an endoscopic approach. In all patients, complete excision of the lesions was achieved. CONCLUSION: Colloid cysts, though benign, present surgical challenges because of its deep midline location. Complete excision of the colloid cyst carries an excellent prognosis. Surgery is a safe and effective treatment option for this benign lesion.

scholarly journals Comparative analysis of anterior third ventricle approaches

2020 ◽  
pp. 544-549
Author(s):  
Deepak Kumar Singh ◽  
Kuldeep Yadav ◽  
Rakesh Kumar ◽  
Arun Kumar Singh ◽  
Vipin Kumar Chand
Keyword(s):  
Third Ventricle ◽  
Medical Science ◽  
Optic Chiasm ◽  
Visual Disturbance ◽  
Colloid Cyst ◽  
Subtotal Resection ◽  
Common Symptom ◽  
Anatomical Landmarks ◽  
Transcallosal Approach ◽  
Transforaminal Approach

Background. Third ventricle tumors are uncommon and account for only 0.6 - 0.9% of all the brain tumors7. In 1921, Dandy was the first neurosurgeon who successfully removed a colloid cyst from the third ventricle through a posterior transcallosal approach. Despite their unfavourable locations, these tumours can be removed successfully by proper knowledge of anatomical landmarks and by choosing the appropriate approach. Methods. We performed a retrospective analysis of all patients (17 patients) who underwent surgery for anterior third ventricular masses between March 2018 to March 2020 in the Dr Ram Manohar Lohia Institute of Medical Science Lucknow, Uttar Pradesh. Results: The most common symptom in our cases was headache, which was present in all (100%) patients, nausea/vomiting in 7 (41%), history of recurrent episodes of drop attacks in 4 (23%), h/o seizure in 2 (11.7%), visual disturbance in 1 (5.4%), memory disturbance in 1 (5.4%) and urinary incontinence in 1 (5.4%) patient. 6 patients were operated with transcallosal-transforaminal approach, 1 patient was operated with transcallosal interforniceal approach, 3 patients were operated with transcortical-transforaminal approach, 1 patient was operated with subfrontal translamina terminalis approach, 1 patient was operated with transcallosal-transchoroidal approach, 5 patients were operated with endoscopically. Gross total excision was achieved in 15 (88%) patients while in 2 (11.7%) patients subtotal resection was done due to their adherence to choroid plexus and optic chiasm. The most common post-operative complication was endocrine dysfunction in the form of diabetes insipidus. Conclusions. Anterior Third ventricular tumours are mostly benign and best treatment modality is surgical resection. When we analyzed the results of various approaches, we found that despite their unfavourable location, the results were satisfactory for different tumours of different location in the anterior third ventricle, when treated with the carefully planned microsurgical or endoscopic approach with proper knowledge of anatomical landmarks.

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