Resection Of Hypothalamic Hamartomas Research Articles

Full-Endoscopic Surgery for Hypothalamic Hamartoma Resection.

Hypothalamic hamartomas (HH) are rare developmental anomalies of the inferior hypothalamus that often cause refractory epilepsy, including gelastic seizures. Surgical resection is an effective method to treat drug-resistant epilepsy and endocrinopathy in a suitable patient group. Open surgery, endoscopic surgery, ablative procedures, and stereotactic radiosurgery can be utilized. In this study, we aimed to describe the full-endoscopic approach for HH resection. The technique involves the use of an intraoperative ultrasonography (USG) system, a 30° rigid endoscope system that has an outside diameter of 2.7 mm with two working channels, a stylet that has an outer diameter of 3.8 mm, a monopolar coagulation electrode, a fiberoptic light guide, and the endovision system. Microforceps and monopolar electrocautery are the two main surgical instruments for HH removal. The protocol is easy to apply after a particular learning curve has been passed and shorter than open surgical approaches. It leads to less blood loss. Full-endoscopic surgery for HH is a minimally invasive technique that can be applied safely and effectively with good seizure and endocrinological outcomes. It provides low surgical site pain and early mobilization.

Hypothalamic hamartoma surgery in a setting with limited resources

BackgroundThree years ago (in 2020), we at the epilepsy center in Shiraz, Iran, started an endeavor to initiate a surgical program for patients with hypothalamic hamartoma (HH). We discussed that although minimally invasive techniques are desired, they are not available in the nation. We decided to proceed with open disconnection and resection surgery techniques. The current manuscript presents the results of the HH surgery program at our center as a case series. MethodsThis study included all patients with a diagnosis of HH who were referred to Shiraz Epilepsy Center with drug-resistant epilepsy and who underwent HH surgery from October 2020 to January 2023 at our epilepsy center, Namazi Hospital, Shiraz, Iran. ResultsSeven patients were included. All patients had gelastic seizures. Four patients (57%) underwent total resection of HH, and the lesions were disconnected and partially resected in three other patients (43%). Three patients (43%) became seizure-free after surgery, and three patients (43%) had more than 50% reduction in their seizure frequencies. Three patients (43%) had no post-operative complications. Only one patient (14.3%) suffered from a permanent postoperative complication (right hemiparesis). The mortality rate was zero. Five parents (71%) were satisfied with the surgery outcomes. ConclusionHypothalamic hamartoma surgery is feasible even in centers with limited resources if a close collaboration exists between the epileptology and neurosurgery teams. Careful planning based on the expertise of the team members and the available resources is required to foster success.

Minimally invasive procedures for hypothalamic hamartoma-related epilepsy: a systematic review and meta-analysis.

Hypothalamic hamartoma (HH) is a rare, nonmalignant, heterotopic developmental malformation that consists of a mixture of normal neurons and glial cells. Resection of HHs has been associated with high rates of mortality and morbidity. Therefore, minimally invasive ablation methods could be the best treatment option for HH. The most frequently used minimally invasive options for HH ablation are radiofrequency thermocoagulation (RFT), laser ablation (LA), and stereotactic radiosurgery. To investigate three minimally invasive procedures in the treatment of refractory seizures related to HH, the authors conducted a systematic search in March 2022 in the MEDLINE, Embase, Scopus, and Web of Science databases in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Seizure freedom was the primary outcome of interest. The authors defined seizure freedom as Engel class I or International League Against Epilepsy class 1 or 2 or as the reported term "seizure freedom." The secondary outcome was long-term complications reported in studies. Both random- and fixed-effects models were used to calculate the pooled proportion of seizure freedom and complication rate with 95% confidence intervals. A modified version of the Joanna Briggs Institute (JBI) Critical Appraisal to assess the risk of bias was used. The authors included 15 studies with 422 patients (RFT, n = 190; LA, n = 171; and Gamma Knife Radiosurgery [GKRS], n = 61). Generally, the mean incidences of overall seizure freedom after minimally invasive procedures were 77% (95% CI 0.74-0.81) and 68% (95% CI 0.57-0.79) using fixed- and random-effects models, respectively. The mean incidence of overall seizure freedom after RFT was 69% (95% CI 0.63-0.75), and the mean incidences of overall seizure freedom after LA and GKRS were 87% (95% CI 0.82-0.92) and 44% (95% CI 0.32-0.57), respectively. The total complication rate with minimally invasive procedures was 13% (95% CI 0.01-0.26). The complication rate in each treatment was as follows: 5% (95% CI 0.0-0.12) for RFT, 20% (95% CI 0.0-0.47) for LA, and 22% (95% CI 0-0.65) for GKRS. Meta-regression analysis showed an association between older age and higher complication rates in the LA group. In this meta-analysis, LA showed superiority in seizure freedom over the other two methods. The complication rate associated with RFT was less than those in the other two methods; however, this difference was not statistically significant.

Stereotactic radiofrequency thermocoagulation and resective surgery for patients with hypothalamic hamartoma.

In this study, the authors compared the efficacy and safety of stereotactic radiofrequency thermocoagulation (SRT) and resective surgery (RS) for patients with hypothalamic hamartoma (HH). The authors included all patients with HHs who were treated by SRT or hamartoma resection. Seizure outcomes were assessed by blinded observers according to the International League Against Epilepsy (ILAE) classification. Favorable seizure outcomes were defined as ILAE classes 1 and 2, and unfavorable seizure outcomes were defined as ILAE classes 3-6. Twenty-nine patients who underwent SRT or RS met the inclusion criteria; 3 were excluded because they had completed less than 12 months of follow-up. Most of the patients (20 of 26; 76.9%) had small HHs (i.e., maximum HH diameter less than 20 mm). The patients' follow-up time ranged from 12 to 66 months (median 60 months). At the last follow-up, favorable outcomes were observed in 9 patients (69.2%) who had undergone SRT and 10 patients (76.9%) who had undergone HH resection. No significant difference was found in seizure outcomes between SRT and RS recipients. Patients with giant HHs were more likely than patients with smaller tumors to undergo multiple resections (p = 0.043, univariate logistic regression; significant). However, no significant difference was found between SRT and RS recipients in terms of the number of procedures per patient. SRT recipients had fewer and less-severe adverse events than RS recipients. For patients with small HHs, SRT provides similar seizure outcomes to RS with a less invasive procedure. Patients who underwent SRT experienced fewer and lighter adverse effects than patients who had RS. Patients with giant HHs were more likely to undergo multiple HH resections.

Endoscopic transsphenoidal resection of hypothalamic hamartoma

This abstract discusses two clinical cases of hypothalamic hamartoma (HH) which have not yet been described in any Russian or international papers, and presents the details of endoscopic resection technique with a transsphenoidal approach. Patient R., 16 years old male with a 9-year history, epileptic seizures every 5–6 days. On MRI- 1.5 × 1.4 × 1.4 cm HH and FCD of the right occipital lobe. Three seizures were detected during video-EEG monitoring. Patient M., 23 years old female with a 16-year history of illness, epileptic seizures once in a week. On MRI - 2.44 × 2.79 × 2.68 cm HH. Two seizures were detected during video-EEG. In both cases surgical excision by transsphenoidal endoscopic approach was performed. First patient was discharged in satisfactory condition on the 7th day after surgery, and didn't have neurological deficit, electrolyte disorders (hypo- or hypernatremia, polyuria, polydipsia), visual disorders (hemianopsia or reduced visual acuity). Second patient in the early postoperative period had electrolyte disorders (hyponatremia), which were reversed after using hypo-osmolar solutions and mineralocorticoids. There were no visual impairments (hemianopia or visual acuity reduction). The patient was discharged in satisfactory condition on the 7th day after the surgery. In 18 months after surgery both patients are seizure free (Engel I). Clinical observation data suggest that transsphenoidal resection of HH in patients with drug-resistant epilepsy is possible. Our experience shows that refractory epilepsy associated with HH can be safely and effectively treated with transsphenoidal resection of HH, with the possibility of radical cure and disease control.

Correction to: Open resection of hypothalamic hamartomas for intractable epilepsy revisited, using intraoperative MRI

The authors apologize to have sent a final manuscript draft omitting "Athanasius Chawira" from the list of authors. The correct list of authors is given in this article.The original article has been corrected.

Pure endoscopic management of epileptogenic hypothalamic hamartomas.

Hypothalamic hamartomas (HH) are rare congenital malformations located in the region of the tuber cinereum and third ventricle. Their usual clinical presentation is characterized by gelastic/dacrystic seizures which often become pharmaco-resistant and progress to secondary focal/generalized intractable epilepsy causing mostly in children cognitive and behavioral problems (particularly in cases of progressive epileptic encephalopathy) and precocious puberty. Whereas gelastic seizures can be surgically controlled either by resection of the lesion or disconnection (tissue-destructive) procedures, aimed at functionally prevent the spreading of the epileptic burst; generalized seizures tend to respond better to HH excision rather than isolated neocortical resections, which generally fail to control them. Prospective analysis of 14 consecutive patients harboring HH treated in an 8-year period; 12 patients had unilateral and two bilateral HH. All patients were managed by pure endoscopic excision of the HH. The mean operative time was 48min and mean hospital stay was 2days; perioperative blood loss was negligible in all cases. Two patients showed a transient diabetes insipidus (DI); no transient or permanent postoperative neurological deficit or memory impairment was recorded. Complete HH excision was achieved in 10/14 patients. At a mean follow-up of 48months, no wound infection, meningitis, postoperative hydrocephalus, and/or mortality were recorded in this series of patients. Eight patients became seizure free (Engel class I), 2 other experienced worthwhile improvement of disabling seizures (Engel class II); 2 patients were cured from gelastic attacks while still experiencing focal dyscognitive seizures; and 2, having bilateral HH (both undergoing unilateral HH excision), did not experience significant improvement and required later on a temporal lobectomy coupled to amygdalohyppocampectomy. Overall, the followings resulted to be predictive factors for better outcomes in terms of seizure control: (1) cases of unilateral, Delalande class B, HH, (2) shorter history of epilepsy. Endoscopic resection of HH proved, in our series, to be effective in achieving complete control or in reducing the frequency of seizures. Furthermore, this approach has confirmed its minimally invasive nature with a very low morbidity rate: of note, it allowed to better preserve short-term memory and hypothalamic function.

Technical aspects of pediatric epilepsy surgery: Report of a multicenter, multinational web-based survey by the ILAE Task Force on Pediatric Epilepsy Surgery.

Surgical techniques may vary extensively between centers. We report on a web-based survey aimed at evaluating the current technical approaches in different centers around the world performing epilepsy surgery in children. The intention of the survey was to establish technical standards. A request was made to 88 centers to complete a web-based survey comprising 51 questions. There were 14 questions related to general issues, 13 questions investigating the different technical aspects for children undergoing epilepsy surgery, and 24 questions investigating surgical strategies in pediatric epilepsy surgery. Fifty-two centers covering a wide geographic representation completed the questionnaire. The median number of resective procedures per center per year was 47. Some important technical practices appeared (>80% of the responses) such as the use of prophylactic antibiotics (98%), the use of high-speed drills for bone opening (88%), nonresorbable material for bone flap closure (85%), head fixation (90%), use of the surgical microscope (100%), and of free bone flaps. Other questions, such as the use of drains, electrocorticography (ECoG) and preoperative withdrawal of valproate, led to mixed, inconclusive results. Complications were noted in 3.8% of the patients submitted to cortical resection, 9.9% hemispheric surgery, 5% callosotomy, 1.8% depth electrode implantation, 5.9% subdural grids implantation, 11.9% hypothalamic hamartoma resection, 0.9% vagus nerve stimulation (VNS), and 0.5% deep brain stimulation. There were no major differences across regions or countries in any of the subitems above. The present data offer the first overview of the technical aspects of pediatric epilepsy surgery worldwide. Surprisingly, there seem to be more similarities than differences. That aside many of the evaluated issues should be examined by adequately designed multicenter randomized controlled trials (RCTs). Further knowledge on these technical issues might lead to increased standardization and lower costs in the future, as well as definitive practice guidelines.

Nursing cooperation in the operation of hypothalamic hamartoma resection through transcallosal interforniceal approach

Objective To summarize the experience of nursing cooperation during the operation of hypothalamic hamartoma resection through transcallosal interforniceal approach , in order to improve quality of cooperation in the operation and reduce the incidence of surgery-related complications .Methods The situation of nursing cooperation in four patients undergoing hypothalamic hamartoma resection through transcallosal interforniceal approach was analyzed retrospectively , including preoperative preparation , body position management , comprehensive heat preservation measures , collaboration in operation and others .Results Four patients obtained the good curative effect after the surgical treatment , whose symptoms were relieved , without postoperative intracranial infection .There were total removal of three patients and partial removal of one patient because of the special position of tumor .Conclusions Sufficient preoperative preparation , preoperative psychological care and intervention , correct body position , reasonable application of comprehensive heat preservation measures in the operation and proficient operation cooperation are key factors to ensure the success of operation. Key words: Hamartoma; Transcallosal interforniceal approach; Microsurgery; Nursing cooperation in the operation

Clinical characteristics and long-term outcome of surgery for hypothalamic hamartoma in children with refractory epilepsy.

Context:Hypothalamic hamartomas (HH) are ectopic masses of neuronal and glial tissue most commonly presenting with medically refractory gelastic seizures with evolution to other seizure types. They are also associated with cognitive and behavioral problems to varying extent. Surgery has been found to improve quality of life in more than 50% of patients.Aim:To evaluate the clinical characteristics and long-term outcome of surgery in children with HH and refractory epilepsy.Materials and Methods:A retrospective analysis of presurgical, surgical, and postsurgical data of six children who underwent surgery for HH and had at least 3 years follow-up was performed.Results:Six children (male: female = 5:1) aged 3-16 years (at the time of surgery) underwent surgical resection of HH for refractory epilepsy. At last follow-up (range 3-9 years), three children were in Engel's class I, two in Class II, and one in class III outcome. Significant improvement in behavior, quality of life was noted in four children; while the change in intelligence quotient (IQ) was marginal.Conclusions:Medically refractory epilepsy associated with behavioral and cognitive dysfunction is the most common presentation of HH. Open surgical resection is safe with favorable outcome of epilepsy in 50% with significant improvement in behavior and marginal change in cognitive functions.

Asymptomatic hydrocephalus and subdural hygroma after successful resection of hypothalamic hamartoma with central precocious puberty

Asymptomatic hydrocephalus and subdural hygroma after successful resection of hypothalamic hamartoma with central precocious puberty

Combined open microsurgical and endoscopic resection of hypothalamic hamartomas

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.

Transcallosal endoscopic resection of hypothalamic hamartoma in a case with Pallister‐Hall syndrome

Pallister-Hall syndrome (PHS) is a very rare syndrome characterized by hypothalamic hamartoma (HH), polydactyly, panhypopituitarism, imperforate anus and other visceral anomalies. Contrary to patients with isolated HH, neurological dysfunction and precocious puberty are uncommon and seizures are usually well controlled with anticonvulsant medication. Therefore, conservative management of HH is advised. To the best of our knowledge, seven cases of PHS with surgical resection of the HH have so far been reported. Five patients were either seizure-free or had >90% seizure reduction postoperatively. Here, we present a case of PHS of a patient who also underwent transcallosal endoscopic resection of the HH with a subsequent 70% reduction in seizure frequency.

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

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. 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. 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. 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.

Syndrome of alternating hypernatremia and hyponatremia after hypothalamic hamartoma surgery

In this paper, the authors' goal was to describe the occurrence of alternating hypernatremia and hyponatremia in pediatric patients who underwent resection of hypothalamic hamartomas (HHs) for epilepsy. Hypernatremia in patients after pituitary or hypothalamic surgery can be caused by diabetes insipidus (DI), whereas hyponatremia can occur due to a syndrome of inappropriate antidiuretic hormone, cerebral salt wasting, or excessive administration of desmopressin (DDAVP). The triphasic response after surgery in the pituitary region can also explain variations in sodium parameters in such cases. One hundred fifty-three patients with HH who underwent surgery were enrolled in a prospective study to monitor outcomes. Of these, 4 patients (2.6%) were noted to experience dramatic alterations in serum sodium values. The medical records of these patients were identified and evaluated. Patients' ages at surgery ranged from 1.2 to 6.0 years. All patients were girls. Two patients had Delalande Type IV lesions (of 16 total Type IV lesions surgically treated) and 2 had Type III lesions (of 39 total Type III lesions). All patients had a history of gelastic seizures refractory to medication. Seizure frequency ranged from 3 to 300 per day. After surgery, all patients experienced hypernatremia and hyponatremia. The largest fluctuation in serum sodium concentration during hospitalization in a single patient was 53 mEq/L (range 123-176 mEq/L). The mean absolute difference in maximum and minimum sodium values was 38.2 mEq/L. All patients exhibited an initial period of immediate DI (independent of treatment) after surgery followed by a period of hyponatremia (independent of treatment), with a minimum value occurring between postoperative Days 5 and 8. All patients then returned to a hypernatremic state of DI, and 3 patients still require DDAVP for DI management. A second occurrence of hyponatremia lasting several days without DDAVP administration occurred in 2 patients during their hospitalization between periods of hypernatremia. One patient stabilized in the normal range of sodium values prior to discharge from rehabilitation without the need for further intervention. At last follow-up, 3 patients are seizure-free. Severe instability of sodium homeostasis with hypernatremia and hyponatremia is seen in up to 2.6% of children undergoing open resection of HH. This risk appears to be related to HH type, with a higher risk for Types III (2 [5.1%] of 39) and IV (2 [12.5%] of 16) lesions. Here, the authors describe alternating episodes of hypernatremia and hyponatremia in the postoperative period following HH surgery. Management of this entity requires careful serial assessment of volume status and urine concentration and will often require alternating salt replacement therapy with DDAVP administration.

Predictors of inpatient complications and outcomes following surgical resection of hypothalamic hamartomas

Background:Our aim was to identify the preoperative factors associated with a greater risk of poor inpatient outcomes in those undergoing resection of hypothalamic hamartomas.Methods:We performed a multi-institutional retrospective cohort analysis via the Nationwide Inpatient Sample (1998 – 2007). Patients of any age who underwent resection of hypothalamic hamartomas were identified by ICD-9 coding. The primary outcomes included inpatient complications, length of stay (LOS), and total charges. Multivariate regression models were constructed to analyze the outcomes.Results:Two hundred and eighty-two patients were identified with a mean age of 27.7 years, with most being male (53.2%), Caucasian (78.9%), privately insured (69.3%), and treated electively (74.7%) at academic centers (91.7%). A majority (82.2%) had Elixhauser comorbidity scores of < 1, indicating few comorbidities. No inpatient deaths were reported. Mean LOS was 7.39 days and the mean total hospital charges were $53,935. Overall, 19.5% developed an inpatient complication, primarily stroke (16.7%). Female gender, ethnic / racial minorities, higher comorbidity scores, private insurance, and non-academic hospitals were associated with greater LOS and total charges. Private insurance (Odds Ratio, OR: 1.59, P = 0.045) and academic hospitals (OR: 1.43, P = 0.008) were associated with significantly higher odds of any complication. Minority race / ethnicity was associated with a minimal increase in the odds of postoperative stroke (OR: 1.02, P < 0.001) relative to Caucasians.ConclusionsThrough an analysis of a 10-year multi-institutional database, we have described the surgical outcomes of patients undergoing resection of hypothalamic hamartomas. Results demonstrate significant inpatient morbidity, particularly postoperative stroke. Patient- and institution-level factors should be considered in determining the perioperative risk for such patients.

Surgical resection of hypothalamic hamartomas for severe behavioral symptoms

Hypothalamic hamartomas (HHs) are associated with treatment-resistant epilepsy. Many patients also experience severe and sometimes disabling psychiatric problems. The most common behavioral symptoms consist of paroxysms of uncontrolled anger related to poor frustration tolerance. These can include violence, resulting in disrupted family or school relationships, and legal consequences including incarceration. In a large cohort of patients undergoing surgical resection of HHs for refractory epilepsy, 88% of families described an improvement in overall behavioral functioning [1]. Here, we describe four patients (three males, mean age=11.9years) who underwent surgical resection of HHs largely for behavioral indications. Three patients had relatively well controlled seizures, and one had no history of epilepsy. All patients had striking improvement in their psychiatric comorbidity. HH resection can result in significant improvement in behavioral functioning, even in patients with relatively infrequent seizures. Further investigation under approved human research protocols is warranted.

Hypothalamic hamartoma in adults

Objective To study the clinical features of hypothalamic hamartoma (HH) in adults. Methods A retrospective review of 27 cases of hypothalamic hamartoma in adults was performed, which account for 12.62% in all 214 hypothalamic hamartoma patients diagnosed in Beijing Tiantan Hospital from 1994 to 2008. Results The mean age of the patients diagnosed as hypothalamic hamartoma was 29.22 years old ranged from 18 to 66 years old, the rate of male to female was 2∶1. Eight patients (29.63%) were asymptomatic and 19 patients (70.37%) were symptomatic. The onset of symptom developed before adult life were found in 14 patients (73.68%), the other 5 patients (26.32%) developed a milder epileptic seizures in adult life which ranged from 19 to 51 years old. Most patients presented grand mal epilepsy. Hypothalamic hamartoma was diagnosed by the combination of medical history with CT and MRI which presented space occupying lesion at interpeduncular cistern or in the third ventricle. The first symptom presented in 19 cases of symptomatic patients were as follow: precocious puberty in 3 cases (15.79% ), gelastic seizure in 9 patients (47.37%), absence seizure in 2 patients (10.52%) and generalized tonic⁃clonic seizure in 5 patients (26.32%). Two patients combined with mental retardation. Among the 9 patients with gelastic seizure, 2 patients had generalized tonic⁃clonic seizure at the time of onset of gelastic seizure, 6 patients presented with generalized tonic⁃clonic seizure at the mean time of 10.70 years (ranged from 6 months to 21 years) after the onset of gelastic seizure, one patient presented with gelastic seizure only. All the patients performed CT and MRI, and the time of follow⁃up was 14 months to 9 years and 10 months. Six patients underwent resection of hypothalamic hamartoma through pterional approach or callosal⁃septum pellucidum⁃interforniceal approach. Three cases were cured (Engel class Ⅰ). Seizures were almost free in 2 cases (Engel classⅡ). Seizures were not changed in one case (Engel class Ⅴ). Two patients underwent γ⁃knife surgery: one case showed no change at seizure onset and another one showed reduction of seizure frequency (Engel class Ⅳ). Antiepileptic drugs were used in 9 patients, but no effect was seen on gelastic seizure. Conclusion Adults with hypothalamic hamartoma usually present with mild epilepsy and seldom occur intellectual disturbance. Treatment should be given with caution. DOI：10.3969/j.issn.1672-6731.2010.03.019

Resumption of normal onset of puberty after resection of hypothalamic hamartoma

Objective To study the effect of the resection of hypothalamic hamartoma ( HH) on the normal start of puberty. Method Three girls whose HHs were totally removed were followed - up for long term to study the onset of spontaneous menses, general health and the development of intelligence. Results The precocious puberty was arrested immediately after the resection of HH in three girls and the start of spontaneous menses were begun in the 8. 0th,8.3th and 7.5th years postsurgery with regular menses. The height stature,body weight and intelligence development were all normal. Conclusions The hypothalamic hamartoma may be a ectopic second hypothalamus and the resection of HH may have no effects on the normal start of puberty. Key words: Hypothalamus; Hamartoma; Postsurgery; Resumption onset of puberty

Long-term cognitive outcome after transcallosal resection of hypothalamic hamartoma in older adolescents and adults with gelastic seizures

Hypothalamic hamartomas (HHs) are typically associated with pharmacologically intractable gelastic seizures, which often develop into complex partial and/or generalized tonic–clonic seizures. The transcallosal, anterior interforniceal (TAIF) approach to HH resection has been shown to be safe and to result in good seizure frequency reduction. We documented postsurgical cognitive functioning and examined whether using the TAIF approach with older adolescents and adults affected long-term cognitive outcome. Six older adolescents and adults, who had undergone resection of an HH using the TAIF approach, underwent comprehensive neuropsychological assessment at least 2 years postoperatively. Four of these individuals also had neuropsychological evaluation preoperatively. All participants who were assessed pre- and postoperatively demonstrated long-term improvement in perceptual/visuospatial function after surgery. Three of the four participants also demonstrated lasting reduction in memory function postoperatively. The TAIF approach to HH resection is associated with a high risk of long-term memory impairment in older adolescents and adults.