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Noritsugu Kunihiro, Takeo Goto, Kenichi Ishibashi, and Kenji Ohata


Retrochiasmatic craniopharyngiomas are surgically challenging tumors. Retrochiasmatic craniopharyngiomas with complicated conditions such as large diameter, major calcification, or significant extension to the third ventricle or posterior fossa present surgical challenges; moreover, recurrent retrochiasmatic craniopharyngiomas are particularly formidable challenges. Although the transpetrosal approach to retrochiasmatic craniopharyngiomas published by Hakuba in 1985 can provide unique advantageous exposure of the retrochiasmatic area to allow safe neurovascular dissection and facilitate radical tumor removal, the procedure is viewed as complicated and time consuming and has a high risk of damaging hearing functions. The authors have modified Hakuba's technique to minimize petrosectomy and reduce surgical complications and have applied this modified approach to retrochiasmatic craniopharyngiomas with complicated conditions. In this study, the authors describe their technique and surgical outcomes to elucidate the role of this modified transpetrosal approach for retrochiasmatic craniopharyngiomas with complicated conditions. This is the first study to report surgical outcomes of the transpetrosal approach for retrochiasmatic craniopharyngiomas.


Between 1999 and 2011, the minimum anterior and posterior combined (MAPC) transpetrosal approach, which is a modification of Hakuba's transpetrosal approach, was applied in 16 cases of retrochiasmatic craniopharyngiomas with complicated conditions. Eight cases were recurrent tumors, 4 had previously received radiotherapy, 11 had a large diameter, 10 had large calcification, 15 had superior extension of the tumor into the third ventricle, and 10 had a posterior extension of the tumor that compressed the midbrain and pons. In all 16 patients, more than 2 of these complicated conditions were present. The follow-up duration ranged from 0.8 to 12.5 years (mean 5.3 years). Surgical outcomes assessed were the extent of resection, surgical complications, visual function, endocrinological status, and neuropsychological function. Five-year and 10-year recurrence-free survival rates were also calculated.


Gross-total or near-total resection was achieved in 15 cases (93.8%). Facial nerve function was completely maintained in all 16 patients. Serviceable hearing was preserved in 15 cases (93.8%). Visual function improved in 13 out of 14 cases (92.9%) that had visual disturbance before surgery. None of the patients experienced deterioration of their visual function. Twelve cases had endocrinological deficit and received hormonal replacement before surgery. New endocrinological deficit occurred in 2 cases (12.5%). Neuropsychological function was maintained in 14 cases (87.5%) and improved in 1 case (6.3%). One case that had received previous conventional radiotherapy treatment showed a gradual decline in neuropsychological function. The 5-year and 10-year recurrence-free survival rates were both 86.5%.


The authors obtained good results by using the MAPC transpetrosal approach for the removal of retrochiasmatic craniopharyngiomas with complicated conditions. The MAPC transpetrosal approach should be considered as a therapeutic option for selected cases of retrochiasmatic craniopharyngiomas with complicated conditions.

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Saya Koh, Takehiro Uda, Noritsugu Kunihiro, Ichiro Kuki, Takeshi Inoue, Toshiyuki Kawashima, Hiroshi Uda, Ryoko Umaba, Kosuke Nakajo, Yoko Nakanishi, Satoru Sakuma, Toshiyuki Seto, Shin Okazaki, Hisashi Kawawaki, and Takeo Goto


Surgery is a treatment option for medically intractable epileptic spasms (ESs). However, outcomes of ES after surgery are not well understood, especially when surgeries aimed at seizure palliation are included. The purpose of the present study was to 1) investigate the proportion of favorable postoperative ES outcomes, 2) explore the preoperative factors related to favorable postoperative ES outcomes, and 3) examine the timing of ES recurrence after disconnection surgeries, including both curative and palliative indications.


This retrospective study included patients who underwent disconnection surgery for medically intractable ES at the authors’ institution between May 2015 and April 2021. Patients with suggested focal-onset ES based on preoperative evaluations initially underwent lobar disconnection. Patients with suggested generalized or unknown-onset ES underwent corpus callosotomy (CC). If evaluations after initial CC showed focalized or lateralized change, they were considered secondarily revealed focal-onset ES, and lobar disconnection was performed. ES outcomes were evaluated using the International League Against Epilepsy classification. ES outcomes were divided into classes 1–4 as favorable outcomes and classes 5 and 6 as unfavorable outcomes. The relationship between the favorable postoperative ES outcomes and the following preoperative factors was analyzed: sex, age at onset (< or > 1 year), duration between seizure onset and initial surgery (< or > 2 years), type of seizure at onset (ES or others), presence of other types of seizures, substrate, hypsarrhythmia, and MRI abnormalities. The period between the last surgery and ES recurrence was also analyzed.


A total of 41 patients were included, of whom 75.6% achieved favorable ES outcomes. A longer seizure duration between seizure onset and initial surgery, presence of hypsarrhythmia, and positive MRI findings led to poorer postoperative ES outcomes (p = 0.0028, p = 0.0041, and p = 0.0241, respectively). A total of 60.9% of patients had ES recurrence during the follow-up period, and their ES recurred within 13 months after the last surgery.


Disconnection surgery is an effective treatment option for medically intractable ES, even when the preoperative evaluation suggests a generalized or unknown onset.

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Takehiro Uda, Ichiro Kuki, Takeshi Inoue, Noritsugu Kunihiro, Hiroharu Suzuki, Hiroshi Uda, Toshiyuki Kawashima, Kosuke Nakajo, Yoko Nakanishi, Shinsuke Maruyama, Takashi Shibata, Hiroshi Ogawa, Shin Okazaki, Hisashi Kawawaki, Kenji Ohata, Takeo Goto, and Hiroshi Otsubo


Epileptic spasms (ESs) are classified as focal, generalized, or unknown onset ESs. The classification of ESs and surgery in patients without lesions apparent on MRI is challenging. Total corpus callosotomy (TCC) is a surgical option for diagnosis of the lateralization and possible treatment for ESs. This study investigated phase-amplitude coupling (PAC) of fast activity modulated by slow waves on scalp electroencephalography (EEG) to evaluate the strength of the modulation index (MI) before and after disconnection surgery in children with intractable nonlesional ESs. The authors hypothesize that a decreased MI due to surgery correlates with good seizure outcomes.


The authors studied 10 children with ESs without lesions on MRI who underwent disconnection surgeries. Scalp EEG was obtained before and after surgery. The authors collected 20 epochs of 3 minutes each during non–rapid eye movement sleep. The MI of the gamma (30–70 Hz) amplitude and delta (0.5–4 Hz) phase was obtained in each electrode. MIs for each electrode were averaged in 4 brain areas (left/right, anterior/posterior quadrants) and evaluated to determine the correlation with seizure outcomes.


The median age at first surgery was 2.3 years (range 10 months–9.1 years). Two patients with focal onset ESs underwent anterior quadrant disconnection (AQD). TCC alone was performed in 5 patients with generalized or unknown onset ESs. Two patients achieved seizure freedom. Three patients had residual generalized onset ESs. Disconnection surgeries in addition to TCC consisted of TCC + posterior quadrant disconnection (PQD) (1 patient); TCC + AQD + PQD (1 patient); and TCC + AQD + hemispherotomy (1 patient). Seven patients became seizure free with a mean follow-up period of 28 months (range 5–54 months). After TCC, MIs in 4 quadrants were significantly lower in the 2 seizure-free patients than in the 6 patients with residual ESs (p < 0.001). After all 15 disconnection surgeries in 10 patients, MIs in the 13 target quadrants for each disconnection surgery that resulted in freedom from seizures were significantly lower than in the 26 target quadrants in patients with residual ESs (p < 0.001).


In children with nonlesional ESs, PAC for scalp EEG before and after disconnection surgery may be a surrogate marker for control of ESs. The MI may indicate epileptogenic neuronal modulation of the interhemispheric corpus callosum and intrahemispheric subcortical network for ESs. TCC may be a therapeutic option to disconnect the interhemispheric modulation of epileptic networks.