Sukwoo Hong, Daisuke Hirokawa, Kenichi Usami and Hideki Ogiwara
The goal of this study was to clarify the long-term outcome of endoscopic third ventriculostomy (ETV) in pediatric hydrocephalus in light of the ETV Success Score (ETVSS), shunt dependency, and intellectual development.
The authors retrospectively analyzed pediatric patients with hydrocephalus who underwent ETV between 2002 and 2012 and who were followed for longer than 5 years as a single-center cohort. The data of the patients’ pre- and postoperative status were collected. The relationships between ETVSS and the full-scale IQ as well as shunt dependency were analyzed. The usefulness of ETVSS for repeat ETV and the change of radiological parameters of ventricle size before and after ETV were also analyzed. The success of ETV was defined as no requirement for further CSF diversion procedures.
Fifty ETVs were performed in 40 patients. The average ETVSS was 61 and the success rate at 6 months was 64%. The mean follow-up was 9.9 years (5.2–15.3 years), and the long-term success rate of ETV was 50%. The Kaplan-Meier survival curve continued to show a statistically significant difference among patients with a low, moderate, and high ETVSS, even after 6 months (p = 0.002). After 15 months from the initial ETV, no patients required additional CSF diversion surgery. There was no statistical significance between ETVSS and the long-term full-scale IQ or shunt dependency (p = 0.34 and 0.12, respectively). The radiological improvement in ventricle size was not associated with better future educational outcome.
The ETVSS was correlated with the long-term success rate. After 15 months from the initial ETV, no patients required an additional CSF diversion procedure. The ETVSS was not considered to be correlated with long-term intellectual status.
Hideki Ogiwara, Nobuhito Morota, Masahiro Joko and Kengo Hirota
The endoscopic treatment of a suprasellar arachnoid cyst (SAC) consists mainly of ventriculocystostomy (VC) and ventriculocystocisternostomy (VCC). However, the most appropriate treatment for these cysts has not been fully determined yet. The authors report on the effectiveness of endoscopic VC for SACs communicating with the basal cisterns, which is demonstrated by preoperative CT cisternography.
The authors retrospectively analyzed the surgical results of patients with an SAC treated using endoscopic fenestration. The patient charts were reviewed for demographic data, clinical presentations, surgical therapies, and clinical outcomes.
Six consecutive patients with SAC were surgically treated using endoscopic fenestration (VC or VCC) between March 2004 and February 2011. The mean age was 18.5 months (range 5 days to 37 months). Five patients were previously untreated, and 1 patient had previously undergone ventriculoperitoneal shunt placement. Five patients underwent preoperative CT cisternography, and communication between the SAC and the basal cisterns was demonstrated in 3 patients, very slight communication in 1, and no communication in 1. Four patients, including the 3 with communication between the SAC and basal cisterns, underwent VC. Two patients with very slight or no communication underwent VCC. In all patients the SAC decreased in size and hydrocephalus improved postoperatively. Five patients (3 treated with VC and 2 with VCC [83%]) have been stable without reoperation (mean follow-up 32.7 months). All 3 patients with an SAC communicating with the basal cisterns have been stable without reoperation following VC.
Preoperative cisternography may be useful for selecting the optimal endoscopic treatment method for SAC. If an SAC communicates with the basal cisterns, a VC could be an effective, safe, and simpler treatment option.
Elizabeth C. Tyler-Kabara
Nobuhito Morota, Satoshi Ihara, Hideki Ogiwara and Goichiro Tamura
Chondrodysplasia punctata (CDP), a rare skeletal dysplasia, can lead to cervical spine instability and deformity. However, an optimal neurosurgical intervention has yet to be established. Thus, a retrospective study was conducted to assess the efficacy of various surgical interventions for children with CDP.
The authors retrospectively reviewed 9 cases of CDP in which cervical decompression with or without posterior fusion was performed between April 2007 and May 2016. Patient demographics, preoperative clinical conditions, radiographic findings, surgical procedures, and the postoperative course were analyzed in detail.
A total of 12 operations were carried out in 9 patients (8 male, 1 female) during the study period. The patients’ ages at the initial surgery ranged from 2 months to 2 years. Seven of the children had CDPX1, 1 had CDPX2, and 1 had tibia-metacarpal type CDP (CDP-TM). The lesion occurred at the craniovertebral junction (CVJ) in 7 cases and involved a subaxial deformity in 2 cases. The initial surgery was C-1 laminectomy with occipitocervical fusion (OCF) followed by halo external fixation in 5 cases, OCF alone in 1 case, and C-1 laminectomy alone in 3 cases. Three children required additional surgery. In one of these cases, a staged operation was required because the patient’s head was too small to attach a halo ring at the time of the initial procedure (C-1 laminectomy). In another case, OCF was performed 11 months after C-1 laminectomy because of intramedullary signal change on serial MRI, although the child remained asymptomatic. In the third case, additional posterior fusion was performed 17 months after an initial laminectomy and OCF due to newly developed cervical dislocation caudal to the original fusion. This last patient required a third operation 9 months after the second because of deep wound infection. Surgery improved the motor function of all 7 children with CDPX1, but 3 children who had already suffered respiratory failure preoperatively required continued respiratory support. At the time of this report, 7 of the 9 children were alive and in stable condition. One child died due to restrictive respiratory insufficiency, and another died in an accident unrelated to CDP.
Surgical decompression with or without fusion for CVJ and subaxial cervical lesions in infants and toddlers with CDP generally saves lives and increases the likelihood of motor function recovery. However, in this case series the patients’ preoperative condition had a strong effect on postoperative respiratory function. The surgery was not straightforward, and a second operation was required in some cases. Nevertheless, the findings indicate that early surgical intervention for CDP with cervical involvement is feasible, suggesting that the role of neurosurgery should be reevaluated.
Hideki Ogiwara, Masahiro Joko, Michiko Takado, Kodai Uematsu, Masahiro Kameda, Natsu Sasaki, Masashi Kitagawa and Nobuhito Morota
The untethering of a tethered spinal cord in patients with a tight filum terminale is a relatively simple procedure that can prevent or improve neurological symptoms. Postoperatively, patients are usually kept in the horizontal decubitus position to prevent a CSF leak. However, the optimal period for keeping patients flat has not been determined yet. The authors compared 2 cohorts with different periods of horizontal decubitus; one with 72 hours and the other with 8 days.
The authors retrospectively analyzed surgical results in 2 cohorts of pediatric patients who had tethered spinal cord with a tight filum terminale. One cohort was maintained flat for 8 days and the other cohort for 72 hours postoperatively. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy, and clinical course.
Three hundred fifty-four patients underwent sectioning of a tight filum terminale. Of those, 238 were kept lying flat for 8 days postoperatively, and 116 were maintained flat for 72 hours. Magnetic resonance imaging was performed 1 to 2 weeks after the surgery. None of the patients in either cohort developed a CSF leak. Pseudomeningocele, which was confirmed by MRI, developed in 1 patient who had been kept flat for 8 days. The occurrence rates of a CSF leak and pseudomeningocele were not significantly different in either cohort.
Keeping patients flat for longer than 72 hours did not change the rate of postoperative CSF leakage or pseudomeningocele. Seventy-two hours or less would be an appropriate period for maintaining patients flat after transection of a tight filum terminale.
Junya Iwama, Hideki Ogiwara, Chikako Kiyotani, Keita Terashima, Kentaro Matsuoka, Hideto Iwafuchi and Nobuhito Morota
Because of their large size and high vascularity, complete removal of brain tumors in infants and young children is often difficult. In most cases the degree of resection is associated with prognosis. Neoadjuvant chemotherapy may facilitate resection by reducing the vascularity of the tumor. The authors evaluated the effectiveness of neoadjuvant chemotherapy in the management of these tumors.
The authors performed a retrospective review of infants and young children who underwent tumor removal after neoadjuvant chemotherapy.
Nine consecutive patients underwent resection after neoadjuvant chemotherapy during the period February 2004 to December 2012. The mean age at diagnosis was 18 months (range 2–50 months). The average largest tumor diameter was 71 mm (range 30–130 mm) at initial surgery. Five patients underwent partial resection, and 4 underwent biopsy as the initial surgery. The histopathological diagnoses were ependymoma in 2 patients, anaplastic ependymoma in 1, primitive neuroectodermal tumor (PNET) in 2, choroid plexus carcinoma in 1, atypical teratoid/rhabdoid tumor (AT/RT) in 1, glioblastoma in 1, and embryonal tumor with abundant neuropil and true rosettes in 1. After 2–4 courses of multiagent chemotherapy (mainly with vincristine, cyclophosphamide, etoposide, and cisplatin), the second-look surgery was performed. In 1 patient with a PNET, intratumoral hemorrhage was observed after 2 courses of chemotherapy. The mean interval between the initial and the second-look surgery was 3 months. The tumor volume was reduced to varying degrees in 5 patients (56%) after chemotherapy. Intraoperatively, the vascularity of the tumor was considerably reduced, and the tumor was more circumscribed in all cases. Gross-total resection was achieved in 8 patients (89%) and neartotal resection in 1 (11%). Histopathological examination demonstrated fibrotic tissue circumscribing the tumor in 6 of 9 cases (67%). The average blood loss was 20% of the estimated blood volume, and 3 patients (33%) required a blood transfusion. There was no surgical mortality. One patient had transient dysphasia postoperatively. The mean follow-up period was 28 months. At the last follow-up, 2 patients (22%) had died (1 died of tumor progression and 1 of sepsis), and 4 patients (44%) had no tumor recurrence.
Neoadjuvant chemotherapy for brain tumors in infants and young children was effective in reduction of tumor vascularity and clarification of the tumor-brain interface, which significantly facilitated maximal tumor resection.
Hideki Ogiwara, Douglas R. Nordli Jr., Arthur J. DiPatri Jr., Tord D. Alden, Robin M. Bowman and Tadanori Tomita
Ganglioglioma is the most common neoplasm causing focal epilepsy, accounting for approximately 40% of all epileptogenic tumors and for 1–4% of all pediatric CNS tumors. The optimal surgical treatment for pediatric epileptogenic ganglioglioma has not been fully established. The authors present their experience in the surgical management of these lesions.
The authors retrospectively analyzed seizure outcome and surgical results of pediatric patients with ganglioglioma treated with resection. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy, and follow-up.
The 30 patients (17 boys, 13 girls) had a history of medically intractable epilepsy. Total resection of tumor was achieved with or without adjacent epileptogenic tissue resection in all patients except 1, who underwent cyst fenestration and biopsy. Intraoperative electrocorticography (ECoG) was used in 21 patients. If an active spike focus or profound attenuation was observed in adjacent tissues, resection of those tissues was performed in addition to complete tumor resection (lesionectomy). The interval between onset of seizures and surgery ranged from 1 month to 9 years (mean 1.6 years). Patient age at the time of surgery ranged from 9 months to 16.3 years (mean 8.6 years). Twenty-five patients (83.3%) had complex partial seizures and 5 (16.7%) had simple partial seizures. Eighteen tumors (60%) were temporal (14 temporomesial, 4 temporolateral), and 12 (40%) were extratemporal. The mean follow-up period was 3.4 years (range 1 month–8.16 years). In 2 cases (6.7%), tumor recurrence was observed. Outcome was Engel Class I in 27 cases (90.0%; 14 temporomesial, 4 temporolateral, 9 extratemporal) and Engel Class II in 3 (10.0%; all extratemporal). Tumor resection allowed good seizure control, especially in the 18 cases of temporal ganglioglioma (all Engel Class I postoperatively). Eleven patients underwent removal of extratumoral epileptogenic tissue (anterior temporal neocortex resection in 10, anterior temporal neocortex resection with anterior hippocampectomy in 1) in addition to lesionectomy using intraoperative ECoG.
Lesionectomy with adjacent temporal neocortex resection using intraoperative ECoG provided good seizure control of pediatric temporal ganglioglioma. For extratemporal ganglioglioma, lesionectomy alone can provide fairly good seizure control. Considering the memory function of the hippocampus, lesionectomy with adjacent temporal neocortical resection can be a safe, feasible, and effective treatment option for epileptogenic gangliogliomas in pediatric patients.
Hideki Ogiwara, Arleta Lyszczarz, Tord D. Alden, Robin M. Bowman, David G. McLone and Tadanori Tomita
Untethering of a tethered spinal cord (TSC) by transecting or removing a fatty filum terminale is a relatively simple procedure that can prevent or ameliorate neurological symptoms, and the postoperative prognosis is usually good. Progressive neurological deterioration caused by recurrent tethering has been rarely reported. The authors present their experience in cases in which a sectioned fatty filum terminale has become retethered.
The authors retrospectively analyzed the surgical results of pediatric patients with fatty filum terminale–TSC treated by transection of the filum. The patients' charts were reviewed for demographic data, clinical presentation, surgical therapy, and follow-up data.
Of the 225 children who underwent TSC release by sectioning the fatty filum from 1992 to 2005, there were 6 patients (2.7%; 3 males, 3 females) in whom the fatty filum retethered. The mean age at the first diagnosis of TSC was 5.2 years (range 2 months–12.3 years). The mean duration from the first untethering procedure to retethering was 5.4 years. The mean age at the time of retethering was 10.6 years (range 7–17.5 years). Symptoms of retethering were urinary incontinence, low-back pain, difficulty walking, constipation, leg pain, and worsening foot deformity. Patients underwent cystometrography at the time retethering was indicated by increased bladder capacity, large post-void residual volume, decreased bladder capacity, increase in filling pressure, and poor sensation of filling. Magnetic resonance imaging revealed adherence of the rostral stump of the sectioned filum to the midline dorsal dural surface. All patients underwent the second untethering procedure. Four patients improved neurologically and experienced no retethering thereafter (mean follow-up period 5.5 years). Two patients experienced additional retethering after temporary improvement following the second untethering procedure.
Retethering of the spinal cord is a rare condition occurring after the sectioning of a fatty filum terminale. Awareness of this rare sequela is necessary for appropriate long-term management of TSC caused by a fatty filum terminale. Cystometrography is useful for detecting the lesion and confirming the diagnosis of retethering.