Neurosurgical treatment of gangliogliomas in children and adolescents

Acta Neurochirurgica (2018) 160:1207–1214

The object of this study was to delineate long-term results of the surgical treatment of pediatric tumors classified as ganglioglioma or gangliocytoma.

Methods A cohort of consecutive patients 19 years or younger who had undergone primary resection of CNS tumors during the years 1980–2016 at a single institution were reviewed in this retrospective study of surgical morbidity, mortality, and academic achievement and/or work participation. Grossmotor function and activities of daily living were scored using the Barthel Index (BI).

Results Patient records for 32 consecutive children and adolescents who had undergone resection for a ganglioglioma were included in this study. Of the 32 patients, 13 were in the first decade at the first surgery, whereas 19 were in the second decade. The male/female ratio was 1.0 (16/16). No patient was lost to follow-up. The tumor was localized to the supratentorial compartment in 26 patients, to the posterior fossa in 5 patients, and to the spinal cord in 1 patient. Only two of the tumors were classified as anaplastic. Of the 30 low-grade tumors, 2 were classified as gangliocytomas, 6 were desmoplastic infantile gangliogliomas, and 22 were ordinary gangliogliomas. The aim of primary surgery was gross-total resection (GTR) and was achieved in 23 patients (71.9%). Altogether, 43 tumor resections were performed. Eight patients underwent a second resection from 1 to 10 years after primary surgery and three of these also had a third resection from 2 to 24 years after initial surgery. The reason for further resection was clinical (seizure control failure/recurrence of epilepsy or progressive neurological deficit) and/or residual tumor progression/recurrence. There was no operative mortality in this series and all 32 patients are alive with follow-up periods from 0.5 to 36 years (median 14 years). Observed 14-year survival is thus 100%. One out of two children with primary anaplastic tumor received local radiotherapy (proton) postoperatively. The other 31 patients did not have any kind of non-surgical adjuvant therapy. Twenty-one out of 26 children with supratentorial tumor had epilepsy as one of their presenting symptoms. Nineteen of these became seizure-free after initial surgery (18 of them after GTR), but 3 patients experienced recurrence of seizures within some years. Functional outcome in terms ofADL, schooling, and work participation was gratifying in most patients. Five patients have persistent hydrocephalus (HC), treated with ventriculoperitoneal (VP) shunts.

Conclusion Low-grade gangliogliomas (GGs) can be surgically treated with good long-term results including seizure and tumor control as well as school and working participation.


Corridor-Based Endonasal Endoscopic Surgery for Pediatric Skull Base Pathology


Operative Neurosurgery 10:273–293, 2014

Pediatric anatomy is more restricted, and the propagation of endonasal endoscopic approaches in the pediatric population has been limited.

OBJECTIVE: To demonstrate the feasibility of the endonasal endoscopic approach in a variety of age groups and to perform measurements of the corridors and spaces available for surgery as a guide for case selection.

METHODS: Only patients ,18 years were included. The choice of operative corridor/ approach is described in relation to pathological entity and location. Preoperative/ postoperative visual fields and endocrine panels, extent of resection, as well as postoperative long-term complications are described. Prospective magnetic resonance image-based anatomic measurements of key distances were performed to determine age-dependent surgical indications and limitations.

RESULTS: Forty purely endoscopic procedures were performed in 33 pediatric patients (5-18 years of age) harboring a variety of skull base lesions, from benign tumors to congenital malformations. For the 20 patients in whom gross total resection was the intended goal of surgery, gross total resection was attained in 15 (75%). There were 2 infections (5%) and no cerebrospinal fluid leaks. Significant improvement was shown in 58.3% of patients with visual deficits. Hormone overproduction resolved in 75% of patients, while preoperative hormone insufficiency only improved in 29.2%. Wider intercarotid distance at the superior clivus (P = .01) and shorter nare-dens working distance (P = .001) predicted improved outcomes and fewer postoperative complications.

CONCLUSION: Endonasal endoscopic skull base approaches are viable in the pediatric population, they are not impeded by sphenoid sinus aeration, and they have minimal risk of cerebrospinal fluid leak and meningitis. Outcomes and complications can be predicted based on specific radio anatomical skull base measurements rather than age.

Endoscopic endonasal skull base surgery in the pediatric population


J Neurosurg Pediatrics 11:227–241, 2013

The use of endoscopic endonasal surgery (EES) for skull base pathologies in the pediatric population presents unique challenges and has not been well described. The authors reviewed their experience with endoscopic endonasal approaches in pediatric skull base surgery to assess surgical outcomes and complications in the context of presenting patient demographics and pathologies.

Methods. A retrospective review of 133 pediatric patients who underwent EES at our institution from July 1999 to May 2011 was performed.

Results. A total of 171 EESs were performed for skull base tumors in 112 patients and bony lesions in 21. Eightyfive patients (63.9%) were male, and the mean age at the time of surgery was 12.7 years (range 2.3–18.0 years). Skull base tumors included angiofibromas (n = 24), craniopharyngiomas (n = 16), Rathke cleft cysts (n = 12), pituitary adenomas (n = 11), chordomas/chondrosarcomas (n = 10), dermoid/epidermoid tumors (n = 9), and 30 other pathologies. In total, 19 tumors were malignant (17.0%). Among patients with follow-up data, gross-total resection was achieved in 16 cases of angiofibromas (76.2%), 9 of craniopharyngiomas (56.2%), 8 of Rathke cleft cysts (72.7%), 7 of pituitary adenomas (70%), 5 of chordomas/chondrosarcomas (50%), 6 of dermoid/epidermoid tumors (85.7%), and 9 cases of other pathologies (31%). Fourteen patients received adjuvant radiotherapy, and 5 received chemotherapy. Sixteen patients (15.4%) showed tumor recurrence and underwent reoperation. Bony abnormalities included skull base defects (n = 12), basilar invagination (n = 4), optic nerve compression (n = 3) and trauma (n = 2); preexisting neurological dysfunction resolved in 12 patients (57.1%), improved in 7 (33.3%), and remained unchanged in 2 (9.5%). Overall, complications included CSF leak in 14 cases (10.5%), meningitis in 5 (3.8%), transient diabetes insipidus in 8 patients (6.0%), and permanent diabetes insipidus in 12 (9.0%). Five patients (3.8%) had transient and 3 (2.3%) had permanent cranial nerve palsies. The mean follow-up time was 22.7 months (range 1–122 months); 5 patients were lost to follow-up.

Conclusions. Endoscopic endonasal surgery has proved to be a safe and feasible approach for the management of a variety of pediatric skull base pathologies. When appropriately indicated, EES may achieve optimal outcomes in the pediatric population

Endoscopic Treatment of Isolated Fourth Ventricle: Clinical and Radiological Outcome

Neurosurgery 70:847–859, 2012 DOI: 10.1227/NEU.0b013e318236717f

Treatment of an isolated fourth ventricle should be considered when clinical symptoms or a significant mass effect occur.

OBJECTIVE: To report clinical and radiographic outcomes after endoscopic transaqueductal or transcisternal stent placement into the fourth ventricle. METHODS: In 19 patients (age, 34th week of gestation-20 years; median age, 17.5 months), 22 endoscopic procedures were performed. Either an aqueductoplasty or, in cases with a supratentorially extended fourth ventricular component, an interventricular fenestration was performed. In all patients, a stent connected to the cerebrospinal fluid–diverting shunt was placed through the fenestration. Surgical complications and radiological and clinical outcomes are reported.

RESULTS: All 19 patients had a mean follow-up of 26.9 ± 18.2 months. No persisting neurological complications were observed; 27.3% of patients experienced complete resolution of presenting symptoms, whereas 68.3% demonstrated partial resolution. Symptoms with short duration (< 4 weeks) resolved completely, whereas long-standing symptoms partially improved. Short-term shunt complications (n = 2; insufficient catheter placement and subdural hygroma) and a need for long-term stent revisions (n = 3; stent retraction and shunt revision for other causes) were observed. The mean fourth ventricular volume was reduced after surgery (44.2 ± 25.8 to 23.1 ± 21.9 mL; P < .01). Pontine diameter increased from 0.9 ± 0.3 to 1.2 ± 0.3 cm (P < .01) after surgery. Both effects were still demonstrated on later radiological follow-up of 24.4 ± 14.2 months (fourth ventricular size, 24.7 ± 28.1 mL; P < .01; pontine diameter, 1.3 ± 0.3 cm; P < .01).

CONCLUSION: The clinical and radiological outcomes after endoscopic aqueductoplasty and interventriculostomy in children with an isolated fourth ventricle indicate that this procedure is feasible, effective, and safe.

Outcomes in pediatric patients with Chiari malformation Type I followed up without surgery

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2011.1.PEDS10341

The natural history of untreated Chiari malformation Type I (CM-I) is poorly defined. The object of this study was to investigate outcomes in pediatric patients with CM-I who were followed up without surgical intervention.

Methods. The authors retrospectively reviewed 124 cases involving patients with CM-I who presented between July 1999 and July 2008 and were followed up without surgery. The patients ranged in age from 0.9 to 19.8 years (mean 7 years). The duration of follow-up ranged from 1.0 to 8.6 years (mean 2.83 years). Imaging findings, symptoms, and findings on neurological examinations were noted at presentation and for the duration of follow-up.

Results. The mean extent of tonsillar herniation at presentation was 8.35 mm (range 5–22 mm). Seven patients had a syrinx at presentation. The syrinx size did not change in these patients on follow-up imaging studies. No new syrinxes developed in the remaining patients who underwent subsequent imaging. The total number of patients with presenting symptoms was 81. Of those 81 patients, 67 demonstrated symptoms that were not typical of CM-I. Of the 14 patients with symptoms attributed to CM-I, 9 had symptoms that were not severe or frequent enough to warrant surgery, and surgery was recommended in the remaining 5 patients. Chiari malformation Type I was also diagnosed in 43 asymptomatic patients who had imaging studies performed for various reasons. No new neurological deficits were noted in any patient for the duration of follow-up.

Conclusions. The majority of patients with CM-I who are followed up without surgery do not progress clinically or radiologically. Longer follow-up of this cohort will be required to determine if symptoms or new neurological findings develop over the course of many years.

Variability among pediatric neurosurgeons in the threshold for ventricular shunting in asymptomatic children with hydrocephalus

J Neurosurg Pediatrics 7:000–000, 2011.DOI: 10.3171/2010.11.PEDS10275

The thresholds for shunting CSF in children with asymptomatic hydrocephalus are unclear; there are neither guidelines nor sufficient research to determine what degree of hydrocephalus should be treated. The authors hypothesize that 1) pediatric neurosurgeons currently have high thresholds for recommending treatment for these children, but 2) there is significant variability among these treatment thresholds.

Methods. The authors surveyed attendees of the Joint Pediatric Neurosurgery Section meeting in Spokane, Washington, in December 2008, regarding their treatment thresholds for 22 clinical scenarios. Each participant was provided an illustration of 5 imaging studies (3 slices each) showing progressively larger ventricles. For each scenario, respondents were asked to indicate the minimum ventricular size they would treat, if any. Responses were quantified from 1 to 6 from smaller to larger, with 6 being no treatment, and a mean theoretical treated ventricular size (MTTVS) was calculated for each scenario.

Results. Respondents were relatively conservative in recommending treatment, with MTTVSs of 3.7–6.0; in 13 scenarios, the MTTVS was greater than 5.0 (larger than the largest presented ventricular size). For scenarios in which a mean frontooccipital ratio could be calculated, the value ranged from 0.55 to 0.67 (moderate to severe hydrocephalus). Although there were clear majority responses for each scenario, there was also significant variability. There were no patterns of association with the respondent’s age, training, board certification, or type or location of practice.

Conclusions. This study demonstrates that pediatric neurosurgeons’ thresholds for treating asymptomatic children with hydrocephalus are generally high, but there is also significant variability.

Surgical management of giant pediatric craniopharyngiomas

J Neurosurg Pediatrics 6:000–000, 2010.DOI: 10.3171/2010.8.PEDS09385

Prior work by the authors’ group and reports of other authors suggest worse functional outcomes and decreased survival in children with larger craniopharyngiomas. The purpose of this study was to assess the oncological, endocrinological, and functional outcomes in children who underwent radical resection of giant craniopharyngiomas (defined as 5 cm or greater in largest diameter).

Methods. Between 1986 and 2006, 26 children under the age of 18 (14 boys, 12 girls; mean age 10.5 years) underwent radical resection of giant craniopharyngiomas performed by the senior author. Data were retrospectively collected to assess the outcome of surgical treatment.

Results. Twenty (77%) of 26 patients underwent gross-total resection (GTR) confirmed by intraoperative inspection and postoperative imaging. All primary tumors (17 of 17) and 3 (33%) of 9 recurrent tumors were treated with GTR. There was no operative mortality, and 18 of 26 patients (69%) were alive at a mean follow-up of 8.9 years (median 9.3 years). Disease control was achieved in 21 (84%) of the 25 patients followed up for more than 6 months and was more successful in patients who underwent GTR (95%) than in those who underwent STR (50%, p = 0.03). New-onset diabetes insipidus (DI) occurred in 63.2% of patients (73% of patients had DI postoperatively). New or worsened deficits in visual acuity and visual fields occurred in 16% and 28%, respectively, of the 25 patients for whom postoperative visual data were available. Five patients (19%) experienced significant, permanent neurological deficits, and 5 (19%) had mild to moderate deficits. New or worsened hypothalamic disturbance occurred in 35% and 22% of patients, respectively, but obesity developed in only 15%.

Conclusions. In this retrospective series, radical resection of giant craniopharyngiomas in children was found to lead to excellent rates of disease control with acceptable or good functional outcomes but slightly higher rates of neurological complications compared with rates in patients with smaller tumors. Radical resection is less successful in recurrent tumors that reach very large sizes, especially previously irradiated tumors, with resultant diminished survival.

Reinfection following initial cerebrospinal fluid shunt infection

J Neurosurg Pediatrics 6:000–000, 2010. DOI: 10.3171/2010.5.PEDS09457

Significant variation exists in the surgical and medical management of CSF shunt infection. The objectives of this study were to determine CSF shunt reinfection rates following initial CSF shunt infection in a large patient cohort and to determine management, patient, hospital, and surgeon factors associated with CSF shunt reinfection.

Methods. This retrospective cohort study included children who were in the Pediatric Health Information System (PHIS) database, who ranged in age from 0 to 18 years, and who underwent uncomplicated initial CSF shunt placement in addition to treatment for initial CSF shunt infection between January 1, 2001, and December 31, 2008. The outcome was CSF shunt reinfection within 6 months. The main predictor variable of interest was surgical approach to treatment of first infection, which was determined for 483 patients. Covariates included patient, hospital, surgeon, and other management factors.

Results. The PHIS database includes 675 children with initial CSF shunt infection. Surgical approach to treatment of the initial CSF shunt infection was determined for 483 children (71.6%). The surgical approach was primarily shunt removal/new shunt placement (in 286 children [59.2%]), but a substantial number underwent externalization (59 children [12.2%]), of whom a subset went on to have the externalized shunt removed and a new shunt placed (17 children [3.5% overall]). Other approaches included nonsurgical management (64 children [13.3%]) and complete shunt removal without shunt replacement (74 children [15.3%]). The 6-month reinfection rate was 14.8% (100 of 675 patients). The median time from infection to reinfection was 21 days (interquartile range [IQR] 5–58 days). Children with reinfection had less time between shunt placement and initial infection (median 50 vs 79 days, p = 0.06). No differences between those with and without reinfection were seen in patient factors (patient age at either shunt placement or initial infection, sex, race/ethnicity, payer, indication for shunt, number of comorbidities, distal shunt location, and number of shunt revisions at first infection); hospital volume; surgeon volume; or other management factors (for example, duration of intravenous antibiotic use). Nonsurgical management was associated with reinfection, and complete shunt removal was negatively associated with reinfection. However, reinfection rates did not differ between the 2 most common surgical approaches: shunt removal/new shunt placement (44 [15.4%] of 286; 95% CI 11.4%–20.1%) and externalization (total 12 [20.3%] of 59; 95% CI 11.0%–32.8%). Externalization followed by shunt removal/new shunt placement (5 [29.4%] of 17; 95% CI 10.3%–56.0%) and nonsurgical management (15 [23.4%] of 64; 95% CI 13.8%–35.7%) had higher, but nonstatistically significant, reinfection rates. The length of stay was shorter for nonsurgical management.

Conclusions. Surgical approach to treatment of initial CSF shunt infection was not associated with reinfection in this large cohort of patients.

Decompression of Chiari malformation with and without duraplasty: morbidity versus recurrence

J Neurosurg Pediatrics 5:474-478, 2010. DOI: 10.3171/2010.1.PEDS09218

The optimal surgical management of Chiari malformation (CM) is evolving. Evidence continues to accrue that supports decompression without duraplasty as an effective treatment to achieve symptomatic relief and anatomical decompression. The risks and benefits of this less invasive operation need to be weighed against decompression with duraplasty.

Methods. The authors performed a retrospective review of all CM decompressions from 2003 to 2007. All operations were performed by a single surgeon at a single institution. Data were analyzed for outcome, postoperative morbidity, and recurrence.

Results. Of 121 unique patients, 56 underwent posterior fossa decompressions without duraplasty (PFD) and 64 patients underwent posterior fossa decompressions with duraplasty (PFDD). Of the 56 PFD patients, 7 (12.5%) needed a subsequent PFDD for symptomatic recurrence. Of the 64 patients who underwent a PFDD, 2 (3.1%) needed a repeated PFDD for symptomatic recurrence. Patients treated with PFDD had an average operative time of 201 minutes in contrast to 127 minutes for those who underwent PFD (p = 0.0001). Patients treated with PFDD had average hospital stays of 4.0 days, whereas that for patients treated with PFD was 2.7 days (p = 0.0001). While in the hospital, patients treated with PFDD used low-grade narcotics, intravenous narcotics, muscle relaxants, and antiemetic medications at statistically significant differing rates.

Conclusions. While PFD was associated with a higher rate of recurrent symptoms requiring repeated decompression, this may be justified by the significantly lower morbidity rate. Clearer delineation of the trade-off between morbidity and recurrence may be used to help patients and their families make decisions regarding care.

In pursuit of prognostic factors in children with pilocytic astrocytomas

Childs Nerv Syst (2010) 26:19–28 DOI 10.1007/s00381-009-0990-8

This study described a 23-year experience in the treatment of children with pilocytic astrocytomas (piloA) with the aim of identifying putative clinical, histopathological, and/or immunohistochemical features that could be related to the outcome of these patients.

Methods Clinical data of 31 patients under 18 years of age with piloA were obtained from 1984 to 2006.

Results The mean age at the time of surgery was 7.8± 4.2 years (1 to 17 years), and the mean follow-up was 5.7± 5.4 years (1 to 20 years). The most common site of tumor formation was the cerebellum (17), followed by brainstem (4), optic chiasmatic hypothalamic region (4), cerebral hemisphere (3), cervical spinal cord (2), and optic nerve (1). Gross total resection (GTR) was achieved in 23 (74.1%), mainly in those with tumors located in the cerebellum and cerebral hemispheres (P=0.02). The global mortality rate was 6.4%. Nine patients were reoperated. Rosenthal fibers, eosinophilic granular bodies, microvascular proliferation, and lymphocytic infiltration were observed in most cases. The mean Ki-67LI was 4.4 ± 4.5%. In all cases, Gal-3 expression in tumor cells was observed with variable staining pattern.

Conclusions Aside from GTR, no other clinical, histopathological, or immunohistochemical features were found to be related to the prognosis. We postulate that strict follow- up is recommended if piloA is associated with high mitotic activity/Ki67-LI, or if GTR cannot be achieved at surgery. Tumor recurrence or progression of the residual lesion should be strictly observed. In some aspects, childhood piloA remains an enigmatic tumor.

Neurosurgical management of intracranial epidermoid tumors in children

J Neurosurg Pediatrics 4:91-96, 2009

Epidermoid tumors are benign lesions representing 1% of all intracranial tumors. There have been few pediatric series of intracranial epidermoid tumors reported previously. The authors present their experience in the management of these lesions.

The neurosurgical database at the Hospital for Sick Children was searched for children with surgically managed intracranial epidermoid tumors. The patients’ charts were reviewed for demographic data, details of clinical presentation, surgical therapy, and follow-up. Ethics board approval was obtained for this study.

Seven children, all girls, were identified who met the inclusion criteria between 1980 and 2007. The average age at surgery was 11.2 years (range 8–15 years), and the mean maximal tumor diameter was 2.1 cm. Headache was the most common presenting symptom, and 1 tumor was found incidentally. Most patients had normal neurological examinations, but meningism was found in 2 cases. There were 3 cerebellopontine angle lesions, 1 pontomedullary lesion, and 3 supratentorial tumors. Hydrocephalus developed in 1 patient after aseptic meningitis, and she underwent shunt placement. There were no operative deaths. Complete resection could be performed in 2 patients. One patient experienced a small recurrence that did not require a repeated operation, while 1 subtotally resected lesion recurred and the patient underwent a second operation.

Conclusions: Intracranial epidermoid tumors are rare in the pediatric population. Total resection is desirable to minimize the risk of postoperative aseptic meningitis, hydrocephalus, and tumor recurrence. Aggressive neurosurgical resection may be associated with cranial nerve or ischemic deficits, however. In these cases, neurosurgical judgment at the time of surgery is warranted to ensure maximum resection while minimizing postoperative neurological deficits.