Seizure-free and neuropsychological outcomes after temporal lobectomy with amygdalohippocampectomy in pediatric patients with hippocampal sclerosis

2012 ◽  
Vol 10 (2) ◽  
pp. 103-107 ◽  
Author(s):  
Sumeet Vadera ◽  
Varun R. Kshettry ◽  
Patricia Klaas ◽  
William Bingaman
Keyword(s):  
Pediatric Patients ◽  
Hippocampal Sclerosis ◽  
Pediatric Population ◽  
Cleveland Clinic ◽  
Intractable Epilepsy ◽  
Cortical Dysplasia ◽  
Temporal Lobectomy ◽  
Low Grade ◽  
Neuropsychological Outcomes

Object Temporal lobe epilepsy is an uncommon clinical syndrome in the pediatric population. The most common underlying pathologies include low-grade gliomas, cortical dysplasia, and, less commonly, hippocampal sclerosis (HS). There is a paucity of data on neuropsychological and seizure-free outcomes in these patients after temporal lobectomy. In this study, the authors reviewed their seizure-free and neuropsychological outcomes after temporal lobectomy for pediatric HS. Methods The authors retrospectively reviewed the medical records of pediatric patients with HS who underwent anterior temporal lobectomy and amygdalohippocampectomy between 1998 and 2011 at the Cleveland Clinic. Results of neuropsychological assessment before and after surgery and seizure-free outcome at last follow-up were obtained. Results Forty-five patients met the inclusion criteria. Thirty-four (76%) patients had pathology of HS alone and 10 (22%) had HS and cortical dysplasia. The mean duration of follow-up was 60.2 months. Eighty-four percent of patients had postoperative Engel Class I or II outcomes. Neuropsychological outcomes remained unchanged or minimally improved postoperatively. Conclusions Seizure-free outcomes in pediatric HS are similar to historical rates in adult HS. Neuropsychological assessments remain stable after temporal lobectomy. Standard temporal lobectomy should be considered in pediatric patients with medically intractable epilepsy secondary to HS.

Tailored Temporal Lobectomy for Medically Intractable Epilepsy

Neurosurgery ◽  
2012 ◽  
Vol 71 (3) ◽  
pp. 703-709 ◽  
Author(s):  
Steven M. Falowski ◽  
David Wallace ◽  
Andres Kanner ◽  
Michael Smith ◽  
Michael Rossi ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Hippocampal Sclerosis ◽  
Intractable Epilepsy ◽  
Temporal Lobectomy ◽  
Class I ◽  
Visual Field Defects ◽  
Seizure Outcome ◽  
Temporal Lobe Resection ◽  
Generalized Epilepsy

Abstract BACKGROUND: There have been only a few large series that have used a tailored temporal lobectomy. OBJECTIVE: To clarify whether tailoring a temporal lobe resection will lead to equivalent epilepsy outcomes or have the same predictive factors for success when compared with standard resections. METHODS: Retrospective analysis of 222 patients undergoing a tailored temporal lobe resection. Demographic measures and typical factors influencing outcome were evaluated. RESULTS: Pathology included 222 cases. With a mean follow-up of 5.4 years, 70% of patients achieved Engel class I outcome. A significant factor predicting Engel class I outcome on multivariate analysis was lesional pathology (P = .04). Among patients with hippocampal sclerosis, extent of lateral neocortical resection and hippocampal resection were not statistically associated with Engel class I outcome (P = .93 and P = .24). However, an analysis of Engel class subgroups a to d showed that patients who had a complete hippocampectomy in the total series were more likely to achieve an Engel class Ia outcome (P = .04). This was also true among patients with hippocampal sclerosis (P = .03). Secondarily, generalized seizure (P = .01) predicted outcome less than Engel class I. Predictive of poor outcome was the need for preoperative electrodes (P = .02). Complications included superior quadrant visual field defects, 2 cases of permanent dysphasia, and 3 wound infections. CONCLUSION: Predictors of successful seizure outcome for a tailored temporal lobectomy are similar to standard lobectomy. Patients with secondarily generalized epilepsy and cases in which preoperative subdural electrodes were thought necessary were less likely to achieve class I outcome. Among Engel class I cases, those who had a complete hippocampectomy were more likely to achieve Engel class Ia outcome.

Epilepsy Surgery of the Temporal Lobe in Pediatric Population: A Retrospective Analysis

Neurosurgery ◽  
2011 ◽  
Vol 70 (3) ◽  
pp. 684-692 ◽  
Author(s):  
Miguel Angel Lopez-Gonzalez ◽  
Jorge Alvaro Gonzalez-Martinez ◽  
Lara Jehi ◽  
Prakash Kotagal ◽  
Ann Warbel ◽  
...  
Keyword(s):  
Temporal Lobe ◽  
Epilepsy Surgery ◽  
Pediatric Patients ◽  
Hippocampal Sclerosis ◽  
Pediatric Population ◽  
Cleveland Clinic ◽  
Seizure Freedom ◽  
Complication Rates ◽  
Long Term Outcome

Abstract Background: There is still some reluctance to refer pediatric patients for epilepsy surgery, despite evidence of success in retrospective series. Objective: To describe surgical experience and long-term outcome in pediatric temporal lobe epilepsy (TLE) at a single institution. Methods: Retrospective review of pediatric (<18-years-old) TLE patients who underwent surgery between November 1996 and December 2006 at Cleveland Clinic Epilepsy Center. Cox proportional hazard modeling was used to assess outcome predictors. Results: One hundred thirty pediatric patients met study criteria. Mean time between seizure onset and surgery was 6.3 years. Invasive evaluation was used in 32 patients (24.5%). Hippocampal sclerosis was present in 70 patients (53.8%), either alone or associated in dual pathology. The complication rate was 7%. The seizure-freedom rates at 1, 2, 5, and 12 years were 76%, 72%, 54%, and 41%, respectively (Kaplan-Meier). With the use of the Engel outcome classification, 98 (75.3%) patients were class I, 11 (8.5%) class II, 9 (7%) class III, and 12 (9.2%) were class IV at last follow-up. Only 4 (3.1%) patients underwent reoperations. Antiepileptic drugs (AEDs) were discontinued in 36 patients (28.3%) in a mean period of 18 months (SD ± 17 months; range, 6-102 months). Although left-sided resection, lower number of preoperative AED trials (≤4), and tumor pathology correlated with favorable seizure outcomes, extensive surgical resection remained the only significant outcome predictor after multivariate analysis (P = .007; HR = 0.13 [95% confidence interval 0.007–0.64]). Conclusion: Careful selection of surgical candidates by multidisciplinary evaluations is required. Long-term seizure control is achieved successfully with acceptable low complication rates.

scholarly journals Surgical Treatment of Epilepsy in Pediatric Patients

2000 ◽  
Vol 27 (2) ◽  
pp. 106-110 ◽  
Author(s):  
Elaine Wyllie
Keyword(s):  
Pediatric Patients ◽  
Focal Epilepsy ◽  
Hippocampal Sclerosis ◽  
Intractable Epilepsy ◽  
Postoperative Outcome ◽  
Seizure Outcome ◽  
Low Grade ◽  
Epileptogenic Zone ◽  
Developmental Issues ◽  
Relative Risks

ABSTRACT:Surgery has become an accepted treatment modality for carefully selected adults with intractable focal epilepsy. More recently, increasing numbers of pediatric patients with intractable epilepsy are also being referred for surgical consideration. Key elements of surgical candidacy include medically intractable focal epilepsy, a localized epileptogenic zone, and a low risk for new postoperative neurologic deficits. The most common etiologies of the epilepsies in pediatric surgical candidates are malformation of cortical development and low grade tumor but some patients with childhood onset temporal lobe epilepsy due to hippocampal sclerosis also present for early surgery. Based on results from several recent pediatric surgical series, the chance for favorable seizure outcome after surgery is not adversely affected by young age, with seizure-free postoperative outcome reported for 60% to 65% of infants, 59% to 67% of children, and 69% of adolescents, compared to 64% reported in a large, predominantly adult series. Some subgroups of patients have higher percentages of seizure-free outcome, including those with hippocampal sclerosis or low grade tumor. In addition to seizures, developmental issues are also a major concern in children with intractable epilepsy. Few quantitative data are available, but some anecdotal experience suggests that surgical relief of catastrophic epilepsy may result in resumption of developmental progression after surgery, although the rate of development often remains abnormal. In one series, best developmental outcomes were seen in patients with earliest surgery and highest level of preoperative development. For each patient, the timing of surgery must be carefully considered, based on a full assessment of the relative risks and benefits, derived from a detailed presurgical evaluation.

Is postresective intraoperative electrocorticography predictive of seizure outcomes in children?

2012 ◽  
Vol 9 (5) ◽  
pp. 546-551 ◽  
Author(s):  
Carter D. Wray ◽  
Sharon S. McDaniel ◽  
Russell P. Saneto ◽  
Edward J. Novotny ◽  
Jeffrey G. Ojemann
Keyword(s):  
Epilepsy Surgery ◽  
Pediatric Patients ◽  
Intractable Epilepsy ◽  
Temporal Lobectomy ◽  
Pediatric Epilepsy ◽  
Seizure Freedom ◽  
Low Grade ◽  
Subtle Difference ◽  
Epileptiform Discharges ◽  
Pediatric Epilepsy Surgery

Object Intraoperative electrocorticography (ECoG) is commonly used to guide the extent of resection, especially in lesion-associated intractable epilepsy. Interictal epileptiform discharges on postresective ECoG (post-ECoG) have been predictive of seizure recurrence in some studies, particularly in adults undergoing medial temporal lobectomy, frontal lesionectomy, or low-grade glioma resection. The predictive value of postresective discharges in pediatric epilepsy surgery has not been extensively studied. Methods The authors retrospectively examined the charts of all 52 pediatric patients who had undergone surgery with post-ECoG and had more than 1 year of follow-up between October 1, 2003, and October 1, 2009. Results Of the 52 pediatric patients, 37 patients showed residual discharges at the end of their resection and 73% of these patients were seizure free, whereas 15 patients had no residual discharges and 60% of them were seizure-free, which was not significantly different (p = 0.36, chi-square). Conclusions Electrocorticography-guided surgery was associated with excellent postsurgical outcome. Although this sample size was too small to detect a subtle difference, absence of epileptiform discharges on post-ECoG does not appear to predict seizure freedom in all pediatric patients referred for epilepsy surgery. Future studies with larger study samples would be necessary to confirm this finding and determine whether post-ECoG may be useful in some subsets of pediatric epilepsy surgery candidates.

Long-term seizure outcomes after pediatric temporal lobectomy: does brain MRI lesion matter?

2019 ◽  
Vol 24 (2) ◽  
pp. 200-208
Author(s):  
Ravindra Arya ◽  
Francesco T. Mangano ◽  
Paul S. Horn ◽  
Sabrina K. Kaul ◽  
Serena K. Kaul ◽  
...  
Keyword(s):  
Repeated Measures ◽  
Mixed Model ◽  
Linear Mixed Model ◽  
Hippocampal Sclerosis ◽  
Brain Mri ◽  
Temporal Lobectomy ◽  
Seizure Freedom ◽  
Seizure Onset

OBJECTIVEThere is emerging data that adults with temporal lobe epilepsy (TLE) without a discrete lesion on brain MRI have surgical outcomes comparable to those with hippocampal sclerosis (HS). However, pediatric TLE is different from its adult counterpart. In this study, the authors investigated if the presence of a potentially epileptogenic lesion on presurgical brain MRI influences the long-term seizure outcomes after pediatric temporal lobectomy.METHODSChildren who underwent temporal lobectomy between 2007 and 2015 and had at least 1 year of seizure outcomes data were identified. These were classified into lesional and MRI-negative groups based on whether an epilepsy-protocol brain MRI showed a lesion sufficiently specific to guide surgical decisions. These patients were also categorized into pure TLE and temporal plus epilepsies based on the neurophysiological localization of the seizure-onset zone. Seizure outcomes at each follow-up visit were incorporated into a repeated-measures generalized linear mixed model (GLMM) with MRI status as a grouping variable. Clinical variables were incorporated into GLMM as covariates.RESULTSOne hundred nine patients (44 females) were included, aged 5 to 21 years, and were classified as lesional (73%), MRI negative (27%), pure TLE (56%), and temporal plus (44%). After a mean follow-up of 3.2 years (range 1.2–8.8 years), 66% of the patients were seizure free for ≥ 1 year at last follow-up. GLMM analysis revealed that lesional patients were more likely to be seizure free over the long term compared to MRI-negative patients for the overall cohort (OR 2.58, p < 0.0001) and for temporal plus epilepsies (OR 1.85, p = 0.0052). The effect of MRI lesion was not significant for pure TLE (OR 2.64, p = 0.0635). Concordance of ictal electroencephalography (OR 3.46, p < 0.0001), magnetoencephalography (OR 4.26, p < 0.0001), and later age of seizure onset (OR 1.05, p = 0.0091) were associated with a higher likelihood of seizure freedom. The most common histological findings included cortical dysplasia types 1B and 2A, HS (40% with dual pathology), and tuberous sclerosis.CONCLUSIONSA lesion on presurgical brain MRI is an important determinant of long-term seizure freedom after pediatric temporal lobectomy. Pediatric TLE is heterogeneous regarding etiologies and organization of seizure-onset zones with many patients qualifying for temporal plus nosology. The presence of an MRI lesion determined seizure outcomes in patients with temporal plus epilepsies. However, pure TLE had comparable surgical seizure outcomes for lesional and MRI-negative groups.

Seven years follow-up of corneal cross-linking (CXL) in pediatric patients: Evaluation of treated and untreated eye

2021 ◽  
pp. 112067212110206
Author(s):  
Iliya Simantov ◽  
Lior Or ◽  
Inbal Gazit ◽  
Biana Dubinsky-Pertzov ◽  
David Zadok ◽  
...  
Keyword(s):  
Visual Acuity ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Corneal Thickness ◽  
Cross Linking ◽  
Uncorrected Distance Visual Acuity ◽  
Mild Reduction ◽  
Change In The Mean ◽  
The Mean

Background: Retrospective cohort study evaluating long term keratoconus progression amongst cross-linking (CXL) treated pediatric patients in the treated and the fellow untreated eyes. Methods: Data on 60 eyes of 30 patients, 18 years old or younger, who underwent CXL in at least one eye was collected and analyzed. Follow-up measurements taken from the treated and untreated eye up to 7 years after CXL treatment, were compared to baseline measurements. Parameters included uncorrected distance visual acuity (UCDVA), best-corrected spectacle visual acuity (BCSVA), manifest refraction, pachymetry, corneal tomography, and topography. Results: Mean age of patients was 16 ± 2.1 years. For the treated eyes, during follow-up period mean UCDVA had improved (from 0.78 ± 0.22 at baseline to 0.58 ± 0.26 logMAR at 7 years; p = 0.13), as well as mean BCSVA (from 0.23 ± 0.107 at baseline to 0.172 ± 0.05 logMAR at 7 years; p = 0.37). The mean average keratometry showed a significant flattening (from 49.95 ± 4.04 to 47.94 ± 3.3 diopters (D); p < 0.001), However there was no change in the mean maximal keratometry. The mean minimal corneal thickness (MCT) showed a significant mild reduction of 26 µm ( p = 0.006). Although statistically insignificant, the mean manifest cylinder was also reduced to 2D ( p = 0.15). During the follow-up period, eight untreated eyes (26.6%) deteriorated and underwent CXL, while only one treated eye (3.33%) required an additional CXL. Conclusion: CXL is a safe and efficient procedure in halting keratoconus progression in the pediatric population, the fellow eye needs to be carefully monitored but only a 25% of the patients will require CXL in that eye during a period of 7 years.

Endoscopic transcervical odontoidectomy for pediatric basilar invagination and cranial settling

2008 ◽  
Vol 1 (4) ◽  
pp. 337-342 ◽  
Author(s):  
Matthew J. McGirt ◽  
Frank J. Attenello ◽  
Daniel M. Sciubba ◽  
Ziya L. Gokaslan ◽  
Jean-Paul Wolinsky
Keyword(s):  
Pediatric Patients ◽  
Pediatric Population ◽  
Tube Feeding ◽  
Basilar Invagination ◽  
Craniocervical Junction ◽  
Consecutive Series ◽  
Initial Experience ◽  
Prolonged Intubation ◽  
Alternative Approach

✓ Pediatric basilar invagination and cranial settling have traditionally been approached through a transoral–transpharyngeal route with or without extended maxillotomy or mandibulotomy for resection of the anterior portion of C-1 and the odontoid. The authors hypothesize that application of a recently described endoscopic transcervical odontoidectomy (ETO) technique would allow an alternative approach for the treatment of ventral pathological entities at the craniocervical junction in pediatric patients. The authors performed ETO in a consecutive series of pediatric patients presenting with myelopathy or bulbar dysfunction resulting from basilar invagination or cranial settling. All clinical, radiographic, surgical, and follow-up data were prospectively collected. The initial experience with ETO in the pediatric population is analyzed and outcomes are reported. Three patients required ETO for basilar invagination and 1 required ETO with anterior C-1 arch and distal clivus resection for cranial settling. All patients presented with myelopathy. One patient was wheelchair bound with severe quadriparesis. The mean age was 14 ± 3 years (mean ± standard deviation [SD]) in the 2 male and 2 female patients. The ETO and posterior fusion were performed as a 2-stage procedure in 2 (50%) and as a single-stage procedure in 2 (50%) cases. Prolonged intubation or postoperative placement of a gastrostomy tube was not needed in any case. The postoperative hospitalization lasted 9 ± 4 days (mean ± SD). At last follow-up (mean 5 months), head and neck pain had resolved and motor strength had improved or stabilized in all cases. All 4 children were independently functioning and ambulatory at the last follow-up. In the authors' initial experience, ETO has allowed ventral brainstem decompression without the need for prolonged intubation, worsening dysphagia requiring enteral tube feeding, or prolonged hospitalization, and has resulted in cosmetically appealing results. The ETO technique allows an alternative approach for the treatment of ventral pathological entities at the craniocervical junction in pediatric patients.

scholarly journals Safety and efficacy of stereoelectroencephalography in pediatric focal epilepsy: a single-center experience

2018 ◽  
Vol 22 (4) ◽  
pp. 444-452 ◽  
Author(s):  
Hannah E. Goldstein ◽  
Brett E. Youngerman ◽  
Belinda Shao ◽  
Cigdem I. Akman ◽  
Arthur M. Mandel ◽  
...  
Keyword(s):  
Pediatric Patients ◽  
Surgical Intervention ◽  
Focal Epilepsy ◽  
Wound Care ◽  
Pediatric Population ◽  
Attractive Alternative ◽  
Safety And Efficacy ◽  
Single Center Experience ◽  
A Minor

OBJECTIVEPatients with medically refractory localization-related epilepsy (LRE) may be candidates for surgical intervention if the seizure onset zone (SOZ) can be well localized. Stereoelectroencephalography (SEEG) offers an attractive alternative to subdural grid and strip electrode implantation for seizure lateralization and localization; yet there are few series reporting the safety and efficacy of SEEG in pediatric patients.METHODSThe authors review their initial 3-year consecutive experience with SEEG in pediatric patients with LRE. SEEG coverage, SOZ localization, complications, and preliminary seizure outcomes following subsequent surgical treatments are assessed.RESULTSTwenty-five pediatric patients underwent 30 SEEG implantations, with a total of 342 electrodes placed. Ten had prior resections or ablations. Seven had no MRI abnormalities, and 8 had multiple lesions on MRI. Based on preimplantation hypotheses, 7 investigations were extratemporal (ET), 1 was only temporal-limbic (TL), and 22 were combined ET/TL investigations. Fourteen patients underwent bilateral investigations. On average, patients were monitored for 8 days postimplant (range 3–19 days). Nearly all patients were discharged home on the day following electrode explantation.There were no major complications. Minor complications included 1 electrode deflection into the subdural space, resulting in a minor asymptomatic extraaxial hemorrhage; and 1 in-house and 1 delayed electrode superficial scalp infection, both treated with local wound care and oral antibiotics.SEEG localized the hypothetical SOZ in 23 of 25 patients (92%). To date, 18 patients have undergone definitive surgical intervention. In 2 patients, SEEG localized the SOZ near eloquent cortex and subdural grids were used to further delineate the seizure focus relative to mapped motor function just prior to resection. At last follow-up (average 21 months), 8 of 15 patients with at least 6 months of follow-up (53%) were Engel class I, and an additional 6 patients (40%) were Engel class II or III. Only 1 patient was Engel class IV.CONCLUSIONSSEEG is a safe and effective technique for invasive SOZ localization in medically refractory LRE in the pediatric population. SEEG permits bilateral and multilobar investigations while avoiding large craniotomies. It is conducive to deep, 3D, and perilesional investigations, particularly in cases of prior resections. Patients who are not found to have focally localizable seizures are spared craniotomies.

scholarly journals Intractable Temporal Lobe Epilepsy (TLE) From Tuberculoma; Microneurosurgical Management of by Amygdalohippocampectomy with Lesionectomy Plus Standard Anterior Temporal lobectomy: A Case Report and Short Review of Literature

2019 ◽  
Vol 16 (2) ◽  
pp. 106-109
Author(s):  
Forhad Hossain Chowdhury ◽  
Mohammod Raziul Haque ◽  
AFM Momtazul Haque
Keyword(s):  
Temporal Lobe Epilepsy ◽  
Temporal Lobe ◽  
Medial Temporal Lobe ◽  
Venous Malformation ◽  
Intractable Epilepsy ◽  
Complex Partial Seizure ◽  
Temporal Lobectomy ◽  
Mesial Temporal Sclerosis ◽  
Low Grade ◽  
Presenting Symptoms

Patient presenting as a case of Temporal Lobe Epilepsy (TLE) are usually resistant to antiepileptic drugs and surgery is the treatment of choice. This type of epilepsy may be due to Mesial Temporal Sclerosis (MTS), tumors [i.e. low grade glioma, Arterio-venous Malformation (AVM) etc], trauma, infection (Tuberculosis) etc. Here we report a case of surgically treated TLE that was due to a large tuberculoma in medial temporal lobe. Intractable epilepsy caused by tuberculoma is rare. The only presenting symptoms was Complex partial seizure (Psychomotor epilepsy) for which the patient underwent scalp EEG (Electro Encephalography) and MRI (Magnetic resonance imaging) of brain. The patient was managed by amygdalohippocampectomy with lesionectomy plus standard anterior lobectomy. Postoperatively she was on anti-tubercular therapy and on carbamazepine. The case was seizure and disease free till last follow up. Journal of Surgical Sciences (2012) Vol. 16 (2) : 106-109

Pediatric cerebellopontine angle and internal auditory canal tumors

2013 ◽  
Vol 12 (4) ◽  
pp. 317-324 ◽  
Author(s):  
Michelle A. Holman ◽  
William R. Schmitt ◽  
Matthew L. Carlson ◽  
Colin L. W. Driscoll ◽  
Charles W. Beatty ◽  
...  
Keyword(s):  
Family History ◽  
Diagnostic Criteria ◽  
Cerebellopontine Angle ◽  
Pediatric Patients ◽  
Pediatric Population ◽  
Internal Auditory Canal ◽  
Neurofibromatosis Type ◽  
History Of

Object The aim in this study was to describe the clinical presentation, differential diagnosis, and risk for neurofibromatosis Type 2 (NF2) in pediatric patients presenting with cerebellopontine angle (CPA) and internal auditory canal (IAC) tumors. Methods The authors conducted a retrospective study at a tertiary care academic referral center. All patients with an age ≤ 18 years who had presented with an extraaxial CPA or IAC tumor between 1987 and 2012 were included in the study cohort. Data regarding symptoms, diagnosis, tumor characteristics, and NF2 status were collected and analyzed. Results Sixty patients (55% female, 45% male) harboring 87 tumors were identified. The mean age at diagnosis was 12.8 years (median 14.0 years, range 0.9–18.9 years). Schwannomas were the most commonly identified lesions (57 of 87 tumors, including 52 vestibular, 3 facial, and 2 trigeminal schwannomas), followed by meningiomas (5 of 87) and epidermoid cysts (4 of 87). Six malignant tumors were diagnosed, including small-cell sarcoma, squamous cell carcinoma, malignant meningioma, atypical rhabdoid-teratoid tumor, endolymphatic sac tumor, and malignant ganglioglioma. Headache, followed by hearing loss and imbalance, was the most common presenting symptom, whereas dysphagia, otalgia, and facial pain were uncommon. Neurofibromatosis Type 2 was diagnosed in 20 (61%) of 33 patients with vestibular schwannoma (VS), while the other 13 patients (39%) had sporadic tumors. Nineteen of the 20 patients with NF2 met the diagnostic criteria for that disorder on initial presentation, and 15 of them presented with bilateral VS. At the last follow-up, 19 of the 20 patients subsequently diagnosed with NF2 demonstrated bilateral VSs, whereas 1 patient with a unilateral VS and multiple other NF2-associated tumors has yet to demonstrate a contralateral VS to date. Only 1 patient presenting with an isolated unilateral VS and no family history of NF2 demonstrated a contralateral VS on subsequent radiological screening. Conclusions Cerebellopontine angle and IAC tumors in the pediatric population are rare. There are several noteworthy differences between the adult and pediatric populations harboring these lesions. While VS is the most common pathology in both age groups, the lesion was found in only 60% of the pediatric patients in the present study. Unlike in adults, VSs in the pediatric population were associated with NF2 in over one-half of all cases. The majority of pediatric patients with NF2 fulfilled the diagnostic criteria at initial presentation; however, approximately 7% of patients presenting with a seemingly sporadic (no family history of NF2) unilateral VS will meet the criteria for NF2 later in life. Finally, malignancies account for a significantly higher percentage (10%) of cases among pediatric patients. These findings underscore the importance of early screening and close radiological follow-up and may be helpful in patient counseling.

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