Surgical Management of Cavernous Malformations Presenting with Drug-Resistant Epilepsy

Abstract BACKGROUND: Multiple cerebral cavernous malformations (mCCMs) are known as potentially epileptogenic lesions. Treatment might be multimodal. Management of patients with mCCMs and epilepsy is challenging. OBJECTIVE: To evaluate (1) algorhythmic therapeutic sequences in patients with epilepsy associated to mCCMs, (2) whether there are predictive parameters to anticipate the development of drug-resistant epilepsy, and (3) seizure after epilepsy surgery compared to conservatively-treated drug-resistant patients. METHODS: All inpatients and outpatients with epilepsy associated to mCCMs from 1990 to 2010 and follow-up >12 months available were retrospectively analyzed. RESULTS: Twenty-three patients matched inclusion criteria. Epilepsy became drug-resistant in 18/23 (78%) patients. No predictors were found for development of drug-resistant epilepsy. Median follow-up for both groups was 7.8 years. Nine patients did not qualify for surgical therapy and were treated conservatively. One patient of this cohort (11%) was seizure-free (International League Against Epilepsy [ILAE] class 1). Surgical treatment was performed in 9 patients; 7/9 (78%) of these patients were seizure-free (ILAE class 1) after epilepsy surgery for at least 12 months compared with 1/9 patients in the non-operated group. In 7/9 cases (78%) the largest CCM was resected. In 8/9 (89%) not all CCMs were resected. CONCLUSION: After initial diagnosis of epilepsy associated to mCCMs, a primary conservative approach is reasonable. Surgical treatment can be successful in a large fraction of cases with drug-resistant epilepsy where an epileptogenic lesion is identified. Cases where surgery is not undertaken are likely to remain intractable.

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Preoperative evaluation and surgical management of infants and toddlers with drug-resistant epilepsy

Neurosurgical FOCUS ◽

10.3171/2018.7.focus18220 ◽

2018 ◽

Vol 45 (3) ◽

pp. E3 ◽

Cited By ~ 6

Author(s):

Jonathan Pindrik ◽

Nguyen Hoang ◽

Luke Smith ◽

Mark Halverson ◽

Mary Wojnaroski ◽

...

Keyword(s):

Literature Review ◽

Surgical Management ◽

Epilepsy Surgery ◽

Preoperative Evaluation ◽

Case Series ◽

Developmental Outcomes ◽

Drug Resistant ◽

Infants And Toddlers ◽

Study Team ◽

Drug Resistant Epilepsy

OBJECTIVEDespite perioperative risks, epilepsy surgery represents a legitimate curative or palliative treatment approach for children with drug-resistant epilepsy (DRE). Several factors characterizing infants and toddlers with DRE create unique challenges regarding optimal evaluation and management. Epilepsy surgery within children < 3 years of age has received moderate attention in the literature, including mainly case series and retrospective studies. This article presents a systematic literature review and explores multidisciplinary considerations for the preoperative evaluation and surgical management of infants and toddlers with DRE.METHODSThe study team conducted a systematic literature review based on Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, targeting studies that investigated children < 3 years of age undergoing surgical treatment of DRE. Using the PubMed database, investigators selected peer-reviewed articles that reported seizure outcomes with or without developmental outcomes and/or perioperative complications. Studies were eliminated based on the following exclusion criteria: sample size < 5 patients; and inclusion of patients > 3 years of age, when demographic and outcomes data could not be separated from the cohort of patients < 3 years of age.RESULTSThe study team identified 20 studies published between January 1990 and May 2017 that satisfied eligibility criteria. All selected studies represented retrospective reviews, observational studies, and uncontrolled case series. The compiled group of studies incorporated 465 patients who underwent resective or disconnective surgery (18 studies, 444 patients) or vagus nerve stimulator insertion (2 studies, 21 patients). Patient age at surgery ranged between 28 days and 36 months, with a mean of 16.8 months (1.4 years).DISCUSSIONThe study team provided a detailed summary of the literature review, focusing on the etiologies, preoperative evaluation, surgical treatments, seizure and developmental outcomes, and potential for functional recovery of infants and toddlers with DRE. Additionally, the authors discussed special considerations in this vulnerable age group from the perspective of multiple disciplines.CONCLUSIONSWhile presenting notable challenges, pediatric epilepsy surgery within infants and toddlers (children < 3 years of age) offers significant opportunities for improved seizure frequency, neuro-cognitive development, and quality of life. Successful evaluation and treatment of young children with DRE requires special consideration of multiple aspects related to neurological and physiological immaturity and surgical morbidity.

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Surgical management of glioneuronal tumors with drug-resistant epilepsy

Acta Neurochirurgica ◽

10.1007/s00701-011-1050-1 ◽

2011 ◽

Vol 153 (8) ◽

pp. 1551-1559 ◽

Cited By ~ 17

Author(s):

Kareem A. Zaghloul ◽

Johannes Schramm

Keyword(s):

Surgical Management ◽

Drug Resistant ◽

Drug Resistant Epilepsy ◽

Glioneuronal Tumors

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Cavernoma-related epilepsy in cavernous malformations located within the temporal lobe: surgical management and seizure outcome

Neurosurgical FOCUS ◽

10.3171/2020.1.focus19920 ◽

2020 ◽

Vol 48 (4) ◽

pp. E6 ◽

Cited By ~ 1

Author(s):

Patrick Schuss ◽

Julia Marx ◽

Valeri Borger ◽

Simon Brandecker ◽

Ági Güresir ◽

...

Keyword(s):

Surgical Management ◽

Temporal Lobe ◽

Preoperative Evaluation ◽

Patient Characteristics ◽

University Hospital ◽

Temporal Lobectomy ◽

Seizure Outcome ◽

Drug Resistant ◽

Cavernous Malformations ◽

Number Of Patients

OBJECTIVECavernoma-related epilepsy (CRE) is a frequent symptom in patients with cerebral cavernous malformations (CCMs). Reports on surgical management and seizure outcome of epileptogenic CCM often focus on intracranial cavernoma in general. Therefore, data on CCMs within the temporal lobe are scarce. The authors therefore analyzed their institutional data.METHODSFrom 2003 to 2018, 52 patients suffering from CCMs located within the temporal lobe underwent surgery for CRE at University Hospital Bonn. Information on patient characteristics, preoperative seizure history, preoperative evaluation, surgical strategies, postoperative complications, and seizure outcome was assessed and further analyzed. Seizure outcome was assessed 12 months after surgery according to the International League Against Epilepsy (ILAE) classification and stratified into favorable (ILAE class I) versus unfavorable (ILAE classes II–VI).RESULTSOverall, 47 (90%) of 52 patients with CCMs located in the temporal lobe and CRE achieved favorable seizure outcome. Pure lesionectomy was performed in 5 patients, extended lesionectomy with resection of the hemosiderin rim in 38 patients, and anterior temporal lobectomy in 9 patients with temporal lobe CCM. Specifically, 36 patients (69%) suffered from drug-resistant epilepsy (DRE), 3 patients (6%) from chronic CRE, and 13 patients (25%) sustained sporadic CRE. In patients with DRE, favorable seizure outcome was achieved in 32 (89%) of 36 patients. Patients with DRE were significantly older than patients with CCM-associated chronic or sporadic seizures (p = 0.02). Furthermore, patients with DRE more often underwent additional amygdalohippocampectomy following the recommendation of presurgical epileptological evaluation.CONCLUSIONSFavorable seizure outcome is achievable in a substantial number of patients with epileptogenic CCM located in the temporal lobe, even if patients suffered from drug-resistant CRE. For adequate counseling and monitoring, patients with CRE should undergo a thorough pre- and postsurgical evaluation in dedicated epilepsy surgery programs.

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