Preemptive strategies and lessons learned from complications encountered with microvascular decompression for hemifacial spasm.

OBJECTIVE: Microvascular decompression (MVD) is the only curative treatment modality for hemifacial spasm (HFS). Although generally considered to be safe, this surgical procedure is surrounded by many risks and possible complications. The authors present the spectrum of complications that they met in their case series, the possible causes, and the strategies recommended to minimize them. METHODS: The authors reviewed a prospectively maintained database for MVDs performed from 2005 until 2021 and extracted relevant data including patient demographics, offending vessel(s), operative technique, outcome, and different complications. Descriptive statistics with uni- and multivariable analyses for the factors that may influence the seventh, eighth, and lower cranial nerves were performed. RESULTS: Data from 420 patients were obtained. Three hundred seventeen of 344 patients (92.2%) with a minimum follow-up of 12 months had a favorable outcome. The mean follow-up (standard deviation) was 51.3 ± 38.7 months. Immediate complications reached 18.8% (79/420). Complications persisted in only 7.14% of patients (30/420) including persistent hearing deficits (5.95%) and residual facial palsy (0.95%). Temporary complications included CSF leakage (3.10%), lower cranial nerve deficits (3.57%), meningitis (0.71%), and brainstem ischemia (0.24%). One patient died because of herpes encephalitis. Statistical analyses showed that the immediate postoperative disappearance of spasms and male gender are correlated with postoperative facial palsy, whereas combined vessel compressions involving the vertebral artery (VA) and anterior inferior cerebellar artery can predict postoperative hearing deterioration. VA compressions could predict postoperative lower cranial nerve deficits. CONCLUSIONS: MVD is safe and effective for treating HFS with a low rate of permanent morbidity. Proper patient positioning, sharp arachnoid dissection, and endoscopic visualization under facial and auditory neurophysiological monitoring are the key points to minimize the rate of complications in MVD for HFS.

Cavernoma-Related Epilepsy: Postoperative Epilepsy Outcome and Analysis of the Predictive Factors, Case Series.

BACKGROUND: Epilepsy is common in patients harboring cavernous malformation, and surgery is reported to be an effective treatment. However, few patients still experience seizures after surgery. We analyzed the outcome and predictive factors after cavernoma-related epilepsy (CRE) surgery. METHODS: A database was created for all patients with CRE treated surgically from 2003 to 2020 at a university hospital. A chart review, perioperative epilepsy workup, surgical strategies, and postoperative and follow-up notes were analyzed. Postoperative seizure outcome was evaluated according to the Engel classification. RESULTS: Thirty-seven patients (40.5% women; mean age 39.1 ± 14.5 years) were studied. The mean follow-up time was 5.6 ± 3.9 years. Among 37 patients, 32 (86.5%) achieved Engel class I at the last follow-up. Engel class II was found in 1 (2.7%), Engel class III in 1 (2.7%), and Engel class IV in 3 (8.1%) cases. Engel class Ia was observed in 23 patients (62.2%). None of the patients had a worse seizure outcome after the operation (Engel class IVc). Univariate and multivariate analysis showed that short-standing, sporadic, or low-frequent (≤3) seizures were the only variables significantly associated with seizure freedom, whereas longstanding, drug-resistant, or high-frequent seizure history were associated with worse seizure outcomes. CONCLUSIONS: Surgical treatment results in favorable seizure control in most patients after CRE surgery. Long duration of epilepsy before surgery, high seizure frequency, and drug-resistant epilepsy could have a negative effect on seizure outcome (Engel class II-IV). Therefore, early surgical resection of the cavernoma after careful presurgical planning is recommended to achieve an optimal result.

The value of intraoperative indocyanine green angiography in microvascular decompression for hemifacial spasm to avoid brainstem ischemia.

PURPOSE: Despite being rarely reported, ischemic insults resulting from compromising small brainstem perforators following microvascular decompression (MVD) remain a potential devastating complication. To avoid this complication, we have been using indocyanine green (ICG) angiography intraoperatively to check the flow within the small brainstem perforators. We aim to evaluate the safety and usefulness of ICG videoangiography in MVD. METHODS: We extracted retrospective data of patients who received ICG videoangiography from our prospectively maintained database for microvascular decompression. We noted relevant data including demographics, offending vessels, operative technique, outcome, and complications. RESULTS: Out of the 438 patients, 15 patients with a mean age (SD) of 53 ± 10.5 years underwent intraoperative ICG angiography. Male:female was 1:1.14. The mean disease duration prior to surgery was 7.7 ± 5.3 years. The mean follow-up (SD) was 50.7 ± 42.0 months. In 14 patients, the offending vessel was an artery, and in one patient, a vein. Intraoperative readjustment of the Teflon pledget or sling was required in 20% (3/15) of the cases. No patient had any sort of brainstem ischemia. Eighty percent of the patients (12/15) experienced complete resolution of the spasms. 86.7% (13/15) of the patients reported a satisfactory outcome with marked improvement of the spasms. Three patients experienced slight hearing affection after surgery, which improved in two patients later. There was no facial or lower cranial nerve affection. CONCLUSION: Intraoperative ICG is a safe tool for evaluating the flow within the brain stem perforators and avoiding brainstem ischemia in MVD for hemifacial spasm.

Endoscopic Transcortical-Transventricular Approach in Treating Third Ventricular Craniopharyngiomas-Case Series With Technical Note and Literature Review.

BACKGROUND: A paradigm shift toward less radicality in the treatment of craniopharyngioma (CP) has increasingly gained recognition. In selected patients, a less invasive approach aiming at cyst decompression may be suitable. OBJECTIVE: To present our experience with the endoscopic transcortical-transventricular approach (ETTA) for the treatment of CP located in the third ventricle, describe our surgical technique, and explore the results reported in the literature. METHODS: Data for third ventricular CPs treated using ETTA in 2 tertiary medical centers between 2006 and 2020 were retrospectively reviewed. Cystic, mixed lesion, new, and recurrent cases were included. ETTA aimed at cyst fenestration, subtotal or partial tumor resection, managing the hydrocephalus, or a combination of these modalities. Fractionated radiotherapy (RT) was performed (54 Gy in 30 fractions). RESULTS: The described approach was performed in 13 patients with a mean age of 30.2 years. Tumor excision was attempted in 10 patients. Cystic fenestration alone was conducted in 3 patients, followed by intracystic catheter implantation in 2 patients. Permanent diabetes insipidus developed in 3 patients, and 1 patient experienced a surgical site infection. The recurrence rate for ETTA + RT (pre-ETTA in 33.3%) was 2/9 (22.2%) after a mean follow-up of 21.3 months. Four patients did not undergo RT after ETTA, and all these patients experienced recurrences after a mean follow-up of 15.5 months. Hydrocephalus resolved in all 6 patients who presented with accompanying hydrocephalus. CONCLUSION: ETTA combined with adjuvant RT is a safe and effective option in the management of cystic or predominantly cystic CP extending into the third ventricle. This procedure also effectively treats the accompanying hydrocephalus.

Long-term outcome and quality of life after CNS cavernoma resection: eloquent vs. non-eloquent areas.

The aim of this study is to analyze the long-term quality of life after surgery of cavernoma. A monocentric retrospective study was conducted on 69 patients with cavernoma treated microsurgically between 2000 and 2016. The eloquence was adopted from Spetzler-Martin definition. A most recent follow-up was elicited between 2017 and 2019, in which the quality of life (QoL) was evaluated with the Short Form-12 questionnaire (SF12). Forty-one lesions were in eloquent group (EG), 22 in non-eloquent group (NEG), 3 in orbit, and 3 in the spinal cord. Postoperative worsening of the modified Rankin scale (mRS) occurred in 19.5% of cases in EG versus 4.5% in NEG. After a mean follow-up of 6.5 years (SD 4.6), the neurological status was better or unchanged compared to baseline in 85.4% of EG and 100% of NEG. Regarding QoL assessment of 44 patients (EG n = 27, NEG n = 14) attended the last follow-up. Patients after eloquent cavernoma resection reported a non-inferior QoL in most SF12 domains (except for physical role) compared to NEG. However, they reported general health perception inferior to norms, which was affected by the limited physical and emotional roles. At a late follow-up, the surgical morbidity was transient in the NEG and mostly recovered in the EG. The QoL comparison between eloquent and non-eloquent cavernomas created interesting and new data after prolonged follow-up. These results add value for decision-making as well as patient counseling for future encountered cases. Preoperative evaluation of QoL is recommended for future studies to assess QoL dynamics.

Novel Pathogenic Variants in a Cassette Exon of CCM2 in Patients With Cerebral Cavernous Malformations.

Autosomal dominant cerebral cavernous malformation (CCM) represents a genetic disorder with a high mutation detection rate given that stringent inclusion criteria are used and copy number variation analyses are part of the diagnostic workflow. Pathogenic variants in either CCM1 (KRIT1), CCM2 or CCM3 (PDCD10) can be identified in 87-98% of CCM families with at least two affected individuals. However, the interpretation of novel sequence variants in the 5'-region of CCM2 remains challenging as there are various alternatively spliced transcripts and different transcription start sites. Comprehensive genetic and clinical data of CCM2 patients with variants in cassette exons that are either skipped or included into alternative CCM2 transcripts in the splicing process can significantly facilitate clinical variant interpretation. We here report novel pathogenic CCM2 variants in exon 3 and the adjacent donor splice site, describe the natural history of CCM disease in mutation carriers and provide further evidence for the classification of the amino acids encoded by the nucleotides of this cassette exon as a critical region within CCM2. Finally, we illustrate the advantage of a combined single nucleotide and copy number variation detection approach in NGS-based CCM1/CCM2/CCM3 gene panel analyses which can significantly reduce diagnostic turnaround time.

Surgical management of an adult manifestation of Ewing sarcoma of the spine-a case report.

Ewing sarcoma (ES) is a primary malignant bone tumor. Its occurrence in adults is uncommon. Even rarer is the occurrence in the spine. The prognosis of this tumor in adult patients is unfavorable. In this report, we present the case of a 56-year-old woman with ES localized at the upper thoracic spine, presented with acute incomplete paraplegia. The patient underwent emergency decompressive hemilaminectomy and tumor debulking surgery to decompress the spinal cord. As adjuvant therapy, she received radio-chemotherapy followed by en bloc resection via a combined dorsoventral approach. In the course of the disease, the patient showed a continuous neurological improvement from AISA B to E over the follow up period of 18 months. To date there has been no evidence of tumor recurrence. We report our experience in treating ES of the spine in an adult patient presenting with neurological deficits. We highlight the advantage of en bloc resection of the tumor after spinal stabilization to treat the local disease and alleviating the presenting symptoms.