RESUMEN
Background: Microvascular decompression (MVD) through a retrosigmoid approach is considered the treatment of choice in cases of hemifacial spasm (HFS) due to neurovascular conflict (NVC). Despite the widespread of neuronavigation and intraoperative neuromonitoring (IONM) techniques in neurosurgery, their contemporary application in MVD for HFS has been only anecdotally reported. Methods: Here, we report the results of MVD performed with a combination of neuronavigation and IONM, including lateral spread response (LSR) in 20 HFS patients. HFS clinical outcome and different surgical-related factors, such as craniotomy size, surgical duration, mastoid air cell (MAC) opening, postoperative cerebral spinal fluid (CSF) leakage, sinus injury, and other complications occurrence, and the length of hospitalization (LOS) were studied. Results: Postoperatively, residual spasm persisted only in two patients, but at the latest follow-up (FU) (mean: 12.5 ± 8.98 months), all patients had resolution of symptoms. The mean surgical duration was 103.35 ± 19.36 min, and the mean LOS was 2.21 ± 1.12 days. Craniotomy resulted in 4.21 ± 1.21 cm2 in size. Opening of MAC happened in two cases, whereas no cases of CSF leak were reported as well as no other complications postoperatively and during FU. Conclusion: MVD for HFS is an elective procedure, and for this reason, surgery should integrate all technologies to ensure safety and efficacy. The disappearance of LSR is a crucial factor for identifying the vessel responsible for NVC and for achieving long-term resolution of HFS symptoms. Simultaneously, the benefits of using neuronavigation, including the ability to customize the craniotomy, contribute to reduce the possibility of complications.
RESUMEN
BACKGROUND: Intramedullary melanocytomas are exceedingly rare, with only twenty-four cases reported up to now. They present as local invasive tumors despite their benign biological behavior. Attempting a complete safe resection often results in severe post-operative neurological deficits, as in our case presented here. METHODS: A systematic review was conducted across the PubMed and Scopus databases including studies published till February 2024. RESULTS: A total of 19 studies were included, encompassing 24 cases. A similar distribution between sexes was noted (M:F 13:11), with ages ranging from 19 to 79 years. The thoracic segment was most affected, and intermediate-grade melanocytoma (19 cases) was the most common histotype. Radiographically, intramedullary melanocytomas usually appear as hyperintense hemorrhagic lesions peripheral to the central canal with focal nodular enhancement. Intraoperatively, they are black-reddish to tan and are tenaciously adherent lesions. In the sampled studies, IONM employment was uncommon, and post-operative new-onset neurological deficits were described in 16 cases. Adjuvant RT was used in four cases and its value is debatable. Recurrence is common (10 cases), and adjuvant therapies (RT or repeated surgery) seem to play a palliative role. CASE PRESENTATION: A 68-year-old woman presented with a three-year history of worsening spastic paraparesis and loss of independence in daily activities (McCormick grade 4). An MRI revealed an intramedullary tumor from Th5 to Th7, characterized by T1-weighted hyperintensity and signs of recent intralesional hemorrhage. Multimodal neuromonitoring, comprising the D-Wave, guided the resection of a black-tan-colored tumor with hyper-vascularization and strong adherence to the white matter. During final dissection of the lesion to obtain gross total resection (GTR), a steep decline in MEPs and D-Wave signals was recorded. Post-operatively, the patient had severe hypoesthesia with Th9 level and segmental motor deficits, with some improvement during neurorehabilitation. Histopathology revealed an intermediate-grade melanocytoma (CNS WHO 2021 classification). A four-month follow-up documented the absence of relapse. CONCLUSIONS: This literature review highlights that intramedullary T1 hyperintense hemorrhagic thoracic lesions in an adult patient should raise the suspicion of intramedullary melanocytoma. They present as locally aggressive tumors, due to local invasiveness, which often lead to post-operative neurological deficits, and frequent relapses, which overwhelm therapeutic strategies leading to palliative care after several years.