Leukoencephalopathy with cerebral calcifications and cysts: the neurosurgical perspective. Literature review.

PURPOSE: Labrune syndrome is a rare white matter disease characterised by angiomatous leukoencephalopathy, diffuse intracranial calcifications and supra- and infra-tentorial parenchymal cysts. The clinical worsening is often related to cyst expansion, and surgery may be advocated for symptomatic management in about one-third of cases. However, no consensus exists on surgical timing, the most effective procedure, and long-term results. MATERIAL AND METHODS: Electronic databases, such as PubMed/ MEDLINE and Google Scholar, were analysed for studies published to April 2022 using the search term "Labrune syndrome OR leukoencephalopathy with calcifications and cysts OR brain calcifications OR brain cysts AND therapy OR surgery RESULTS: We found 28 studies reported in the literature, and we added a new case we treated at our Institution, reaching a series of 37 patients. All the patients in this series underwent surgical intervention. We aimed to review all the pertinent literature to discuss clinical-radiological features and etiopathogenetic insights, addressing the specific issue of the surgical options, clinical results and prognosis. CONCLUSION: LCC is a rare neurodegenerative disorder without effective medical treatment. Surgery remains the only therapeutic option to control the disease to reduce the mass effect of growing cystic lesions. Almost half of the patients who underwent surgery required further approaches, with great concern for the associated disabilities. Several procedures have been described, with no evidence of the most effective. Individual-based surgical planning must be advocated, tailoring the approach to limit side effects. Mini-invasive neuroendoscopic approaches may be considered to achieve those satisfactory results.

Cauda equina syndrome from intradural metastasis of a non-neural tumor: case report and review of literature.

BACKGROUND: Cauda equina syndrome (CES) is a challenging condition and it can be caused by variable entities. Leptomeningeal carcinomatosis (LC) is a multifocal seeding of the leptomeninges by malignant cells and it is observed in 1-8% of patients with solid tumors. Diagnosis of intradural metastases of the cauda equina is often delayed due to the non-specific characteristics of this condition but also to the delay of presentation of many patients. Cauda equina metastases usually occur in advanced cancers, but rarely can be the first presentation of disease. CASE DESCRIPTION: A 63-year-old man presented with 6 months history of low back pain and 20 d history of bilateral sciatica, hypoesthesia of the legs and the saddle, flaccid paraparesis and bowel incontinence determine by multiple nodular small lesions on the entire cauda equina with contrast-enhancement. Total-body CT showed a millimetric lesion at the lung. The patient underwent L2-L5 laminectomy and subtotal removal and histological examination showed a small cell lung carcinoma metastasis. CONCLUSIONS: In the literature, 54 cases of CES from non-CNS tumor metastasis are described. The diagnosis is challenging, back pain, with or without irradiation to the lower limbs, is the most frequently reported disturbance. In about 30% of patients there is no known malignancy and CES is the first clinical presentation. Treatment of choice is surgery, followed by radiotherapy and less frequently adjuvant chemotherapy. The surgical removal is almost always incomplete and functional outcome is often not satisfactory. Prognosis is poor.

Holocordic Spinal Sbdural Hematoma: A Challenging Management In Emergency: Literature Review.

OBJECTIVE: Spinal subdural hematoma (SSH) is a rare entity and the etiology has yet to be elucidated. Holocordic spinal hematomas represent an exception. The management of these patients remains controversial, due to their rarity and their extremely poor prognosis. METHODS: Electronic databases (MEDLINE, Scopus, and PubMed) were analyzed for studies published through April 2021, searching holocordic spinal subdural hematoma. Case reports, case series, and literature reviews were included. RESULTS: We found only 7 cases describing holocordic SSH reported in the literature, and we added the description of 1 case we managed at our institution, reaching a total of 8 cases. We discuss clinical and radiologic features, etiologic hypothesis, treatment strategies, and prognostic factors. CONCLUSIONS: The correct treatment of holocordic hematomas is not yet well standardized. Complete evacuation is not amenable, but surgical evacuation based on magnetic resonance imaging has shown to be the most effective therapeutic option, crucial in terms of prognosis, even if performed with some delay.

Hypertrophic Pachymeningitis and Hydrocephalus-The Role of Neuroendoscopy: Case Report and Review of the Literature.

BACKGROUND: Hypertrophic pachymeningitis (HP) is a rare primary or secondary inflammatory disorder that manifests with thickening of dura mater involving predominantly the tentorium and falx in the focal form. Hydrocephalus has not been reported in association with secondary HP. CASE DESCRIPTION: A 61-year-old woman presented with intracranial HP of the posterior fossa secondary to Wegener granulomatosis not responsive to corticosteroid therapy. Owing to the association of noncommunicating hydrocephalus, endoscopic third ventriculostomy was performed. There was immediate improvement of neurologic symptoms and no postoperative complications. The patient had very good clinical and radiologic outcome at 1-year follow-up. CONCLUSIONS: In a case of noncommunicating hydrocephalus related to focal HP of the posterior fossa, endoscopic third ventriculostomy was effective in resolving symptoms related to increased intracranial pressure. The physiopathogenetic mechanisms and therapeutic strategies were discussed along with a review of the most relevant literature.

Brain Granuloma: Rare Complication of a Retained Catheter.

BACKGROUND: Ventriculoperitoneal (VP) shunt is the most frequent treatment for hydrocephalus. VP shunt malfunction is a very common problem in neurosurgical practice, often requiring shunt revision procedures. In some cases, complete removal of a VP shunt may present difficulties, leading the surgeon to leave the ventricular catheter in situ. This decision is often made to avoid possible adverse events, primarily risk of life-threatening hemorrhage. However, a nonfunctioning catheter left in the ventricular system may lead to further complications. CASE DESCRIPTION: We report the case of an adult man who presented with a tumor-like cystic lesion, with mural and nodular postcontrast enhancement, caused by a huge granuloma formed around the tip of a retained ventricular catheter. CONCLUSIONS: This occurrence is extremely rare, and this is the first reported case in the literature. Preoperative differential diagnosis was challenging and included infection, metastasis, dysembryogenetic lesions, and rare reversible porencephalic cysts. According to several physiopathogenetic theories recently reported in the literature, the granuloma may develop as the result of persistent chronic inflammatory reactions between the ventricular catheter left in situ and the brain parenchyma. Application of neuroendoscopic techniques is improving management of VP shunt revisions, allowing safe removal of catheters stuck or lost in the ventricular system. Neuroendoscopy may represent an additional option to avoid possible complications related to retained ventricular catheters.

Recurrent lumbar disc herniation: Is there a correlation with the surgical technique? A multivariate analysis.

PURPOSE: The recurrence of a lumbar disc herniation (LDH) is a common cause of poor outcome following lumbar discectomy. The aim of this study was to assess a potential relationship between the incidence of recurrent LDH and the surgical technique used. Furthermore, we tried to define the best surgical technique for the treatment of recurrent LDH to limit subsequent recurrences. MATERIALS AND METHODS: A retrospective study was conducted on 979 consecutive patients treated for LDH. A multivariate analysis tried to identify a possible correlation between (1) the surgical technique used to treat the primary LDH and its recurrence; (2) technique used to treat the recurrence of LDH and the second recurrence; and (3) incidence of recurrence and clinical outcome. Data were analyzed with the Pearson's Chi-square test for its significance. RESULTS: In 582 cases (59.4%), a discectomy was performed, while in 381 (40.6%), a herniectomy was undertaken. In 16 cases, a procedure marked as "other" was performed. Among all patients, 110 (11.2%) had a recurrence. Recurrent LDH was observed in 55 patients following discectomy (9.45%), in 45 following herniectomy (11.8%), and in 10 (62.5%) following other surgery. Our data showed that 90.5% of discectomies and 88.2% of the herniectomies had a good clinical outcome, whereas other surgeries presented a recurrence rate of 62.5% (Pearson's χ2< 0.001). No statistical differences were observed between discectomy or herniectomy, for the treatment of the recurrence, and the incidence for the second recurrences (P > 0.05). A significant statistical correlation emerged between the use of other techniques and the incidence for the second recurrences (P < 0.05). CONCLUSIONS: The recurrence of an LDH is one of the most feared complications following surgery. Although the standard discectomy has been considered more protective toward the recurrence compared to herniectomy, our data suggest that there is no significant correlation between the surgical technique and the risk of LDH recurrence.