Optimizing Therapeutic Strategies for Syringomyelia Associated with Tethered Cord Syndrome: A Comprehensive Review.

This review highlights the need for therapeutic guidelines for syringomyelia associated with tethered cord syndrome (TCS) caused by spinal dysraphism (SD). A comprehensive literature review was conducted, selecting twelve articles to analyze common therapeutic strategies. Surgical cord untethering alone has recently become a preferred treatment, with 45 ± 21.1% of patients experiencing remission or improvement, 47 ± 20.4% unchanged and asymptomatic, and 4 ± 8% worsened. Untethering with direct surgical drainage for the syrinx had better outcomes than untethering alone (78% vs. 45%, p = 0.05). Terminal syringostomy was beneficial for syrinxes extending to the filum terminale but not for asymptomatic small syrinxes with a syrinx index < 0.4. Syrinx shunting was recommended for symptomatic large syrinxes (>2 cm in length and syrinx index > 0.5). Various shunt procedures for syrinxes are still advocated, mainly for refractory syringomyelia in Chiari malformation, posttraumatic cases, SD, or other causes. Personalized surgical methods that address the root cause of syringomyelia, particularly those improving cerebrospinal fluid flow, offer promising results with minimized complications. Ongoing studies are required to enhance management strategies for syringomyelia associated with TCS, optimize patient outcomes, and reduce the risk of recurrent symptoms.

Delayed symptomatic TFV in neonatal posthemorrhagic hydrocephalus-pathophysiology and therapeutic strategy: A case report and review of the literature.

INTRODUCTION: Trapped fourth ventricle (TFV), which is a rare neurosurgical condition with multifactorial etiology, requires a prompt diagnosis and appropriate therapeutic method selection. We report a case of post-hemorrhagic hydrocephalus and TFV incited/worsened by prematurity, sepsis, acute respiratory distress syndrome (ARDS), mechanical ventilation, and concomitant fourth ventricle outlets stenosis; which displayed a delayed onset. This article addresses the proposed pathophysiology and the clinical importance of appropriate therapeutic strategies with a mini-review of the literature. CASE PRESENTATION: We encountered a case involving a premature Asian male newborn with sepsis and posthemorrhagic hydrocephalus who required ventriculoperitoneal shunt surgery. However, after three years, the baby was diagnosed with a trapped fourth ventricle and subsequently underwent retrograde endoscopic surgery with stent insertion. DISCUSSION: TFV is traditionally known as a complication of lateral ventricle shunting. However, in rare cases such as our neonate patient, it develops as a consequence of multiple pathophysiological processes including ventricular system inflammation along with associated anatomic and physiologic alterations, which necessitates prompt diagnosis and a case-specific therapeutic strategy. CONCLUSION: Understanding the multifactorial pathophysiological mechanisms leading to the development of TFV is crucial. The presence of comorbidities such as prematurity, neonatal sepsis, and ARDS increased the risk of intraventricular hemorrhage and subsequent inflammation and further exacerbated obstructions in cerebrospinal fluid pathways. When posthemorrhagic TFV is accompanied by collapsed lateral ventricles, the optimal treatment approach is retrograde endoscopic fenestration with stent insertion. This treatment option has proven effective in alleviating the condition and restoring proper cerebrospinal fluid flow.

Endothelial marker profiles in cerebral radiation-induced vasculopathy: A comparative immunohistochemical analysis.

Radiation therapy results in radiation-induced vasculopathy, characterized by alterations in the vascular architecture stemming from radiation exposure. The exact molecular pathways and associated pathologies of this condition have yet to be comprehensively understood. This study aimed to identify specific markers' roles in cerebral vascular endothelial injury pathogenesis after radiosurgery and explore their unique expression patterns in diverse pathologies post-stereotactic radiosurgery. A retrospective cohort study was conducted to assess the expression profiles of endothelial markers via immunohistochemical analysis in 25 adult patients (13 males and 12 females) who had undergone neurosurgical resection for various central nervous system pathologies following stereotactic radiosurgery or radiotherapy from 2001 to 2015. Our findings revealed strong immunohistochemical expression of ICAM-1 and E-selectin across various disease states, while MMP-9, PAI-1, and eNOS exhibited moderate expression levels. In contrast, VCAM-1 and P-Selectin had the weakest expression across all groups. Notably, while individual markers showed significant variations in expression levels when comparing different diseases (P < .001), no substantial differences were found in the overall immunohistochemical expression patterns across the 5 distinct pathologies studied (P = .407, via 2-way ANOVA). Despite the varied long-term effects of radiotherapy on the vascular endothelium, a common thread of inflammation runs through the pathology of these conditions. The distinct patterns of marker expression identified in our study suggest that different markers play unique roles in the development of radiation-induced vasculopathy. These findings offer insights that could lead to the development of novel preventive strategies and treatments.

A Rare Occurrence of Primarily Extranodal Spinal Epidural Lymphoma With Spinal Cord Compression and Invasion to the Thoracic Cavity.

A 41-year-old man suffered from progressive radiculomyelopathy caused by spinal epidural mass primarily encasing the spinal cord at the cervicothoracic vertebrae that extended into the thoracic cavity through the neural foramen. An urgent decompressive laminectomy and epidural tumor resection were performed to prevent neurological deterioration and effective spinal cord decompression. The histopathologic diagnosis was diffuse large B-cell lymphoma. As first-line treatment for stage II extranodal lymphoma, he received 6 cycles of R-CHOP (rituximab/cyclophosphamide, hydroxydaunorubicin, Oncovin, and prednisone) chemotherapy. Consequently, follow-up positron-emission tomography CT and MR images demonstrated a complete metabolic response (Deauville score 1). This rare occurrence of primarily extranodal spinal epidural lymphoma with limited disease will be presented in a literature review.

Sacrococcygeal teratomas: a case-based review from the perspective of individual multidisciplinary experts.

PURPOSE: Sacrococcygeal teratoma (SCT) is a rare congenital tumor originating from a variant of extragonadal germ cell neoplasm. Herein, we present three cases of neonatal SCTs undergoing surgical resection and reconstruction. We also review the literature to discuss the importance of proper perinatal management and timely surgical intervention depending on the tumor type and maturity to prevent malignant transformation and recurrence and ensure functional outcomes. METHODS AND RESULTS: Three cases of SCT were retrospectively analyzed. All infants underwent complete surgical resection of the SCT and coccygectomy during their neonatal/infancy period, followed by pelvic floor and buttock reconstruction while minimizing buttock contour deformity and undesirable skin scar. Two of the cases were histopathologically diagnosed as predominantly cystic mature teratomas of Altman types I and IV, and the third was a mature teratoma of Altman type II. There were no complications or tumor recurrence during the average follow-up period of 5.3 years. CONCLUSIONS: This case-based review highlights the role of multidisciplinary team approaches, including prenatal monitoring, oncologic resection, and adequate reconstruction according to the type of tumor and anomaly. Optimal perinatal evaluation allows promising oncologic and functional outcomes in terms of timely intervention to eliminate tumor recurrence and malignant transformation. Complete oncologic surgical resection of SCTs should also include proper functional preservation strategies, such as the reconstruction of the pelvic floor, cosmetic buttock contouring, and preservation of bladder and bowel sphincter function.